Seminars in neurology
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Status epilepticus (SE) is a neurological emergency that requires prompt diagnosis and treatment, as delay is associated with a higher likelihood of poor response to treatment and worse outcome. Lorazepam has been well established as a first-line therapy. Subsequent steps are less established, and there are many reasonable options, including intravenous fosphenytoin, valproate, midazolam, propofol, and phenobarbital. ⋯ For refractory SE, continuous intravenous midazolam and propofol, separately or in combination, are rapidly effective, with pentobarbital remaining the gold standard for prolonged cases. If a patient does not awaken after treatment, urgent electroencephalogram (EEG) should be obtained to rule out nonconvulsive seizure activity. In refractory SE, continuous EEG monitoring is required to recognize recurrence of seizure activity, as most seizures will be nonconvulsive.
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The inflammatory myopathies, including dermatomyositis, inclusion body myositis, and polymyositis, are poorly understood autoimmune diseases affecting skeletal muscle. Dermatomyositis is a disease mainly of skin and muscle, but may affect lung and other tissues. Proximal or generalized weakness or skin rash are the typical presenting features. ⋯ Dermatomyositis and polymyositis are treated with corticosteroids and a variety of agents. Osteoporosis and opportunistic infections pose a significant risk during treatment of patients. This review discusses the clinical manifestations, pathology, and treatment approaches for the inflammatory myopathies.
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The inherited myopathies and muscular dystrophies are a diverse group of muscle diseases presenting with common complaints and physical signs: weakness, motor delay, and respiratory and bulbar dysfunction. The myopathies are caused by genetic defects in the contractile apparatus of muscle, and defined by distinctive histochemical or ultrastructural changes on muscle biopsy. ⋯ Diagnosis of these disorders is contingent on a targeted history and examination, biochemical and neurophysiological assessment, muscle biopsy, and genetic testing. Treatment is focused on symptomatic management and rehabilitation, and monitoring for disease complications.
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Seminars in neurology · Feb 2008
ReviewAcute disseminated encephalomyelitis: current understanding and controversies.
Acute disseminated encephalomyelitis (ADEM) is an uncommon monophasic idiopathic inflammatory demyelinating disease. Available diagnostic criteria do not reliably distinguish it from first presentations of relapsing diseases such as multiple sclerosis (MS) and neuromyelitis optica (NMO). ⋯ We review pathological differences between ADEM and MS in terms of their utility in the diagnosis of ADEM. Finally, we present a practical approach for management of patients suspected of having ADEM when the diagnosis is uncertain.
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Movement disorders are not commonly seen during pregnancy. As a result, there are few studies on whether disease manifestations are affected by the hormonal changes that occur during pregnancy or on the teratogenicity of commonly used medications for movement disorders on the developing fetus. This article discusses movement disorders that are seen only during pregnancy (chorea gravidarum) or that may present during pregnancy (restless legs syndrome), the effect that pregnancy has on symptoms and treatment (in Parkinson's disease, essential tremor, dystonia, tic disorders, and Wilson's disease), and the role of genetic testing for movement disorders in genetic counseling for pregnant women.