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Welander远端肌病为病理上类似于其他肌营养不良、常染体显性遗传的原发性肌病,早期即有神经原性损害.作者对6个该病家庭9位中青年早期患者作了研究,包括详尽的神经、感觉检查、运动、感觉神经传导速度(NCV)、感觉神经动作电位(NAP)、肌电图(EMG)和肌活检.患者男4例、女5例,年龄23～47岁(平均31岁).8例主诉手指活动笨拙,拇、食指对夹无力.检查:9例手指伸肌无力,7例足背屈无力、足跟行走困难.温度觉减退:下肢远端9例,手部7例;感觉减退6例,触觉减
Welander distal myopathy is pathologically similar to other muscular dystrophy, autosomal dominant primary myopathy, early neurogenic damage that is caused by the author of the disease in the family of nine young patients with early childhood (NV), electromyography (EMG) and muscle biopsy were included in the study, including detailed neurological, sensorimotor, motor, sensory nerve conduction velocity (NCV), and muscle biopsy. There were 4 males and 5 females with a mean age of 23 ~ 47 years old (average 31 years old) .8 cases complained of clumsiness of fingers activities, weakness of the thumb and index finger pairs.Check: 9 cases of weakness of the fingers extensor, 7 cases of dorsiflexion weakness, heel difficult to walk. 9 cases in the end and 7 cases in the hand. Six cases had sensory loss and tactile subtraction