Respiratory muscle weakness in peripheral neuropathies.

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J Peripher Nerv Syst. 2010 Dec;15(4):307-13

Respiratory muscle weakness in peripheral neuropathies.

Burakgazi AZ, Höke A.

Department of Neurology, Washington University, Washington, DC, USA

Department of Neurology, s Hopkins School of Medicine, Baltimore, MD, USA.

Abstract

Common peripheral neuropathies do not usually cause diaphragmatic weakness and

subsequent respiratory compromise. However, respiratory involvement is

relatively common in Guillain-Barré syndrome (GBS). Experience in GBS has led to

a standardized approach to manage respiratory problems in peripheral

neuropathies. Diaphragmatic weakness is not common in chronic inflammatory

demyelinating polyneuropathy and extremely rare in multifocal motor neuropathy.

The linkage has been described between certain subtypes of Charcot-Marie-Tooth

(CMT) disease such as CMT2C and CMT4B1 and diaphragmatic weakness. A correlation

usually has not been found between electrophysiologic findings and clinical

respiratory signs or spirometric abnormalities in peripheral neuropathies except

in amplitudes of evoked phrenic nerve responses. Careful and frequent assessment

of respiratory function by a qualified team of healthcare professionals and

physicians is essential. Criteria established for mechanical ventilation in GBS

cases may be applied to other peripheral neuropathies with respiratory

compromise as necessary.