Neuromuscular Junction and Muscle

Robert B. Darnell and Jerome B. Posner

in Paraneoplastic Syndromes

Published on behalf of Oxford University Press

Published in print April 2010 | ISBN: 9780199772735
Published online April 2013 | e-ISBN: 9780199322916 | DOI:

Series: Contemporary Neurology Series

Neuromuscular Junction and Muscle

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Paraneoplastic disorders of the neuromuscular junction and muscle are relatively common (Table 9–1). These disorders can cause either hypoactivity of muscles (weakness) or hyperactivity1 (Table 9–2). Much of the time these disorders are not paraneoplastic but are instead nonparaneoplastic autoimmune disorders. However, in some disorders, the likelihood of cancer is high enough to warrant significant consideration. For example, myasthenia gravis is paraneoplastic in 10% to 15% of instances; Lambert-Eaton myasthenic syndrome (LEMS) in 50% to 60%; dermatomyositis in 15%; and polymyositis in 9%. Thus, in most instances, the development of one of these neuromuscular junction or muscle syndromes requires a search for an underlying neoplasm. The three paraneoplastic disorders of the neuromuscular junction are LEMS, myasthenia gravis, and neuromyotonia. The first two cause muscle weakness, and the third causes muscle hyperactivity. Figure 9–1 illustrates the large number of proteins involved in neuromuscular transmission and some of the disorders that affect them.

Chapter.  15147 words.  Illustrated.

Subjects: Neurology

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