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目的从临床、电生理及病理方面对遗传性压迫易感性神经病(HNPP)进行探讨,以提高对本病的认识.方法4例经临床及腓肠神经活检确诊,肌电图检测临床受累或未受累的肢体的感觉或运动传导速度;腓肠神经活检标本分别做HE、MASON染色和FLEMMING染色光镜观察.结果肌电图为广泛神经传导异常,甚至出现在临床未受累的神经支配区;腓神经活检显示部分神经纤维明显增粗,轴索正常.结论HNPP与遗传关系密切,但也有散发,电生理检查是重要的筛选手段,神经活检见到髓鞘增粗或典型的腊肠样结构是重要的确诊手段“,”s Objective To study the clinical,electrophysiological and neuropathological features of HNPP. Methods 4 cases of HNPP were diagnosed by sural nerve biopsy.Electromyography was recorded in clinical affected or unaffected nerve.Sural nerve biopsies were done and specimens were analyzed by HE,Masson and Flemmings myelin staining respectively. Results Electromyography demonstrated that the nerve conduction velocities were slow in clinical affected and unaffected areas.The nerve biopsies showed the pre...