Issue Overview
No abstract available (Source: CONTINUUM: Lifelong Learning in Neurology)
Source: CONTINUUM: Lifelong Learning in Neurology - December 1, 2019 Category: Neurology Tags: ISSUE OVERVIEW Source Type: research

Key Points For Issue
No abstract available (Source: CONTINUUM: Lifelong Learning in Neurology)
Source: CONTINUUM: Lifelong Learning in Neurology - December 1, 2019 Category: Neurology Tags: KEY POINTS FOR ISSUE Source Type: research

List of Abbreviations
No abstract available (Source: CONTINUUM: Lifelong Learning in Neurology)
Source: CONTINUUM: Lifelong Learning in Neurology - December 1, 2019 Category: Neurology Tags: LIST OF ABBREVIATIONS Source Type: research

Learning Objectives
No abstract available (Source: CONTINUUM: Lifelong Learning in Neurology)
Source: CONTINUUM: Lifelong Learning in Neurology - December 1, 2019 Category: Neurology Tags: LEARNING OBJECTIVES AND CORE COMPETENCIES Source Type: research

Erratum
No abstract available (Source: CONTINUUM: Lifelong Learning in Neurology)
Source: CONTINUUM: Lifelong Learning in Neurology - December 1, 2019 Category: Neurology Tags: ERRATUM Source Type: research

Postreading Self-Assessment and CME Test—Preferred Responses
No abstract available (Source: CONTINUUM: Lifelong Learning in Neurology)
Source: CONTINUUM: Lifelong Learning in Neurology - December 1, 2019 Category: Neurology Tags: SELF-ASSESSMENT AND CME Source Type: research

Postreading Self-Assessment and CME Test
No abstract available (Source: CONTINUUM: Lifelong Learning in Neurology)
Source: CONTINUUM: Lifelong Learning in Neurology - December 1, 2019 Category: Neurology Tags: SELF-ASSESSMENT AND CME Source Type: research

Instructions for Completing CME and Tally Sheet
No abstract available (Source: CONTINUUM: Lifelong Learning in Neurology)
Source: CONTINUUM: Lifelong Learning in Neurology - December 1, 2019 Category: Neurology Tags: SELF-ASSESSMENT AND CME Source Type: research

Lambert-Eaton Myasthenic Syndrome, Botulism, and Immune Checkpoint Inhibitor–Related Myasthenia Gravis
This article reviews the pathophysiology, epidemiology, clinical presentation, diagnosis, and treatment of Lambert-Eaton myasthenic syndrome (LEMS) and of botulism, and immune-related myasthenia gravis (MG) occurring in the context of immune checkpoint inhibitor therapy for cancer. RECENT FINDINGS The suspicion that LEMS is rare but also likely underdiagnosed is supported by recent epidemiologic data. A validated, LEMS-specific scale now exists to assess and monitor disease, and symptomatic and immunomodulatory treatments are available. As presynaptic disorders of neuromuscular transmission, LEMS and botulism share elec...
Source: CONTINUUM: Lifelong Learning in Neurology - December 1, 2019 Category: Neurology Tags: REVIEW ARTICLES Source Type: research

Myasthenia Gravis and Congenital Myasthenic Syndromes
This article reviews the importance of prompt recognition of the typical signs and symptoms, best tests to confirm the diagnosis, currently available acute and chronic treatment modalities, the role of thymectomy, and the natural history of the disease. Special consideration related to the diagnosis and management in women during pregnancy and in children will also be reviewed. This article also includes an overview of congenital myasthenic syndromes. RECENT FINDINGS Recent significant efforts in standardizing and improving the care of patients with MG have occurred, as well as new momentum in developing new drugs for p...
Source: CONTINUUM: Lifelong Learning in Neurology - December 1, 2019 Category: Neurology Tags: REVIEW ARTICLES Source Type: research

Mitochondrial and Metabolic Myopathies
This article provides an overview of mitochondrial and metabolic biology, the genetic mechanisms causing mitochondrial diseases, the clinical features of mitochondrial diseases, lipid myopathies, and glycogen storage diseases, all with a focus on those syndromes and diseases associated with myopathy. Over the past decade, advances in genetic testing have revolutionized patient evaluation. The main goal of this review is to give the clinician the basic understanding to recognize patients at risk of these diseases using the standard history and physical examination. RECENT FINDINGS Primary mitochondrial disease is the cur...
Source: CONTINUUM: Lifelong Learning in Neurology - December 1, 2019 Category: Neurology Tags: REVIEW ARTICLES Source Type: research

Toxic Myopathies
This article reviews the pathogenesis, clinical features, and management of toxic myopathy related to common medications, critical illness, and illicit substances. RECENT FINDINGS Muscle symptoms are common among statin users and are usually reversible after discontinuation of the statin; rarely, however, statins trigger an immune-mediated necrotizing myopathy that persists and requires immunomodulatory therapy. Autoantibodies targeting 3-hydroxy-3-methylglutaryl coenzyme A reductase can distinguish the toxic and immune-mediated forms. Immune checkpoint inhibitors, increasingly used in the treatment of advanced cancer, ...
Source: CONTINUUM: Lifelong Learning in Neurology - December 1, 2019 Category: Neurology Tags: REVIEW ARTICLES Source Type: research

Episodic Muscle Disorders
This article reviews the episodic muscle disorders, including benign cramp-fasciculation syndrome, the periodic paralyses, and the nondystrophic myotonias. The core diagnostic criteria for a diagnosis of primary periodic paralysis, including clues to distinguish between the hypokalemic and hyperkalemic forms, and the distinctive elements that characterize Andersen-Tawil syndrome are discussed. Management of patients with these disorders is also discussed. RECENT FINDINGS Childhood presentations of periodic paralysis have recently been described, including atypical findings. Carbonic anhydrase inhibitors, such as dichlor...
Source: CONTINUUM: Lifelong Learning in Neurology - December 1, 2019 Category: Neurology Tags: REVIEW ARTICLES Source Type: research

Myotonic Muscular Dystrophies
This article describes the clinical features, pathogenesis, prevalence, diagnosis, and management of myotonic dystrophy type 1 and myotonic dystrophy type 2. RECENT FINDINGS The prevalence of myotonic dystrophy type 1 is better understood than the prevalence of myotonic dystrophy type 2, and new evidence indicates that the risk of cancer is increased in patients with the myotonic dystrophies. In addition, descriptions of the clinical symptoms and relative risks of comorbidities such as cardiac arrhythmias associated with myotonic dystrophy type 1 have been improved. SUMMARY Myotonic dystrophy type 1 and myotonic dyst...
Source: CONTINUUM: Lifelong Learning in Neurology - December 1, 2019 Category: Neurology Tags: REVIEW ARTICLES Source Type: research

Facioscapulohumeral Muscular Dystrophies
This article reviews the phenotype and pathophysiology of the disease as well as the recent efforts in clinical outcome measures and clinical trials. RECENT FINDINGS As the name implies, FSHD involves weakness of facial muscles, muscles that fix the scapula, and muscles overlying the humerus (biceps and triceps). The distinctive phenotype of FSHD occurs secondary to two different genetic mechanisms. FSHD type 1 (FSHD1) is due to a deletion on chromosome 4q, leading to hypomethylation and derepression of DUX4. FSHD type 2 (FSHD2) is due to mutations in SMCHD1 with resulting hypomethylation of the same subtelomeric region...
Source: CONTINUUM: Lifelong Learning in Neurology - December 1, 2019 Category: Neurology Tags: REVIEW ARTICLES Source Type: research

Congenital Muscular Dystrophy and Congenital Myopathy
This article summarizes the clinical and genetic aspects of these disorders. RECENT FINDINGS Historically, diagnoses of congenital muscular dystrophy and congenital myopathy have been made by clinical features and histopathology; however, recent advances in genetics have changed diagnostic practice by relying more heavily on genetic findings. This article reviews the clinical and genetic features of the most common congenital muscular dystrophies including laminin subunit alpha 2 (LAMA2)–related (merosin deficient), collagen VI–related, and α-dystroglycan–related congenital muscular dystrophies a...
Source: CONTINUUM: Lifelong Learning in Neurology - December 1, 2019 Category: Neurology Tags: REVIEW ARTICLES Source Type: research

The Dystrophinopathies
This article reviews the epidemiology, clinical manifestations, genetic cause, management, and new and emerging therapies for this condition. RECENT FINDINGS New studies have highlighted how oral corticosteroids have changed the natural history of the disease, prolonging ambulation in boys with Duchenne muscular dystrophy and reducing the risk of developing scoliosis and subsequent surgical correction, improving cardiac health, and increasing long-term survival. Additionally, recent publications have provided insights into how newer and emerging treatment options are becoming more common for this condition. With gene th...
Source: CONTINUUM: Lifelong Learning in Neurology - December 1, 2019 Category: Neurology Tags: REVIEW ARTICLES Source Type: research

The Limb-Girdle Muscular Dystrophies
This article defines and describes LGMDs, delineates a diagnostic strategy, and discusses treatment of the LGMDs. recent findings In 2018, the definition of the LGMDs was further refined, and a new nomenclature was proposed. Diagnosis of the LGMDs was long guided by the distinctive clinical characteristics of each particular subtype but now integrates use of genetics—with next-generation sequencing panels, exomes, and full genome analysis—early in the diagnostic assessment. Appreciation of the phenotypic diversity of each LGMD subtype continues to expand. This emphasizes the need for precision genetic diagno...
Source: CONTINUUM: Lifelong Learning in Neurology - December 1, 2019 Category: Neurology Tags: REVIEW ARTICLES Source Type: research

Sporadic Inclusion Body Myositis and Other Rimmed Vacuolar Myopathies
This article reviews the clinical, laboratory, and histopathologic features of sporadic inclusion body myositis (IBM) and explores its pathogenic overlap with inherited myopathies that have IBM-like pathology. RECENT FINDINGS Sporadic IBM is the most common acquired muscle disease in patients older than 50 years of age and is becoming more prevalent because of the increasing age of the population, the emerging development of more inclusive diagnostic criteria, and the advent of a diagnostic autoantibody. No effective therapy is known, and the pathogenic mechanism remains unclear. Some pathogenic insight can be gleaned f...
Source: CONTINUUM: Lifelong Learning in Neurology - December 1, 2019 Category: Neurology Tags: REVIEW ARTICLES Source Type: research

Immune-Mediated Myopathies
This article summarizes the clinical features, diagnostic evaluation, and management of the common immune-mediated myopathies: dermatomyositis, antisynthetase syndrome, immune-mediated necrotizing myopathy, and overlap myositis. RECENT FINDINGS The identification of myositis-specific autoantibodies has improved the characterization of the subtypes of myositis and associated clinical phenotypes, as the severity of muscle involvement, extramuscular manifestations, and risk of malignancy may vary among the subtypes of autoimmune myopathies. SUMMARY The understanding and diagnostic accuracy of the subtypes of autoimmune ...
Source: CONTINUUM: Lifelong Learning in Neurology - December 1, 2019 Category: Neurology Tags: REVIEW ARTICLES Source Type: research

Approach to Muscle and Neuromuscular Junction Disorders
This article provides a diagnostic approach based on clinical history and neurologic examination leading to a narrow set of diagnostic tests. RECENT FINDINGS Numerous discoveries in recent years have facilitated clinician access to more advanced laboratory and genetic testing to pinpoint the exact diagnosis in patients with muscle or neuromuscular junction disorders. Large-scale genetic testing has become much less expensive, and free testing has become available for many of the rare conditions because of increased research and the availability of effective therapies for these rare disorders. SUMMARY The approach to ...
Source: CONTINUUM: Lifelong Learning in Neurology - December 1, 2019 Category: Neurology Tags: REVIEW ARTICLES Source Type: research

Actin Lessons
No abstract available (Source: CONTINUUM: Lifelong Learning in Neurology)
Source: CONTINUUM: Lifelong Learning in Neurology - December 1, 2019 Category: Neurology Tags: EDITOR’S PREFACE Source Type: research

Contributors
No abstract available (Source: CONTINUUM: Lifelong Learning in Neurology)
Source: CONTINUUM: Lifelong Learning in Neurology - December 1, 2019 Category: Neurology Tags: CONTRIBUTORS Source Type: research

Table of Contents
No abstract available (Source: CONTINUUM: Lifelong Learning in Neurology)
Source: CONTINUUM: Lifelong Learning in Neurology - December 1, 2019 Category: Neurology Tags: Table of Contents Source Type: research

Issue Overview
No abstract available (Source: CONTINUUM: Lifelong Learning in Neurology)
Source: CONTINUUM: Lifelong Learning in Neurology - October 1, 2019 Category: Neurology Tags: ISSUE OVERVIEW Source Type: research

Key Points For Issue
No abstract available (Source: CONTINUUM: Lifelong Learning in Neurology)
Source: CONTINUUM: Lifelong Learning in Neurology - October 1, 2019 Category: Neurology Tags: KEY POINTS FOR ISSUE Source Type: research

List of Abbreviations
No abstract available (Source: CONTINUUM: Lifelong Learning in Neurology)
Source: CONTINUUM: Lifelong Learning in Neurology - October 1, 2019 Category: Neurology Tags: LIST OF ABBREVIATIONS Source Type: research

Learning Objectives
No abstract available (Source: CONTINUUM: Lifelong Learning in Neurology)
Source: CONTINUUM: Lifelong Learning in Neurology - October 1, 2019 Category: Neurology Tags: LEARNING OBJECTIVES AND CORE COMPETENCIES Source Type: research

Postreading Self-Assessment and CME Test—Preferred Responses
No abstract available (Source: CONTINUUM: Lifelong Learning in Neurology)
Source: CONTINUUM: Lifelong Learning in Neurology - October 1, 2019 Category: Neurology Tags: SELF-ASSESSMENT AND CME Source Type: research

Postreading Self-Assessment and CME Test
No abstract available (Source: CONTINUUM: Lifelong Learning in Neurology)
Source: CONTINUUM: Lifelong Learning in Neurology - October 1, 2019 Category: Neurology Tags: SELF-ASSESSMENT AND CME Source Type: research

Instructions for Completing CME and Tally Sheet
No abstract available (Source: CONTINUUM: Lifelong Learning in Neurology)
Source: CONTINUUM: Lifelong Learning in Neurology - October 1, 2019 Category: Neurology Tags: SELF-ASSESSMENT AND CME Source Type: research

Imaging in Neuro-ophthalmology
This article discusses an approach to imaging in patients with neuro-ophthalmologic disorders, with emphasis on the clinical-anatomic localization of lesions affecting afferent and efferent visual function. RECENT FINDINGS Advances in MRI, CT, ultrasound, and optical coherence tomography have changed how neuro-ophthalmic disorders are diagnosed and followed in the modern clinical era. SUMMARY The advantages, disadvantages, and indications for various imaging techniques for neuro-ophthalmologic disorders are discussed, with a view to optimizing how these tools can be used to enhance patient care. (Source: CONTINUUM: L...
Source: CONTINUUM: Lifelong Learning in Neurology - October 1, 2019 Category: Neurology Tags: REVIEW ARTICLES Source Type: research

Infectious Optic Neuropathies
This article reviews common infectious optic neuropathies, focusing on the more common and globally important entities. RECENT FINDINGS Novel infections continue to emerge and drift geographically over time; not infrequently, these have important neurologic or ocular features. Malarial retinal findings comprise a relatively specific set of findings and serve as an invaluable aid in the diagnosis of cerebral malaria. Therapy continues to evolve and is best formulated in concert with an infectious disease expert. SUMMARY Infectious optic neuropathies are less common than inflammatory or ischemic optic neuropathies; may...
Source: CONTINUUM: Lifelong Learning in Neurology - October 1, 2019 Category: Neurology Tags: REVIEW ARTICLES Source Type: research

Paraneoplastic Syndromes in Neuro-ophthalmology
This article discusses the varied types of paraneoplastic syndromes that commonly have neuro-ophthalmologic manifestations. Diagnostic considerations and therapeutic options for individual diseases are also discussed. RECENT FINDINGS Paraneoplastic syndromes can affect the afferent and efferent visual systems. Paraneoplastic syndromes may result in reduced visual acuity from retinal degeneration, alterations in melanocyte proliferation and uveal thickening, or acquired nystagmus. Ocular motor abnormalities related to paraneoplastic syndromes may present with symptoms from opsoclonus or from neuromuscular junction diseas...
Source: CONTINUUM: Lifelong Learning in Neurology - October 1, 2019 Category: Neurology Tags: REVIEW ARTICLES Source Type: research

Nystagmus and Saccadic Intrusions
This article provides an overview of nystagmus and saccadic intrusions with the goal of facilitating recognition and differentiation of abnormal eye movements to assist with accurate diagnosis of neurologic disease and evidence-based specific treatment of oscillopsia. Myriad advances have been made in the understanding of several types of nystagmus and saccadic intrusions, even in the past 5 to 10 years, especially regarding underlying pathophysiology, leading to pharmacologic advances rooted in physiologic principles. RECENT FINDINGS Specific recent advances in the study of nystagmus and saccadic intrusions include (1)...
Source: CONTINUUM: Lifelong Learning in Neurology - October 1, 2019 Category: Neurology Tags: REVIEW ARTICLES Source Type: research

Approach to Diplopia
This article provides a foundation for formulating an approach to the patient with diplopia and includes practical examples of developing the differential diagnosis, effectively using confirmatory examination techniques, determining an appropriate diagnostic strategy, and (where applicable) providing effective treatment. RECENT FINDINGS Recent population-based analyses have determined that diplopia is a common presentation in both ambulatory and emergency department settings, with 850,000 such visits occurring annually. For patients presenting to an outpatient facility, diagnoses are rarely serious. However, potentially...
Source: CONTINUUM: Lifelong Learning in Neurology - October 1, 2019 Category: Neurology Tags: REVIEW ARTICLES Source Type: research

Higher Cortical Visual Disorders
This article reviews the disorders that result from disruption of extrastriate regions of the cerebral cortex responsible for higher visual processing. For each disorder, a historical perspective is offered and relevant neuroscientific studies are reviewed. RECENT FINDINGS Careful analysis of the consequences of lesions that disrupt visual functions such as facial recognition and written language processing has improved understanding of the role of key regions in these networks. In addition, modern imaging techniques have built upon prior lesion studies to further elucidate the functions of these cortical areas. For exa...
Source: CONTINUUM: Lifelong Learning in Neurology - October 1, 2019 Category: Neurology Tags: REVIEW ARTICLES Source Type: research

Chiasmal and Postchiasmal Disease
This article reviews the anatomy, symptoms, examination findings, and causes of diseases affecting the optic chiasm, optic tracts, optic radiations, and occipital lobes. RECENT FINDINGS Modern ophthalmic imaging can be used to monitor the effects of diseases of the optic chiasm and tract on the retinal ganglion cells. It can also be used to visualize transsynaptic degeneration of the anterior visual pathway in the setting of acquired retrogeniculate lesions. Visual prostheses that directly stimulate the occipital lobe are a potential strategy for rehabilitation that is in active clinical trials. SUMMARY Detecting and...
Source: CONTINUUM: Lifelong Learning in Neurology - October 1, 2019 Category: Neurology Tags: REVIEW ARTICLES Source Type: research

Idiopathic Intracranial Hypertension
This article reviews the diagnostic criteria, clinical features, neuroimaging findings, differential diagnosis, and management options for this condition. RECENT FINDINGS Recent population studies have found that the annual incidence of idiopathic intracranial hypertension is increasing in association with obesity rates, whereas recent scientific studies indicate a possible role for androgen sex hormones and adipose tissue in the pathogenesis of the disease. Prospective clinical trials have demonstrated a role for weight loss, acetazolamide, and topiramate in the management of mild disease. A recently begun randomized m...
Source: CONTINUUM: Lifelong Learning in Neurology - October 1, 2019 Category: Neurology Tags: REVIEW ARTICLES Source Type: research

Toxic-Metabolic and Hereditary Optic Neuropathies
PURPOSE OF REVIEW The diagnosis of visual loss from toxic-metabolic and hereditary optic neuropathies may be delayed in some cases because of a failure to elicit important information in the clinical history or to recognize typical examination findings. An understanding of the features specific to each type of toxic-metabolic and hereditary optic neuropathy, and of the underlying mechanism of insult to the optic nerve, could lead to earlier recognition, diagnosis, and treatment (when available). RECENT FINDINGS Understanding of the role of mitochondria in toxic-metabolic and hereditary optic neuropathies is growing, p...
Source: CONTINUUM: Lifelong Learning in Neurology - October 1, 2019 Category: Neurology Tags: REVIEW ARTICLES Source Type: research

Optic Neuritis
This article discusses the clinical presentation, evaluation, and management of the patient with optic neuritis. Initial emphasis is placed on clinical history, examination, diagnostic testing, and medical decision making, while subsequent focus is placed on examining specific inflammatory optic neuropathies. Clinical clues, examination findings, neuroimaging, and laboratory testing that differentiate autoimmune, granulomatous, demyelinating, infectious, and paraneoplastic causes of optic neuritis are assessed, and current treatments are evaluated. RECENT FINDINGS Advances in technology and immunology have enhanced our ...
Source: CONTINUUM: Lifelong Learning in Neurology - October 1, 2019 Category: Neurology Tags: REVIEW ARTICLES Source Type: research

Ischemic Optic Neuropathy
This article provides an overview of the three major classes of ischemic optic neuropathy, including information on risk factors, differential diagnosis, evaluation, and management. RECENT FINDINGS Optical coherence tomography provides precise anatomic imaging in ischemic optic neuropathy, showing neural loss weeks before it is visible on examination. Refinements of optical coherence tomography reveal optic nerve microvasculature and may assist in understanding pathogenesis and verifying diagnosis. New diagnostic algorithms and cranial vascular imaging techniques help define the likelihood of GCA in patients with ischem...
Source: CONTINUUM: Lifelong Learning in Neurology - October 1, 2019 Category: Neurology Tags: REVIEW ARTICLES Source Type: research

The Pupil
PURPOSE OF REVIEW The goal of this article is to review the anatomy and physiology of pupillary function and then employ that information to develop a comprehensive framework for understanding and diagnosing pupillary disorders. RECENT FINDINGS The contribution of rods and cones to the pupillary light reflex has long been known. A third photosensitive cell type, the intrinsically photosensitive retinal ganglion cell, has recently been discovered. This cell type employs melanopsin to mediate a portion of the pupillary light reflex independent of rods and cones (the postillumination pupillary response) and photic regula...
Source: CONTINUUM: Lifelong Learning in Neurology - October 1, 2019 Category: Neurology Tags: REVIEW ARTICLES Source Type: research

Cicadic Intrusions
No abstract available (Source: CONTINUUM: Lifelong Learning in Neurology)
Source: CONTINUUM: Lifelong Learning in Neurology - October 1, 2019 Category: Neurology Tags: EDITOR’S PREFACE Source Type: research

Contributors
No abstract available (Source: CONTINUUM: Lifelong Learning in Neurology)
Source: CONTINUUM: Lifelong Learning in Neurology - October 1, 2019 Category: Neurology Tags: CONTRIBUTORS Source Type: research

Table of Contents
No abstract available (Source: CONTINUUM: Lifelong Learning in Neurology)
Source: CONTINUUM: Lifelong Learning in Neurology - October 1, 2019 Category: Neurology Tags: Table of Contents Source Type: research

Issue Overview
No abstract available (Source: CONTINUUM: Lifelong Learning in Neurology)
Source: CONTINUUM: Lifelong Learning in Neurology - August 1, 2019 Category: Neurology Tags: ISSUE OVERVIEW Source Type: research

Key Points for Issue
No abstract available (Source: CONTINUUM: Lifelong Learning in Neurology)
Source: CONTINUUM: Lifelong Learning in Neurology - August 1, 2019 Category: Neurology Tags: KEY POINTS FOR ISSUE Source Type: research

List of Abbreviations
No abstract available (Source: CONTINUUM: Lifelong Learning in Neurology)
Source: CONTINUUM: Lifelong Learning in Neurology - August 1, 2019 Category: Neurology Tags: LIST OF ABBREVIATIONS Source Type: research

Learning Objectives
No abstract available (Source: CONTINUUM: Lifelong Learning in Neurology)
Source: CONTINUUM: Lifelong Learning in Neurology - August 1, 2019 Category: Neurology Tags: LEARNING OBJECTIVES AND CORE COMPETENCIES Source Type: research