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16岁女孩5年来走路逐渐困难.体检发现痉挛步态,下肢无力和肌张力增高.膝和踝反射活跃,双侧踝阵挛和双侧巴彬斯基征.正中神经感觉及腓骨神经运动纤维的传导速度均为低限正常值.四兄、一姐和母亲的下肢肌张力增高,膝和踝反射活跃.姐和两兄有巴彬斯基征和踝阵挛.电子显微镜所见:大多数神经纤维正常,但有些粗大有髓纤维的髓鞘发生断裂,且不能完全包围
16-year-old girl walked gradually difficult for 5 years.Patients with spastic gait, lower extremity weakness and muscular tension were found on physical examination.Knee and ankle reflexes were active, bilateral ankle clonus and bilateral Babinski’s sign.Central nerve sensory and peroneal nerve fibers Of the conduction velocity of the lower limit of normal .Four brothers, a sister and her mother’s lower extremity muscle tension, knee and ankle reflex active sister and the two brothers Babinski sign and ankle clonus Electron Microscopy seen: large Most nerve fibers are normal, but myelin sheaths of some coarse myelinated fibers break and can not completely surround