慢性炎症性脱髓鞘性多发性神经病呈家族性发病者未见报导。本文报导一家族中有姐妹二人同患本病。姐来诊时,9岁,主诉行走困难,但智能正常。母亲怀孕、分娩和出生后期发育均正常。14个月时开始走路,但步态不稳,随后渐出现马蹄足和脊柱侧弯。查体发现有:四肢肌萎缩,肌力弱,腱反射消失;肢体远端有感觉减退。周围神经无增粗现象。二年后,其妹在4岁9个月时以同样症状就诊。表现与其姐类似。家族中无多发性神经病的病史,仅其母呈轻度高弓足。二例患者的血尿常规检查及多种肌酶、溶酶体酶和细菌学检查均阴性。2例均示运动神经传导速度减慢;第一例CSF 蛋白增加至630mg/dl;常规免疫学检查正常,HLA 检查例1A_2B_7DR_5/ Chronic inflammatory demyelinating polyneuropathy is a familial disease has not been reported. This article reports a family of two sisters with the disease. When attending the clinic, he was 9 years old and complained of difficulty in walking, but intelligence was normal. Mother’s pregnancy, childbirth and late-stage development are normal. Walking started at 14 months, but the gait was not stable, followed by gradual emergence of the clubfoot and scoliosis. Physical examination found: limb atrophy, weak muscle strength, tendon reflex disappeared; distal sensory loss. Peripheral nerves without thickening phenomenon. Two years later, her sister visits the same symptoms at the age of 4 and 9 months. The performance is similar to that of her sister. Family no history of multiple neuropathy, only the mother showed mild high arch feet. Two patients with routine examination of hematuria and a variety of muscle enzymes, lysosomal enzymes and bacteriological examination were negative. 2 cases showed decreased motor nerve conduction velocity; the first case of CSF protein increased to 630mg / dl; routine immunological examination was normal, HLA test cases 1A_2B_7DR_5 /