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多数儿童周围神经病为遗传所致,目前尚无有效疗法.慢性炎性脱髓鞘性多发性神经病(CIDP)为散发的后天性疾病,但二者具有相同的临床特征.因此,家族受累的证据就成为鉴别诊断的关键.作者报道3例家族性显性遗传性神经病,并伴有炎性脱髓鞘性神经病的特征,用皮质类甾醇治疗收到良好效果.例1男性,10岁,因下肢无力就诊.检查发现脊柱侧弯,腓肠肌无力,弓形足,跟腱反射消失.14岁时严重近端无力,共济失调步态.其母亲为遗传性运动、感觉神经病(HMSN)Ⅱ型.实验室检查:
The majority of children with peripheral neuropathy are inherited and no effective therapy is available at present.Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired acquired disease, but both have the same clinical features.Therefore, evidence of familial involvement Has become the key to differential diagnosis.3 The authors reported 3 cases of familial dominant hereditary neuropathy, accompanied by inflammatory demyelinating neuropathies, corticosteroid treatment received good results.Example 1 male, 10 years old, due to Lower extremity weakness examination found that scoliosis, gastrocnemius weakness, bow foot, Achilles tendon reflex disappeared .14 years old, severe proximal weakness, ataxia gait .My mother is hereditary exercise, sensory neuropathy (HMSN) type Ⅱ. Laboratory examination: