Journal article
Motor unit number estimate of distal and proximal muscles in Charcot-Marie-Tooth disease
Muscle & nerve, Vol.28(2), pp.161-167
08/2003
DOI: 10.1002/mus.10419
PMID: 12872319
Abstract
In order to determine the utility of motor unit number estimation (MUNE) in assessing axonal loss in chronic inherited neuropathies, we determined MUNEs in 54 patients with Charcot-Marie-Tooth (CMT) disease (29 patients with CMT-1A, 13 with CMT-X, and 12 with CMT-2) by using spike-triggered averaging (STA) of the ulnar-innervated abductor digiti minimi/hypothenar muscles (ADM) and the musculo-cutaneous innervated biceps/brachialis (BB) muscles. MUNEs were analyzed in relationship to the corresponding compound muscle action potential (CMAP) amplitudes as well as to clinical strength. Proximal muscles, which appeared strong clinically, had evidence of chronic denervation/reinnervation, although to a lesser extent than weak distal hand muscles, supporting the concept that axonal loss in CMT occurs in a length-dependent fashion. The reduction in ADM-MUNE strongly correlated with clinical weakness in the hand. Both the ADM-MUNE and BB-MUNE were abnormal more often than CMAP amplitude, probably reflecting extensive motor unit reconfiguration and enlargement that maintains CMAP amplitude despite severe motor unit loss. This study suggests that MUNE can assess motor unit loss in CMT and may better reflect axonal loss than CMAP amplitude. The STA technique of MUNE may be useful in longitudinal studies of proximal and distal motor unit changes in CMT.
Details
- Title: Subtitle
- Motor unit number estimate of distal and proximal muscles in Charcot-Marie-Tooth disease
- Creators
- Richard A Lewis - Department of Neurology, Wayne State University School of Medicine, 4201 St Antoine, Detroit, Michigan 48201, USA. RAlewis@med.wayne.eduJun LiDarren R FuerstMichael E ShyKaren Krajewski
- Resource Type
- Journal article
- Publication Details
- Muscle & nerve, Vol.28(2), pp.161-167
- Publisher
- United States
- DOI
- 10.1002/mus.10419
- PMID
- 12872319
- ISSN
- 0148-639X
- eISSN
- 1097-4598
- Language
- English
- Date published
- 08/2003
- Academic Unit
- Neurology; Molecular Physiology and Biophysics; Stead Family Department of Pediatrics; Iowa Neuroscience Institute
- Record Identifier
- 9984020762502771
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