RECURRENT FAMILIAL NEUROPATHY WITH LIABILITY TO PRESSURE PALSIES - REPORTS OF 2 CASES AND ULTRASTRUCTURAL NERVE STUDY

被引：23

作者：

DUBI, J

REGLI, F

BISCHOFF, A

SCHNEIDER, C

CROUSAZ, GD

机构：

[1] CTR HOSP UNIV VAUDOIS,DEPT NEUROL,CH-1011 LAUSANNE,SWITZERLAND

[2] UNIV BERN,INSELSPITAL,DEPT NEUROL,CH-3010 BERN,SWITZERLAND

来源：

JOURNAL OF NEUROLOGY | 1979年 / 220卷 / 01期

关键词：

Electrophysiological study; Hereditary neuropathy; Metabolic polyneuropathy; Traumatic nerves lesions; Ultrastructural nerve study;

D O I：

10.1007/BF00313147

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Electrophysiological, histological and ultrastructural studies on two patients (one family) with familial neuropathy and a liability to pressure palsies are reported. There was slowing of nerve conduction velocities and increase of distal latencies of clinically affected and clinically non-affected nerves. These alterations were confirmed by biopsy of the sural nerve which had the characterized signs of the disease. Pathogenetically there seems to be an endogeneous metabolic defect of the Schwann cell to synthetize a normal myelin sheath, which is responsible for the particular susceptibility to mechanical damage. © 1979 Springer-Verlag.

引用

页码：43 / 55

页数：13