Type 1 and 2 differences research from China

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Abstract from Zhonghua Nei Ke Za Zhi 2002 Nov;41(11):736-8

The clinical and pathologic features of Charcot-Marie-Tooth disease.

Xiao B, Xie J, Yang X, Wu Z, Xiao J, Li J.

Department of Neurology, Xiangya Hospital, the Central South University,

Changsha 410008, China.

OBJECTIVE: To study the clinical and pathological features of

Charcot-Marie-Tooth disease (CMT). METHODS: The general clinical data,

the changes of neuroelectrophysiology and the pathological features of

neural and muscular biopsy of 20 CMT patients were comprehensively

analyzed. RESULTS: The clinical manifestations in the two types of CMT

were nearly the same, but the changes of neuroelectrophysiology and the

pathological characteristics of the neural and muscular biopsy in the

two

types were obviously different. 16 cases of CMT type 1 were

characterized by decreased sensory nerve conduction velocity (SNCV < 30

m/s) and are associated with demyelinating hypertrophic neuropathy. 4

cases of CMT type 2 were characterized by normal SNCV and associated

with axonopathy. CONCLUSION: Neuroelectrophysiology and

neural and muscular biopsy are important for the diagnosis of CMT.