Abstract:
BACKGROUND:Charcot Marie Tooth type 1a (CMT1a) is a primarily demyelinating neuropathy, characterized by slowly progressive muscle weakness, atrophy, and sensory loss, and is most pronounced in both feet and hands. There is increasing evidence that muscle weakness is determined by motor axonal dysfunction. OBJECTIVE:To investigate in patients with CMT1a whether motor axon loss, as estimated with motor unit number estimation (MUNE) and compound muscle action potential (CMAP), is related to hand function and manual dexterity. METHODS:Hand function, manual dexterity, and axon loss were studied in 48 patients with proven CMT1a. Using high-density surface EMG on the thenar muscles, MUNE was determined and CMAPs were measured. RESULTS:Pinch strength, clawing of the fingers, and manual dexterity correlated significantly with MUNE and CMAP (amplitude and area), while sensory impairments did not. Grip strength correlated significantly with CMAP amplitude but did not become significant with MUNE and CMAP area. Neurophysiologic variables were particularly associated with fine motor function of the hand. CONCLUSIONS:Motor axon loss is likely to be the major cause of hand dysfunction and impaired manual dexterity in Charcot Marie Tooth type 1a (CMT1a). In a clinical setting, the evaluation of the hands of patients with CMT1a should thus be mainly directed toward the evaluation of fine motor functions.
journal_name
Neurologyjournal_title
Neurologyauthors
Videler AJ,van Dijk JP,Beelen A,de Visser M,Nollet F,van Schaik INdoi
10.1212/01.wnl.0000327643.05073.ebsubject
Has Abstractpub_date
2008-10-14 00:00:00pages
1254-60issue
16eissn
0028-3878issn
1526-632Xpii
71/16/1254journal_volume
71pub_type
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