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.

先证者(Ⅳ10) 男,21岁,先天性裂手伴并指畸形,于出生后4个月行左手中、环指并指分指术.查体见左手虎口发育不全,食指及中指屈曲畸形,右手中指及第3掌骨缺失、分裂至腕骨,拇指发育不良,拇指、示指完全并指(图1)。

先证者(Ⅱ2) 女,49岁.主因双下肢麻木、无力反复发作10余年人院.于久坐或提重物后发作,主要症状为小腿前外侧及后侧、足背麻木,抬脚费力,足趾不能背曲.发病初症状时重时轻,能自行缓解,查体:颅神经无异常,双侧腓浅神经分布区痛觉减低,深感觉减退,双足及足趾不能背曲,足不能外展.四肢腱反射对称减低,病理征未引出.余神经系统检查均正常。