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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Continuum (Minneap Minn) · Dec 2017
ReviewPalliative Care, End-of-Life Care, and Advance Care Planning in Neuro-oncology.
Despite new therapeutic approaches, most patients with high-grade glioma face limited overall survival and have a high symptom burden throughout their disease trajectory, especially in the end-of-life phase. This article provides an overview of the role of palliative care in neuro-oncology. Management recommendations are made for neurologic symptoms in patients with advanced brain tumors, including headaches, nausea, and fatigue. Special attention is given to how and when to involve subspecialty palliative care and hospice services to improve symptom management during active tumor treatment and in the end-of-life phase of patients with brain tumors. Advance care planning and end-of-life goals should be addressed early in the disease trajectory; this article provides a road map for these discussions. ⋯ Palliative care can address typical symptoms, such as fatigue, nausea, and headaches that have the potential to severely disable patients with brain tumors. Advance care planning should be introduced proactively and early in the disease trajectory to ensure a dignified death and improved caregiver bereavement.
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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.