Continuum : lifelong learning in neurology
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Continuum (Minneap Minn) · Dec 2017
Review2016 World Health Organization Classification of Central Nervous System Tumors.
Since 1979, the World Health Organization (WHO) has periodically published a consensus classification and grading of tumors of the central nervous system (CNS) to ensure uniform histopathologic diagnostic criteria worldwide. In 2016, the WHO published an update of the fourth edition of the classification of CNS tumors. This article summarizes the major changes in the update and discusses their impact on clinical practice. ⋯ The use of integrated phenotypic and genotypic parameters for the classification of CNS tumors introduces greater objectivity to the diagnosis but also requires more widespread availability of molecular testing. It is hoped that these changes will lead to greater diagnostic accuracy with more biologically homogeneous diagnostic entities and improved patient management and determination of prognosis.
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As a group, benign tumors account for the majority of primary neoplasms affecting the central nervous system (CNS). This article reviews the epidemiology, clinical presentation, neuroimaging features, and management of the most common of these tumors: meningiomas, schwannomas, and pituitary adenomas. ⋯ As a group, nonmalignant neoplasms are the most common neoplasms affecting the CNS in adults. Because of their unique neuroanatomic location or containment within the bony skull, these histologically benign lesions can cause significant neurologic morbidity. Management with a multidisciplinary team that includes neurologists, neuro-oncologists, radiologists, neurosurgeons, radiation oncologists, otolaryngologists, pathologists, neuropsychologists, physiatrists, and others is necessary for the optimal management of these lesions.
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This article provides a review of the clinical phenotypes and evaluation of peripheral nerve hyperexcitability syndromes. These rare diagnoses include cramp-fasciculation syndrome, Isaacs syndrome, and Morvan syndrome. Recent investigations have led to an understanding of the autoimmune underpinnings of these conditions and their specific associated antibodies. As the presentation of peripheral nerve hyperexcitability syndromes includes muscle stiffness, twitches, and spasms, which are also shared with certain central nervous system and myopathic conditions, the differential diagnosis of peripheral nerve hyperexcitability syndromes is reviewed. ⋯ The diagnosis of peripheral nerve hyperexcitability syndromes requires a high index of suspicion, support from the physical examination, familiarity with the spectrum of symptoms associated with peripheral nerve hyperexcitability syndromes, and recognition of diagnostic EMG features. Voltage-gated potassium channel complex antibodies are associated with these conditions. Optimum treatment and autoimmune pathogenesis remain areas of active research.
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Continuum (Minneap Minn) · Oct 2017
ReviewDiagnosis and Clinical Management of Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders.
This article reviews the clinical features, diagnostic pathway, therapies, and current understanding of the pathophysiology of amyotrophic lateral sclerosis (ALS). The spectrum of motor neuron diseases is reviewed, and the clinical heterogeneity of ALS is described. ⋯ ALS is a progressive fatal multisystem neurodegenerative disease primarily affecting motor neurons. Clinical recognition of suspicious symptoms and the appropriate laboratory evaluation are essential to limit diagnostic delay and avoid unnecessary testing and procedures. ALS has broad genetic and hypothesized environmental causes and phenotypic variability. Recognizing related motor neuron diseases will prevent misdiagnosis while allowing proper disease counseling. Although ALS cannot be cured, implementation of appropriate symptomatic treatment is valuable.
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Sleep-disordered breathing encompasses a broad spectrum of sleep-related breathing disorders, including obstructive sleep apnea (OSA), central sleep apnea, as well as sleep-related hypoventilation and hypoxemia. Diagnostic criteria have been updated in the International Classification of Sleep Disorders, Third Edition and the American Academy of Sleep Medicine Manual for Scoring Sleep and Associated Events. Neurologic providers should have basic knowledge and skills to identify at-risk patients, as these disorders are associated with substantial morbidity, the treatment of which is largely reversible. ⋯ Sleep-disordered breathing is highly prevalent and grossly underrecognized. The adverse medical and psychosocial consequences of OSA and other sleep-related breathing disorders are considerable. The impact of sleep therapies on highly prevalent neurologic disorders associated with substantial morbidity and health care costs is becoming increasingly recognized.