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运动神经元病、脊髓性肌萎缩、背髓灰质炎后肌萎缩、氨基已糖苷酯酶A缺乏、铅中毒及多灶性运动神经病(MMN)均以无感觉障碍的进行性肢体无力及肌萎缩为主要临床表现,但只有MMN是可以治疗的疾病,其诊断依据包括进行性非对称性的下运动神经元瘫痪,电生理检查提示多灶性运动纤维脱鞘伴部分运动传导阻滞,感觉诱发电位正常,血清抗糖脂抗体(antiglycolipid antibodies)滴度升高。血清抗糖脂抗体的出现、免疫抑制剂治疗有效及诸多与CIDP相同的电生理特点提示MMN的发病机制与免疫介导有关、强地松和血浆交换未取得满意疗效。环磷酰胺(cyclophosphamide,CTX)是目前报道的唯一可改善MMN症状的免疫抑制剂,但常有严重的副作用。本文作者应用人体免疫球蛋白(HIG)治疗了9例MMN,取得较好疗效。
Motor neuron disease, spinal muscular atrophy, postmortem muscular atrophy, aminoglycoside esterase A deficiency, lead poisoning and multifocal motor neuropathy (MMN) are characterized by non-sensory progressive limb weakness and amyotrophy As the main clinical manifestations, but only MMN is a treatable disease, its diagnosis based on progressive asymmetry of lower motor neurons paralysis, electrophysiological examination suggests that multifocal motor fiber dehiscence with partial motor block, sensory evoked Normal potentials, elevated serum anti-glycolipid antibodies. The emergence of serum anti-glycolipid antibodies, the effectiveness of immunosuppressive agents and many of the same electrophysiological characteristics of CIDP suggest that the pathogenesis of MMN is associated with immune mediation, and that there is no satisfactory response to prednisone and plasma exchange. Cyclophosphamide (CTX) is currently the only immunosuppressant reported to improve MMN symptoms, but often has serious side effects. The authors applied human immunoglobulin (HIG) treatment of 9 cases of MMN, and achieved good results.