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本文报告一家系四代58个成员中19人(32.7％)患HMSN,其中男11例、女8例,发病年龄2～20岁(平均16.2岁),病程8～60年(平均29.2年)。其临床表现、EMG和肌活检等改变均符合HMSNⅠ型的特征。呈常染色体显性遗传。
In this paper, 19 HCCNs (32.7%) were reported in a family of 58 members of four generations, including 11 males and 8 females with a mean age of 16.2 years and a mean duration of 2 years to 20 years (range, average of 29.2 years) . The clinical manifestations, EMG and muscle biopsy and other changes are in line with the characteristics of HMSN Ⅰ type. An autosomal dominant inheritance.