The clinical analysis of neuroelectrophysiologic diagnosis in bereditary neuropathy with liability to pressure palsies: four cases of one pedigree

Abstract

Objective To investigate the electrophysiological changes of peripheral nerves in both patients with hereditary neuropathy with liability to pressure palsies(HNPP).Methods The nerve conduction velocities of a family consisting of a proband and 4 members were tested,and the proband was detected by biopsy.Results The proband's median nerve,ulnar nerve,sural sensory nerve conduction velocity (SCV) were decreased.The median nerve,ulnar nerve,common peroneal nerve motor nerve conduction velocity(MCV) of proband were decreased.The sensory fibers of the most frequently involved nerve sural nerve,sural nerve damage to the results:the motor nerve conduction motor nerve evoked potential latency and 18 abnormal rate was 75.0％,the nerve distal to the abnormal rate of MCV was 87.5％.Conclusion Prolongation of distal nerve conduction latencies occurs in essentially all individuals whether symptomatic or asymptomatic.Nerves may be more easily damaged at the site of compression. Key words: Hereditary motor and sensory neuropathies; Electrophysiology; Neural conduction