Seminars in neurology
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Seminars in neurology · Jun 2002
Review Case ReportsNeuromuscular scoliosis: causes of deformity and principles for evaluation and management.
Scoliosis is commonly associated with a variety of neuromuscular disorders including conditions affecting upper and lower motor neurons as well as myopathies. Contained herein is a discussion of the spectrum of neuromuscular disorders that have been associated with scoliosis and related spinal deformities. Management, including surgical treatment in such patients, is summarized including indications, expectations, and impact on trunk balance, pulmonary function, and appearance.
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Seminars in neurology · Dec 2001
ReviewPearls and pitfalls in the intensive care management of Guillain-Barré syndrome.
Approximately one third of patients with Guillain-Barré syndrome (GBS) need to be admitted to the intensive care unit (ICU) because of respiratory failure. A significant proportion of patients require ICU admission because of dysautonomia or because of medical or iatrogenic complications. ⋯ Recognition of autonomic dysfunction and of the rare need for intervention is important to avoid iatrogenic complications. Prognosis determination in GBS is based on the clinical history, epidemiological factors, and neurologic examination.
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Amyotrophic lateral sclerosis (ALS) is a devastating paralytic disorder caused by motor neuron degeneration. A subgroup of familial cases arises from mutations in the gene encoding cytosolic superoxide dismutase (SOD1). ⋯ Also discussed are the status of other genetic forms of ALS and the elusive question of why this disorder so specifically targets motor neurons. Ultimately, it is hoped that insights from these types of studies will improve the prospects for developing meaningful therapies of ALS.
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Ocular myasthenia gravis is a not uncommon autoimmune disorder causing diplopia, ptosis, and weakness of lid closure. The predilection of myasthenia for the ocular muscles may be related to differences between limb and extraocular muscles in either physiological function or antigenicity. Clinically, ocular myasthenia can mimic any form of pupil-sparing ocular motility disorder. ⋯ Treatment consists of symptomatic use of acetylcholinesterase inhibitors and immunosuppression with steroids or azathioprine. Between 50 and 70% of patients with ocular myasthenia will eventually develop generalized disease: there is some retrospective data that steroids or azathioprine may reduce this by about 75%. The role of thymectomy in ocular myasthenia remains unclear.
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In the past 10 years the epidemiology of bacterial meningitis has changed, with a decreased incidence of meningitis caused by Haemophilus influenzae and an increasing incidence of meningitis caused by penicillin- and cephalosporin-resistant strains of Streptococcus pneumoniae. Meningococcal meningitis has become an increasing threat to college students. ⋯ In this article, the pathophysiology, etiology, clinical presentation, differential diagnosis, and management of acute bacterial meningitis are reviewed. The present recommendations for the use of dexamethasone in the treatment of this infection, the use of chemoprophylaxis, and the indications for vaccinations are included.