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Semin Respir Crit Care Med 2002; 23(3): 191-200
DOI: 10.1055/s-2002-33027
Copyright © 2002 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA. Tel.: +1(212) 584-4662

Overview of Neuromuscular Disorders Affecting Respiratory Function

James M. Gilchrist
  • Brown Medical School, Providence, Rhode Island, and Department of Neurology, Rhode Island Hospital, Providence, Rhode Island
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Publication History

Publication Date:
02 August 2002 (online)

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ABSTRACT

A great many neuromuscular disorders affect respiratory function. This article focuses on neuropathic and myopathic disorders that weaken the diaphragm and respiratory muscles. This discussion gives a general overview and attempts to update the reader on recent pathophysiological insights and developments in therapy. Among the disorders discussed, amyotrophic lateral sclerosis, poliomyelitis, postpolio syndrome, Kennedy syndrome, and spinal muscular atrophy are motor neuron diseases. Brachial plexitis and isolated unilateral or bilateral phrenic neuropathies can also weaken the diaphragm significantly. Peripheral neuropathies affecting respiration are primarily acute disorders such as Guillain-Barré syndrome, porphyria, and critical illness neuropathy, but chronic diseases such as chronic inflammatory demyelinating polyneuropathy (CIDP) and Charcot-Marie-Tooth disease (CMT) can also cause respiratory insufficiency. Disorders of neuromuscular transmission such as Lambert-Eaton syndrome, botulism, and myasthenia gravis often affect respiration. Many muscle diseases also affect pulmonary function, including polymyositis/dermatomyositis, muscular dystrophy, hereditary channel disorders, mitochondrial encephalomyopathies, acid maltase deficiency, and congenital myopathy.

KEYWORDS

Neuropathy - myasthenia gravis - muscular dystropy - amyotrophic lateral sclerosis - myopathy

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