• General Medicine
    • 1

      On Being a Physician

      By Elizabeth G Nabel, MD, FACP
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      On Being a Physician

      • ELIZABETH G NABEL, MD, FACPPresident, Brigham and Women’s Hospital, Professor of Medicine, Harvard Medical School, Boston, MA

      The role of a physician as healer has grown more complex, and emphasis will increasingly be on patient and family-centric care. Physicians must provide compassionate, appropriate, and effective patient care by demonstrating competence in the attributes that are essential to successful medical practice. Beyond simply gaining medical knowledge, modern physicians embrace lifelong learning and need effective interpersonal and communication skills. Medical professionalism encompasses multiple attributes, and physicians are increasingly becoming part of a larger health care team. To ensure that physicians are trained in an environment that fosters innovation and alleviates administrative burdens, the Accreditation Council for Graduate Medical Education has recently revamped the standards of accreditation for today’s more than 130 specialties and subspecialties.

      This chapter contains 6 references and 5 MCQs.

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    • 2

      Ethical and Social Issues in Medicine

      By Roberta Springer Loewy, PhD (PHIL, ETHICS); Erich H. Loewy, MD, FACP (deceased); Faith T. Fitzgerald, MD, MACP
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      Ethical and Social Issues in Medicine

      • ROBERTA SPRINGER LOEWY, PHD (PHIL, ETHICS)Professor and Bioethics Education Consultant, VCF, University of California, Davis, Sacramento, CA
      • ERICH H. LOEWY, MD, FACP (DECEASED)Professor and Founding Chair of the Bioethics Program (Emeritus), University of California, Davis, Sacramento, CA
      • FAITH T. FITZGERALD, MD, MACPProfessor of Internal Medicine, University of California, Davis, Sacramento, CA

      So rapidly has the field of health care ethics continued to grow that, when recently “googled,” the term produced 28.2 million hits. The challenge is to address the ethical and social issues in medicine in this very limited article space. It remains an impossible task to present more than a superficial discussion of these complex issues and the complicated cases in which they are to be found. Like good medicine, good ethics cannot be practiced by algorithm. The authors have opted to provide an operational guide to help clinicians sort through the ethical and social quandaries they must face on a daily basis. To that end, the authors have chosen to divide this chapter into the following sections:
      1. A brief description of the biopsychosocial nature of ethics and how it differs from personal morality
      2. A method for identifying and dealing with ethical issues
      3. A discussion of the role of bioethicists and ethics committees
      4. The professional fiduciary role of clinicians
      5. Listings of some of the common key bioethical and legal terms 
      6. A very brief discussion of the terms cited in the above listings


      This chapter contains two tables: common bioethical concepts and legal concepts.

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    • 3

      Practicing Evidence-based Medicine

      By Michael Barnett, MD; Niteesh Choudhry, MD, PhD
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      Practicing Evidence-based Medicine

      • MICHAEL BARNETT, MDFellow in General Internal Medicine, Division of General Internal Medicine and Primary Care, Department of Medicine, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA
      • NITEESH CHOUDHRY, MD, PHDAssociate Professor, Division of Pharmacoepidemiology and Pharmacoeconomics, Department of Medicine, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA

      Today, a plethora of resources for evidence-based medicine (EBM) are available via alert services, compendia, and more. In theory, a clinician researching a topic or looking for information regarding a clinical decision should easily find the literature or synopses needed. However, the real challenge lies in recognizing which resources (out of hundreds or possibly thousands) present the best and most reliable evidence. As well, evidence from research is only part of the decision calculus, and the clinician, not the evidence, makes the final decisions. Medical decision analysis attempts to formalize the process and reduce it to algebra, but it is difficult or impossible to represent all the components of a decision mathematically and validly let alone do so in “real time” for individual patients. This review discusses these challenges and more, including how to ask answerable questions, understand the hierarchy for evidence-based information resources, critically appraise evidence, and apply research results to patient care. Figures show the total number of new articles in Medline from 1965 to 2012, a “4S” hierarchy of preappraised medicine, percentage of physician and medical student respondents with a correct or incorrect answer to a question about calculating the positive predictive value of a hypothetical screening test, a nomogram for Bayes’s rule, an example of nomogram use for pulmonary embolism, and a model for evidence-informed clinical decisions. Tables list selected barriers to the implementation of EBM; Patient, Intervention, Comparison, and Outcome (PICO) framework for formulating clinical questions; guides for assessing medical texts for evidence-based features; clinically useful measures of disease frequency and statistical significance and precision; definitions of clinically useful measures of diagnostic test performance and interpretation; definitions of clinically useful measures of treatment effects from clinical trials; summary of results and derived calculations from the North American Symptomatic Carotid Endarterectomy Trial (NASCET); and selected number needed to treat values for common therapies.

      This review contains 6 highly rendered figures, 9 tables, and 28 references.

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    • 4

      Quality of Care: Performance Measurement and Quality Improvement in Clinical Practice

      By Sonali P. Desai, MD, MPH; Allen Kachalia, MD, JD
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      Quality of Care: Performance Measurement and Quality Improvement in Clinical Practice

      • SONALI P. DESAI, MD, MPHAmbulatory Director, Patient Safety, Center for Clinical Excellence, Associate Director of Quality, Department of Medicine, Division of Rheumatology, Brigham and Women's Hospital, Boston, MA
      • ALLEN KACHALIA, MD, JDAssociate Chief Quality Officer, Co-Director, Center for Clinical Excellence, Brigham and Women's Hospital, Boston, MA

      Attention to the quality of care within the United States health care system has grown tremendously over the past decade. We have witnessed a significant change in how quality improvement and clinical performance measurement are approached. The current focus on quality and safety stems in part from the increasingly clear realization that more services and technological advancement are not automatically equivalent to high-quality care. Much of the discussion about cost and quality in health care is shifting towards the concept of value. Value is defined as health outcomes achieved per dollar spent (in other words, an assessment of the quality of care per cost). This chapter reviews the current state of quality improvement in health care and, because improvement cannot be determined without measurement, reviews several aspects of effective clinical performance measurement. Since many measures are already in place, the chapter describes some of the organizations involved in quality measurement and improvement, as well the approaches they utilize. It looks at the multiple strategies in place to improve quality, from process management to collaboration, from financial incentives to transparency, and reviews newer models of care delivery that may materialize in the near future. Tables list types of quality measures, characteristics to consider when developing a quality measure, and organizations involved in quality improvement and performance measurement. A figure shows strategies used by the federal government to spur performance measurement and quality improvement. This chapter contains 56 references.

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    • 5

      Medical Evaluation of the Surgical Patient

      By Marie Gerhard-Herman, MD; Jonathan Gates, MD
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      Medical Evaluation of the Surgical Patient

      • MARIE GERHARD-HERMAN, MDDepartment of Medicine, Cardiovascular Division, Brigham and Womens Hospital, Boston, MA
      • JONATHAN GATES, MDDirector of the Burn and Trauma Unit, Department of Surgery, Brigham and Womens Hospital, Boston, MA

      Medical evaluation prior to surgery includes risk assessment and the institution of therapies to decrease perioperative morbidity and mortality to improve patient outcomes. The most effective medical consultation for surgical patients begins with an assessment of the individual patient and knowledge of the planned surgery and anesthesia followed by clear communication of a concise and specific recommended plan of perioperative care to the surgical team. This chapter describes anesthetic, cardiac, pulmonary, hepatic, nutritional, and endocrine risk assessment. Perioperative thrombotic management and postoperative care and complications, including fluid management; pulmonary, cardiac, renal complications; and delirium are discussed. Tables outline the American Society of Anesthesiologists class and perioperative mortality risk, a comparison of the Revised Cardiac Risk Index and National Surgery Quality Improvement Program, Duke Activity Status Index, high-risk stress test findings, markers for increased perioperative risk in pulmonary hypertension, aortic stenosis and nonemergent noncardiac surgery, risk factors for pulmonary complications in noncardiac surgery, the Model for End-Stage Liver Disease score to predict postoperative mortality, venous thromboembolism risk factors and options for pharmacologic prophylactic regimens, perioperative management of warfarin, and Brigham and Women’s Hospital guidelines for postoperative blood product replacement. Figures include a care algorithm for noncardiac surgery, an illustration of types of myocardial infarction, and an algorithm for the treatment of postoperative delirium.

      This chapter contains 3 highly rendered figures, 12 tables, 68 references, and 5 MCQs.

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    • 6

      Complementary, Alternative, and Integrative Medicine

      By Josephine P. Briggs, MD
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      Complementary, Alternative, and Integrative Medicine

      • JOSEPHINE P. BRIGGS, MD

      Complementary and alternative medicine (CAM) refers to a group of diverse medical and health care systems, practices, and products that are not considered to be part of conventional or allopathic medicine. Most of these practices are used together with conventional therapies and have therefore been called complementary to distinguish them from alternative practices, which are those used instead of standard care. Although some CAM practices are directed by an alternative health care provider, such as a chiropractor, acupuncturist, or naturopathic practitioner, much of CAM is undertaken as "self-care" and paid for out of pocket. Although much of CAM remains firmly outside the mainstream, a number of physicians are advocating an approach called integrative medicine, which incorporates some CAM practices into conventional care. This chapter reviews information on a variety of self-help and practitioner-based practices that fall under the general umbrella of CAM. The review includes the major therapies currently used in the United States, the settings in which these CAM therapies are used, and the evidence base for the safety and efficacy of some of the most commonly used modalities. The chapter also reviews the current regulatory framework for dietary supplements and for licensure of CAM practitioners and provides advice on sources of evidence-based information and tools for patient education. Figures illustrate CAM use by age, race/ethnicity, and education; the 10 most common CAM therapies among adults in 2007, the 10 most common natural products used by adults in 2002 and 2007; and diseases/conditions for which CAM was most frequently used among adults in 2002 and 2007. Tables describe major complementary and alternative health practices, CAM therapies whose use increased significantly between 2002 and 2007, Internet sources for patient information and education, health care provider information sources, and resources to assess dietary supplement-drug interactions. This chapter has 126 references.

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  • Neurological Symptoms
    • 1

      Headache and Facial Pain

      By Elizabeth W. Loder, MD, MPH
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      Headache and Facial Pain

      • ELIZABETH W. LODER, MD, MPHAssociate Professor of Neurology, Harvard Medical School, Chief, Division of Headache and Pain, Department of Neurology, Brigham and Women’s Hospital, Boston, MA

      Headaches are a near-universal experience, with a 1-year prevalence of 90% and a lifetime prevalence of 99%. Headaches and pain to the head account for roughly 3% of visits to US emergency departments annually, making them the fourth most common reason for seeking emergency care. There are numerous types of headaches, and although the majority are benign, types exist that may result from serious and potentially life-threatening causes. As such, it is important for the physician to consider a broad differential diagnosis for every headache patient. This review discusses the classification of headaches, identifies pain-sensitive structures in the head, discusses the history and examination in patients with headache, and describes many of the primary and secondary headaches. Figures show the areas of the brain sensitive to pain; 1-year prevalence of migraine in men, women, and children; frequency of attacks in migraineurs; prevalence of headaches by age group and in patients with cerebrovascular disorders; and symptoms of idiopathic intracranial hypertension. Tables list the major categories of headache disorders, key elements of the headache history, helpful questions to ask, features of selected primary and secondary headaches, reasons to consider neuroimaging, efficacy of selected over-the-counter medications, triptans available in the United States, medication options for urgent or emergency treatment of migraine, selected preventive medications for migraine, generally accepted indications for preventive treatment, general principles for the use of preventive medications, titration schedules for preventive medication, interval or short-term preventive treatment of menstrual migraine, strategies for managing increase in migraines in patients starting estrogen replacement therapy, transition medications for rapid, temporary suppression of headaches, medications possibly effective for cluster and hypnic headaches, differential diagnosis of the acute, severe, new-onset headache, and etiologies of papilledema and headache.

      This review contains 6 highly rendered figures, 20 tables, and 112 references.

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    • 2

      Tension-type Headache: Epidemiology, Diagnosis, and Pathophysiology

      By Paul Rizzoli, MD, FAAN, FAHS
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      Tension-type Headache: Epidemiology, Diagnosis, and Pathophysiology

      • PAUL RIZZOLI, MD, FAAN, FAHSAssistant Professor of Neurology, Harvard Medical School, Clinical and Fellowship Director John R. Graham Headache Center, Brigham and Women’s Faulkner Hospital, Boston, MA

      Tension-type headache (TTH) is a significant but underappreciated condition that is much more frequent than migraine, 42% versus 11%, and produces significant socioeconomic burden. Why then do research advances in this condition seem to lag?

      One reason is that precise epidemiologic data are lacking, with lifetime prevalence estimates varying from about 13 to 78%. Also, classification is confounded by whether or not to include the occasional but universal headache as TTH. Furthermore, TTH pathophysiology is debated, with some feeling that the pathophysiology of TTH is similar to and on a spectrum with migraine, and some feeling that it is entirely separate and related to peripheral and muscular mechanisms. More recently, central pain mechanisms have also been implicated in the pathophysiology. In addition, a large body of information connects stress and TTH.

      Although TTH varies widely in frequency and severity among and within patients, TTH pain, compared with pain in other headache types, could be characterized generally as more mild in severity and more generalized in location. This review discusses the current epidemiologic data and diagnostic challenges in TTH and the current pathophysiologic mechanisms.

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    • 3

      Other Primary Headache: Physical Exertion and Other (COMING FEBRUARY 2017)

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      Other Primary Headache: Physical Exertion and Other (COMING FEBRUARY 2017)

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    • 4

      Dizziness

      By Kevin A. Kerber, MD, MS; Robert W. Baloh, MD
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      Dizziness

      • KEVIN A. KERBER, MD, MSAssistant Professor, Department of Neurology, University of Michigan, Ann Arbor, MI
      • ROBERT W. BALOH, MDProfessor, Departments of Neurology and Surgery (Head and Neck), UCLA Medical Center, Reed Neurological Research Center, Los Angeles, CA

      Dizziness is the quintessential symptom presentation in all of clinical medicine. It is a common reason that patients present to a physician. This chapter provides background information about the vestibular system, then reviews key aspects of history-taking and examination of the patient, then discusses specific disorders and common presentation types. Throughout the chapter the focus is on neurologic and vestibular disorders. Normal vestibular anatomy and physiology are discussed, followed by recommendations for history-taking and the physical examination. Specific disorders that cause dizziness are explored, along with common causes of non-specific dizziness. Common presentations are discussed, including acute severe dizziness, recurrent attacks, and recurrent positional vertigo. Finally, the chapter looks at laboratory investigations in diagnosis and management. Figures include population prevalence of dizziness symptoms, the anatomy of inner structures, primary afferent vestibular nerve activity, the head thrust test, the Dix-Hallpike maneuver, the supine positional test, the canalith repositioning procedure, and the barbecue roll maneuver. Tables list physiologic properties and clinical features of the components of the peripheral vestibular system, information to be acquired from history of the present illness, common symptoms patients report as dizziness, examination components, distinguishing among common peripheral and central vertigo syndromes, common causes of nonspecific dizziness, types of dizziness presentations, relevant imaging abnormalities on neuroimaging studies, vestibular testing components, and medical therapy for symptomatic dizziness.

      This review contains 8 highly rendered figures, 10 tables, and 68 references.

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    • 5

      Pain Syndromes Other Than Headache

      By Edgar L. Ross, MD
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      Pain Syndromes Other Than Headache

      • EDGAR L. ROSS, MD

      Pain is experienced within a complex biologic, emotional, psychological, and social context that may defy physical examination, diagnostic procedures, and laboratory tests. This chapter aims to empower internists to improve their medical practices in pain management. It provides a scientific background that covers nociception and how sensory processing occurs at multiple levels in the body. Clinical assessment is detailed, as well as diagnostic categories that include mixed or uncertain chronic pain syndromes (back pain, fibromyalgia, postamputation pain, pain from cancer and bone) and neuropathic pain syndromes (polyneuropathy, mononeuropathy multiplex, ganglionopathy, genetic disorders, focal and regional syndromes). Treatment of chronic pain can be surgical or interventional. Pharmacologic treatment for acute and chronic nociceptive pain includes special considerations for geriatric and terminal patients. For treatment of neuropathic pain, medications are the major component. One tables lists iatrogenic nerve injuries that can cause posttraumatic neuralgia and complex regional pain syndrome. Other tables detail stepwise pharmacologic management of neuropathic pain and cite recommendations on opioid use from the Centers for Disease Control and Prevention. One figure illustrates how pain transducers monitor and influence tissue conditions. Other figures show sensory processing in the spinal cord dorsal horn, physical findings in the feet of patients with bilateral foot pain from small-fiber polyneuropathy, illustrate how examination can identify specific nerve injuries causing chronic pain, and provide classification of chronic pain syndromes. This chapter contains 82 references.

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    • 6

      Fatigue and Asthenia

      By Thomas D. Sabin, MD; David M. Dawson, MD
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      Fatigue and Asthenia

      • THOMAS D. SABIN, MDProfessor of Neurology, Tufts University School of Medicine, Boston MA
      • DAVID M. DAWSON, MDProfessor of Neurology, Brigham and Women’s Hospital, Boston MA

      The word fatigue implies a sense of exhaustion, lassitude, or inability to generate drive or energy. In taking a medical history of a patient with fatigue, the examiner should pursue questions of timing and circumstance. A medical or psychiatric diagnosis can be established in two thirds of patients who complain of fatigue, and in many cases, they are remediable. This chapter reviews the history of fatigue, including outbreaks. The clinical picture, diagnostic criteria, special considerations in diagnosis, general medical disorders, prescription drugs, postural orthostatic tachycardia syndrome, neutrally mediated hypotension, Chiari malformation, neurologic causes of fatigue, psychiatric investigation, laboratory investigations, magnetic resonance imaging (MRI) studies, pathophysiology, prognosis, and treatment are detailed. Asthenia is discussed in relation to clinical data, the central nervous system, muscle, and electromyography (EMG). Tables include terms and phrases used for indicating fatigue; definitions, features, and medical causes of chronic fatigue syndrome (CFS); blood tests to evaluate chronic fatigue; symptoms of CFS determined from laboratory studies; MRI studies for detecting CFS; outcomes of MRI studies of CFS; summary of CFS facts; summary of drugs used for fighting fatigue in multiple sclerosis patients; clinical testing for signs of asthenia; considerations when measuring creatine kinase; EMG testing in search of abnormalities; and decisive EMG findings. Figures show maps of sensory loss, an MRI of a relapsing-remitting multiple sclerosis patient, white matter hyperintensities, a diagram of EMG testing, and repetitive stimulation testing for neuromuscular transmission disorder.

      This chapter contains 5 highly rendered figures, 14 tables, 74 references, and 5 MCQs.

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    • 7

      Disorders of Consciousness

      By Eelco F. M. Wijdicks, MD, PhD, FACP; Jennifer E Fugate, DO
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      Disorders of Consciousness

      • EELCO F. M. WIJDICKS, MD, PHD, FACP
      • JENNIFER E FUGATE, DOInstructor of Neurology, Division of Critical Care Neurology, Mayo Clinic, Rochester, MN

      The components of consciousness are classically separated into two major groups: level of arousal and content of thought processes (better known as being awake and aware). Disorders can affect both components or can affect only the content of consciousness. Physicians assess consciousness indirectly by observing a patient’s response to stimuli. This chapter covers the clinical approach to the comatose patient, which discusses history, physical examination (general and neurologic), localization principles, bihemispheric syndromes, and brainstem syndromes. Diagnostic tests discussed include blood tests, tests of other body fluids, and neuroimaging (e.g., computed tomography, magnetic resonance imaging, functional neuroimaging and physiologic testing). Also discussed are prognosis and brain death. Figures illustrate the Full Outline of Unresponsiveness (FOUR) scale, the spectrum of pupil abnormalities and causes, as well as anatomic structures and dorsal and ventral pathways involved with the maintenance of consciousness. Tables include the Glasgow Coma Scale, blood gas abnormalities due to toxins, and prognostic factors in nontraumatic coma. This chapter contains 45 references.

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    • 8

      Sleep Disorders

      By Sudhansu Chokroverty, MD, FRCP, FACP
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      Sleep Disorders

      • SUDHANSU CHOKROVERTY, MD, FRCP, FACPProfessor and Director of Sleep Research, Medical Director of Devry Technology Training Program, Co-Chair Emeritus of Neurology, Department of Neurology, JFK Neuroscience Institute, Edison, NJ, Professor of Neuroscience, Seton Hall University, South Orange, NJ, Clinical Professor of Neurology, Robert Wood Johnson Medical School, New Brunswick, NJ

      Recent research has generated an enormous fund of knowledge about the neurobiology of sleep and wakefulness. Sleeping and waking brain circuits can now be studied by sophisticated neuroimaging techniques that map different areas of the brain during different sleep states and stages. Although the exact biologic functions of sleep are not known, sleep is essential, and sleep deprivation leads to impaired attention and decreased performance. Sleep is also believed to have restorative, conservative, adaptive, thermoregulatory, and consolidative functions. This review discusses the physiology of sleep, including its two independent states, rapid eye movement (REM) and non–rapid eye movement (NREM) sleep, as well as functional neuroanatomy, physiologic changes during sleep, and circadian rhythms. The classification and diagnosis of sleep disorders are discussed generally. The diagnosis and treatment of the following disorders are described: obstructive sleep apnea syndrome, narcolepsy-cataplexy sydrome, idiopathic hypersomnia, restless legs syndrome (RLS) and periodic limb movements in sleep, circadian rhythm sleep disorders, insomnias, nocturnal frontal lobe epilepsy, and parasomnias. Sleep-related movement disorders and the relationship between sleep and psychiatric disorders are also discussed. Tables describe behavioral and physiologic characteristics of states of awareness, the international classification of sleep disorders, common sleep complaints, comorbid insomnia disorders, causes of excessive daytime somnolence, laboratory tests to assess sleep disorders, essential diagnostic criteria for RLS and Willis-Ekbom disease, and drug therapy for insomnia. Figures include polysomnographic recording showing wakefulness in an adult; stage 1, 2, and 3 NREM sleep in an adult; REM sleep in an adult; a patient with sleep apnea syndrome; a patient with Cheyne-Stokes breathing; a patient with RLS; and a patient with dream-enacting behavior; schematic sagittal section of the brainstem of the cat; schematic diagram of the McCarley-Hobson model of REM sleep mechanism; the Lu-Saper “flip-flop” model; the Luppi model to explain REM sleep mechanism; and a wrist actigraph from a man with bipolar disorder.

      This review contains 14 highly rendered figures, 8 tables, 115 references, and 5 MCQs.

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    • 9

      Tension-type Headache: Acute and Preventive Therapies

      By Melissa Rayhill, MD
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      Tension-type Headache: Acute and Preventive Therapies

      • MELISSA RAYHILL, MDAssistant Professor, Department of Neurology, University at Buffalo School of Medicine, the State University of New York at Buffalo

      Tension-type headache (TTH) is an incredibly common condition. The clinician should be careful to distinguish TTH from migraine and from causes of secondary headache. The importance of regular sleep, nutrition, hydration, and appropriate management of life stressors cannot be overemphasized. The mainstays of abortive pharmacologic therapy for TTH are the nonsteroidal antiinflammatory drugs. Most of these drugs are thought to have roughly equivalent efficacies based on many older clinical trials and more recent meta-analyses. The side effects of this drug class can be severe and include renal toxicity and gastrointestinal bleeding; these drugs may also increase cardiovascular risk. Tricyclic antidepressants are thought to be the most effective preventive therapy for TTH, particularly amitriptyline. Other antidepressant medications as well as muscle relaxants may also be beneficial in some patients. A number of other nonpharmacologic and procedural therapies exist, although the evidence supporting the use of these treatments is variable. However, in many patients, these other modalities can be helpful therapeutic adjuncts. In this review, we also discuss the evidence base for physical therapy, acupuncture, trigger-point injections, massage therapy, and psychological therapy. 

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    • 10

      Difficult to Treat (refractory) Chronic Migraine: Outpatient Approaches

      By Lawrence Robbins, MD, (retired)
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      Difficult to Treat (refractory) Chronic Migraine: Outpatient Approaches

      • LAWRENCE ROBBINS, MD, (RETIRED) Assistant Professor of Neurology, Dept. of Neurology, University of Illinois; (retired) Assistant Professor of Neurology, Dept. of Neurology, Rush Medical College, Chicago, Il

      This comprehensive review addresses the many challenges in treating refractory migraine. Issues relating to pathophysiology are covered. A unique “refractory scale for migraine patients” is introduced. The definition and role of medication overuse headache are presented with a much different perspective than is usually found. Issues outside of medication that are covered include active coping, acceptance, resilience, and catastrophizing. A number of outpatient treatments are thoroughly discussed. These include the role of onabotulinum toxin, the application of polypharmacy, when to employ sphenopalatine ganglion  blocks, the role of occipital and trigger-point injections, the implementation of long-acting opioids, the advantages of stimulants, and the possible use of monoamine oxidase inhibitors. Miscellaneous approaches include muscle relaxants, nasal or intravenous ketamine, transcranial magnetic stimulation, memantine, and ergonovine. Finally, many cutting-edge “refractory clinical pearls” are listed. 

      This review contains 8 highly rendered figures, 4 tables, and 25 references.

      Key Words: Headache, migraine, chronic, refractory, medication overuse, alternative, treatments

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    • 11

      Nerve Blocks and Neurostimulation in the Treatment of Migraine

      By Matthew S Robbins, MD
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      Nerve Blocks and Neurostimulation in the Treatment of Migraine

      • MATTHEW S ROBBINS, MDAssociate Professor of Clinical Neurology, Albert Einstein College of Medicine, Chief of Neurology, Jack D. Weiler Hospital, Montefiore Medical Center, Director of Inpatient Services, Montefiore Headache Center, Associate Program Director, Neurology Residency, Bronx, NY

      Peripheral nerve and sphenopalatine ganglion blocks are a safe, effective treatment option for headache disorders, although, despite a wealth of anecdotal experience, the evidence is conflicting for efficacy in chronic migraine prophylaxis. Neurostimulation has emerged as an effective treatment modality for migraine with both noninvasive and minimally invasive options available. Such options include transcutaneous supraorbital nerve stimulation for prophylaxis and single-pulse transcranial magnetic stimulation for the acute treatment of migraine with aura. Although occipital nerve stimulation may be effective for some patients with intractable chronic migraine, the evidence is mixed and procedure-related complications are common. Emerging treatment modalities for acute and preventive treatment of migraine include noninvasive vagus nerve stimulation and implanted sphenopalatine ganglion stimulation.

      This review contains 5 highly rendered figures, 2 tables, and 106 references.

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    • 12

      Migraine: Psychiatric Comorbidities

      By Todd A Smitherman, PhD; Anna Katherine Black, MA; A Brooke Walters Pellegrino, PhD
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      Migraine: Psychiatric Comorbidities

      • TODD A SMITHERMAN, PHDAssociate Professor of Psychology, Department of Psychology, University of Mississippi, Oxford, MS
      • ANNA KATHERINE BLACK, MAGraduate Student, Clinical Psychology, Department of Psychology, University of Mississippi, Oxford, MS
      • A BROOKE WALTERS PELLEGRINO, PHDDirector of Behavioral Medicine, Hartford Healthcare Headache Center, West Hartford, CT

      Psychiatric disorders often co-occur with migraine, and these comorbid conditions compound disability and are risk factors for medication overuse and migraine progression. For these reasons, attention to psychiatric comorbidities in clinical practice is of paramount importance. Assessment of depression, anxiety, and sleep disorders is recommended, focusing on the core cognitive and emotional symptoms of the comorbidities and using measures validated among medical patients. Pharmacologic treatment of migraine and comorbid psychiatric conditions is challenging owing to a lack of agents with proven efficacy for both conditions, side effect profiles that may exacerbate one condition, and potential drug interactions. Existing data suggest that migraineurs with psychiatric symptomatology can obtain positive outcomes with appropriate preventive medications, behavioral interventions for headache or the comorbid condition, or a combination thereof.

      Keywords: anxiety, comorbidity, depression, insomnia, migraine, pharmacotherapy, relaxation, stress management

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    • 13

      Migraine: Behavioral Treatment

      By Elizabeth K Seng, PhD
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      Migraine: Behavioral Treatment

      • ELIZABETH K SENG, PHDAssistant Professor, Ferkauf Graduate School of Psychology, Yeshiva University, New York, NY

      Behavior change is an essential component of any migraine management plan. Behavioral migraine treatments are interventions designed to change a patient’s behavior with the result of a reduction in migraine symptoms and migraine-related disability. Behavioral treatments commonly target medication adherence, behavioral and psychosocial factors known to precipitate migraine (including stress, sleep, and skipping meals), maladaptive cognitive patterns, and comorbid psychiatric symptoms (most commonly depression and anxiety). Guidelines and evidence from randomized clinical trials indicate that biofeedback, relaxation treatments, and cognitive-behavioral therapy are effective preventive migraine treatments. Patient education and self-monitoring are foundational components to any behavioral intervention for migraine. Portable personal technology is increasingly becoming an essential part of migraine patient care and provides another avenue for supporting adherence to medication and behavioral migraine management.  

      Key words: anxiety; behavior; biofeedback; cognitive-behavioral therapy; depression; migraine; psychology; relaxation; sleep; stress

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    • 14

      Postherpetic Neuralgia: A Patient’s and a Physician’s Perspective

      By James H. Diaz, MD, MHA, MPH, DrPH, FACA, DABA, FACPM
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      Postherpetic Neuralgia: A Patient’s and a Physician’s Perspective

      • JAMES H. DIAZ, MD, MHA, MPH, DRPH, FACA, DABA, FACPM

      Herpes zoster can plague anyone who has had varicella or has received the varicella or chickenpox vaccine. The incidence of herpes zoster increases with age and rises exponentially after 60 years of age. Postherpetic neuralgia (PHN) may occur after herpes zoster at any age but typically occurs after 50 years of age, with over 40% of persons over 60 years of age suffering from PHN after a shingles attack. Up to 1 million new cases of herpes zoster and 200,000 new cases of PHN may now be anticipated in the United States every year, with the incidence rate increasing as the population grows and ages with prolonged life expectancies. Although new antiviral medications will improve and shorten the course of herpes zoster, they do not guarantee the prevention of PHN. Given the high prevalence of PHN in an aging population and the availability of primary prevention by vaccination, the objectives of this review are to describe the epidemiology, pathophysiology, and clinical manifestations of zoster and PHN and to recommend a combination of strategies for the clinical management and prevention of PHN.

      Key words: evidence-based pain medicine, herpes zoster, neuropathic pain, postherpetic neuralgia

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    • 15

      Migraine Epidemiology, Impact, and Pathogenesis

      By Amy A Gelfand, MD; Dawn C Buse, PhD
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      Migraine Epidemiology, Impact, and Pathogenesis

      • AMY A GELFAND, MDDirector, Pediatric Headache, Division of Child Neurology, Department of Neurology, UCSF Benioff Children’s Hospital, San Francisco, CA
      • DAWN C BUSE, PHDAssociate Professor, Department of Neurology, Albert Einstein College of Medicine of Yeshiva University, Assistant Professor, Clinical Health Psychology Doctoral Program, Ferkauf Graduate School of Psychology of Yeshiva University, Director of Behavioral Medicine, Montefiore Headache Center Bronx, NY

      Migraine is a common and often disabling neurologic disorder. No longer thought of as neurovascular in etiology, migraine is now known to be a complex disorder of the brain with strong genetic underpinnings. The impact of migraine may extend beyond the affected individual to also impact partners and children. Although many patients search to identify “triggers” of migraine, teasing out such relationships can be remarkably complex. The premonitory phase of a migraine attacks can include symptoms such as food cravings, photophobia, and increased yawning—symptoms that could, for example, lead a person to mistakenly conclude that the migraine attacks are “triggered” by eating chocolate, bright lights, or being tired. We review current evidence on the epidemiology, impact, and pathophysiology of migraine. 

      Key words: epidemiology, impact, migraine, pathophysiology

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    • 16

      Seizure

      By Robert Silbergleit, MD
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      Seizure

      • ROBERT SILBERGLEIT, MDProfessor, Neurological Emergencies Research, Department of Emergency Medicine, Ann Arbor, MI

      A seizure is a sudden change in behavior that is accompanied by electrical discharges in the brain. Many patients presenting with a first-ever seizure are surprised to find that it is a very common event in both children and adults. Epilepsy, a chronic disorder of the brain characterized by recurrent unprovoked seizures, is far less common. Patients who present to the emergency department with seizures vary considerably in underlying etiology, symptoms, and prognosis. Optimal care of the seizure patient in the emergency department requires differentiating those who need little intervention from those requiring intensive resuscitation. This review presents the pathophysiology, stabilization and assessment, diagnosis and treatment, and disposition and outcomes of seizure. Figures show the progression of selected neuronal pathophysiologic mechanisms involved over time during and after status epilepticus, tongue bite from seizure, a general emergency department management strategy for patients with seizure presentations, an electroencephalogram of a patient who experienced convulsive syncope after placement of an intravenous line, and staged treatment of status epilepticus. Tables list key elements of the initial emergency department management of status epilepticus, third-line medications for treatment of seizures, and the Status Epilepticus Severity Score (STESS).

      Key words: acute seizure, convulsion, epilepsy, seizure, status epilepticus

      This review contains 5 highly rendered figures, 3 tables, and 54 references.

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  • Categories of Neurological Diseases
    • 1

      Chorea: Classification, Differential Diagnosis, and Treatment

      By James P Battista, MD; Ruth H Walker, MB, ChB, PhD
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      Chorea: Classification, Differential Diagnosis, and Treatment

      • JAMES P BATTISTA, MDMovement Disorders Division, Department of Neurology, Icahn School of Medicine at Mount Sinai, New York, NY
      • RUTH H WALKER, MB, CHB, PHDMovement Disorders Clinic, Department of Neurology, James J. Peters VAMC, Bronx, NY; Movement Disorders Division, Department of Neurology, Icahn School of Medicine at Mount Sinai, New York, NY

      Chorea is a common hyperkinetic movement disorder that can have a large differential diagnosis. Causes can include genetic and sporadic etiologies, including metabolic abnormalities, autoimmune, structural, paraneoplastic, psychogenic, and iatrogenic causes. The workup can be challenging due to this large number of possible causes, and can include basic metabolic and specialized blood analysis such as genetic testing, in addition to imaging studies and cerebrospinal fluid analysis. Treatment of symptoms outside the realm of reversible causes can be challenging, and is a major focus of current research. This review covers sporadic causes, genetic causes, and management. Figures show a flowchart for evaluation of patients with chorea, a computed tomography scan of a Huntington disease patient showing caudate head atrophy, brain computed tomography scan showing calcification, and acanthocytosis. Videos show mild and moderate Huntington disease, Huntington disease-like 2,  and chorea-acanthocytosis. Tables list metabolic abnormalities that can manifest as chorea, paraneoplastic and nonparaneoplastic syndromes associated with chorea, and reported successful therapies for chorea.

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    • Cognitive and Behavioral Neurology
      • 1

        Neurobiology of Anxiety Disorders

        By Pooja Palkar, MBBS; Eric Hollander, MD
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        Neurobiology of Anxiety Disorders

        • POOJA PALKAR, MBBS Fellow - Autism and Obsessive Compulsive Spectrum & Anxiety and Depression Program, Montefiore Medical Center, Albert Einstein College of Medicine
        • ERIC HOLLANDER, MDDirector - Autism and Obsessive-Compulsive Spectrum Program, and Anxiety and Depression Program, Clinical Professor of Psychiatry and Behavioral Sciences, Albert Einstein College of Medicine and Montefiore Medical Center

        In recent years, advances in the fields of neuroimaging and experimental psychology increased our understanding of the basic mechanisms of classical conditioning and learning, contributing to our knowledge of the neurobiology of anxiety disorders. Research has shown that the amygdala is the cornerstone of fear circuitry and that abnormalities in amygdala pathways can affect the acquisition and expression of fear conditioning. Activation of the amygdala in response to disorder-relevant stimuli has been observed in anxiety disorders. The roles of the hippocampus, nucleus accumbens, periaqueductal gray, and insular and medial prefrontal cortices in response to fear have been identified as well. Neurotransmitters such as serotonin, dopamine, γ-aminobutyric acid, glutamate, and some neurosteroids play an important part in the neurobiology of anxiety disorders. Neuropeptides such as oxytocin, neuropeptide Y, galanin, and cholecystokinin have been shown to modulate stress response. Drugs such as N-methyl-d-aspartate (NMDA) antagonists and blockers of voltage-gated calcium channels in the amygdala are anxiolytic. Fear extinction, which entails new learning of fear inhibition, is the mechanism of effective antianxiety treatments such as d-cycloserine, a partial NMDA agonist. Extinction is thought to occur by the medial prefrontal cortex, which inhibits the lateral amygdala under hippocampal modulation. Harnessing extinction to delink neutral stimuli from aversive responses is an important goal of the psychotherapy and pharmacotherapy of anxiety disorders. Discovery of the role of microRNAs in the etiology of anxiety disorders and their possible utility as targets to treat these disorders is fascinating. In this review, we discuss the neurobiology of anxiety disorders, which will help us better manage them clinically.

        Key words: amygdala, anxiety disorders, neurobiology, fear conditioning, neurocircuitry, neurotransmitters, neuropeptides, neurosteroids, endogenous opioids.

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      • 2

        Cognitive Disorders Other Than Alzheimer Disease

        By Seth A Gale, MD; Kirk R. Daffner, MD, FAAN
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        Cognitive Disorders Other Than Alzheimer Disease

        • SETH A GALE, MDAssociate Neurologist, Division of Cognitive and Behavioral Neurology, Brigham and Women’s Hospital, and Instructor of Neurology, Harvard Medical School, Boston, MA
        • KIRK R. DAFFNER, MD, FAANChief, Division of Cognitive and Behavioral Neurology, Brigham and Women’s Hospital, J. David and Virginia Wimberly Professor of Neurology, Harvard Medical School, Boston, MA

        Higher cognitive functions, such as abstract reasoning, complex decision making, and language, are the mental faculties that separate our species from other animals. When these faculties become impaired as a result of neurologic disease, striking and devastating behavioral changes result. Many neurologic diseases are associated with impaired cognition and behavior, and their etiologies are as varied as their clinical presentations. In this review, the focus is on dementia with Lewy bodies (DLB), the frontotemporal dementias (FTDs), and vascular cognitive impairment (VCI). The review covers the epidemiology and diagnosis of  DBB, FTDs, and VCIs, as well as the etiology and genetics. Figures show neuroimaging in DLB, management of DLB, FTLD clinical syndromes, FTLD clinicopathologic correlations: approximate distribution of pathotypes for behavioral-variant FTD and primary progressive aphasia (PPA) variants, PPA-semantic subtype, PPA-logopenic subtype, post-stroke VCI, and subcortical ischemic vascular disease subtype of VCI. Tables list diagnostic criteria for DLB, FTD genetics: gene/protein relationship, clinical syndrome, salient geatures, VCI: clinical and pathologic features of the main subtypes, summary guidance based on VCI prevention studies, and summary guidance based on VCI treatment studies.

         

        This review contains 8 highly rendered figures, 5 tables, and 254 references.

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      • 3

        Stroke and Other Cerebrovascular Diseases

        By Scott E. Kasner, MD, MSCE, FAHA, FAAN; Christina A Wilson, MD, PhD
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        Stroke and Other Cerebrovascular Diseases

        • SCOTT E. KASNER, MD, MSCE, FAHA, FAANProfessor, Department of Neurology, Perelman School of Medicine, University of Pennsylvania, Director, Comprehensive Stroke Center, University of Pennsylvania Health System, Philadelphia, PA
        • CHRISTINA A WILSON, MD, PHDAssistant Professor, Department of Neurology, University of Florida, Gainesville, FL

        Stroke is a leading cause of neurologic morbidity and mortality, and rapid treatment is key for a good outcome. This review addresses the epidemiology, common presenting symptoms, causes, and treatment of ischemic stroke, intracerebral hemorrhage, and subarachnoid hemorrhage. Current recommendations for the emergent evaluation and treatment of an acute ischemic stroke are highlighted, including recently updated indications and contraindications for intravenous recombinant tissue plasminogen activator administration and recent guidelines for the expanded role of endovascular mechanical embolectomy for stroke due to acute large vessel occlusion. An algorithm of diagnostic evaluations to assist with identification of the cause of ischemic stroke is offered. Evidence-based primary and secondary stroke prevention is discussed, including the ideal choice of antithrombotic based on identified stroke mechanism and optimal risk factor management. Best practice supportive measures for the post-stroke patient are highlighted, including recent guidelines for the management of elevated intracranial pressure. Management of uncommon causes of ischemic stroke is also addressed. 

        Key Words: Intracerebral hemorrhage, ischemic stroke, recombinant tissue plasminogen activator, subarachnoid hemorrhage

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      • 4

        Major Neurocognitive Disorders

        By Deena J Tampi, MSN, MBA-HCA, RN; Rajesh R Tampi, MD, MS, DFAPA
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        Major Neurocognitive Disorders

        • DEENA J TAMPI, MSN, MBA-HCA, RNExecutive director of Behavioral Health Services, Saint Francis Hospital and Medical Center, Hartford, CT.
        • RAJESH R TAMPI, MD, MS, DFAPAProfessor of psychiatry at Case Western Reserve University School of Medicine and vice chairman for education and faculty development and program director in the Psychiatry Residency, Department of Psychiatry, MetroHealth, Cleveland, OH

        Major neurocognitive disorder is the most common neurodegenerative condition in the world and the leading cause of dependence and disability among older adults worldwide. There are numerous etiologies for major neurocognitive disorder, of which Alzheimer disease (AD) is the most common. Available evidence indicates that the risk factors for major neurocognitive disorder include older age, female sex, lower educational attainment, obesity, and vascular risk factors, including smoking, hypertension, diabetes mellitus, and hyperlipidemia. Certain etiologies for major neurocognitive disorder are heritable, especially those due to AD and frontotemporal lobar degeneration. The pathophysiologic changes associated with the various etiologies of major neurocognitive disorder include neuronal loss, senile plaques, neurofibrillary tangles, vascular pathology, and α-synuclein neuronal inclusions. Major neurocognitive disorder remains a clinical diagnosis with a thorough history, appropriate laboratory tests, and standardized rating scales assisting in determining the etiology and severity of the condition. In older adults, major neurocognitive disorder must be differentiated from depression and delirium as these three conditions may have similar clinical presentations or may coexist. Current data indicate that approximately a third of the cases of major neurocognitive disorder, especially those due to AD, may be prevented by controlling potentially modifiable risk factors, including diabetes, depression, smoking, physical inactivity, midlife hypertension, midlife obesity, and low educational attainment. Currently, the only Food and Drug Administration–approved medications are acetylcholinesterase inhibitors and memantine for use in major neurocognitive disorder due to AD and rivastigmine (an acetylcholinesterase inhibitor) for major neurocognitive disorder due to Parkinson disease.

         

        Key words: acetylcholinesterase inhibitors, Alzheimer disease, amyloid precursor protein, frontotemporal lobar degeneration, Lewy body disease, major neurocognitive disorder, memantine, Parkinson disease, tau proteins, vascular disease

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      • 5

        Multiple Sclerosis and Related Disorders

        By J William Lindsey, MD
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        Multiple Sclerosis and Related Disorders

        • J WILLIAM LINDSEY, MDProfessor, Department of Neurology, University of Texas Health Science Center at Houston, Houston, TX

        Multiple sclerosis (MS) is a relatively common cause of neurologic symptoms and disability in young adults. The distinguishing pathologic features of MS are loss of myelin and inflammation in the central nervous system (CNS). The myelin sheath is essential for rapid conduction of nerve signals along large-diameter axons. Oligodendrocytes produce and maintain myelin in the CNS, and Schwann cells produce and maintain myelin in the peripheral nerves. In addition to MS, there are a number of related disorders causing demyelination, inflammation, or both in the CNS. This chapter discusses MS and related disorders, including neuromyelitis optica, optic neuritis, acute disseminated encephalomyelitis, transverse myelitis, Behçet syndrome, neurosarcoidosis, inherited demyelinating diseases (leukodystrophies, cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy [CADASIL]), and virus-induced demyelination (progressive multifocal leukoencephalopathy, subacute sclerosing panencephalitis). The section on MS covers epidemiology, etiology/genetics, pathogenesis, diagnosis, differential diagnosis, management, and prognosis. Figures include organization of the microenvironment of larger-diameter axons, typical magnetic resonance imaging findings in MS and neuromyelitis optica, postgadolinium images of the cervical spine in MS, and an approach to treatment of relapsing-remitting MS. Tables list MS and related disorders, distribution of neurologic deficits at the onset of MS, differential diagnosis of MS, disease-modifying therapies for relapsing-remitting MS, and selected leukodystrophies, as well as diagnostic criteria and selected symptomatic therapies for MS.

         

        This chapter contains 3 highly rendered figures, 7 tables, 82 references, 1 teaching slide set, and 5 MCQs.

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      • 6

        Traumatic Brain and Spinal Cord Injuries

        By Mohit Datta, MD; Geoffrey S.F. Ling, MD, PhD, FAAN
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        Traumatic Brain and Spinal Cord Injuries

        • MOHIT DATTA, MD
        • GEOFFREY S.F. LING, MD, PHD, FAANUniformed Services University of the Health Sciences, Bethesda, MD

        Traumatic brain injury (TBI), the most common cause of death and disability in young adults in the United States, is classified by severity (mild, moderate, or severe). This chapter reviews the pathogenesis of TBI, the types and severity of injuries, and management of TBI. Figures include computed tomography (CT) images of brains showing bi-frontal hemorrhagic contusions with associated peri-hematomal edema, a right-sided hyperdense lenticular-shaped space-occupying lesion with minimal midline shift, and evolution of the CT appearance of a subdural hematoma (from a hyperdense lesion, through to an isodense lesion, to a hypodense space-occupying lesion). Tables present the Glasgow Coma Scale, indications for CT scan in mild TBI (based on the Canadian head rule), guidelines for the management of moderate to severe TBI, and return to play or work criteria for patients suffering from concussion. This chapter contains 61 references.

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      • 7

        Clinical Aspects of Alzheimer Disease

        By David Knopman, MD
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        Clinical Aspects of Alzheimer Disease

        • DAVID KNOPMAN, MDProfessor of Neurology, Department of Neurology, Mayo Clinic, Rochester, MN

        The clinical diagnosis of Alzheimer disease (AD) has been well established, but there is a widespread misunderstanding about the relationship between dementia (a syndrome) and AD (a cause of dementia). AD is the most common etiology that causes dementia in mid- and late life. The prototypical clinical presentation is that of a gradually worsening problem with learning new information, that is, a short-term memory deficit, accompanied by cognitive impairment in other domains, including language, spatial cognition, and executive functioning, as well as changes in personality and behavior. A key element of the diagnosis of dementia is that daily functioning is impaired. The concept of mild cognitive impairment (MCI) as the earliest symptomatic presentation of a dementing illness is now widely accepted. MCI due to AD typically presents with isolated problems with learning and memory without substantial loss of ability to function in daily life.  Less common variants of AD are now recognized and include a disorder in which spatial and visual cognitive dysfunction occurs or in which word-finding problems predominate at the onset of symptoms. Although AD as a cause of dementia is the most common among etiologies, AD often co-occurs with other neurodegenerative diseases and with cerebrovascular disease. The presence of multietiology dementia in which AD is a contributor is particularly common in the eighth decade of life and beyond. 

        Key words: Alzheimer disease, cognitive impairment, dementia, mild cognitive impairment

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      • 8

        Pathophysiology of Alzheimer Disease

        By David Knopman, MD
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        Pathophysiology of Alzheimer Disease

        • DAVID KNOPMAN, MDProfessor of Neurology, Department of Neurology, Mayo Clinic, Rochester, MN

        Genetic discoveries coupled with neuropathologic investigations initially established the central role for β-amyloidosis in Alzheimer disease (AD). Three dominantly inherited genes (APP, PSEN1, and PSEN2) and one common allelic variant with lower penetrance (APOE) account for the majority of the genetic basis for AD. PET biomarkers for AD have been developed in the past decade and are fundamentally altering our view of the disease. The availability of PET tracers, first for amyloid and now for tau, has enabled researchers to develop a model of AD that begins long before people become symptomatic. In persons destined to develop dementia due to AD, brain β-amyloid levels begin to rise 10 to 20 years earlier. Other imaging changes that might precede symptomatic disease include (1) reductions in brain metabolic activity in a group of temporal and parietal cortical association areas that can be demonstrated by [18F]fluorodeoxyglucose-PET scanning; (2) losses of hippocampal volume as measured on structural magnetic resonance imaging; and (3) loss of cortical thickness or cortical volume in temporal and parietal cortical association areas. All of these changes are greatly accentuated once people become symptomatic. Although mild elevations in tau PET abnormalities can also be seen in presymptomatic individuals, it is only when persons become symptomatic that marked elevations in these abnormalities begin to occur in those same temporal and parietal cortical association areas. Cerebrospinal fluid (CSF) biomarkers provide a complementary view, with CSF β-amyloid levels falling (presumably due to aggregation within the cortex) even before amyloid PET abnormalities are visible. CSF total tau and phospho-tau levels begin to rise when persons are much closer to being symptomatic. The sum of these observations has allowed researchers to gain a far more insightful antemortem view of the pathophysiology of AD in humans than had previously been available from neuropathologic investigations. 

        Keywords: β-amyloid, cerebrospinal fluid β-amyloid, cerebrospinal fluid phospho-tau, cortical thickness, [18F]fluorodeoxyglucose–positron emission tomography, hippocampal atrophy, preclinical Alzheimer disease, tau protein 

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      • 9

        Clinical Aspects of Non-alzheimer Disease Dementias

        By David Knopman, MD
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        Clinical Aspects of Non-alzheimer Disease Dementias

        • DAVID KNOPMAN, MDProfessor of Neurology, Department of Neurology, Mayo Clinic, Rochester, MN

        There are a relatively small number of disorders that account for the majority of dementia in the elderly that is not Alzheimer disease (AD): cerebrovascular disease, Lewy body disease (α-synucleinopathies), and the frontotemporal lobar degenerations. Cerebrovascular disease and Lewy body disease account for most non-AD dementia among persons in the eighth decade of life and beyond. These two frequently co-occur with AD but can occur in their pure forms rarely (in the case of dementia associated with cerebrovascular disease) or more commonly (in the case of Lewy body disease). There is no one cognitive or behavioral syndrome associated with cerebrovascular disease; however, attempts to isolate a common theme suggest that cognitive slowing is typical of cerebrovascular contributions to cognitive impairment. Cerebrovascular pathology relevant to cognitive impairment accumulates subclinically more commonly than it causes acute, strokelike declines in cognition. Dementia with Lewy bodies is a multidimensional disorder that includes a nonamnestic dementia, Parkinson disease or at least some parkinsonian features, a disorder of sleep and wakefulness, autonomic disturbances, and depression. The disorders of sleep prominently include rapid eye movement sleep behavior disorder, excessive daytime sleepiness, visual hallucinations, and marked fluctuations in level of alertness. The frontotemporal lobar degenerations are nearly as common as causes of dementia in persons under age 65 as is AD. The group of disorders includes two cognitive syndromes (primary progressive aphasia and behavior variant frontotemporal dementia) and two neuropathologic subtypes (tauopathy and TDP43 proteinopathy) and is associated with three major autosomal dominant genetic mutations (in MAPT, GRN,and C9ORF72).

        Key words: dementia with Lewy bodies, frontotemporal lobar degenerations, vascular cognitive impairment

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      • 10

        Management and Therapeutic Issues in the Dementias

        By David Knopman, MD
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        Management and Therapeutic Issues in the Dementias

        • DAVID KNOPMAN, MDProfessor of Neurology, Department of Neurology, Mayo Clinic, Rochester, MN

        As of 2016, treatment of Alzheimer disease (AD) dementia and the principal non-AD dementias is entirely palliative. Although there are several drugs approved for the treatment of mild to moderate AD dementia, these drugs—the cholinesterase inhibitors and memantine—have rather modest benefits. In general, nonpharmacologic approaches to the management of patients with dementia emphasize support for the caregiver, attention to safety, and providing a supportive and socially enriched environment for the patient. Depression is common in dementias of diverse etiology; lower doses of later-generation antidepressants are effective in controlling depressive symptoms in patients with dementia. Agitation is not a ubiquitous occurrence in patients with dementia, but physically aggressive behavior, hallucinations, and delusions affect a sizable fraction of patients with dementia. There is much controversy regarding the appropriate medication classes to use in cases of agitation, but the antipsychotic agent quetiapine is often effective and unique among its class in not causing parkinsonism or tardive dyskinesia.

        Key words: antidepressants, antipsychotic agents, caregiver support, cholinesterase inhibitors

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    • Neuromuscular Diseases
      • 1

        Motor Neuron Diseases

        By Elena Ratti, MD; Merit E. Cudkowicz, MD, MSc; James D Berry, MD, MPH
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        Motor Neuron Diseases

        • ELENA RATTI, MDClinical Research Fellow, Neurological Clinical Research Institute, Massachusetts General Hospital, Boston, MA
        • MERIT E. CUDKOWICZ, MD, MSCChief of Neurology, Massachusetts General Hospital, Julieanne Dorn Professor of Neurology Harvard Medical School, Neurological Clinical Research Institute, Massachusetts General Hospital, Boston, MA
        • JAMES D BERRY, MD, MPHAssistant Professor of Neurology, Neurological Clinical Research Institute, Massachusetts General Hospital, Boston, MA

        The motor neuron diseases (MNDs) are a family of diseases commonly categorized by their propensity to affect upper or lower motor neurons and by their mode of inheritance. The chapter provides some content on infectious MNDs caused by viral infections affecting the motor neurons in the anterior horn of the spinal cord. However, the chapter devotes most of its attention to the inherited and sporadically occurring MNDs. The majority of research into adult MND focuses on amyotrophic lateral sclerosis (ALS) due to its high prevalence, rapid progression, and phenotypical similarities between its inherited form and its sporadic form. As our knowledge of genetic mechanisms underlying ALS pathology has grown, common themes have emerged. These include abnormalities in RNA biology, axonal transport, protein folding, and inflammatory responses. These themes currently drive much of the direction in ALS experimental therapy development. It is clear that MND is complex and involves several different molecular pathways. Given this complexity, ALS might not be a single disease entity, and if this is the case, treatment approaches may need to be targeted to specific pathologies rather than all ALS patients on a broad scale. Chapter content is enhanced by tables outlining the types of MNDs, criteria for supporting a diagnosis, first-line workup, the genes associated with ALS, ALS efficacy outcome measures, symptom management of ALS, and spinal muscular atrophy classification. Mechanisms of ALS are illustrated, and clinical photographs demonstrate symptoms. This chapter contains 252 references. 

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      • 2

        Radiculopathies and Neuropathies

        By Kathy Chuang, MD; William S David, MD, PhD
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        Radiculopathies and Neuropathies

        • KATHY CHUANG, MDClinical Fellow in Neuromuscular Medicine, Massachusetts General Hospital and Brigham and Women’s Hospital, 165 Cambridge Street, Suite 820, Boston, MA 02114
        • WILLIAM S DAVID, MD, PHDAssociate Professor of Neurology, Harvard Medical School, Director of the EMG Laboratory and Neuromuscular Diagnostic Center, Massachusetts General Hospital, 165 Cambridge Street, Suite 820, Boston, MA 02114

        “Radiculopathies” are disorders of nerve roots, whereas “neuropathies” are disorders of the peripheral nerve. These disorders may involve single roots or nerves, multiple roots or nerves, and even other aspects of the nervous system. This chapter reviews the anatomy and pathophysiology of the peripheral nervous system; the general approach to radiculopathies and neuropathies, including clinical manifestations and localization, diagnostic studies, and treatment; radiculopathies, including anatomy, cervical radiculopathy, lumbosacral radiculopathy, thoracic radiculopathy, and cauda equina syndrome; and neuropathies, including  mononeuropathies and polyneuropathies. Tables describe the innervation of select nerve roots and peripheral nerves, differences between root and nerve lesions, commonly used neuropathic pain medications, distinctive patterns of neuropathy with limited differential diagnoses, differential diagnosis of demyelinating polyneuropathy, drugs that may cause polyneuropathy, and neuropathies associated with diabetes mellitus. Figures show the anatomy of a spinal segment, nerve fascicles, ultrasound images of the median nerve, magnetic resonance imaging of the lumbosacral spine, the Spurling maneuver, and physical examination maneuvers for lumbosacral radiculopathies.

        This chapter contains 6 highly rendered figures, 8 tables, 77 references, and 5 MCQs.

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      • 3

        Neuromuscular Junction Disorders

        By Anthony A Amato, MD; Mohammad Kian Salajegheh, MD
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        Neuromuscular Junction Disorders

        • ANTHONY A AMATO, MDChief, Division of Neuromuscular Disease, Department of Neurology, Brigham and Women’s Hospital, Professor of Neurology, Harvard Medical School, Boston, MA
        • MOHAMMAD KIAN SALAJEGHEH, MDDirector, Peripheral Nerve Clinic, Associate Neurologist, Department of Neurology, Brigham and Women’s Hospital, Assistant Professor of Neurology, Harvard Medical School, Boston, MA

        The three main components of the neuromuscular junction (NMJ) include the presynaptic region, the synaptic cleft, and the postsynaptic region. The NMJ acts as an interface between the motor nerve and muscle by converting the motor nerve electric currents into chemical signals and then back into electric currents in the muscle. This chapter reviews electrodiagnostic testing in NMJ disorders, including repetitive nerve stimulation and single-fiber electromyography. Myasthenia gravis, congenital myasthenic syndromes, Lambert-Eaton myasthenic syndrome, botulism, and organophosphate poisoning and other toxins are discussed, including epidemiology, etiology/genetics, pathogenesis, diagnosis, differential diagnosis, management, complications, and prognosis. Tables include an overview of neuromuscular disorders, drugs with adverse effects on the NMJ, common immunomodulatory agents used for treatment of myasthenia gravis, congenital myasthenic syndromes, and toxins and venoms. Figures illustrate the NMJ structure and function, structure of the presynaptic and postsynaptic regions, electrodiagnostic studies in NMJ disorders, and dysfunction of the NMJ in acetylcholine receptor myasthenia gravis.

        This chapter contains 5 highly rendered figures, 5 tables, 65 references, and 5 MCQs.

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      • 4

        Myopathies

        By Anthony A Amato, MD; Thomas I. Cochrane, MD, MBA
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        Myopathies

        • ANTHONY A AMATO, MDChief, Division of Neuromuscular Disease, Department of Neurology, Brigham and Women’s Hospital, Professor of Neurology, Harvard Medical School, Boston, MA
        • THOMAS I. COCHRANE, MD, MBAAssociate Neurologist, Division of Neuromuscular Disease, Department of Neurology, Brigham and Women’s Hospital, Assistant Professor of Neurology, Harvard Medical School, Boston, MA

        Muscle disease (myopathy) can be acquired or hereditary. Symptoms include skeletal muscle weakness, atrophy, muscle cramps or myalgias, and impaired function of respiratory, pharyngeal, facial, or ocular muscles. Clinicians must identify treatable myopathies and initiate therapy before permanent weakness occurs. For patients with untreatable disorders, proper supportive care, rehabilitation, genetic counseling, and psychological support are critical. This chapter covers common myopathies, including muscular dystrophies; malignant hyperthermia; metabolic myopathies; mitochondrial myopathies and encephalopathies; ion channelopathies, periodic paralyses, and nondystrophic myotonias; and drug-induced myopathies. Clinical presentation, diagnosis, pathogenesis, and therapy are emphasized. Tables describe genetic classification of the limb-girdle and distal muscular dystrophies; proteins involved in myofibrillar myopathy; other distal myopathies; and antirheumatic, antiinflammatory, and immunosuppressive drug-induced myopathy. Figures show the sarcolemmal membrane and enzymatic proteins associated with muscular dystrophies, sarcomeric and nuclear proteins associated with muscular dystrophies, and major metabolic pathways used by muscle.

        This chapter contains 3 highly rendered figures, 4 tables, 107 references, and 5 MCQs.

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    • Movement Disorders
      • 1

        Hyperkinetic Movement Disorders

        By Devin Mackay, MD; Edison Miyawaki, MD, PhD
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        Hyperkinetic Movement Disorders

        • DEVIN MACKAY, MDClinical Fellow in Neurology, Massachusetts General Hospital and Brigham and Women’s Hospital, Harvard Medical School, Boston, MA
        • EDISON MIYAWAKI, MD, PHDAssociate Neurologist, Brigham and Women’s Hospital and Assistant Professor of Neurology, Harvard Medical School, Brigham and Women’s Hospital, Boston, MA

        The hyperkinetic movement disorders include heterogeneous diseases and syndromes, all characterized by one or a variety of excessive, involuntary movements. The hyperkinetic movement disorders are heterogeneous in clinical presentation, but a rational and practical approach to diagnosis exists based on new genetic correlations and targeted laboratory investigations. Treatments informed by a still-developing picture of motor pathophysiology offer significant benefit for these disorders. This chapter discusses choreiform disorders, including patterns in choreiform diagnosis; tremor disorders; paroxysmal disorders, including tics and myoclonus; dystonias, including monogenic primary dystonias; and pathophysiology and treatment in the hyperkinetic movement disorders. Figures include clinical photos, computed tomography scans, and an algorithm representing cortical-subcortical circuitry. Tables delineate definitions, distinguishing clinical features, medications, genetics, protein products, and treatments associated with various disorders.

        This chapter contains 6 figures, 12 tables, 145 references, and 5 Board-styled MCQs.

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      • 2

        Movement Disorders

        By Bradley J. Robottom, MD; Jason S. Hawley, MD; William J. Weiner, MD
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        Movement Disorders

        • BRADLEY J. ROBOTTOM, MDAssistant Professor, Department of Neurology, University of Maryland School of Medicine
        • JASON S. HAWLEY, MDUS Army, Movement Disorders Service, Department of Neurology, Walter Reed Army Medical Center
        • WILLIAM J. WEINER, MDProfessor and Chairman, Department of Neurology, University of Maryland School of Medicine

        The term movement disorders encompasses a wide range of conditions that cause abnormal movements. This review discusses hypokinetic movement disorders, including bradykinesia, akinesia, akinetic rigid syndrome, and Parkinsonism, an akinetic rigid syndrome that is one of the most common of all the movement disorders which is most often a manifestation of Parkinson disease (PD). The review looks at the epidemiology, etiology, pathology, diagnosis, treatment, and management of PD. Other parkinsonian syndromes include progressive supranuclear palsy, corticobasal syndrome, multiple system atrophy, vascular parkinsonism, normal pressure hydrocephalus, drug-induced parkinsonism, and dementia associated with Parkinson disease. Hyperkinetic movement disorders include tremor, dystonia, tics, myoclonus, and chorea. They include Huntington disease, Wilson disease, tardive dyskinesia, Tourette syndrome, and essential tremor. Figures in this review include examples of generalized dystonia, moderate parkinson disease, affected handwriting, Kayser-Fleischer ring, cervical dystonia, head deviation, and writer's cramp. Tables provide clinical definitions, clues to drug-induced parkinsonism, and a list of drugs that can cause parkinsonism. This review contains 129 references.

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      • 3

        Inherited Ataxias

        By Susan Perlman, MD
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        Inherited Ataxias

        • SUSAN PERLMAN, MDClinical Professor of Neurology, David Geffen School of Medicine at UCLA, 300 UCLA Medical Plaza, Suite B200, Los Angeles, CA 90095

        The inherited ataxias are disorders that cause progressive imbalance as a result of pathology in the cerebellum and its various connecting pathways. Autosomal recessive ataxias include Friedreich ataxia, ataxia with isolated vitamin E deficiency, ataxia-telangiectasia, and autosomal recessive ataxia of Charlevoix-Saguenay, among others. A discussion of autosomal dominant ataxias covers spinocerebellar ataxias (SCA) types 1 through 14, dentatorubral pallidoluysian atrophy (DRPLA), and episodic ataxia (EA) syndromes. Clinical features, laboratory studies, differential diagnosis, and management of inherited ataxias are discussed. Tables describe both autosomal recessive ataxias and autosomal dominant ataxias (with known gene loci), childhood– or young adult–onset ataxias with ill-defined genetic abnormalities, phenotypic features that may indicate a specific genotype in the common autosomal dominant ataxias, and normal and expanded ranges of various repetitive nucleotide sequences in inherited ataxias. Figures include a diagrammatic representation of the type of repeat expansions associated with ataxias, aggregates of ataxin 3, a schematic of some of the proposed pathogenic mechanisms in the polyglutamine ataxias, and dystonia in a patient with SCA3. A sidebar offers selected Internet resources for information on ataxias. This chapter contains 64 references.

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      • 4

        Parkinson Disease: Epidemiology, Pathology, and Clinical Diagnosis

        By Maurizio Zibetti, MD, PhD; Aristide Merola, MD, PhD
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        Parkinson Disease: Epidemiology, Pathology, and Clinical Diagnosis

        • MAURIZIO ZIBETTI, MD, PHD
        • ARISTIDE MEROLA, MD, PHD

        Parkinson disease (PD) is the second most common neurodegenerative disorder after Alzheimer disease. PD predominantly affects elderly people, and most countries are now facing a marked demographic change, with a progressively larger proportion of the population reaching old age. Thus, PD is expected to cause an increasing social and economic burden on society in the near future. The causes of PD are still mostly unknown. There has been a substantial progress in our understanding of the genetic basis of PD in the past 15 years; however, these mutations explain only a small fraction of all PD cases and other non-genetic factors may play a role. This review covers the epidemiology, pathology, and clinical diagnosis of PD. Figures show age-specific incidence of PD, age-specific prevalence of PD, PD risks for exposures reported in high-quality studies, PD and atypical parkinsonism clinical features, and neuroimaging alterations in PD and atypical parkinsonism. Tables list factors associated with increased or decreased risk of PD, Braak staging scheme of α-synuclein pathology in PD, United Kingdom Parkinson’s Disease Society Brain Bank clinical diagnostic for PD, National Institute of Neurological Disorders and Stroke diagnostic criteria for PD, differential diagnosis of parkinsonian syndromes, and “atypical” atypical parkinsonism.

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      • 5

        Parkinson Disease: Treatment

        By Daniel Martinez-Ramirez, MD; Michael S. Okun, MD
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        Parkinson Disease: Treatment

        • DANIEL MARTINEZ-RAMIREZ, MDAssistant Professor, Neurology Clerkship Director, Department of Neurology, University of Florida College of Medicine, Center for Movement Disorders and Neurorestoration, Gainesville, FL, USA
        • MICHAEL S. OKUN, MDProfessor, Department of Neurology, University of Florida College of Medicine, Center for Movement Disorders and Neurorestoration, Gainesville, FL, USA

        Parkinson disease (PD) is a chronic neurodegenerative disorder affecting more than 4 million people worldwide. It is expected that this number will potentially double by 2030 due to the aging population, thus transforming PD into an even bigger worldwide health concern. As PD knowledge has expanded over the past three decades, it is now recognized that multiple brain neurotransmitters and circuits are altered and collectively contribute to a constellation of motor and nonmotor symptoms. The classic symptoms required for the clinical diagnosis of PD are bradykinesia, resting tremor, rigidity, and/or loss of postural reflexes. Due to the multiple brain circuits affected as the disease advances, the nonmotor manifestations become equally, or even potentially, more troublesome for patients and caregivers. This review covers general considerations and aspects of treatment of PD. Figures show expected phases of PD, how to start therapy, and the approach to levodopa-induced complications. Tables list suggested dosages of available medications for the treatment of motor symptoms in PD, classification of levodopa-induced complications, suggested dosages of available medications for the treatment of neuropsychiatric symptoms, and suggested dosages of available medications for the treatment of autonomic symptoms and sleep disorders.

        Key words: levodopa, levodopa-induced complications, movement disorders, neurodegenerative disorders, Parkinson disease

         

        This review contains 3 highly rendered figures, 4 tables, and 226 references.

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      • 6

        Atypical Parkinsonian Syndromes: Tauopathies

        By Hee Kyung Park, MD, PhD; Irene Litvan, MD
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        Atypical Parkinsonian Syndromes: Tauopathies

        • HEE KYUNG PARK, MD, PHDAssistant professor in the Department of Neurology, Inje University Ilsan-Paik Hospital, Goyang, Republic of Korea.
        • IRENE LITVAN, MDTasch Endowed Professor in Parkinson Disease Research in the Department of Neurosciences, University of California San Diego, San Diego, CA

        Atypical parkinsonian disorders, which include two common proteinopathies, tauopathies and α-synucleinopathies, are clinically characterized by a progressive parkinsonism that typically does not respond to levodopa therapy and usually associates with early postural instability, falls, and other atypical features not observed in Parkinson disease. Tauopathies refer to neurodegenerative diseases in which there is an abnormal accumulation of hyperphosphorylated tau. The most frequent tauopathies are progressive supranuclear palsy and corticobasal degeneration. Better recognition of the expanding phenotypes of these disorders has led to the development of new diagnostic criteria. Furthermore, better knowledge about the pathogenesis (cell-to-cell transmission of pathologic tau) has resulted in advances in novel disease-modifying therapies that target tau. This review addresses the basic concepts of and recent issues in tauopathies, including their clinical phenotypes, genetic features, biomarkers, and novel experimental therapies. 

        Key words: atypical parkinsonian disorders, corticobasal degeneration, progressive supranuclear palsy, proteinopathies, tauopathies 

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      • 7

        Tics

        By Justyna R Sarna, MD, PhD ; Tamara Pringsheim, MD, MSc
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        Tics

        • JUSTYNA R SARNA, MD, PHD Clinical Assistant Professor, Division of Neurology, Department of Clinical Neurosciences, Hotchkiss Brain Institute, University of Calgary, Calgary, AB
        • TAMARA PRINGSHEIM, MD, MSC Assistant Professor, Division of Neurology, Department of Clinical Neurosciences, Hotchkiss Brain Institute, University of Calgary, Calgary, AB

        Tourette syndrome (TS) was originally described and conceptualized by Gilles de la Tourette in 1885. Since then, our understanding of tic disorders has grown immensely and continues to evolve at a rapid pace. Tics are abrupt, usually brief and repetitive, nonrhythmic movements (motor tics) and sounds (vocal tics). Motor tics can be further subdivided into simple and complex motor tics. Simple tics are sudden, meaningless movements most commonly involving eye blinking, facial grimacing, mouth gestures, and shoulder shrugs. Complex motor tics typically involve a series of stereotyped movements that may appear to be purposeful. Vocal (also called phonic) tics are similarly subdivided into simple and complex types. This review covers disease definition/subclassification, epidemiology, etiology/genetics, pathophysiology and pathogenesis, diagnosis, differential diagnosis, and treatment of tic disorders. Figures show a risperidone safety monitoring template for children, an aripiprazole safety monitoring template for children, and adult antipsychotic safety monitoring recommendations. The video shows how to perform the extrapyramidal symptom rating scale. Tables list classification of tic disorders, medication dosing suggestions, and TS deep brain stimulation guidelines.

        This review contains 3 highly rendered figures, 1 video, 3 tables, and 115 references.

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      • 8

        Myoclonus

        By Hiroshi Shibasaki, MD, PhD; Marina A J Koning-Tijssen, MD, PhD
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        Myoclonus

        • HIROSHI SHIBASAKI, MD, PHDEmeritus Professor, Department of Neurology and Human Brain Research Center, Kyoto University School of Medicine, Kyoto, Japan
        • MARINA A J KONING-TIJSSEN, MD, PHDProfessor, Department of Neurology, University Medical Center Groningen, Groningen, The Netherlands

        Myoclonus is defined as sudden, brief, jerky, shocklike, involuntary movements involving the extremities, face, and trunk, without loss of consciousness. Myoclonus is one of the most commonly encountered involuntary movements, and it can be seen in association with a variety of conditions. This review covers  the epidemiology, classification, and diagnosis of myoclonus, as well as a thorough discussion of cortical myoclonus, myoclonus of brainstem origin, myoclonus of spinal cord origin, and myoclonus of undetermined origin. Figures show the principle of jerk-locked back-averaging, example data of jerk-lock averaging, schematic diagrams of cortical reflex myoclonus and spontaneous cortical myoclonus, a polygraphic electromyogram (EMG) recorded from a patient with postanoxic, reticular reflex myoclonus, surface negative slow electroencephalographic (EEG) potentials recorded before psychogenic jerks and voluntary movements mimicking the jerks in a patient with a diagnosis of psychogenic truncal movements, and an EEG-EMG polygraph in a patient with Creutzfeldt-Jakob disease. Tables list the classification of myoclonus based on the estimated site of origin, causes of cortical myoclonus, and causes of progressive myoclonus epilepsy and gene abnormalities.

         

        Key words: cortical myoclonus, EEG-EMG polygraph, epilepsy syndromes, involuntary movements, myoclonus

         

        This review contains 6 highly rendered figures, 3 tables, and 63 references.

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      • 9

        Tremors

        By Alfonso Fasano, MD, PhD; Günther Deuschl, MD, PhD
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        Tremors

        • ALFONSO FASANO, MD, PHDAssociate Professor of Medicine, Division of Neurology, University of Toronto, Morton and Gloria Shulman Movement Disorders Clinic and the Edmond J. Safra Program in Parkinson’s Disease, Toronto Western Hospital, Toronto, ON, Canada
        • GÜNTHER DEUSCHL, MD, PHDProfessor of Neurology, Department of Neurology, Christian-Albrechts-University, University-Hospital Schleswig-Holstein, Kiel, Germany

        Tremor is the most common movement disorder and denotes a rhythmic and involuntary movement of one or several regions of the body. This review covers disease definition, essential tremor, enhanced physiologic tremor, parkinsonian tremor, dystonic tremor, orthostatic tremor, cerebellar tremor, Holmes tremor, neuropathic tremor, palatal tremor, drug-induced and toxic tremors, functional tremor, rare tremor syndromes, tremorlike conditions, and treatment of tremor. Figures show action tremor assessment, the central nervous system circuits of tremor, magnetic resonance imaging findings in specific tremor conditions, general management of tremor patients, an algorithm for the treatment of parkinsonian tremor, and an algorithm for the treatment of dystonic tremor and primary writing tremor. Tables list types of tremor according to the condition of activation, tremor conditions in newborns and during childhood, clinical features of the most common tremor syndromes, motor signs other than tremor and nonmotor features of essential tremor patients, Movement Disorder Society consensus criteria for the diagnosis of essential tremor, genetic and environmental causes of essential tremor, causes of enhanced physiologic tremor, drugs and toxins known to cause tremor, paroxysmal tremors, pseudorhythmic myoclonus in the differential diagnosis of tremor, and pharmacologic management of essential tremor.

         

        Key words: essential tremor, movement disorder, pathologic tremor, physiologic tremor, tremor

         

        This review contains 6 highly rendered figures, 7 videos, 11 tables, and 163 references.

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      • 10

        Ataxias

        By Hélio A G Teive, MD, PhD; Orlando G P Barsottini, MD, PhD
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        Ataxias

        • HÉLIO A G TEIVE, MD, PHDAssociate Professor of Neurology, Internal Medicine Department, Neurology Service, Ataxia Unit, Universidade Federal do Paraná
        • ORLANDO G P BARSOTTINI, MD, PHDAdjunct Professor of Neurology, Department of Neurology and Neurosurgery, Division of General Neurology and Ataxia Unit, Universidade Federal de São Paulo

        Ataxia is a disorder of balance and coordination. The most common forms are cerebellar ataxia and afferent/sensory ataxia. Ataxias can be classified as primary or secondary, as well as hereditary, nonhereditary degenerative, and sporadic. This review covers the primary (congenital, hereditary, and nonhereditary degenerative ataxias) and secondary cerebellar ataxias and afferent/sensory ataxias. Hereditary ataxias are classified as autosomal recessive cerebellar ataxias (such as Friedreich ataxia and ataxia-telangiectasia), autosomal dominant cerebellar ataxias (currently known as spinocerebellar ataxias, such as spinocerebellar ataxia type 3 or Machado-Joseph disease), episodic ataxias, X-linked cerebellar ataxias, and mitochondrial ataxias. Idiopathic degenerative cerebellar ataxias include the cerebellar form of multiple system atrophy (MSA-C) and idiopathic late-onset cerebellar ataxias. Secondary cerebellar ataxias or acquired ataxias include ataxias due to exogenous or endogenous nongenetic causes, such as toxic, paraneoplastic, immune mediated, nutritional, infectious, and secondary to focal injury to the cerebellum. Afferent/sensory ataxia represents a special group of ataxias with many possible causes and is associated with peripheral neuropathies, dorsal root ganglionopathies, sensory nerve root involvement, and posterior spinal column involvement. Figures show several clinical and radiologic (magnetic resonance imaging) signs of autosomal recessive, autosomal dominant ataxias, and multiple system atrophy (type C). Tables list the most common neurologic signs of cerebellar and afferent/sensory ataxias, the main forms of autosomal recessive and autosomal dominant cerebellar ataxias, and the most common causes of secondary cerebellar and afferent/sensory ataxias.

        Key words: afferent/sensory ataxias, ataxias, autosomal recessive cerebellar ataxias, congenital ataxias, nonhereditary degenerative ataxias, secondary cerebellar ataxias, spinocerebellar ataxias

        This review contains 5 highly rendered figures, 4 tables, and 99 references.

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      • 11

        Huntington Disease and Other Genetic Causes of Choreas

        By Francisco Cardoso , MD, PhD, FAAN; Ainhi Ha, MBBS, PhD, FRACP; Débora Maia, MD; Victor S C Fung, PhD, FRACP
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        Huntington Disease and Other Genetic Causes of Choreas

        • FRANCISCO CARDOSO , MD, PHD, FAANProfessor of Neurology, Movement Disorders Unit, Neurology Service, The Federal University of Minas Gerais, Belo Horizonte, MG, Brazil
        • AINHI HA, MBBS, PHD, FRACPStaff Specialist Neurologist, Department of Neurology, Westmead Hospital, Westmead, Australia
        • DÉBORA MAIA, MDConsultant, Movement Disorders Unit, Neurology Service, The Federal University of Minas Gerais, Belo Horizonte, MG, Brazil
        • VICTOR S C FUNG, PHD, FRACPClinical Associate Professor, Sydney Medical School, University of Sydney, & Director, Movement Disorders Unit, Department of Neurology, Westmead Hospital, Sydney, Australia

        The aim of this review is to provide an overview of Huntington disease (HD) and other genetic choreas with an emphasis on clinical presentation, diagnosis, treatment, and expected outcome. Chorea is a syndrome characterized by brief, abrupt, involuntary movements resulting from a continuous flow of random muscle contractions. The first step in approaching a subject with chorea is to define the underlying etiology because the natural history and management vary accordingly. Age at onset, body distribution, other neurologic features, and family history are important in establishing the cause of chorea. HD is the most common etiology of genetic choreas worldwide. It is a progressive neurodegenerative disorder transmitted as an autosomal dominant trait characterized by a combination of movement disorders, cognitive decline, and behavioral abnormalities that causes progressive disability and death. When an HD phenotype test is negative for this condition, other causes, such as neuroacanthocytosis; spinocerebellar ataxia 17; Huntington disease–like syndrome 2, 3, or 4; benign hereditary chorea; and dentatorubral-pallidoluysian atrophy, as well as others, should be investigated. 

        Key words: Huntington, movement disorders, genetic choreas, neurodegenerative disorder

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      • 12

        Dystonia

        By Federico Micheli, MD, PhD ; Carolina Candelaria Ramírez Gómez, MD; Cristian Calandra, MD
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        Dystonia

        • FEDERICO MICHELI, MD, PHD Parkinson Disease and Movement Disorders Unit Hospital de Clínicas “José de San Martín” University of Buenos Aires Buenos Aires, Argentina
        • CAROLINA CANDELARIA RAMÍREZ GÓMEZ, MDParkinson Disease and Movement Disorders Unit Hospital de Clínicas “José de San Martín” University of Buenos Aires Buenos Aires, Argentina
        • CRISTIAN CALANDRA, MDParkinson Disease and Movement Disorders Unit Hospital de Clínicas “José de San Martín” University of Buenos Aires Buenos Aires, Argentina

        Diagnosing dystonia requires a solid understanding of the phenomenology of this movement disorder as well as successful classification and a systematic search for the etiology. We describe the current definition, classification, and methodology for reaching an accurate diagnosis. This review covers the most relevant clinical features of disorders where dystonia is present, emphasizing the affected genes most recently identified, as well as other pathophysiologic factors involved in the generation of this condition. Finally, advances in medical treatment and surgery for dystonia are discussed.

        Key words: antipsychotics, botulinum toxin, combined, deep brain stimulation, dopa-responsive dystonia, dystonia, focal, isolated, neurodegenerative, phenomenology, specific task, triggers

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    • Epilepsy
      • 1

        Epilepsy and Related Disorders

        By Barbara Dworetzky, MD; Jong Woo Lee, MD, PhD
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        Epilepsy and Related Disorders

        • BARBARA DWORETZKY, MDAssociate Professor of Neurology, Harvard Medical School, Chief, Division of Epilepsy, EEG, and Sleep Neurology, Director, The Edward B. Bromfield Epilepsy Program, Brigham and Women’s Hospital, Boston, MA
        • JONG WOO LEE, MD, PHDAssistant Professor of Neurology, Harvard Medical School, Director, ICU EEG Monitoring, The Edward B. Bromfield Epilepsy Program, Brigham and Women’s Hospital, Boston, MA

        Epilepsy is a chronic disorder of the brain characterized by recurrent unprovoked seizures. A seizure is a sudden change in behavior that is accompanied by electrical discharges in the brain. Many patients presenting with a first-ever seizure are surprised to find that it is a very common event. A reversible or avoidable seizure precipitant, such as alcohol, argues against underlying epilepsy and therefore against treatment with medication. This chapter discusses the epidemiology, etiology, and classification of epilepsy and provides detailed descriptions of neonatal syndromes, syndromes of infancy and early childhood, and syndromes of late childhood and adolescence. The pathophysiology, diagnosis, and differential diagnosis are described, as are syncope, migraine, and psychogenic nonepileptic seizures. Two case histories are provided, as are sections on treatment (polytherapy, brand-name versus generic drugs, surgery, stimulation therapy, dietary treatments), complications of epilepsy and related disorders, prognosis, and quality measures. Special topics discussed are women?s issues and the elderly. Figures illustrate a left midtemporal epileptic discharge, wave activity during drowsiness, cortical dysplasias, convulsive syncope, rhythmic theta activity, right hippocamal sclerosis, and right temporal hypometabolism. Tables describe international classifications of epileptic seizures and of epilepsies, epilepsy syndromes and related seizure disorders, differential diagnosis of seizure, differentiating epileptic versus nonepileptic seizures, antiepileptic drugs, status epilepticus protocol for treatment, when to consider referral to a specialist, and quality measures in epilepsy. This chapter contains 111 references.

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  • Neurology in General Medicine and Surgery
    • 1

      Neurologic Complications of Cancer

      By Eudocia Q Lee, MD, MPH; Soma Sengupta, MD, PhD; Patrick Y Wen, MD, PhD
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      Neurologic Complications of Cancer

      • EUDOCIA Q LEE, MD, MPHInstructor in Neurology, Harvard Medical School, Center for Neuro-Oncology, Dana-Farber Cancer Institute, and Division of Neurology, Department of Neurology, Brigham and Women’s Hospital, Boston, MA
      • SOMA SENGUPTA, MD, PHDNeuro-Oncology Fellow, Dana-Farber Cancer Institute, Boston, MA
      • PATRICK Y WEN, MD, PHDProfessor of Neurology, Harvard Medical School, Director, Center for Neuro-Oncology, Dana-Farber Cancer Institute, and Division of Neuro-Oncology, Department of Neurology, Brigham and Women’s Hospital, Boston, MA

      Neurologic complications from cancer and its therapies can significantly impact mortality and morbidity. Early recognition and intervention are key to preventing permanent neurologic injury. This review discusses common and uncommon neurologic complications of cancer, including central nervous system metastases, treatment-related neurotoxicities (including immunotherapies), and paraneoplastic syndromes. Workup and management of these neurologic complications are also addressed.

      Key words: cancer, chemotherapy, metastases, nervous system complications, paraneoplastic syndromes

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    • 2

      Metabolic Encephalopathy

      By Matthew McCoyd, MD; Sean Ruland, DO; José Biller, MD, FACP, FAAN, FANA, FAHA
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      Metabolic Encephalopathy

      • MATTHEW MCCOYD, MDAssistant Professor, Department of Neurology, Loyola University Chicago, Stritch School of Medicine, Maywood, IL 60153
      • SEAN RULAND, DOAssociate Professor, Department of Neurology, Loyola University Chicago, Stritch School of Medicine, Maywood, IL 61053
      • JOSÉ BILLER, MD, FACP, FAAN, FANA, FAHAProfessor and Chair, Department of Neurology, Loyola University Chicago, Stritch School of Medicine, Maywood, IL 60153

      Encephalopathy is a general term used to describe diffuse cerebral dysfunction and may be due to myriad primary central nervous system and systemic etiologies. Acute encephalopathy has a rapid onset between hours and days and is commonly due to toxic and metabolic factors that may lead to reversible or permanent cerebral injury. The hallmark of toxic and metabolic encephalopathies is impaired arousal (i.e., hypokinetic delirium), although occasionally an agitated delirium may manifest. Toxic and metabolic encephalopathies may range in severity from the acute confusional state to frank coma. As permanent injury may occur, an organized approach is needed to make an accurate and rapid diagnosis of potentially treatable etiologies. This chapter covers the diagnostic approach to metabolic encephalopathies, specific etiologies (including septic, toxic, and hypoxic-ischemic encephalopathy; electrolyte and acid-base disturbances; and end-organ failure), nutritional deficiencies, and encephalopathy related to endocrine disorders. Figures depict characteristic changes in the posterior brain white matter on imaging, cortical laminar necrosis after severe hypoxic-ischemic insult, osmotic demyelination syndrome of the basis pontis, triphasic frontal slowing, and necrosis of mammillary bodies in a patient with Wernicke encephalopathy. Tables outline the initial management and assessment of patients with acute encephalopathy of uncertain cause, Glasgow Coma Scale, Full Outline of Unresponsiveness (FOUR) score, and West Haven classification for severity of hepatic encephalopathy.

       

      This chapter contains 5 highly rendered figures, 4 tables, 50 references, 1 teaching slide set, and 5 MCQs.

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    • 3

      Neuro-ophthalmology

      By Sashank Prasad, MD
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      Neuro-ophthalmology

      • SASHANK PRASAD, MDAssistant Professor of Neurology, Harvard Medical School, Brigham and Women’s Hospital, Boston, MA

      Neuro-ophthalmic disorders are capable of causing significant morbidity, requiring the clinician to develop and maintain nuanced skills that facilitate timely diagnosis and effective management. Virtually any neurologic disease can present with symptoms of either afferent or efferent visualpathway dysfunction. This chapter emphasizes principles of accurate localization and discusses key clinical clues that serve to generate an appropriate differential diagnosis. It specifically addresses acute monocular visual loss from retinal and optic nerve diseases, papilledema, chronicoptic neuropathies, visual field deficits, supranuclear and infranuclear eye movement disturbances, and nystagmus. Figures include an algorithm for the evaluation of acute monocular visual loss, computed tomography scans, and a large gallery of diagnostic photos.

      This chapter contains 48 figures, 2 tables, 168 references, 5 Board-styled MCQs, and 1 teaching slide set.

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    • 4

      Uroneurology

      By Jai H Seth, MBBS, MRCS, BSc, MSc; Jalesh N. Panicker, MBBS, MD, DM, MRCP
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      Uroneurology

      • JAI H SETH, MBBS, MRCS, BSC, MSCSpecialist Registrar and Research Fellow in Urology, Department of Uro-Neurology, National Hospital for Neurology and Neurosurgery, Institute of Neurology, London, United Kingdom
      • JALESH N. PANICKER, MBBS, MD, DM, MRCPConsultant in Uro-Neurology, Department of Uro-Neurology, National Hospital for Neurology and Neurosurgery, Institute of Neurology, London, United Kingdom

      The function of the pelvic organs, including the lower urinary tract (LUT), is controlled by a complex network of nerves. This leaves patients with neurologic disease vulnerable to LUT and pelvic organ dysfunction. Physicians often come across urogenital complaints in their patients with neurologic disease, the symptoms of which can result in significant distress and loss of dignity and quality of life. Due to the health and economic burden that accompanies neurogenic pelvic organ dysfunction, it is important for clinicians to understand the common forms of dysfunction, essential investigations, and modes of management. This chapter covers bladder dysfunction from a physician’s perspective. Topics include neurologic control of the LUT, large bowel, and sexual functions; male and female sexual response; neurogenic bladder dysfunction and its management; diagnostic evaluation; management of neurogenic sexual dysfunction; management of erectile dysfunction; ejaculatory dysfunction; sexual dysfunction in women; and fecal incontinence. Figures illustrate efferent innervation of the LUT, neurologic detrusor overactivity, a urethral pressure profile in a patient with Fowler syndrome, an example bladder diary, an example bladder scan, and normal and obstructed flow patterns. Tables list common causes of injury at the suprapontine, suprasacral, and infrasacral levels and storage and voiding systems.

       

      This chapter contains 6 highly rendered figures, 2 tables, 53 references, 1 teaching slide set, and 5 MCQs.

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    • 5

      Hepatic Encephalopathy, Chronic Hepatic Encephalopathy (portosystemic Encephalopathy), and Acute Liver Failure

      By Norton Greenberger, MD; Amir A. Qamar, MD
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      Hepatic Encephalopathy, Chronic Hepatic Encephalopathy (portosystemic Encephalopathy), and Acute Liver Failure

      • NORTON GREENBERGER, MD
      • AMIR A. QAMAR, MD

      Hepatic encephalopathy, also referred to as portosystemic encephalopathy (PSE), is a complex neuropsychiatric disorder resulting from chronic parenchymal liver disease or liver failure, often in conjunction with portosystemic shunts, either naturally occurring or surgically created. PSE is characterized by changes in personality, level of consciousness, motor function, and cognition. This chapter discusses the pathophysiology, pathogenesis, diagnosis, and treatment of hepatic encephalopathy as well as general considerations, clinical findings, complications, treatment, specific causes, and prognosis regarding acute liver failure (ALF). Tables describe the causes of cirrhosis of the liver, grades of hepatic encephalopathy, diagnostic criteria and precipitating factors for PSE, a comparison of PSE patients with spontaneous shunts and cirrhotic controls, factors that may result in overt PSE in patients with well-preserved liver function, considerations in the management of PSE, and causes of ALF. Figures illustrate the A-deletion and square, spiral, star, and signature tests; triphasic waves; and magnetic resonance images of a patient with PSE. Graphs show the relationship between rifaximin and risk of an episode of hepatic encephalopathy, causes of ALF, and incidence of acetaminophen-related ALF.

      This chapter contains 6 highly rendered figures, 8 tables, and 14 references.

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    • 6

      Neurogastroenterology

      By Ronald F. Pfeiffer, MD
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      Neurogastroenterology

      • RONALD F. PFEIFFER, MDProfessor and Vice Chair, Department of Neurology, University of Tennessee Health Science Center, Memphis, TN

      Contrary to what one may assume, neurology and gastroenterology are similar to each other in many ways. A process found in one system can also affect the other, whether it be gastrointestinal (GI) dysfunction present in neurologic diseases or neurologic dysfunction present in GI diseases. Several disorders from both fields of medicines are highlighted in this review, ranging from stroke, Parkinson disease, and multiple sclerosis (MS) as examples of GI dysfunction in neurologic disease, to celiac disease and inflammatory bowel disease as examples of neurologic dysfunction in GI disease. Rare disorders such as Whipple disease (WhD), chronic acquired hepatocerebral degeneration (CAHD), and Wilson disease (WD) should also be considered for their neurologic dysfunctions as manifested extraintestinally. Tables give an overview of clinical features of the various disorders presented in this review.

       

      This module contains ­4 highly rendered figures, 9 tables, 349 references, and 5 MCQs. 

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    • 7

      Neurologic Consequences of Electrolyte Disorders

      By Nidyanandh Vadivel, MB, BCh; Gearoid M McMahon, MBBCh; Julian L. Seifter, MD
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      Neurologic Consequences of Electrolyte Disorders

      • NIDYANANDH VADIVEL, MB, BCHNephrologist, Division of Nephrology, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA
      • GEAROID M MCMAHON, MBBCHNephrology Fellow, Division of Nephrology, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA
      • JULIAN L. SEIFTER, MDNephrologist, Division of Nephrology, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA

      Characteristically, several fluid and electrolyte disorders have dysfunction of the central nervous system (CNS) as a major complication. The brain, within a closed space, cannot respond quickly or effectively to prevent the acute stresses of osmotic swelling or contraction of extracellular and/or intracellular spaces. Disorders of many other ions and neutral molecules can lead to pathology, including cognitive abnormalities, encephalopathy, falls, and seizures. This chapter looks at osmolar change and brain volume regulation and the importance of sodium (Na), potassium, phosphate, and magnesium. The section on sodium looks at its importance to serum osmolality and discusses hyponatremia and hypernatremia, syndrome of inappropriate antidiuretic hormone (SIADH) and cerebral salt wasting, dialysis disequilibrium syndrome, and the CNS effects of elevated ammonia and altered glucose levels. The section on potassium discusses the ratio between the intracellular and extracellular potassium concentrations and looks at hypokalemia and hyperkalemia. Phosphate plays a vital role in energy metabolism, and this section explores hypercalcemia and hypocalcemia. Magnesium’s role in cell proliferation and energy metabolism is discussed, with subsections on hypomagnesemia and hypermagnesemia. Tables list symptoms of electrolyte disorders, ways to distinguish between SIADH and cerebral salt wasting, risk factors for osmotic demyelination, and expansion and contraction syndromes. Figures include a graph showing serum Na against urine osmolality in a patient with symptomatic hyponatremia, and a chart of a woman with hyperammonemia and severe metabolic acidosis. This chapter contains 2 highly rendered figures, 4 tables, 65 references, 5 MCQs.

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    • 8

      Injuries to the Central Nervous System

      By Krista Keachie, MD; Kee D. Kim, MD; Marike Zwienenberg-Lee, MD; Kiarash Shahlaie, MD, PhD
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      Injuries to the Central Nervous System

      • KRISTA KEACHIE, MDResident Physician, Department of Neurological Surgery, University of California Davis, School of Medicine, Sacramento, CA
      • KEE D. KIM, MDAssociate Professor, Department of Neurological Surgery, University of California Davis, School of Medicine, Sacramento, CA
      • MARIKE ZWIENENBERG-LEE, MDAssistant Professor, Department of Neurological Surgery, University of California Davis, School of Medicine, Sacramento, CA
      • KIARASH SHAHLAIE, MD, PHDAssistant Professor, Department of Neurological Surgery, University of California Davis, School of Medicine, Sacramento, CA

      Traumatic brain injury (TBI) is the leading cause of death and disability in the world. In the first few hours after head injury, rapid diagnostic evaluation and appropriate neurosurgical consultation are of the utmost importance for patients with TBI. To optimize outcome in patients with TBI, a collaborative, multidisciplinary, evidence-based, protocol-directed approach to patient care should be used by modern-day trauma centers. This review covers emergency department, operative, and intensive-care unit management for patients with head injury; penetrating and blast TBI; diagnosis, initial management, and treatment of spinal cord injury (SCI); and  head injury pathophysiology. Tables outline complete neurologic injury in SCI patients by type of injury; average direct costs of a 25-year-old with SCI; Glasgow Coma Scale (GCS); indicators necessitating noncontrast head computed tomography scan in patients with loss of consciousness and GCS score of 14 or 15; patient outcomes correlated by PbtO2 values; prophylactic and therapeutic measures to prevent elevation of intracranial pressure (ICP); column failure in the four types of major thoracolumbar spinal injury; indications for the surgical treatment of burst fracture; and changes in cerebral blood flow, cerebral blood volume, ICP, and arteriovenous oxygen content difference associated with primary reduction of selected variables.

      Figures show the causes of SCI in the United States, gender divide in SCI, anatomic elements involved in SCI, clinical guidelines for the resuscitation and initial treatment of patients with severe TBI, treatment algorithm for brain tissue oxygenation, tiered therapy for ICP management, protocol for trauma patients with suspected spinal cord injury, key muscles to test for motor examination, protocol for cervical spine and thoracolumbar spine clearance in trauma patients, injury mechanisms of lower cervical spine fracture, spinal instability, and the Monro-Kellie doctrine.

      This review contains 14 figures, 9 tables, 136 references, and 5 Board-styled MCQs.

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    • 9

      Injuries to the Neck

      By David H. Wisner, MD, FACS; Joseph M. Galante, MD
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      Injuries to the Neck

      • DAVID H. WISNER, MD, FACSLloyd and Rosemargaret Donant Professor and Acting Chairman, Department of Surgery, University of California, Davis, CA
      • JOSEPH M. GALANTE, MDAssistant Professor of Surgery, Department of Surgery, Division of Trauma and Emergency Surgery, University of California, Davis, Sacramento, CA

      Injuries to the neck can be the result of blunt and penetrating trauma. Both mechanisms can cause devastating injuries, with high associated rates of morbidity and mortality. Airway management in trauma does not differ based on the mechanism of injury, and so the initial priority is to ensure an adequate airway through cricothyrotomy or tracheotomy. For penetrating neck trauma, initial management is evaluated in accordance with Advanced Trauma Life Support (ATLS) guidelines. Thereafter, the management of penetrating trauma of the stable patients is provided and includes carotid artery exploration and repair, vertebral artery exploration and repair, endovascular repair, jugular vein injuries, treatment of the pharynx and esophagus, and treatment of the larynx and trachea. Blunt trauma is described and includes injuries to the aerodigestive tract and cerebrovascular and vertebral injuries. Figures show an algorithm outlining operative management of known or suspected injuries to the carotid arteries, jugular vein, pharynx, and esophagus; the three separate zones of the neck; common incisions made along the sternocleidomastoid muscle; important anatomical structures of the neck; and an algorithm outlining management of known injuries to the vertebral artery. This chapter contains 31 references.

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    • 10

      Injuries to the Face and Jaw

      By Mark E. Engelstad, DDS, MD; Richard A. Hopper, MD, MS
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      Injuries to the Face and Jaw

      • MARK E. ENGELSTAD, DDS, MDChief, Oral and Maxillofacial Surgery, Harborview Medical Center, University of Washington
      • RICHARD A. HOPPER, MD, MSService Chief, Craniofacial Plastic Surgery, Harborview Medical Center, Chief, Plastic Surgery, Surgical Director, Craniofacial Center, Seattle Children’s Hospital, Associate Professor, University of Washington, Seattle, WA

      Facial injuries occur most often through violence or vehicular accident. The main priorities after injury are to assess the airway and check for hemorrhage, followed by assessment of vision, a check for bony trauma or malocclusion, followed by assessment for soft tissue injuries. Most facial injury survey will be done via inspection, palpation, or diagnostic imaging, which includes plain radiography, CT scan, or MRI. Injuries to the face and jaw can be of the soft tissue variety. These include injuries to the facial and trigeminal nerve, parotid duct, scalp, eyelid, lacrimal duct, eyebrow, external ear, nasal passages, or lips. Treatment of maxillofacial fractures include dentoalveolar injuries, mandibular fractures, maxillary fractures, zygomatic fractures, orbital fractures, nasal fractures, nasoorbitoethmoid fractures, and frontal sinus fractures. Figures show common injuries and fractures. This chapter includes 28 references.

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    • 11

      Stroke and Transient Ischemic Attack

      By Billy J. Kim, MD; Thomas S. Maldonado, MD
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      Stroke and Transient Ischemic Attack

      • BILLY J. KIM, MDVascular Surgery Fellow, New York University School of Medicine, New York, NY
      • THOMAS S. MALDONADO, MDAssistant Professor of Surgery, New York University School of Medicine, New York, NY

      Stroke is damage to the central nervous system caused by an interruption in blood supply. Strokes may be ischemic (failure of oxygen and nutrients to reach the affected brain) or hemorrhagic (damaging the brain by cutting off connecting pathways and causing localized or generalized pressure injury). Incidence and risk factors are presented. The clinical evaluation is addressed and includes history and physical and neurologic examination. Investigative studies are presented, including both imaging and laboratory testing. Hemorrhagic stroke is presented and can be intracerebral or subarachnoid. The systemic hypoperfusion of ischemic stroke is discussed, and includes lacunar infarcts, cardiac embolism, and atherothrombosis. Indications for and the timing of therapy is presented including surgical therapy (carotid endarterectomy and carotid angioplasty and stenting) and thrombolytics. A table lists the causes of stroke as recorded in the National Institute of Neurological and Communicative Disorders and Stroke Data Bank. This chapter contains 189 references.

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    • 12

      HIV and AIDS

      By Daniel R. Kuritzkes, MD, FACP
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      HIV and AIDS

      • DANIEL R. KURITZKES, MD, FACP

      In the quarter-century since the first report of AIDS in the United States, HIV infection has spread throughout the population, disproportionately affecting black women, Hispanic women, and men who have sex with men. The prognosis for persons infected with HIV has improved dramatically with the introduction and evolution of highly active antiretroviral therapy (HAART). The underlying principle of HAART is that a combination of potent antiretrovirals, each of which requires different mutations in the HIV genome for resistance to develop, can suppress replication sufficiently to prevent mutation and the emergence of resistance. The prospect that currently available antiretroviral therapy (ART) regimens may suppress HIV replication indefinitely provides the hope that infected patients will have life expectancies similar to those of age-matched uninfected individuals. For these patients, HIV care has shifted from an emphasis on treatment and prevention of the complications of HIV disease itself to a focus on suppression of HIV replication and management of short- and long-term complications of HIV, ART toxicities, and aging. This chapter describes the epidemiology, pathophysiology and pathogenesis, prevention, diagnosis, and management of acute and chronic HIV infection and AIDS, with figures and tables illustrating each chapter section.


      This review contains 9 highly rendered figures, 22 tables, and 248 references.

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    • 13

      Delirium in the Emergency Department: Diagnosis, Evaluation, and Management

      By Maura Kennedy, MD, MPH
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      Delirium in the Emergency Department: Diagnosis, Evaluation, and Management

      • MAURA KENNEDY, MD, MPHDivision Chief, Geriatric Emergency Medicine, Department of Emergency Medicine, Massachusetts General Hospital; Assistant Professor of Emergency Medicine, Harvard Medical School, Boston, MA

      Delirium, an acute confusional state characterized by disturbances in attention, cognition, and arousal, is present in 7 to 10% of older emergency department (ED) patients, underdiagnosed in the ED setting, and associated with increased short-term mortality. Delirium is typically precipitated by a physiologic stressor, such as an acute medical illness, a new medication, or a change in environment. The keys to the care and management of delirious patients are timely diagnosis of delirium and identification and treatment of the precipitating cause. The medical evaluation should include a formal delirium assessment that includes tests of attention and targeted diagnostic tests to identify the underlying etiology, such as infection, metabolic derangement, neurologic emergencies, new medications, and/or toxidromes. Pharmacologic treatment of delirium should be limited to patients who are severely agitated and at risk for substantial harm to self and/or others and patients with delirium secondary to alcohol withdrawal. Typical and atypical psychotics at low doses are first line for use in severely agitated patients. Benzodiazepines may worsen delirium and should be reserved for treatment of patients with delirium secondary to alcohol withdrawal or if sedation is required for critical imaging and/or procedures. ED physicians should also be conscious of and strive to minimize iatrogenic precipitants of delirium.

       

      This review contains 2 figures, 10 tables and 53 references

      Key words: aged, agitation, arousal attention, confusion, delirium, delirium/diagnosis, delirium/etiology, delirium/therapy, dementia complications, geriatrics, risk factors

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    • 14

      Brain Failure and Brain Death

      By Sharven Taghavi, MD, MPH; Ali Salim, MD
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      Brain Failure and Brain Death

      • SHARVEN TAGHAVI, MD, MPHClinical Fellow, Division of Trauma, Burns, and Surgical Critical Care, Brigham & Women’s Hospital, Boston, MA
      • ALI SALIM, MDChief, Division of Trauma, Burns, and Surgical Critical Care, Brigham & Women’s Hospital, Boston, MA

      Brain failure consists of a wide spectrum of central nervous system pathologies with many different neurologic manifestations. The causes of brain failure include several disease processes that result in decreased supply of blood and oxygen to the brain or metabolic derangements that affect the central nervous system. Brain failure usually results in some altered level of consciousness. Brain failure and brain death result in several pathophysiologic changes. The definition of brain death is controversial and evolving. However, clear guidelines to determine brain death have been established. These guidelines state that three cardinal findings be present to establish brain death: (1) coma or unresponsiveness, (2) absence of brainstem reflexes, and (3) apnea. Several clinical parameters must be met when these findings are made. Adjunctive studies such as four-vessel cerebral angiography, electroencephalography, and nuclear brain scintigraphy can help make a diagnosis of brain death. When brain death is established, suitability for organ transplantation should be evaluated. After obtaining consent, potential organ donation should be optimized for possible donation.


      This review contains 2 figures, 3 tables, and 69 references.

      Key Words: brain failure, brain death, consciousness, coma, death, delirium, organ donation



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    • Neurologic Infectious Disease
      • 1

        Encephalitis

        By Shamik Bhattacharyya, MD; Jennifer L. Lyons, MD
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        Encephalitis

        • SHAMIK BHATTACHARYYA, MDDivision of Neurological Infections, Department of Neurology Brigham and Women’s Hospital and Harvard Medical School Boston, MA
        • JENNIFER L. LYONS, MDDivision of Neurological Infections, Department of Neurology Brigham and Women’s Hospital and Harvard Medical School Boston, MA

        In 2013, the International Encephalitis Consortium proposed clinical criteria for acute encephalitis consisting of 24 hours of altered level of consciousness, lethargy, or personality change and at least three additional supportive features. Although viruses are the most common cause of acute encephalitis, not all encephalitides are acute, viral, or even infectious. Chronic encephalitis can be pathologically similar to acute encephalitis, but the causative agents and clinical manifestations vary. Management of encephalitis is largely supportive; however, for many common encephalitides primary preventive approaches exist. This module reviews the epidemiology, manifestations, diagnosis, management, and prevention of various encephalitides, including herpes family encephalitis (herpes simplex virus, varicella-zoster virus, cytomegalovirus, human herpesvirus 6), arbovirus encephalitis (West Nile virus, eastern equine encephalitis virus, tick-borne encephalitis virus, Japanese encephalitis virus), other encephalitides associated with viruses (influenza virus, human immunodeficiency virus, John Cunningham virus, rabies virus), encephalitides associated with bacteria (Mycoplasma pneumonia, Listeria monocytogenes), and autoimmune encephalitis (acute disseminated encephalomyelitis, paraneoplastic and other autoimmune encephalitides, immune reconstitution inflammatory syndrome). Tables include the International Encephalitis Consortium’s supportive feature of encephalitis, differential diagnosis for magnetic resonance imaging (MRI) findings in the patient with suspected encephalitis, diagnostic considerations for triaging workup of infection-associated encephalitis, differential diagnosis of arboviral infections by location of travel or residence, and preventive strategies for select infectious encephalitis. Figures include MRIs showing patients with herpes simplex encephalitis, varicella-zoster virus, eastern equine encephalitis, HIV, Listeria monocytogenes, acute disseminated encephalomyelitis, acute hemorrhagic leukoencephalitis, and immune reconstitution inflammatory syndrome.

        This module contains 8 highly rendered figures, 5 tables, 78 references, and 5 MCQs.

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      • 2

        Acute Viral Meningitis

        By Jared R. Brosch, MD; Karen L. Roos, MD
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        Acute Viral Meningitis

        • JARED R. BROSCH, MDDeptment of Neurology, Assistant Professor of Neurology, Indiana University School of Medicine, Indianapolis, IN
        • KAREN L. ROOS, MDJohn and Nancy Nelson Professor of Neurology and Professor of Neurological Surgery, Indiana University School of Medicine, Indianapolis, IN

        Acute viral meningitis refers to inflammation of the meninges of the brain in response to a viral pathogen. Viruses cause meningitis, encephalitis, myelitis, or a combination of these, meningoencephalitis or encephalomyelitis. Viral meningitis is typically a self-limited disorder with no permanent neurologic sequelae. This chapter reviews the epidemiology, etiology, diagnosis, differential diagnosis, treatment, complications, and prognosis. Tables describe Wallgren’s criteria for aseptic meningitis, important arboviral infections found in North America, herpes family viruses and meningitis, classic cerebrospinal fluid (CSF) abnormalities with viral meningitis, Centers for Disease Control and Prevention criteria for confirming arboviral meningitis, basic CSF studies for viral meningitis, and etiology of CSF pleocytosis. Figures depict common causes of viral meningitis, nuchal rigidity, examination for Kernig sign, and Brudzinski sign for meningeal irritation.

        This chapter contains 4 highly rendered figures, 7 tables, 16 references, and 5 MCQs.

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      • 3

        Acute Bacterial Meningitis

        By Karen L. Roos, MD
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        Acute Bacterial Meningitis

        • KAREN L. ROOS, MDJohn and Nancy Nelson Professor of Neurology and Professor of Neurological Surgery, Indiana University School of Medicine, Indianapolis, IN

        Acute bacterial meningitis is a life-threatening infection. By definition, meningitis is an infection of the meninges and the subarachnoid space. Bacterial meningitis is associated with an inflammatory response that involves the meninges, the subarachnoid space, the brain parenchyma, and the cerebral arteries and veins. As such, bacterial meningoencephalitis is the more accurate descriptive term. This chapter discusses the epidemiology, etiology, pathophysiology and pathogenesis, diagnosis, differential diagnosis, treatment, complications, and prognosis of the disease. The discussion of diagnosis covers clinical manifestations, physical examination findings, laboratory tests, and imaging studies. The discussion of treatment covers empirical therapy, specific antimicrobial therapy, and dexamethasone therapy. Graphs compare causative organisms and clinical manifestations of community-acquired meningitis. Illustrations depict proper patient positioning for detecting nuchal rigidity, Kernig sign, Brudzinski sign, and lumbar puncture, as well as a sagittal view of a lumbar puncture needle as it is advanced into the subarachnoid space. An algorithm delineates the approach to the patient with symptoms and signs of bacterial meningitis. Tables outline bacterial pathogens based on predisposing and associated conditions, cerebrospinal fluid diagnostic studies for meningitis, the appearance of the organism on a Gram stain, empirical antimicrobial therapy based on predisposing and associated conditions, recommendations for specific antibiotic therapy in bacterial meningitis, and recommended doses for antibiotics commonly used in the treatment of bacterial meningitis.

         

        This chapter contains 8 highly rendered figures, 6 tables, 75 references, 1 teaching slide set, and 5 MCQs.

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      • 4

        Central Nervous System Diseases Due to Slow Viruses and Prions

        By Francisco González-Scarano, MD
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        Central Nervous System Diseases Due to Slow Viruses and Prions

        • FRANCISCO GONZÁLEZ-SCARANO, MD

        Several central nervous system diseases whose common elements include a long incubation period and a progressive clinical course were once called slow virus infections, because most of them are in fact caused by viruses. However, one group of these CNS diseases is now believed to be caused by abnormally configured proteins known as prions; rather than an etiologic designation, therefore, on the whole these diseases are better characterized by their chronicity, their transmissibility, and at this point, their inexorably deteriorating natural history. This chapter reviews the more common of these: HIV-associated dementia (HAD or HIVD), human T cell lymphotropic virus type 1 (HTLV-1)-associated myelopathy, Creutzfeldt-Jakob disease (CJD), progressive multifocal leukoencephalopathy (PML), and subacute sclerosing encephalitis (SSPE), which is associated with a variant of measles virus. Figures illustrate the pathogenesis and the pathology of HIV dementia, propagation of scrapie prion protein (PrP) in brain neurons, and spongiform brain changes of CJD. Tables list the stages of HAD and the clinical and pathologic characteristics distinguishing classic CJD and varient CJD.

        This module contains ­5 highly rendered figures, 2 tables, 57 references, and 5 MCQs. 

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      • 5

        Brain and Spinal Abscesses

        By Allan R Tunkel, MD, PhD; W Michael Scheld, MD
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        Brain and Spinal Abscesses

        • ALLAN R TUNKEL, MD, PHDProfessor of Medicine, Drexel University College of Medicine, Philadelphia, PA, Chair, Department of Medicine, Monmouth Medical Center, Long Branch, NJ
        • W MICHAEL SCHELD, MDProfessor of Medicine and Neurosurgery, University of Virginia School of Medicine, Charlottesville, VA

        Brain and spinal abscesses are focal infections of the central nervous system that are often associated with significant morbidity and mortality if not recognized early and managed in a timely manner. In patients with brain abscess, the clinical manifestations run the gamut from indolent to fulminant; most are related to the size and location of the space-occupying lesion within the brain and the virulence of the infecting organism. Untreated spinal epidural abscess usually progresses through four stages: backache and focal vertebral pain, nerve root pain, spinal cord dysfunction, and complete paralysis. Magnetic resonance imaging is the diagnostic neuroimaging procedure of choice in patients with brain and spinal abscesses; on diffusion-weighted images, restricted diffusion may be seen and may help distinguish abscesses from necrotic neoplasms. Aspiration of the abscess is important to facilitate microbiologic diagnosis; after aspiration and submission of specimens for special stains, histopathologic examination, and culture, empirical antimicrobial therapy should be initiated based on stains of the aspirated specimen and the probable pathogenesis of infection. Once the infecting pathogen is isolated, antimicrobial therapy can be modified for optimal treatment. Surgical therapy is often required for the optimal approach to patients with brain and spinal abscesses.

        This review contains 6 figures, 5 tables, and 72 references.

        Key words: antimicrobial therapy for central nervous system infections, brain abscess, epidural abscess, focal intracranial infections, head trauma, infections in immunocompromised hosts, spine infections, subdural empyema, toxoplasmosis 

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      • 6

        Primary and Metastatic Central Nervous System Malignancies

        By Fabio M. Iwamoto, MD; Howard A. Fine, MD
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        Primary and Metastatic Central Nervous System Malignancies

        • FABIO M. IWAMOTO, MDAssistant Clinical Investigator, Neuro-Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD
        • HOWARD A. FINE, MDSenior Investigator and Chief, Neuro-Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD

        About 13,000 deaths each year in the United States are attributed to primary central nervous system (CNS) malignancies. An estimated 20% of patients with cancer eventually develop clinically apparent CNS metastases, and an estimated 170,000 cases of brain metastases are diagnosed in the United States yearly. Autopsy studies suggest that as many as 50% of patients dying from advanced cancer may have metastasis to the CNS. This chapter provides an overview of primary and metastatic CNS malignancies with in-depth discussion of gliomas, primary CNS lymphoma, meningioma, brain metastases, leptomeningeal metastases, and metastatic epidural spinal cord compression. Discussions cover epidemiology, etiology, diagnosis, and treatment of gliomas, including surgery, radiotherapy, and chemotherapy for both newly diagnosed gliomas and recurrent gliomas. The epidemiology, diagnosis, treatment and prognosis for primary CNS lymphomas are reviewed, as well as the epidemiology, etiology, diagnosis, treatment, and prognosis for meningiomas. Epidemiology, diagnosis, and prognosis for brain metastases are briefly discussed, and the section on treatment includes surgery, stereotactic radiosurgery, and whole-brain radiotherapy for patients with three or fewer brain metastases. The sections on leptomeningeal metastases and metastatic epidural spinal cord compression cover diagnosis, treatment, and prognosis. This chapter contains 126 references.

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      • 7

        Viral Zoonoses

        By Lyle R Petersen, MD, MPH; Duane J. Gubler, ScD
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        Viral Zoonoses

        • LYLE R PETERSEN, MD, MPHDirector, Division of Vector-Borne Diseases, Centers for Disease Control and Prevention, Fort Collins, CO
        • DUANE J. GUBLER, SCDProfessor, Program on Emerging Infectious Diseases, DUKE-NUS Graduate Medical School, Singapore

        Human infection by zoonotic viruses—pathogens that normally infect animals—may result in no obvious illness, a nonspecific viral syndrome, or more severe illness typically characterized by hemorrhagic fever, encephalitis, or rash arthralgia. Transmission usually occurs by direct contact with or a bite from an infected animal or arthropod. Viral families discussed include Flaviviridae, Bunyaviridae, Arenaviridae, Filoviridae, Togaviridae, Rhabdoviridae, Paramyxoviridae, and Reoviridae, with consideration given to the epidemiology, diagnosis, treatment, and prevention of specific viruses. Hemorrhagic fevers addressed include dengue fever, dengue hemorrhagic fever, yellow fever, Crimean-Congo hemorrhagic fever, and Rift Valley fever; hantavirus infections; and the Marburg and Ebola viruses. Encephalitic fever–causing viruses discussed include La Crosse; Japanese; Murray Valley; St. Louis; tick-borne; West Nile; Powassan; eastern, western, and Venezuelan equine; rabies; Nipah; Barmah Forest; and Colorado tick fever. Rash arthralgia may be caused by the Barmah Forest, Chikungunya, Mayaro, O’nyong-nyong, Ross River, and dengue viruses. Other viral zoonoses considered include monkey B virus, ruminant and primate poxvirus, Newcastle, and foot-and-mouth diseases, as well as vesicular stomatitis virus infection. A diagram depicts the generalized arbovirus maintenance cycle. Tables list the important viral zoonoses that cause human disease, the principal hantaviruses that cause human disease, the arenaviruses that cause significant human illness, and the viral zoonoses endemic in the United States. 

        This review contains 1 highly rendered figure, 4 tables, and 81 references.

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    • Other Topics in Neurology
      • 1

        Neurology of Rheumatic Diseases

        By Richard B. Rosenbaum, MD
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        Neurology of Rheumatic Diseases

        • RICHARD B. ROSENBAUM, MDMedical Director, The Portland Parkinson’s Program of The Oregon Clinic and Legacy Health System, Affiliate Professor of Neurology, Oregon Health and Sciences University

        This review examines the neurologic complications of several rheumatic diseases, including rheumatoid arthritis, seronegative spondyloarthritis, systemic lupus erythematosus, scleroderma and related diseases, vasculitis, spinal osteoarthritis and spondylosis, and primary Sjögren syndrome. Tables describe the categories of neurologic complications of lupus, the prevalence of neurologic manifestations among patients with lupus, distribution of nerve involvement among patients with mononeuritis multiplex caused by microscopic polyangiitis, sensitivity of combined superficial peroneal nerve and peroneus brevis muscle in patients with suspected vasculitis, sites of central nervous system involvement among patients with parenchymal neuro-Behçet syndrome, and localization of cervical radiculopathy. Figures include a radiograph of a patient with rheumatoid arthritis, magnetic resonance imaging showing diffuse pachymeningeal enhancement and the cervical patient with lupus, and photographs of a muscle biopsy of a patient with vasculitis nephropathy and anterior ischemic optic neuropathy secondary to temporal arteritis.

        This review contains 5 highly rendered figures, 6 tables, and 77 references.

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      • 2

        Neurosarcoidosis

        By Jeffrey M Gelfand, MD, MAS; Barney Stern, MD
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        Neurosarcoidosis

        • JEFFREY M GELFAND, MD, MASAssistant Professor of Clinical Neurology, UCSF Comprehensive Center for Multiple Sclerosis and Neuroinflammation, Department of Neurology, University of California-San Francisco, San Francisco, CA
        • BARNEY STERN, MDProfessor and Interim Chairman, Department of Neurology, University of Maryland, Baltimore, MD

        Sarcoidosis is an inflammatory disorder of unclear cause characterized by nonnecrotizing (noncaseating) granulomatous inflammation. Neurosarcoidosis—involvement of the nervous system by the granulomatous inflammatory process of sarcoidosis—affects approximately 5 to 15% of people with sarcoidosis and is a major cause of morbidity from the disease. Neurosarcoidosis is often invoked in the differential diagnosis of suspected inflammatory neurologic syndromes. This chapter discusses the epidemiology, etiology, genetics, pathophysiology and pathogenesis, diagnosis and differential diagnosis, treatment, complications, and prognosis of neurosarcoidosis. Figures show magnetic resonance imaging features of neurosarcoidosis. Tables outline a clinical framework for categorizing

        neurosarcoidosis and neurologic organ system involvement by sarcoidosis.

         

        This chapter contains 1 highly rendered figure, 2 tables, 124 references, 1 teaching slide set, and 5 MCQs.

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  • Special Topics in Neurology
    • 1

      Blunt Cerebrovascular Injuries

      By Clay Cothren Burlew, MD, FACS
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      Blunt Cerebrovascular Injuries

      • CLAY COTHREN BURLEW, MD, FACSProgram Director, Trauma and Acute Care Surgery Fellowship, Department of Surgery, Denver Health Medical Center, Associate Professor of Surgery, University of Colorado, Denver, CO

      Blunt cerebrovascular injuries (BCVIs) are increasingly recognized in trauma patients, with 1 to 3% of all blunt trauma patients being diagnosed with a carotid artery injury or a vertebral artery injury. Specific injury patterns are associated with BCVI and serve as the trigger for injury screening in asymptomatic patients. Multislice (> 64-slice) computed tomographic angiography is the routine imaging test performed to identify BCVI. Once an injury is identified, antithrombotic treatment almost universally prevents BCVI-related stroke. Endovascular therapy for BCVI is reserved for those patients who are markedly symptomatic or have an enlarging pseudoaneurysm on repeat imaging.

      Key Words: blunt cerebrovascular injuries, blunt trauma, carotid artery injury, stroke, vertebral artery injury

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    • 2

      Women's Neurology

      By M. Angela O’Neal, MD
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      Women's Neurology

      • M. ANGELA O’NEAL, MDDirector of Women’s Neurology, Department of Neurology, Brigham and Women’s Hospital, Boston, MA

      This chapter reviews some of the features important to consider in women with neurologic disease, including medication and disease effects on both reproductive health and pregnancy/fetal development, as well as hormonal effects on neurologic disease. A case-based approach is used to discuss diseases that affect women throughout their life cycle (multiple sclerosis [MS] and epilepsy), disorders that affect only women (eclampsia), and those that affect women preferentially (migraine, cerebral venous thrombosis, reversible cerebral vasospasm, and Alzheimer disease). The epidemiology, differential diagnosis, pathophysiology, management, and prognosis are reviewed for each disorder. Tables include US Food and Drug Administration pharmaceutical pregnancy categories, learning objectives, migraine with aura, alternative diagnostic criteria for migraine without aura, migraine aura versus transient ischemic attacks, red flags to secondary headache, abortive headache therapy in pregnancy, migraine preventive medications and pregnancy, MS therapies in pregnancy, pregnancy consulting points for MS patients on a disease-modifying therapy, and general recommendations for women with epilepsy and pregnancy. Figures show fluid-attenuated inversion recovery and gradient echo magnetic resonance images of the right anterior parietal region; a magnetic resonance venogram demonstrating occlusion in the superior sagittal sinus; posterior reversible encephalopathy syndrome; and computed tomographic (CT) images of a hemorrhage in the left parietal region, left frontal subarachnoid bleeding, and left middle cerebral beading.

      This chapter contains 5 highly rendered figures, 11 tables, 29 references, and 5 MCQs.

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    • 3

      Neurocritical Care

      By Terrance T. Kummer, MD, PhD; Allan H Ropper, MD
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      Neurocritical Care

      • TERRANCE T. KUMMER, MD, PHDNeurocritical Care Fellow, Massachusetts General Hospital and Brigham and Women’s Hospital, Department of Neurology, Harvard Medical School, Boston, MA
      • ALLAN H ROPPER, MDProfessor of Neurology, Brigham and Women’s Hospital, Department of Neurology, Harvard Medical School, Boston, MA

      Neurologic critical care encompasses the management of many nervous system diseases when they present in the extremes of severity. Core conditions managed in the neuroscience intensive care unit (ICU) include stroke, cerebral hemorrhage, status epilepticus (SE), myasthenia gravis (MG), Guillain-Barré syndrome (GBS), traumatic brain and spinal cord injury, and high-risk postoperative neurosurgical patients. The skills and knowledge base required to care for patients with such conditions, and the life-threatening complications associated with them, are drawn from both traditional neurology and from critical care medicine. This chapter covers specialized monitoring in the neurologic intensive care unit and special conditions such as acute intracranial hypertension, acute ischemic stroke, intracerebral hemorrhage, venous sinus thrombosis, myasthenia gravis, GBS, seizure and status epilepticus, spinal cord injury, and traumatic brain injury. The chapter includes 8 tables and 5 figures. Tables provide common etiologies of acute intracranial hypertension; general prophylactic measures, medical interventions, surgical interventions, and stepwise treatment protocol for acute intracranial hypertension; drugs that can exacerbate weakness in myasthenia gravis, cholinergic drug dosage equivalents and duration of action, and antiseizure medications used in status epilepticus. Figures illustrate the Monro-Kellie Doctrine, intracranial pressure waveform and plateau waves, typical herniation patterns, large stroke with malignant edema, and examples of nontraumatic intracerebral hemorrhage. This chapter contains 114 references.

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    • 4

      Trauma: Head and Facial Trauma

      By Christopher R. Tainter, MD, RDMS; Raghu Seethala, MD
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      Trauma: Head and Facial Trauma

      • CHRISTOPHER R. TAINTER, MD, RDMSAssistant Clinical Professor, Department of Emergency Medicine, Department of Anesthesia, Division of Critical Care, University of California, San Diego, San Diego, CA
      • RAGHU SEETHALA, MDAttending Physician, Emergency Department and Surgical Intensive Care Unit, Brigham and Women’s Hospital, Instructor of Emergency Medicine, Emergency Medicine, Harvard Medical School, Boston, MA

      Head and facial trauma includes a wide variety of heterogeneous injuries that vary according to cause, severity, management, and outcomes. These injuries are classified as head injuries (the scalp, skull, and brain) and facial injuries (to the eyes, bony structures, nose, mouth, teeth, tongue, and glands and the accompanying nerves, muscles, and vasculature). This review covers the epidemiology, anatomy, assessment and stabilization, diagnosis, treatment and disposition, and outcomes for traumatic brain injury (TBI), scalp and cranial vault trauma, ocular trauma, and facial trauma. Figures show computed tomographic scans showing a large left frontal lobe intraparenchymal hemorrhage, a traumatic subarachnoid hemorrhage, a subdural hematoma, a right occipital epidural hematoma, and a left orbital floor “blowout” fracture; an illustration of the brain herniation syndromes; the anatomy of the globe; and the Le Fort classification scheme of midface fractures. Tables list the Glasgow Coma Scale, clinical decision aids for computed tomography after mild TBI, the PECARN clinical decision rule for children with mild TBI, the graduated return to play protocol, and anticoagulant and antiplatelet agent reversal.

      This review contains 8 highly rendered figures, 5 tables, and 75 references.

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    • 5

      Ears

      By Peter Pruitt, MD; Thomas Osborne Stair, MD
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      Ears

      • PETER PRUITT, MDEmergency Medicine Resident, Brigham and Women’s / Massachusetts General Hospital Harvard Affiliated Emergency Medicine Residency, Boston, MA
      • THOMAS OSBORNE STAIR, MDDepartment of Emergency Medicine, Brigham and Women’s Hospital, Boston, MA

      As the structure of the ear is made of little more than cartilage, a limited blood supply, and a thin layer of skin, trauma in this area can easily manifest from a variety of causes. Some common examples of trauma involve laceration, piercing (intentional or otherwise), infection causing chondritis, blunt trauma causing necrosis, rupture of the tympanic membrane, perforation of the ear drum, and acoustic trauma that may result in hearing disorders such as tinnitus and high-frequency hearing loss. Acute hearing loss shows in two forms: conductive hearing loss and sensorineural hearing loss, the latter of which is caused by damage to the anatomic or neurologic structures of the ear dedicated to hearing. Sensorineural hearing loss generally has a poor prognosis and mandates prompt referral to an otolaryngologist. For information relating to Meniere disease, please refer to the Emergency Medicine topic review “Mouth and Oropharynx/Upper Airway.” Figures show illustrations of the inner ear and right ear serous otitis media, the canalith repositioning procedure (Epley maneuver), and the Dix-Hallpike maneuver. Tables list differential diagnosis for ear pain, ear equipment for the primary care office, and steps for the Epley maneuver to relocate otolith in benign paroxysmal positional vertigo (BPPV).

      This review contains ­4 highly rendered figures, 3 tables, 28 references, and 5 MCQs. 

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    • 6

      Toxic Plants and Mushrooms

      By Marie King, MD, PhD; Richard Church, MD
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      Toxic Plants and Mushrooms

      • MARIE KING, MD, PHDEmergency Physician, Harrington Hospital, Southbridge, MA
      • RICHARD CHURCH, MDAssistant Professor, Emergency Medicine and Toxicology, University of Massachusetts, Worcester, MA

      Many species of plants and mushrooms exist that, when consumed, can induce poisoning in individuals, causing a range of side effects. As the toxins do not always correspond to an antidote, it is important to have the ability to identify each harmful species to determine the appropriate treatment. This review gives an overview of some of the more prevalent toxic plants and mushrooms, detailing their principles of toxicity, recommendations for immediate stabilization, keys to proper diagnosis and definitive therapy, and patient disposition and outcomes. Figures show photographs of the various toxic plants and mushrooms featured. Tables show a list of toxic species for both plants and mushrooms, including their common names, the toxins contained within, their effects, and the corresponding antidote (if any).

      This review contains ­16 highly rendered figures, 2 tables, 44 references, and 5 MCQs. 

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    • 7

      Toxic Gases

      By Stephanie T Weiss, MD, PhD; Kathryn W Weibrecht, MD
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      Toxic Gases

      • STEPHANIE T WEISS, MD, PHDAssociate Director, Emergency Medicine, Morton Hospital and Medical Center, Taunton, MA
      • KATHRYN W WEIBRECHT, MDAssociate Director, Emergency Medicine, Morton Hospital, Taunton, MA

      This review looks at the potential causes, diagnoses, and possible treatments for three asphyxiant gases: carbon monoxide, hydrogen cyanide, and hydrogen sulfide, Exposure to these gases can lead to central nervous system depression, unconsciousness, and death due to tissue hypoxia. These gases are among the most common causes of fatalities related to toxic gas poisoning, with carbon monoxide responsible for 36% and hydrogen sulfide 7.7%. It is necessary to remove victims affected by poisoning immediately from the source of the toxic gas, administer oxygen, and assess their stability. As symptoms of these gases can differ widely, ranging from broad and unspecific to highly morbid, and may require different levels of care, the correct diagnosis should also rely on inferences from the patient history and the context of the admission, including evidence of fire and chemical reactions. Normobaric oxygen and hyperbaric oxygen are the two main treatments for carbon monoxide, although studies have been inconclusive in regards to the effectiveness of hyperbaric oxygen. The Cyanokit (containing hydroxocobalamin) is considered to be more effective for hydrogen cyanide when compared with the Cyanide Antidote Kit due to the former’s low toxicity and high effectiveness. Hydrogen sulfide is often used as a suicide agent, the mortality of which is close to 100%. Figures show the mechanisms by which the asphyxiant gases carry out their negative effects on the human body. Tables show the half-life of carboxyhemoglobin with oxygen therapy and a comparison between the Cyanide Antidote Kit and the Cyanokit.

      This review contains 3 highly rendered figures, 2 tables, 43 references, and 5 MCQs. 

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    • 8

      Principles of Neurologic Ethics

      By Thomas I. Cochrane, MD, MBA
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      Principles of Neurologic Ethics

      • THOMAS I. COCHRANE, MD, MBAAssociate Neurologist, Division of Neuromuscular Disease, Department of Neurology, Brigham and Women’s Hospital, Assistant Professor of Neurology, Harvard Medical School, Boston, MA

      Many neurologic diseases affect the brain and thus cognition and personality. Many people consider some neurologic conditions “worse than death” and would prefer not to be kept alive using medical technology if there is no reasonable chance of recovery. The clinician caring for patients with neurologic disease will frequently be confronted with decisions about whether to initiate or continue life-sustaining therapies. And because neurologic disease often impairs the ability to understand and to decide, clinicians are more often called on to make decisions for patients who cannot decide for themselves. This chapter covers general principles, including respect for autonomy, beneficence, nonmaleficence, justice, informed consent, and implied consent. Discussion about making decisions for others includes competence and decision-making capacity, advance directives (powers of attorney and living wills), substituted judgment, and best interests. Decisions about life-sustaining treatment include withholding versus withdrawing, acts versus omissions, and limiting life-sustaining treatment for the patient with no surrogate. Also covered are decisions in the face of prognostic uncertainty, and futility, as well as commonly encountered problems such as states of severely disordered consciousness, coma, vegetative state, minimally conscious state, and brain death (including ethical controversies). Organ donation after both brain death and cardiac death is discussed. The section on dementia covers feeding tubes for patients with advanced dementia, the locked-in state, and neuroenhancement. Tables include elements of informed consent, elements of competence, assumed ethical priority of potential surrogates, steps that aid decision making in the face of prognostic uncertainty, and the American Academy of Neurology’s criteria for determination of brain death. This chapter includes 23 references.

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    • 9

      Neuroimaging for the Clinician

      By Joshua P Klein, MD, PhD
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      Neuroimaging for the Clinician

      • JOSHUA P KLEIN, MD, PHDChief, Division of Hospital Neurology, Department of Neurology, Brigham and Women’s Hospital, and Assistant Professor of Neurology, Harvard Medical School, Boston, MA

      Modern neuroimaging has revolutionized the practice of neurology by allowing visualization and monitoring of evolving pathophysiologic processes. High-resolution magnetic resonance imaging (MRI) can now resolve structural abnormalities on a near-cellular level. Advances in functional imaging can assess the in vivo metabolic, vascular, and functional states of neuronal and glial populations in real time. Given the high density of data obtained from neuroimaging studies, it is essential for the clinician to take an active role in understanding the nature and significance of imaging abnormalities. This chapter reviews computed tomography and MRI techniques (including angiography and advanced sequences), specialized protocols for investigating specific diagnoses, risks associated with imaging, disease-specific imaging findings with general strategies for interpretation, and incidental findings and artifacts. Figures include computed tomography, T1- and T2-weighted signal intensity, diffusion-weighted magnetic resonance imaging, magnetic resonance spectroscopy, imaging in epilepsy and dementia, extra-axial versus intra-axial lesions, typical lesions of multiple sclerosis, spinal imaging, spinal pathology, vascular pathology, intracranial hemorrhage, and common imaging artifacts. Tables list Hounsfield units, patterns of enhancement from imaging, advanced techniques in imaging, magnetic resonance imaging sequences, and the evolution of cerebral infarction and intraparenchymal hemorrhage on magnetic resonance imaging. This chapter contains 213 references.

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    • 10

      Spinal Disorders

      By James Rainville, MD
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      Spinal Disorders

      • JAMES RAINVILLE, MDAssistant Clinical Professor, Department of Physical Medicine and Rehabilitation, Harvard Medical School, Chief, Physical Medicine and Rehabilitation, New England Baptist Hospital, Medical Director, The Spine Center, New England Baptist Hospital, Boston, MA

      Physicians of many disciplines encounter patients complaining of neck pain, back pain, and radicular symptoms. Although the prognoses for spinal problems are generally favorable and most patients recover quickly, in many cases, symptoms recur or persist over time. Sharing information with patients can help health care providers develop a rationale for treatment that stresses the role of patients in their recovery. This chapter discusses spine anatomy and function, age-related degeneration of spine structures, the epidemiology and natural history of degenerative spine disorders, the basic neurology of pain, and treatment choices that may optimize recovery. A table describes common patterns of symptoms and signs of radiculopathies. A graph illustrates pain scores for leg pain from acute lumbar radiculopathies.

      This chapter contains 1 highly rendered figure, 1 table, 323 references, and 5 MCQs.

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    • 11

      The Cognitive Psychology of Diagnostic Errors

      By Lauren E. Benishek, PhD; Sallie J. Weaver, PhD; David E. Newman-Toker, MD, PhD
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      The Cognitive Psychology of Diagnostic Errors

      • LAUREN E. BENISHEK, PHDResearch Fellow, Armstrong Institute for Patient Safety and Quality and Department of Anesthesiology & Critical Care Medicine, Johns Hopkins University School of Medicine, Baltimore, MD
      • SALLIE J. WEAVER, PHDAssistant Professor, Armstrong Institute for Patient Safety and Quality, and Department of Anesthesiology & Critical Care Medicine, Johns Hopkins University School of Medicine, Carey School of Business, Baltimore, MD
      • DAVID E. NEWMAN-TOKER, MD, PHDAssociate Professor, Department of Neurology, Johns Hopkins Hospital, Baltimore, MD

      Health care involves complex decision making, often under uncertain, ambiguous, and time-sensitive conditions. Clinicians typically face the greatest uncertainty when making diagnostic decisions; common, undifferentiated symptoms paired with increasing prevalence of complex comorbidities, continuously and rapidly evolving scientific evidence, and often fragmented information systems are just a few of the hurdles clinicians must navigate as part of daily diagnostic decision making. In this review, the current state of the science concerning the cognitive psychology of diagnostic errors is discussed, including models of diagnostic reasoning, common errors: heuristics and biases, and practical implications and interventions. Figures show a conceptual model for diagnostic errors; diagnostic and therapeutic cycles; relationships among heuristics, biases, premature closure, and diagnostic errors; Reason’s (2000) Swiss cheese model; and tradeoffs versus improvements in diagnostic performance as illustrated by the receiver operating characteristic curve. Tables list important reasons for understanding the foundational cognitive models of diagnostic reasoning; a glossary of key diagnostic error–related definitions; three models of cognitive decision making; a summary of clinical reasoning models; steps of diagnostic decision making; examples of diagnostic errors resulting from representativeness, availability, and anchoring and adjustment; categories of countermeasures for error reduction interventions; examples of cognitively, systems-, and patient-focused countermeasures for selected biases; a summary of cognitively focused countermeasures to cognitive bias; key problem “classes” where problem- or context-specific solutions might be applied;types of system-focused countermeasures; and patient-focused countermeasures to avoid diagnostic error.

       

      This review contains 5 highly rendered figures, 12 tables, and 120 references.

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    • 12

      The Neurologic Examination

      By Nicholas J Beimer, MD; Douglas J Gelb, MD, PhD
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      The Neurologic Examination

      • NICHOLAS J BEIMER, MDClinical Instructor, Department of Neurology, University of Michigan, Ann Arbor, MI
      • DOUGLAS J GELB, MD, PHDProfessor, Department of Neurology, University of Michigan, Ann Arbor, MI

      All physicians, regardless of their medical specialty or the setting in which they treat patients, must be able to perform a neurologic examination. In the outpatient office, up to 9 to 10% of all symptoms suggest the possibility of neurologic disease and up to 5% of emergency department visits are due to primary neurologic disease. The neurologic examination is critical in triaging these patients, selecting diagnostic tests, and indicating management. This review covers how to think about the neurologic examination, the screening examination, and diagnosis-focused neurologic examinations with an emphasis on stroke, epilepsy, encephalopathy and coma, neurodegenerative diseases, neuromuscular disease, and functional disorders. The figure shows a conceptual approach to the neurologic examination. The tables list components of the screening neurologic examination, neurologic examination focus points for suspected stroke and suspected epilepsy, lateralization and localization of common seizure semiologies, and neurologic examination focus points for encephalopathy/coma, suspected neurodegenerative disease, suspected neuromuscular disease, and suspected functional neurologic disorders.

      This review contains 1 highly rendered figure, 8 tables, and 14 references.

      Key words: Neurologic examination, neurodegenerative disease, neuromuscular disease, neurologic screening

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    • 13

      Nutritional Support in the Patient With Traumatic and Nontraumatic Brain Injury

      By Barbara Magnuson Woodward, PharmD, CNSC ; Aaron M. Cook, PharmD, BCPS
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      Nutritional Support in the Patient With Traumatic and Nontraumatic Brain Injury

      • BARBARA MAGNUSON WOODWARD, PHARMD, CNSC Coordinator, Nutrition Support Service, UK HealthCare, Associate Adjunct Professor, Department of Pharmacy Practice & Science , University of Kentucky, College of Pharmacy, Lexington, KY
      • AARON M. COOK, PHARMD, BCPS Clinical Coordinator, Neuroscience, Pulmonary/Critical Care, UK HealthCare, Associate Adjunct Professor, Department of Pharmacy Practice & Science, University of Kentucky, College of Pharmacy, Lexington, KY

      The multiple metabolic alterations following neurologic injuries create several unique challenges for clinicians providing nutrition support. The hypermetabolic and hypercatabolic state following traumatic brain injury (TBI) has been well studied over the past three decades. This review discusses several unique issues to incorporate into the nutrition support plan for both acute traumatic and nontraumatic brain injuries. The initial energy expenditure assessment varies drastically among the different neurologic injuries, from the lowest with anoxic brain injury and spinal cord injury to the highest with traumatic subdural hemorrhagic injury. Measuring energy expenditure with indirect calorimetry becomes a critical component of the assessment due to wide-ranging metabolic needs and effects from various drug therapies. An excess or a lack of adequate nutrition support clearly results in worse neurologic and nutrition status outcomes. The nutrition status and preexisting comorbidities of the neurologically injured patient further compound the complexity of the nutrition plan. Anticipating and correcting for refeeding syndrome in the malnourished stroke patient or alcoholic hypermetabolic TBI patient avoid further metabolic, cardiac, and ventilator complications. Fluid balance must be continuously monitored and hyperglycemia avoided to prevent cerebral edema. Planning and implementing an aggressive nutrition support regimen inclusive of drug therapy, appropriate fluid status, electrolyte replacement, and close glucose monitoring incorporate the expertise of several health care disciplines to ensure optimal outcomes from the critical care setting. Nutrition support plans become one of the primary focuses during recovery following a neurologic injury. Proper swallow assessments and techniques to recognize dysphagia and avoid aspiration and its complications with pneumonia become paramount during the stroke rehabilitation phase. Due to the variety of acute neurologic injuries, health care providers must address each of the distinctive issues for an individualized nutrition support therapy to ensure optimal outcomes and minimize complications

      This review contains 1 figure, 4 tables, and 58 references.

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    • 14

      Spine and Spinal Cord Injury

      By Miguel Arribas, MD; Michael A. Cole, MD
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      Spine and Spinal Cord Injury

      • MIGUEL ARRIBAS, MDEmergency Medicine Resident, Department of Emergency Medicine, Michigan Medicine, University of Michigan
      • MICHAEL A. COLE, MDAssistant Professor, Department of Emergency Medicine, University of Michigan Medical School, Michigan Medicine, University of Michigan

      Spinal injury has the potential to dramatically change a patient’s life. Prompt diagnosis, appropriate supportive medical care, early transfer to a spinal injury center and, if necessary, surgical intervention within 24 hours are essential to optimizing outcomes.  Clinical decision rules aid in determining the need for imaging. When needed, non-contrast enhanced CT is the initial imaging test of choice with MRI being used in patients with neurologic findings, significant pathology on CT, and/or high suspicion for injury. CT or MRI with intravenous contrast is preferred in penetrating trauma. Radiographs are of limited utility in evaluating spinal injury in adults. Classification of spinal injury based on appearance on imaging and neurologic exam is important for surgical management decisions. Cervical injury may lead to respiratory distress requiring early intubation. Hypotension is most often a result of hemorrhage from concomitant traumatic injuries to other organ systems. Crystalloid, blood products, atropine and norepinephrine should be used as needed to avoid systolic BP< 90 mm Hg or heart rate< 60 BPM and maintain a MAP of 85-90 mm Hg. Steroid administration within the first 8 hours of significant spinal injury is controversial and the decision to administer steroids should be made through consultation with patient, family and spinal specialist.

      The review contains 8 figures, 2 videos, 13 tables, and 59 references.

      Keywords: blunt trauma, neurologic assessment, penetrating trauma, spinal anatomy, spinal cord injury, spinal injury, steroid use, vertebrae, vertebral anatomy, vertebral injury

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  • Child Neurology
    • 1

      Metabolic Disorders

      By Lance Rodan, MD, FRCP(C)
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      Metabolic Disorders

      • LANCE RODAN, MD, FRCP(C)Division of Genetics and Genomics, Department of Medicine, Pediatrics, Children’s Hospital Boston, Boston, MA

      Inborn errors of metabolism (IEM) are individually rare but have a collective incidence of approximately one in 1000. Most IEM can manifest with neurologic symptoms. It is crucial for the pediatric neurologist to be familiar with the evaluation and management of these disorders because many are amenable to specific treatments. This review provides a category-based approach to the diagnosis and treatment of IEM organized by metabolic pathway and organelle. Categories include disorders of mitochondrial fatty acid oxidation and carnitine metabolism, urea cycle disorders, organic acidemias, aminoacidopathies, lysosomal disorders, peroxisomal disorders, vitamin- and diet-responsive metabolic epilepsies, and neurotransmitter disorders. Multiple summary tables for quick reference are provided. Figures show mitochondrial β-oxidation and carnitine cycle; urea cycle; T2-weighted magnetic resonance images (MRI) of ornithine transcarbamylase deficiency presenting with hyperammonemic encephalopathy, propionic academia, methylmalonic academia, glutaric acidemia type 1, ethylmalonic encephalopathy, mitochondrial complex 1 deficiency, pyruvate dehydrogenase complex E3 deficiency, untreated biotin-thiamine-responsive basal ganglia disease, homocysteinemia and low plasma methionine (suspected remethylation defect), attenuated Krabbe disease, Hunter syndrome, and GM1-gangliosidosis; branched-chain amino acid catabolic pathway; lysine, hydroxylysine, and tryptophan catabolic pathway; intracellular cobalamin metabolism; metabolism of homocysteine; diffusion-weighted imaging of maple syrup urine disease, nonketotic hyperglycinemia, and poorly controlled phenylketonuria; sphingolipid metabolic pathway; skeletal surveys of Hurler syndrome demonstrating features of dysostosis multiplex and rhizomelic chondrodysplasia punctata type 1; MRI of Salla disease; peroxisomal oxidation reactions, and the biogenic amine biosynthetic pathway. Tables list fatty acid oxidation and carnitine disorders, metabolic myopathies presenting with recurrent rhabdomyolysis, urea cycle disorders, organic acidemias, IEM associated with abnormal head size, cobalamin disorders, aminoacidopathies, IEM associated with abnormal odor, lysosomal disorders, lysosomal disease Symptom categories (Not mutually exclusive), peroxisomal disorders, IEM associated with brain malformations, vitamin- and diet-responsive epilepsies, neurotransmitter disorders, and IEM associated with brain mineralization.

      This review contains 26 highly rendered figures, 15 tables, and 71 references.

      Key words: Inborn errors of metabolism, organic acidemia, cobalamin disorders, aminoacidopathy, lysosomal disorders

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    • 2

      Time Is on Our Side: Age as a Diagnostic and Pathologic Clue in Pediatric Movement Disorders

      By Marisela E. Dy, MD; Bhooma R. Aravamuthan, MD, DPhil; Jeff L. Waugh, MD, PhD
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      Time Is on Our Side: Age as a Diagnostic and Pathologic Clue in Pediatric Movement Disorders

      • MARISELA E. DY, MDMovement Disorders Fellow, Department of Neurology, Boston Children’s Hospital, Massachusetts General Hospital, Harvard Medical School, Boston, MA
      • BHOOMA R. ARAVAMUTHAN, MD, DPHILChild Neurology Resident, Department of Neurology, Boston Children’s Hospital, Harvard Medical School, Boston, MA
      • JEFF L. WAUGH, MD, PHDMedical Director, Pediatric Movement Disorders and Deep Brain Stimulation Program, Department of Neurology, Boston Children’s Hospital Massachusetts General Hospital Harvard Medical School Boston, MA

      Pediatric movement disorders encompass a heterogeneous array of neurologic symptoms and syndromes and may be acquired or inherited. Movement phenomenology and age at symptom onset provide important clues in identifying the underlying etiology and establishing a diagnosis. Movement disorders are classified as hyperkinetic (increased movement) or hypokinetic (reduced amplitude and/or rate of movement). In children, hyperkinetic movement disorders are substantially more common than hypokinetic disorders; in adults, a mix of hyper- and hypokinetic disorders is the norm. We present a framework for evaluating a child with abnormal movements and describe a broad range of pediatric movement disorders: chorea, neurotransmitter-associated syndromes, tic disorders, stereotypies, primary dystonias, and dystonic cerebral palsy. Recently described genes associated with pediatric movement disorders, such as ADCY5-related dyskinesia, NKX2.1-associated chorea and dystonia, and the primary dystonias (DYT2 and DYT26), are highlighted. The developmental regulation of neurotransmitter metabolism, a dynamic process that leads to dramatic shifts in neurotransmitter availability in childhood and adolescence, is also reviewed. The myelination and maturation of long-range neuronal networks are described, and the timing of these changes is related to the timing of childhood movement disorders. Incorporating age and timing into a framework for assessing abnormal movements in children has the potential to improve both clinical and research approaches to these disorders. Understanding the genetic and pathophysiologic bases of abnormal movements may aid clinicians in rapidly recognizing and distinguishing these disorders. Recognizing the clinical specificity and age dependency of abnormal movements in children may guide researchers in identifying the molecular underpinnings of these disorders. 

      Key words: Movement disorders, gene expression, childhood development, childhood hyperkinetic disorders

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    • 3

      Acquired Demyelinating Syndromes in Children

      By Laura Adang, MD, PhD; Brenda Banwell, MD
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      Acquired Demyelinating Syndromes in Children

      • LAURA ADANG, MD, PHDAssistant Professor of Neurology and Pediatrics, The Children’s Hospital of Philadelphia
      • BRENDA BANWELL, MDChief of Child Neurology, Professor of Neurology and Pediatrics, The Children’s Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania

      Collectively, pediatric acquired demyelinating syndromes encompass a diverse clinical spectrum and include disorders such as multiple sclerosis (MS), acute disseminated encephalomyelitis, acute transverse myelitis, optic neuritis, and neuromyelitis optica spectrum disorders (NMOSDs). Acquired demyelination may occur as a monophasic illness or represent chronic demyelinating disease (such as MS or NMOSD) and thus prompt identification and classification is essential for proper treatment and guidance. As such, approximately 3% of all patients affected by MS experience their first attack during childhood. In general, one quarter of children diagnosed with an acute demyelinating syndrome will be ultimately confirmed to have MS. The likelihood that an incident demyelinating attack represents the first attack of MS is increased in adolescents, females, patients with positive cerebrospinal fluid oligoclonal bands, and those with clinically silent T2 bright and T1 hypointense lesions on brain magnetic resonance imaging at onset. Early therapeutic intervention for MS and NMOSD is recommended to reduce relapses, modify relapse severity, and, it is hoped, limit future disability and reduce demyelination-related central nervous system injury. Of particular clinical relevance in children, there are many mimics of demyelinating disease that must be considered.

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    • 4

      Pediatric Headache Disorders

      By Marc T DiSabella, DO; Raquel Langdon, MD
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      Pediatric Headache Disorders

      • MARC T DISABELLA, DOAssistant professor of child neurology at The George Washington School of Medicine and Health Sciences, and director of pediatric headache at the Center for Neurosciences and Behavioral Health, Children’s National Medical Center, Washington, DC
      • RAQUEL LANGDON, MDInstructor of child neurology at The George Washington School of Medicine and Health Sciences and co-director of Pediatric Headache and Concussion at the Center for Neurosciences and Behavioral Health, Children’s National Medical Center, Washington, DC

      Headache is the most common neurologic disorder in the population, and most children will experience headaches recurrently throughout childhood and adolescence. Current estimates suggest that one in four children and adolescents experience migraine, one of the most severe forms of headache. It is imperative to differentiate primary headache disorders, including migraine and tension-type headache, from secondary headaches, including posttraumatic and medication overuse headache, to successfully diagnose and manage symptoms. Headache results in significant disability in children, including significant social stigma, school absenteeism, and avoidance of normal activities (eg, athletic and social activities). Headache can be successfully managed by providing patients and their families with a variety of techniques, including healthy lifestyle habits, cognitive-behavioral therapy, selected medications in the appropriate setting, and possibly complementary therapies, which may include acupuncture, physical therapy, and nutraceuticals.

      Key words: headache, migraine, concussion, lifestyle modification

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  • Integrative Reviews
    • 1

      Clinical Trial Design and Statistics

      By Julie Ann Sosa, MA, MD, FACS; April K.S. Salama, MD; Samantha M. Thomas, MS
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      Clinical Trial Design and Statistics

      • JULIE ANN SOSA, MA, MD, FACSAssociate Professor of Surgery, Divisions of Endocrine Surgery and Surgical Oncology, Department of Surgery, Yale University School of Medicine, New Haven, CT
      • APRIL K.S. SALAMA, MDAssistant Professor of Medicine, Division of Medical Oncology, Duke University School of Medicine, Durham, NC
      • SAMANTHA M. THOMAS, MSBiostatistician, Department of Biostatistics & Bioinformatics, Duke Cancer Institute, Durham, NC

      A clinical trial is a planned experiment designed to prospectively measure the efficacy or effectiveness of an intervention by comparing outcomes in a group of subjects treated with the test intervention with those observed in one or more comparable group(s) of subjects receiving another intervention.  Historically, the gold standard for a clinical trial has been a prospective, randomized, double-blind study, but it is sometimes impractical or unethical to conduct such in clinical medicine and surgery. Conventional outcomes have traditionally been clinical end points; with the rise of new technologies, however, they are increasingly being supplemented and/or replaced by surrogate end points, such as serum biomarkers. Because patients are involved, safety considerations and ethical principles must be incorporated into all phases of clinical trial design, conduct, data analysis, and presentation. This review covers the history of clinical trials, clinical trial phases, ethical issues, implementing the study, basic biostatistics for data analysis, and other resources. Figures show drug development and clinical trial process, and type I and II error. Tables list Food and Drug Administration new drug application types, and types of missing data in clinical trials.

      This review contains 2 highly rendered figures, 2 tables, and 38 references

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    • SAM Allergy & Immunology
      • 1

        Drug Allergies

        By James L Baldwin, MD; Aimee L. Speck, MD
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        Drug Allergies

        • JAMES L BALDWIN, MDDivision Chief, Division of Allergy and Clinical Immunology, Department of Medicine, University of Michigan School of Medicine, Ann Arbor, MI
        • AIMEE L. SPECK, MDFellow, Division of Allergy and Clinical Immunology, University of Michigan School of Medicine, Ann Arbor, MI

        Adverse drug reactions (ADRs) are an important public health problem. An ADR is defined by the World Health Organization as an unintended, noxious response to a drug that occurs at a dose usually tolerated by normal subjects. The classification of ADRs by Rawlins and Thompson divides ADRs into two major subtypes: (1) type A reactions, which are dose dependent and predictable, and (2) type B reactions, which are uncommon and unpredictable. The majority of ADRs are type A reactions, which include four subtypes: overdosage or toxicity, side effects, secondary effects, and interactions. Type B reactions constitute approximately 10 to 15% of all ADRs and include four subtypes: drug intolerance, idiosyncratic reactions, pseudoallergic reactions, and drug hypersensitivity reactions. This chapter reviews the epidemiology of ADRs, risk factors for drug hypersensitivity reactions, the classification of drug reactions, diagnostic tests, reactions to specific drugs, and management of the patient with drug allergy. Figures illustrate drugs as haptens and prohaptens, the Gell and Coombs system, the four basic immunologic mechanisms for drug reactions, the chemical structure of different β-lactam antibiotics, penicillin skin testing, sulfonamide metabolism and haptenation, nonsteroidal antiinflammatory drug effects, and patient management. Tables outline the classification of ADRs, drugs frequently implicated in allergic drug reactions, and reagents and concentrations recommended for prick and intradermal skin testing.


        This chapter contains 8 highly rendered figures, 3 tables, 83 references, 5 MCQs, and 1 teaching slide set.

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      • 2

        Introduction to Clinical Immunology: Overview of the Immune Response, Autoimmune Conditions, and Immunosuppressive Therapeutics for Rheumatic Diseases

        By Steven K. Lundy, PhD; Alison Gizinski, MD; David A. Fox, MD
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        Introduction to Clinical Immunology: Overview of the Immune Response, Autoimmune Conditions, and Immunosuppressive Therapeutics for Rheumatic Diseases

        • STEVEN K. LUNDY, PHDResearch Assistant Professor, Division of Rheumatology, Department of Internal Medicine, University of Michigan Medical School, Ann Arbor, MI
        • ALISON GIZINSKI, MDAssistant Professor, Division of Rheumatology, Department of Internal Medicine, University of Arkansas for Medical Sciences, Little Rock, AR
        • DAVID A. FOX, MDProfessor, Division of Rheumatology, Department of Internal Medicine, University of Michigan Medical School and Health System, Ann Arbor, MI

        The immune system is a complex network of cells and mediators that must balance the task of protecting the host from invasive threats. From a clinical perspective, many diseases and conditions have an obvious link to improper functioning of the immune system, and insufficient immune responses can lead to uncontrolled acute and chronic infections. The immune system may also be important in tumor surveillance and control, cardiovascular disease, health complications related to obesity, neuromuscular diseases, depression, and dementia. Thus, a working knowledge of the role of immunity in disease processes is becoming increasingly important in almost all aspects of clinical practice. This review provides an overview of the immune response and discusses immune cell populations and major branches of immunity, compartmentalization and specialized immune niches, antigen recognition in innate and adaptive immunity, immune tolerance toward self antigens, inflammation and innate immune responses, adaptive immune responses and helper T (Th) cell subsets, components of the immune response that are important targets of treatment in autoimmune diseases, mechanisms of action of biologics used to treat autoimmune diseases and their approved uses, and mechanisms of other drugs commonly used in the treatment of autoimmune diseases. Figures show the development of erythrocytes, platelets, lymphocytes, and other immune system cells originating from hematopoietic stem cells that first reside in the fetal liver and later migrate to the bone marrow, antigen–major histocompatibility complex recognition by T cell receptor control of T cell survival and activation, and Th cells as central determinants of the adaptive immune response toward different stimuli. Tables list cell populations involved in innate and adaptive immunity, pattern recognition receptors with known ligands, autoantibody-mediated human diseases: examples of pathogenic mechanisms, selected Food and Drug Administration–approved autoimmune disease indications for biologics, and mechanism of action of biologics used to treat autoimmune diseases.

         

        This review contains 3 highly rendered figures, 5 tables, and 64 references.

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    • SAM Cardiovascular Medicine
      • 1

        Atrial Fibrillation

        By Gregory F. Michaud, MD; Roy M. John, MD, PhD
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        Atrial Fibrillation

        • GREGORY F. MICHAUD, MDDirector, Center for the Advanced Management of Atrial Fibrillation, Brigham and Women’s, Hospital, Assistant Professor of Medicine, Harvard Medical School, Boston, MD
        • ROY M. JOHN, MD, PHDAssociate Director EP Laboratory, Director, Experimental Research, Cardiac Arrhythmia Service, Brigham and Women’s Hospital, Assistant Professor of Medicine, Harvard Medical School, Boston, MA

        Atrial fibrillation (AF) is an abnormal rhythm characterized by chaotic atrial electrical activity resulting in loss of atrial contraction, an irregular and unpredictable heart rate, and a tendency for thrombus formation. The prevalence of AF is estimated at 1 to 2%, but it’s likely higher than that because one-third of patients may have no symptoms and might never seek medical attention. Data suggest that 1 in 4 people over the age of 40 will develop AF in their lifetime. About 10% of patients over age 80 have experienced the arrhythmia, and some estimates predict the prevalence will double in the next 50 years. This chapter discusses the pathophysiology, genetics, diagnosis, classification, and treatment of AF. Figures show atrial fibrillation and coarse atrial fibrillation plus common right atrial flutter. One algorithm is for oral anticoagulation therapy, and a second shows a recommended hierarchical choice of antiarrhythmic therapies versus catheter ablation for recurrent symptomatic atrial fibrillation. Tables list classification, diagnostic evaluation of, clinical consequences of, and conditions often associated with atrial fibrillation. Three scoring systems are included: 1) for congestive heart failure, hypertension, diabetes, stroke, and transient ischemic attack; 2) to assess the risk of bleeding with oral anticoagulation, and 3) data and proportion of patients from the Euro Heart Survey. Other tables include long-term anticoagulation guidelines for atrial fibrillation, intravenous drugs used for acute rate control, oral drugs used for chronic rate control, and antiarrhythmic drugs for conversion of atrial fibrillation and/or maintenance of sinus rhythm. In addition, there’s a summary of randomized trials weighing rate control and rhythm control strategies, plus schemes for categorizing thromboembolism risk. This chapter contains 129 references

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      • 2

        Venous Thromboembolism

        By Samuel Z. Goldhaber, MD
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        Venous Thromboembolism

        • SAMUEL Z. GOLDHABER, MDProfessor of Medicine, Harvard Medical School Director, Venous Thromboembolism Research Group

        Venous thromboembolism, which involves venous thrombosis and pulmonary embolism, is a leading cause of morbidity and mortality in hospitalized patients and is being seen with increasing frequency in outpatients. This chapter discusses the risk factors, etiology, classification, pathophysiology, natural history, prognosis, diagnosis (including venous thrombosis, recurrent venous thrombosis, and pulmonary embolism), prophylaxis, and treatment of venous thromboembolism (including the pharmacology of antithrombotic agents), as well as venous thromboembolism in pregnancy and miscellaneous thromboembolic disorders (including thrombosis of unusual sites). Tables in the chapter present a model for determining clinical suspicion of deep vein thrombosis, test results that effectively confirm or exclude deep vein thrombosis, a model for determining a clinical suspicion of pulmonary embolism, test results that effectively confirm or exclude pulmonary embolism, drug and food interactions with warfarin by level of supporting evidence and direction of interaction, risk categories for venous thromboembolism, and recommendations for prophylaxis. Figures include a venogram illustrating thrombi in the left iliac vein, perfusion scans showing multiple perfusion defects, a computed tomographic pulmonary angiogram demonstrating intraluminal filling defects, a diagnostic approach for suspected pulmonary embolism, and an algorithm for selecting duration of anticoagulation after venous thromboembolism. There are 105 references in this chapter.

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    • SAM Dermatology
      • 1

        Cutaneous Manifestations of Systemic Diseases

        By Mark Lebwohl, MD
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        Cutaneous Manifestations of Systemic Diseases

        • MARK LEBWOHL, MDSol and Clara Kest Professor and Chairman, Department of Dermatology, Mount Sinai School of Medicine, New York City, NY

        This chapter reviews key cutaneous manifestations of systemic diseases that should be recognized by most physicians and highlights recent developments in the diagnosis and management of those disorders. In many of the disorders presented, workup and therapy of the underlying systemic condition are essential to a favorable outcome. Cardiopulmonary, vascular, endocrinologic, gastrointestinal, hematologic, immunodeficiency, infectious, neurologic, renal, and rheumatologic diseases are discussed. Many of the syndromes characterized by physical symptoms are accompanied by photographs demonstrating the manifestations for the reader's reference.

        This chapter contains 28 figures, 104 references, and 5 Board-styled MCQs.

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    • SAM Endocrinology & Metabolism
      • 1

        Diagnosis and Treatment of Dyslipidemia

        By John D. Brunzell, MD, FACP; R Alan Failor, MD
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        Diagnosis and Treatment of Dyslipidemia

        • JOHN D. BRUNZELL, MD, FACPProfessor Emeritus, Active, Department of Medicine, Division of Metabolism, Endocrinology and Nutrition, University of Washington, Seattle, WA 98195
        • R ALAN FAILOR, MDClinical Professor, Department of Medicine, Division of Metabolism, Endocrinology and Nutrition, University of Washington, Seattle, WA 98195

        Disorders of lipoprotein metabolism in conjunction with the prevalence of high-fat diets, obesity, and physical inactivity have resulted in an epidemic of atherosclerotic disease in the United States and other developed countries. The interaction of genetic disorders with these adverse environmental factors leads to the premature development of atherosclerosis. This chapter discusses lipoprotein composition and metabolism and the genetic and environmental defects that can lead to dyslipidemias. Primary disorders of dyslipidemia discussed include the metabolic syndrome, familial combined hyperlipidemia, familial hypertriglyceridemia, familial hypercholesterolemia (both heterozygous and homozygous), familial defective apolipoprotein B-100, a disorder causing increased levels of lipoprotein(a), and remnant removal disease. Secondary disorders discussed include those caused by endocrine disorders, renal disorders, and gastrointestinal disorders. The clinical manifestations of hyperlipidemia and how they correspond to specific lipid disorders are presented, including abnormal levels of lipoproteins, cutaneous manifestations (e.g., xanthomas), signs and symptoms that constitute the chylomicronemia syndrome, and nonalcoholic fatty liver disease. Management principles, including diet and exercise modification and drug therapy, are discussed for patients with elevated cholesterol levels, elevated triglyceride levels, low levels of high-density lipoprotein cholesterol, and atherosclerosis accompanied by normal lipid levels. Primary and secondary prevention of coronary artery disease using statins, bile-acid sequestrants, fibrates, and niacin are covered. Management recommendations for patients of varied age groups are reviewed, including screening for hypercholesterolemia in children, the management of dyslipidemia in women, and the management of dyslipidemia in older patients. The eight tables present the major apolipoproteins and their functions, the Adult Treatment Panel III (ATP-III) classifications of the various lipid levels, the effects of selected drugs on lipoprotein levels, the clinical features of the metabolic syndrome, the ATP-III low-density lipoprotein (LDL) cholesterol goals and cutpoints for therapeutic lifestyle changes and drug therapy in different risk categories, ATP-III major risk factors that modify LDL cholesterol goals, drug treatment for lipid disorders, and doses of statins required to achieve a 30% to 40% reduction of LDL cholesterol levels. The six figures illustrate aspects of lipid metabolism. This chapter contains 108 references.

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      • 2

        Complications of Diabetes Mellitus

        By Samuel Dagogo-Jack, MD, MBBS, FRCP, FACP
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        Complications of Diabetes Mellitus

        • SAMUEL DAGOGO-JACK, MD, MBBS, FRCP, FACPA. C. Mullins Professor & Chief, Division of Endocrinology, Diabetes and Metabolism, University of Tennessee Health Science Center, Memphis, TN

        The long-term complications of diabetes mellitus include retinopathy, nephropathy, and neuropathy. Diabetic retinopathy can result in loss of vision; nephropathy may lead to end-stage kidney disease (ESKD); and neuropathy poses the risk of foot ulcers, amputation, Charcot joints, sexual dysfunction, and potentially disabling dysfunction of the stomach, bowel, and bladder. Hyperglycemia sufficient to cause pathologic and functional changes in target tissues may be present for some time before clinical symptoms lead to a diagnosis of diabetes, especially in patients with type 2 diabetes. Diabetic patients are also at increased risk for atherosclerotic cardiovascular, peripheral vascular, and cerebrovascular disease. These conditions may be related to hyperglycemia, as well as to the hypertension and abnormal lipoprotein profiles that are often found in diabetic patients. Prevention of these complications is a major goal of current therapeutic policy and recommendations for all but transient forms of diabetes. This chapter describes the pathogenesis, screening, prevention, and treatment of diabetic complications, as well as the management of hyperglycemia in the hospitalized patient. Figures illustrate the pathways that link high blood glucose levels to microvascular and macrovascular complications; fundus abnormalities in diabetic retinopathy; the natural history of nephropathy in type 1 diabetes; cumulative incidence of first cardiovascular events, stroke, or death from cardiovascular disease in patients with type 1 diabetes; the effect of intensive glycemic therapy on the risk of myocardial infarction, major cardiovascular event, or cardiovascular death in patients with type 2 diabetes; and risk of death in patients with type 2 diabetes who receive intensive therapy of multiple risk factors or conventional therapy. Tables describe screening schedules for diabetic complications in adults, foot care recommendations for patients with diabetes, and comparison of major trials of intensive glucose control. This chapter has 238 references.

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      • 3

        The Porphyrias

        By Karl E Anderson, MD, FACP; Attallah Kappas, MD
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        The Porphyrias

        • KARL E ANDERSON, MD, FACPProfessor, Departments of Preventive Medicine and Community Health, Internal Medicine, and Pharmacology and Toxicology, University of Texas Medical Branch, Galveston, TX
        • ATTALLAH KAPPAS, MDSherman Fairchild Professor, Physician-in-Chief, Emeritus, The Rockefeller University, Attending Physician, Rockefeller University Hospital, New York, NY

        The porphyrias are uncommon disorders caused by deficiencies in the activities of the enzymes of the heme biosynthetic pathway. The enzymatic defects that cause porphyrias may be either inherited or acquired, and there is significant interplay between the genetic defect and acquired or environmental factors. Acute forms of the porphyrias may be life threatening and may be misdiagnosed because of the nonspecific nature of the clinical presentation (e.g., acute abdominal pain, psychiatric disturbances, and polyneuropathies). The course of the acute forms of disease is characterized by long latent periods interrupted by acute attacks, which are associated with substantial morbidity and mortality. Porphyrias may be classified as neurovisceral or photosensitive, depending on their prominent clinical characteristic, but in some cases of porphyria, both symptoms are present. Alternatively, the porphyrias can be classified as hepatic or erythropoietic, depending on the principal site of expression of the specific enzymatic defect involved, but the expressions overlap in some porphyrias. The neurovisceral porphyrias correspond with the hepatic porphyrias (ie, acute intermittent porphyria [AIP], variegate porphyria [VP], hereditary coproporphyria [HCP], and ALA deficiency porphyria [ADP]); and the photosensitive porphyrias correspond with the erythropoietic porphyrias (i.e., porphyria cutanea tarda [PCT], hepatoerythropoietic porphyria [HEP], erythropoietic protoporphyria [EPP], and congenital erythropoietic porphyria [CEP]). This chapter covers the classification and pathophysiology of porphyrias and the epidemiology, molecular defects and pathophysiology, diagnosis, and treatment of each of the clinical presentations of porphyria. A table lists the drugs that are safe and unsafe for patients with AIP, VP, HCP, and ADP. Figures illustrate the classification and major symptoms of the porphyrias and the corresponding defect in the biosynthesis of heme that causes the disease; the steps in the biosynthesis of heme; and the mechanisms that precipitate symptoms in AIP. This chapter contains 99 references.

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      • 4

        The Pituitary

        By Shlomo Melmed, MD, FACP
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        The Pituitary

        • SHLOMO MELMED, MD, FACPSenior Vice President, Academic Affairs, Dean of Medical Faculty, Cedars Sinai Medical Center, 8700 Beverly Blvd, Los Angeles, CA 90048

        The pituitary gland plays a prominent role in regulating the critical hormonal functions of growth, development, reproduction, stress homeostasis, and metabolic control; for this reason, the pituitary has been termed the master gland. The anterior pituitary synthesizes and secretes adrenocorticotropic hormone (ACTH), growth hormone (GH), prolactin (PRL), thyroid-stimulating hormone (TSH), follicle-stimulating hormone (FSH), and luteinizing hormone (LH). The posterior pituitary secretes vasopressin (also known as antidiuretic hormone [ADH]) and oxytocin, which are synthesized in the hypothalamus. This chapter describes genetic syndromes associated with pituitary tumors, including multiple endocrine neoplasia type 1. Information is provided on the diagnosis and treatment of disorders associated with the underproduction and hypersecretion of pituitary hormones, including Cushing syndrome, pituitary mass effects, and pituitary failure. Figures illustrate pituitary anatomy and physiology and management of prolactinoma. Tables describe hypothalamic and related pituitary hormones; pituitary hormones, their mediators, and their effects; effects of pituitary adenomas; causes of hyperprolactinemia; dopamine agonists in the treatment of prolactinomas; findings in adult GH deficiency; causes and features of acromegaly; clinical features of Cushing syndrome; causes of pituitary failure; and replacement therapy for hypopituitarism in adults.

        This review contains 4 references, 11 tables, and 43 references.

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    • SAM Ethics and Professionalism
      • 1

        Principles and Practice of Palliative Care

        By Kristen G Schaefer, MD; Rachelle E Bernacki, MD, MS
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        Principles and Practice of Palliative Care

        • KRISTEN G SCHAEFER, MDDirector of Medical Student and Resident Education, Division of Adult Palliative Care, Department of Psychosocial Oncology and Palliative Care, Dana-Farber Cancer Institute, Center for Palliative Care, Harvard Medical School, Division of General Internal Medicine, Department of Medicine, Brigham and Women’s Hospital, Boston, MA
        • RACHELLE E BERNACKI, MD, MSDirector of Quality Initiatives, Division of Adult Palliative Care, Department of Psychosocial Oncology and Palliative Care, Dana-Farber Cancer Institute, Center for Palliative Care, Harvard Medical School, Division of Aging, Department of Medicine, Brigham and Women’s Hospital, Boston, MA

        The trajectories of serious illness and dying have changed in the last century; in the past, patients lived shorter lives and often died quickly of infectious disease, whereas patients in the 21st century live longer and often with prolonged debility in the advanced stages of illness. As a result, patients with serious illness can suffer undertreated symptoms and often feel poorly prepared for the final stages of disease. With more options for advanced life support and other aggressive interventions at the end of life, patients and families face increasingly complex medical decisions in the terminal phase of illness, and the treatments they receive do not always align with their goals and values. Emerging evidence suggests that integrating palliative care into the treatment of advanced illness can improve outcomes, decrease costs, and improve both patient and family satisfaction. Consequently, patient access to high-quality specialty-level palliative care is becoming standard of care at most academic cancer centers and more available in the community. This chapter describes the practice and principles of specialty-level palliative care and outlines specific "generalist" palliative care competencies essential for all physicians caring for patients with serious illness, including prognostication, patient-centered communication, and the navigation of ethical dilemmas in the field of palliative care. Tables outline the philosophy of palliative care, domains of suffering, location of death of hospice patients, the palliative performance scale, median survival times for cancer syndromes, indicators associated with a poorer prognosis in congestive heart failure, the NURSE mnemonic for accepting and responding to emotion, palliative care communication competencies in the intensive care unit, and the SPIKES mnemonic for breaking bad news. Figures depict causes of death in 1900 versus 2010, palliative care through the trajectory of serious illness, theoretical trajectories of disease, life expectancies for women and men, mortality at 1 year post discharge, and a model for patient-centered communication. 


        This chapter contains 6 highly rendered figures, 9 tables, 169 references, 1 teaching slide set, and 5 MCQs.

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    • SAM Geriatric Medicine
      • 1

        Approach to the Geriatric Patient

        By Tia Kostas, MD; Mark Simone, MD; James L Rudolph, MD, SM
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        Approach to the Geriatric Patient

        • TIA KOSTAS, MDAssistant Professor of Medicine, Section of Geriatrics & Palliative Medicine, Department of Medicine, University of Chicago, Chicago, IL
        • MARK SIMONE, MDInstructor of Medicine, Harvard Medical School, Associate Program Director-Primary Care, Mount auburn Hospital Internal Medicine Residency, Director, Quality Improvement, Division of Geriatric Medicine, Department of Medicine, Mount Auburn Hospital, Cambridge, MA
        • JAMES L RUDOLPH, MD, SMAssociate Professor of Medicine, Harvard Medical School, Chief (Interim) Geriatrics and Palliative Care, Director, Boston, GRECC, VA Boston Healthcare System, Jamaica Plain, MA, Acting Clinical Chief, Associate Epidemiologist, Division of Aging, Department of Medicine, Brigham and Women’s Hospital, Boston, MA

        As of 2012, over one in eight Americans is over the age of 65, and this number is rising, particularly in the 85+ age group. This segment of the population has a rate of hospitalization three times higher than that for persons of all ages. General internists and family medicine physicians provide a large portion of care for this age group and should therefore be comfortable using a comprehensive approach to geriatric assessment. This review describes general considerations regarding geriatric care, including the process of taking a functional history and clinical implications of geriatric care. The geriatric assessment process is discussed in terms of physical, cognitive, social, and medical domains. The benefits of geriatric assessment in primary care, specialty care, and hospitalized patients are described. Tables outline activities of daily living, sensory changes with aging, major causes of visual impairment in the geriatric population, major neurocognitive disorder diagnostic criteria, medications to avoid or use with caution based on Beers criteria and Screening Tool of Older individuals’ Potentially inappropriate Prescriptions criteria, U.S. Preventive Services Task Force–recommended services relevant to older adults, and vaccinations in older adults. Figures illustrate the key vulnerabilities of older adults; outcomes linked to functional dependence; common disorders associated with cognitive concerns; domains of cognition and examples of impairment in the Diagnostic and Statistical Manual of Mental Disorders, fifth edition; the social and medical domains of geriatric assessment; barriers to medication adherence in older patients; and resources for medication appropriateness in older adults.

        This review contains 8 highly rendered figures, 8 tables, 110 references, and 5 MCQs.

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    • SAM Hematology
      • 1

        Thrombotic Disorders

        By Lawrence L K Leung, MD
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        Thrombotic Disorders

        • LAWRENCE L K LEUNG, MDChief of Staff, Maureen Lyles D’Ambrogio Professor of Medicine, Stanford University School of Medicine, Chief of Staff, Veterans Affairs Palo Alto Health Care System, Stanford, CA

        The three main elements in the pathophysiology of thrombosis are endothelial injury, a decrease in blood flow, and an imbalance between procoagulant and anticoagulant factors. The latter element can be either hereditary (e.g., antithrombin deficiency) or acquired (e.g., antiphospholipid syndrome). This review details the assessment of patients with thrombotic disorders, hereditary and acquired hypercoagulable states, and the management of venous thromboembolism. Figures show how the degradation of thrombin-activated factor V Leiden by activated protein C (APC) is significantly slower than that of normal activated factor V (factor Va), leading to enhanced thrombin generation; how normal factor V serves as a cofactor of APC in the inhibition of factor VIIIa, whereas factor V Leiden has a poor cofactor function; and how IgG antibodies recognize platelet factor 4–heparin complexes in heparin-induced thrombocytopenia. Tables list inherited and acquired hypercoagulable states, questions for assessing thrombosis, screening tests for patients with suspected hypercoagulable states, clinical features that suggest thrombophilia, frequency and relative risk of venous thrombosis in selected hypercoagulable states, proposed clinical and laboratory criteria for antiphospholipid syndrome, the classification of antiphospholipid antibodies, the 4Ts scoring system for heparin-induced thrombocytopenia, and general guidelines for the management of patients with venous thromboembolism.

        This review contains 2 highly rendered figures, 9 tables, and 168 references.

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      • 2

        Hemophilia

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        Hemophilia

        Hemophilia is a family of rare bleeding disorders characterized by deficiency of clotting factors. Hemophilia A is an inherited deficiency of factor VIII, whereas hemophilia B (Christmas disease) represents a deficiency of factor IX. Both hemophilia A and B are X-linked diseases, with hemophilia A accounting for 80 to 85% of cases and hemophilia B 15 to 20%. Although hemophilia has historically referred to deficiencies of factors VIII and IX, it is important to recognize that similar bleeding disorders can occur with other missing clotting factors, although this is far more rare. This review covers the definition, history, epidemiology, biology/genetics, clinical manifestations, diagnosis, differential diagnosis, treatment, complications, measures of quality of care, and prognosis of hemophilia, as well as future directions. Figures show the clotting cascade, the genetic makeup of severe hemophilia A, an algorithm for diagnosing hemophilia, and hemophilic arthropathy in a patient’s knees. Tables list severity in hemophilia A and B, treatment of acute bleeding in hemophilia A and B, frequency of dosing in acute bleeding, and treatment of acute bleeding with inhibitors.

        This review contains 4 highly rendered figures, 4 tables, and 59 references.

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      • 3

        Von Willebrand Disease

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        Von Willebrand Disease

        Von Willebrand disease is the most common hereditary bleeding disorder. The disease is caused by inherited defects in the concentration, structure, or function of von Willebrand protein, a multimeric protein that mediates the initial adhesion of platelets at sites of vascular injury and binds and stabilizes blood clotting factor VIII in plasma. Defects in von Willebrand factor concentration, structure, or function that were not inherited can also occur; this is termed acquired von Willebrand syndrome. These defects occur as a consequence of other medical disorders (valvular heart disease, thrombocythemia, malignant neoplasms, and myeloproliferative and autoimmune diseases). This review examines the laboratory evaluation, clinical variants, and treatment of von Willebrand disease. Figures show the initial assessment of von Willebrand disease, and a treatment algorithm for von Willebrand disease. Tables list types of von Willebrand disease and medications used to treat von Willebrand disease.

        This review contains 2 highly rendered figures, 2 tables, and 10 references.

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    • SAM Human Genetics
      • 1

        Practice of Genetics in Clinical Medicine

        By Bruce R Korf, MD, PhD, FACMG; Carlos Gallego, MD
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        Practice of Genetics in Clinical Medicine

        • BRUCE R KORF, MD, PHD, FACMGProfessor and Chair, Department of Genetics, University of Alabama at Birmingham, Birmingham, AL
        • CARLOS GALLEGO, MDInternist Geneticist, Huntsville Hospital

        This review provides a general overview of the genetic approach in medical practice, discusses the principles of genetic testing, including interpretation of genetic tests and direct-to-consumer genomic testing, and looks at genetic counseling and approaches to treatment. The internist should become familiar with genetic disorders such as those associated with mutations in single genes or changes in chromosome number or structure. This is the traditional area of focus for medical geneticists and is likely to remain so. The internist should be familiar with basic principles of care for individuals with the more common of these conditions and needs to recognize clues that suggest the presence of these disorders, especially in family history. The section on genetics of common disorders focuses on pharmacogenetics, risk assessment, and prevention. Figures illustrate commonly used standard pedigree symbols and examples of autosomal recessive, autosomal dominant, X-linked recessive, and maternal inheritance. Tables offer different forms of genetic testing and types of gene mutations at genome and DNA levels.

        This review contains 2 figures, 2 tables, and 83 references.

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    • SAM Infectious Diseases
      • 1

        Lyme Disease and Other Spirochetal Zoonoses

        By David C Tompkins, MD; Benjamin J Luft, MD, FACP
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        Lyme Disease and Other Spirochetal Zoonoses

        • DAVID C TOMPKINS, MDVice Chairman, Department of Medicine, Head, Division of Infectious Diseases, Lutheran Medical Center, Brooklyn, NY
        • BENJAMIN J LUFT, MD, FACPEdmund D. Pellegrino Professor, Division of Infectious Diseases, Department of Medicine, SUNY at Stony Brook, Stony Brook, NY

        This review covers the epidemiology, pathology, clinical features, laboratory findings, and treatment for Lyme disease, leptospirosis, relapsing fever, and rat-bite fever. Lyme disease is a tick-borne illness caused by the spirochete Borrelia burgdorferi. Lyme disease is a progressive infectious disease with a wide array of clinical manifestations. In most persons, the initial sign of infection is the development of erythema migrans. In general, three stages of the illness can be distinguished: early localized disease, early disseminated disease, and persisting late disease. A photograph shows an erythema migrans lesion, and an algorithm for diagnosis of Lyme disease is provided. A table lists the antibiotic regimens used for the different stages and manifestations of Lyme disease. Leptospirosis is a worldwide zoonosis caused by spirochetes from the genus Leptospira; these spirochetes are shown in a photomicrograph. The disease is acquired by contact with infected animals or exposure to contaminated soil or freshwater and can cause illness ranging from asymptomatic infection to severe multisystem disease with a significant mortality. Relapsing fever is an acute louse-borne or tick-borne infection caused by spirochetes of the genus Borrelia and is characterized by recurrent febrile episodes separated by asymptomatic intervals. Rat-bite fever is infection caused by Streptobacillus moniliformis in the United States and Europe or by Spirillum minus in Asia. A table details the differing characteristics of rat-bite fever from these two organisms.

        This review contains 6 highly rendered figures, 2 tables, and 124 references.

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      • 2

        Syphilis and the Nonvenereal Treponematoses

        By Michael Augenbraun, MD, FACP
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        Syphilis and the Nonvenereal Treponematoses

        • MICHAEL AUGENBRAUN, MD, FACPAssociate Professor of Medicine, State University of New York Downstate Medical Center, New York, NY

        Syphilis is an infectious disease with complex acute and chronic manifestations that is transmitted primarily through sexual contact. The disease has been recognized for many centuries, although its origin remains unknown. This chapter’s discussion of the epidemiology of syphilis includes figures illustrating the rate of syphilis in the United States by state and county and the rate of syphilis in the United States from 1941 to 2009. The etiology, pathogenesis and disease course, diagnosis, differential diagnosis, treatment, and complications of syphilis are discussed. Special cases of syphilis—in pregnant women, in children, in HIV-infected patients, and congenital syphilis—are also considered. Illustrations include a dark-field microphotograph of treponemes and photographs of a syphilitic chancre, the classic aculopapular rash from spirochetemia, condylomata lata, and a gumma. Tables outline the clinical manifestations of syphilis in adults and the treatment of syphilis. The nonvenereal treponematoses—yaws, endemic syphilis, and pinta—are a group of infections distributed throughout tropical and semitropical areas of the world. They are primarily noted to cause a variety of skin and skeletal lesions. There is little biologic difference between the treponemes that cause these conditions. The epidemiology, pathogenesis, diagnosis, differential diagnosis, and treatment of the nonvenereal treponematoses are discussed.


        This chapter contains 7 highly rendered figures, 2 tables, 60 references, 1 teaching slide set, and 5 MCQs.

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      • 3

        Infective Endocarditis

        By Patrick T. O'Gara, MD, FACC, FAHA, FACP
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        Infective Endocarditis

        • PATRICK T. O'GARA, MD, FACC, FAHA, FACPDirector, Clinical Cardiology, Brigham and Women’s Hospital, Professor of Medicine, Harvard Medical School, Boston, MA

        Infective endocarditis is a microbial infection of a cardiac valve or the mural endocardium caused by bacteria or fungi. Forms of this infection include subacute bacterial endocarditis (SBE) and acute bacterial endocarditis (ABE). Etiology and epidemiology are discussed. There is a section on pathogenesis followed by specific clinical presentations, including endocarditis associated with parenteral drug abuse as well as prosthetic valve endocarditis (PVE). Diagnosis and cardiac complications of endocarditis, treatment, recommendations for prophylaxis, and prognosis are addressed. There are several figures showing manifestations and anatomic relations from the infection. Tables describe microorganisms that cause native valve endocarditis, the etiology of PVE, the Duke criteria for diagnosis of infective endocarditis, antimicrobial therapy for endocarditis in adults, guidelines to prevent endocarditis, and recommendations and regimens for endocarditis prophylaxis.

        This chapter contains 99 references.

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    • SAM Interdisciplinary Medicine
      • 1

        Cardiac Resuscitation

        By Charles N. Pozner, MD; Jennifer L Martindale, MD; Brian C. Geyer, MD, PhD
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        Cardiac Resuscitation

        • CHARLES N. POZNER, MDMedical Director, Neil and Elise Wallace STRATUS Center for Medical Simulation, Brigham and Women’s Hospital, Associate Professor of Medicine (Emergency Medicine), Harvard Medical School, Boston, MA
        • JENNIFER L MARTINDALE, MDHarvard Affiliated Emergency Medicine Residency Program, Brigham and Women’s Hospital, Boston, MA
        • BRIAN C. GEYER, MD, PHDHarvard Affiliated Emergency Medicine Residency Program, Department of Emergency Medicine, Brigham and Women’s Hospital, Boston, MA

        Over 1,000 people suffer sudden cardiac death every day in the United States. About half occur in the hospital, the remainder occurring in the home, outpatient medical environments, or public spaces. The ultimate goal of cardiac resuscitation is survival with full neurologic recovery. Interventions that have the greatest potential to provide this outcome are often those interventions that can be performed by trained laypersons. Delay in the initiation of compressions in a cardiac arrest is one of the leading predictors of mortality. This chapter deliberately opens with a discussion of one of the most vital, yet relatively under-appreciated aspects of cardiac resuscitation—teamwork. Following an overview of cardiac arrest, the chapter covers epidemiology and recognition of cardiac arrest, then activation of the emergency response system. Chest compressions, defibrillation and airway management are discussed, then end-tidal CO2, vascular access, rhythm analysis during cardiac arrest, and drugs used during cardiac resuscitation. The chapter concludes with a look at postresuscitation care, special considerations in cardiac arrest, and future directions. Figures include the foundations of cardiac resuscitation, a management algorithm for the initial treatment of cardiac arrest, coronary perfusion pressure as a function of time, an impedance threshold device incorporated into a bag-valve mask, a colorimetric CO2 detector, four basic phases of a capnogram, sample capnographs, a battery-powered intraosseous drill, electrocardiogram readouts of ventricular arrhythmias, a sonogram of large pericardial effusion, and electrocardiographic appearance of varying degrees of hyperkalemia. Tables summarize recommendations from the American Heart Association, the components of optimal and suboptimal chest compressions, and underlying causes of nonshockable rhythms and their management.

        This chapter contains 11 highly rendered figures, 3 tables, 138 references, 5 MCQs.

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      • 2

        Complementary, Alternative, and Integrative Medicine

        By Helene M. Langevin, MD
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        Complementary, Alternative, and Integrative Medicine

        • HELENE M. LANGEVIN, MDDirector, Osher Center for Integrative Medicine, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA

        Complementary and alternative medicine (CAM) refers to a group of diverse medical and health care systems, practices, and products that are not considered to be part of conventional or allopathic medicine. Common CAM practices (e.g., acupuncture, meditation, and therapeutic massage) are gradually becoming incorporated into conventional care in response to patients looking to alternative sources for information and advice about health matters and increased understanding of various CAM methods through evidence-based testing. However, although the claims of some methods are supported with academic research, well-founded concerns remain in many popularized CAM practices regarding the lack of evidence and placebo effects. It is thus imperative for physicians to be comfortable in discussing CAM-related topics with patients and be able to appropriately and informatively guide them in a way that harnesses potential benefits and avoids potential harm. In this review, the major CAM therapies in the United States are examined, including the settings in which they are being used, evidence base status, and efficacy of some of the most commonly used modalities. Figures show percentages of use for various CAM approaches. Tables show major CAM health practices, CAM therapies with significant increases between 2002 and 2007, Internet sources for evidence-based information and tools for patient education, sources for health care provider information, and resources to assess dietary supplement–drug interactions.

        This review contains 5 highly rendered figures, 5 tables, and 155 references.

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    • SAM Palliative Medicine
      • 1

        Management of Psychosocial Issues in Terminal Illness

        By Jane DeLima Thomas, MD; Eva Reitschuler-Cross, MD; Susan D Block, MD
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        Management of Psychosocial Issues in Terminal Illness

        • JANE DELIMA THOMAS, MDAttending Physician, Department of Psychosocial Oncology and Palliative Care, Dana-Farber Cancer Institute and Brigham and Women’s Hospital, Instructor in Medicine, Harvard Medical School, Boston, MA
        • EVA REITSCHULER-CROSS, MDClinical Assistant Professor of Medicine, University of Pittsburgh, Section of Palliative Care and Medical Ethics, University of Pittsburgh Medical Center, Pittsburgh, PA
        • SUSAN D BLOCK, MDChair, Department of Psychosocial Oncology and Palliative Care, Dana-Farber Cancer Institute and Brigham and Women’s Hospital Co-Director, HMS Center for Palliative Care, Professor of Psychiatry and Medicine, Harvard Medical School, Boston, MA

        Patients facing serious or life-threatening illness experience challenges to their psychological, social, and spiritual lives as well as to their physical function and comfort. Physicians may be accustomed to focusing on the biomedical aspects of illness, but they have a critical role in assessing the patient's psychosocial issues to identify sources of distress and help implement a plan for mitigating them. An appropriate psychosocial assessment requires a methodical and rigorous approach and includes assessment of any psychosocial issue affected by or affecting a patient's experience of illness. This chapter outlines a structured approach to addressing psychosocial issues by discussing (1) the doctor-patient relationship; (2) coping with illness; (3) family dynamics and caregiving; (4) ethnic and cultural issues; (5) religious, spiritual, and existential issues; (6) mental health issues, including adjustment disorder, depression, anxiety, personality disorders, aberrant drug behaviors, and major mental health issues; and (7) grief and bereavement. Tables outline psychosocial assessment questions, factors predisposing patients with serious illness to depression, risk factors for suicide in patients with terminal illness, and classes of antidepressants, anxiolytics, and sedatives. The Alcohol Use Disorders Identification Test-Consumption (AUDIT-C) questionnaire is provided, as well as a list of Web sites with further resources about psychosocial issues in serious illness.


        This chapter contains 1 highly rendered figure, 6 tables, 216 references, and 5 MCQs.

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    • SAM Psychiatry
      • 1

        Mood Disorders

        By Hasan A Baloch, MD; Jair C. Soares, MD
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        Mood Disorders

        • HASAN A BALOCH, MDAssistant Professor, Department of Psychiatry, Medical Director, Pediatric Bipolar Program, CERT-BD, UNC School of Medicine, Chapel Hill, NC
        • JAIR C. SOARES, MDDepartment of Psychiatry and Behavioral Sciences, University of Texas Health Science Center at Houston, McGovern Medical School, Houston, TX

        Affective disorders are among the most common disorders in psychiatry. They are generally classified according to the persistence and extent of symptoms and by the polarity of these symptoms. The two poles of the affective spectrum are mania and depression. Bipolar disorder is characterized by the presence of the mania or hypomania and often depression. Unipolar depression is defined by depression in the absence of a lifetime history of mania or hypomania. These differences are not merely categorical but have important implications for the prognosis and treatment of these conditions. Bipolar disorder, for example, is better treated using mood-stabilizing medication, whereas unipolar depression responds optimally to antidepressant medications. In addition, prognostically, unipolar depression may sometimes be limited to one episode in a lifetime, whereas bipolar disorder is typically a lifelong condition. The course of both conditions, however, is often chronic, and frequently patients can present with unipolar depression only to later develop manic symptoms. A thorough understanding of both conditions is therefore required to treat patients presenting with affective symptomatology. This chapter discusses the epidemiology, etiology and genetics, pathogenesis, diagnosis, and treatment of unipolar depression and bipolar disorder. Figures illustrate gray matter differences with lithium use and the bipolar spectrum. Tables list the pharmacokinetics of commonly used antidepressants and medications commonly used in the treatment of bipolar disorder.

        This review contains 2 figures, 2 tables, and 135 references.

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      • 2

        Management of Somatic Symptoms

        By Andreas Schröder, MD, PhD; Joel E Dimsdale, MD
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        Management of Somatic Symptoms

        • ANDREAS SCHRÖDER, MD, PHDConsultant in Psychiatry, The Research Clinic for Functional Disorders, Aarhus University Hospital, Aarhus, Denmark
        • JOEL E DIMSDALE, MDDistinguished Professor of Psychiatry Emeritus, Department of Psychiatry, University of California, San Diego, La Jolla, CA

        Somatic symptoms that cannot be attributed to organic disease account for 15 to 20% of primary care consultations and up to 50% in specialized settings. About 6% of the general population has chronic somatic symptoms that affect functioning and quality of life. This chapter focuses on the recognition and effective management of patients with excessive and disabling somatic symptoms. The clinical presentation of somatic symptoms is categorized into three groups of patients: those with multiple somatic symptoms, those with health anxiety, and those with conversion disorder. The chapter provides information to assist with making a diagnosis and differential diagnosis. Management includes ways to improve the physician–patient interaction that will benefit the patient, a step-care model based on illness severity and complexity, and psychological and pharmacologic treatment. The chapter is enhanced by figures and tables that summarize health anxiety, symptoms, differential diagnoses, and management strategies, as well as by case studies and examples.

        This review contains  5 highly rendered figures, 10 tables, and 235 references.

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      • 3

        Overview of Anxiety Disorders

        By Jon E Grant, JD, MD, MPH
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        Overview of Anxiety Disorders

        • JON E GRANT, JD, MD, MPH

        Anxiety disorders are the most common psychiatric disorders among adults in the United States. Although anxiety disorders generally result in significant psychosocial impairment, most adults do not seek treatment until many years after the onset of the anxiety disorder. The treatment literature for anxiety disorder has grown tremendously since the 1980s, and both psychotherapy and medications may prove beneficial for people with anxiety disorders. This review presents a general overview of the anxiety disorders.

        This review contains 7 tables, and 33 references.

        Key words: agoraphobia, anxiety disorder, generalized anxiety disorder, panic disorder, separation anxiety disorder, social anxiety disorder, specific phobia, treatment of anxiety

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      • 4

        Clinical Management of Anxiety Disorders

        By Shona Vas, PhD; Pooja N Dave, PhD
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        Clinical Management of Anxiety Disorders

        • SHONA VAS, PHDAssociate Professor, Department of Psychiatry & Behavioral Neuroscience, The University of Chicago
        • POOJA N DAVE, PHDPostdoctoral Fellow, Department of Psychiatry & Behavioral Neuroscience, The University of Chicago, Chicago, IL

        Anxiety disorders are characterized by excessive fear and anxiety accompanied by associated behavioral disturbances that cause significant impairment in social and occupational functioning. Anxiety is a complex mood state that involves physiologic, cognitive, and behavioral components. This review describes the five anxiety disorders most commonly diagnosed in adults: social anxiety disorder, panic disorder, agoraphobia, generalized anxiety disorder, and specific phobia. Diagnostic criteria for these disorders are presented along with empirically supported psychological and pharmacologic treatment approaches. Decades of evidence have indicated that for anxiety disorders of mild to moderate severity, cognitive-behavioral therapy (CBT) should be first-line treatment. CBT interventions for anxiety, including psychoeducation, cognitive restructuring, exposure, applied relaxation/breathing retraining, and skills training, are presented with descriptions of how they may be adapted to particular diagnoses, along with data for their efficacy. Data suggest that selective serotonin and norepinephrine reuptake inhibitors are pharmacologic treatments of choice for anxiety and may be used in combination with CBT for moderate to severe symptoms. d-Cycloserine is an emerging treatment that may enhance outcomes in anxiety disorders by optimizing exposure therapy through the facilitation of fear extinction.

        This review contains 7 figures, 12 tables, and 105 references.

        Key words: agoraphobia, anxiety, generalized anxiety disorder, panic disorder, phobias, social anxiety disorder

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    • SAM Pulmonary & Critical Care Medicine
      • 1

        Chest Wall and Neuromuscular Disease

        By Başak Çoruh, MD; Joshua O Benditt, MD
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        Chest Wall and Neuromuscular Disease

        • BAŞAK ÇORUH, MDSenior Fellow, Division of Pulmonary and Critical Care Medicine, University of Washington, Seattle, WA
        • JOSHUA O BENDITT, MDProfessor, Division of Pulmonary and Critical Care Medicine, University of Washington, Seattle, WA

        Chest wall and neuromuscular diseases encompass a broad spectrum of illnesses that affect the mechanics of breathing. This chapter reviews the physiology of the respiratory system and the impact of these diseases. A brief discussion of various chest wall and neuromuscular diseases is included. The approach to the evaluation of a patient with suspected chest wall or neuromuscular disease, including key aspects of the history, physical examination, and diagnostic testing, is discussed. Respiratory care, including ventilation, cough, swallowing, and sleep, is described. Tables outline neuromuscular diseases affecting the respiratory system, causes of diaphragm weakness and paralysis, and a comprehensive approach to respiratory care in chest wall and neuromuscular disease. Figures include illustrations of the anatomy of the respiratory system and contraction of the diaphragm, pressure measures above and below the diaphragm, radiographic images of chest wall diseases and measurement of the Cobb angle, a computed tomographic scan of a patient with pectus excavatum, a graph showing patterns of pulmonary function testing in chest wall and neuromuscular disease, and photos of mouthpiece ventilation and a mechanical insufflator-exsufflator device. Videos show a fluoroscopic sniff test demonstrating unilateral diaphragm paralysis and the use of a mechanical insufflator-exsufflator device.

        This review contains 9 highly rendered figures, 2 videos, 3 tables, and 40 references.

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    • SAM Rheumatology
      • 1

        Idiopathic Inflammatory Myopathies

        By Frederick W Miller, MD, PhD
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        Idiopathic Inflammatory Myopathies

        • FREDERICK W MILLER, MD, PHDChief, Environmental Autoimmunity Group, Office of Clinical Research, National Institute of Environmental Health Sciences, National Institutes of Health, Bethesda, MD

        The idiopathic inflammatory myopathies (IIMs), also known as myositis syndromes, are a collection of heterogeneous disorders that share the common feature of chronic muscle inflammation of unknown cause. These disorders may occur in adults or children and are sometimes associated with other connective tissue disorders and a variety of cancers. A combined clinical, laboratory, and pathologic evaluation is needed to establish the diagnosis of these acquired systemic connective tissue diseases to rule out the many disorders that mimic IIMs. This module reviews the classification of IIMs, including polymyositis, dermatomyositis, inclusion body myositis, myositis associated with other connective tissue diseases and cancer, and antisynthetase syndrome. The epidemiology; etiology, genetics, and environmental factors; pathophysiology and pathogenesis; diagnosis; differential diagnosis; treatment; and prognosis of IIMs are discussed. Tables describe the criteria for polymyositis, dermatomyositis, and inclusion body myositis; well-characterized subgroups of the IIMs in adults and children; presentation of polymyositis; differential diagnosis of muscle weakness or pain; features that assist in discriminating IIMs from other myopathies; goals for managing IIMs; and key factors for achieving adequate corticosteroid response in IIMs. Figures demonstrate skin findings in IIMs, muscle pathology of IIMs, magnetic resonance imaging of three patients with different IIMs, and treatment approaches to the management of myositis patients.

        This review contains 4 highly rendered figures, 8 tables, and 80 references.

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    • SAE Neurology
      • 1

        Intracerebral Hemorrhage

        By Natalie P. Kreitzer, MD; Opeolu Adeoye, MD, MS
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        Intracerebral Hemorrhage

        • NATALIE P. KREITZER, MDNeurocritical Care Fellow in Training, Department of Emergency Medicine, University of Cincinnati, Cincinnati, OH
        • OPEOLU ADEOYE, MD, MSAssociate Professor of Emergency Medicine and Neurocritical Care, University of Cincinnati, Cincinnati, OH

        Intracerebral hemorrhage can be classified as either secondary (due to trauma, vascular malformations, aneurysms, tumors, or hemorrhagic transformation of ischemic stroke) or primary (without a clear secondary cause). Intracerebral hemorrhage is a neurologic emergency, and leads to significant death and disability each year; care should be expedited and emergency departments should be equipped to appropriately care for and manage these patients. This review covers the risk factors, natural history, pathophysiology, stabilization and assessment, diagnosis and treatment, and disposition and outcomes for patients with intracerebral hemorrhage. Figures show head computed tomographic scans demonstrating a left basal ganglia intracerebral hemorrhage, and an algorithm of management of intracerebral hemorrhage in the emergency department. Tables list some common causes of intracerebral hemorrhage, Boston criteria for diagnosis of cerebral amyloid angiopathy, mechanism of action of common anticoagulants, and suggested reversal agents.

        This review contains 2 highly rendered figures, 4 tables, and 79 references.

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    • SAE Trauma
      • 1

        Spine and Spinal Cord Injury

        By Michael A. Cole, MD
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        Spine and Spinal Cord Injury

        • MICHAEL A. COLE, MDAttending Physician, Department of Emergency Medicine, Brigham and Women’s Hospital, Instructor of Emergency, Harvard Medical School, Boston, MA
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  • Infections
    • 1
    • 2

      Herpesvirus Infections

      By Martin S. Hirsch, MD
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      Herpesvirus Infections

      • MARTIN S. HIRSCH, MDProfessor of Medicine, Harvard Medical School, Boston, MA

      The herpes group of viruses is composed of at least eight human viruses and numerous animal viruses. The human herpesviruses include herpes simplex virus types 1 and 2 (HSV-1 and HSV-2), varicella-zoster virus (VZV), cytomegalovirus (CMV), Epstein-Barr virus (EBV), and human herpesvirus types 6 (HHV-6), 7 (HHV-7), and 8 (HHV-8, also known as Kaposi sarcoma–associated herpesvirus). Human herpesviruses share the properties of latency and reactivation. Members of the group can cause productive lytic infections, in which infectious virus is produced and cells are killed, or nonproductive lytic infections, in which viral DNA persists but complete replication does not occur and cells survive. After acute lytic infections, herpesviruses often persist in a latent form for years; periodic reactivations are followed by recurrent lytic infections. Sites of latency vary: HSV and VZV persist in neural ganglion cells, EBV persists in B cells, and CMV probably remains latent in many cell types. The sites of latency for HHV-6 and HHV-7 have not been identified, although both herpesviruses have been detected in salivary glands. All human herpesviruses have a worldwide distribution. Considerable efforts are being directed toward the development of vaccines and antiviral agents that will be active against herpesviruses. This chapter discusses the epidemiology, pathogenesis, diagnosis, prevention, and treatment of herpes simplex virus and varicella-zoster virus and their clinical syndromes. The descriptions of the clinical syndromes include complications and clinical features, as well as descriptions of symptoms. Tables provide information on chemotherapy for primary genital and mucocutaneous herpes infection, suppression of severe and recurring genital herpes infection, and varicella-zoster infection. Figures provide photographic illustrations of the various clinical syndromes. A sidebar about herpesvirus information on the Internet provides further detail.

      This review contains 123 references, 4 tables, and 6 highly rendered figures.

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