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
