Pure motor chronic inflammatory demyelinating polyneuropathy
Pure motor chronic inflammatory demyelinating polyneuropathy
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DOI：
10.1007/s004150170093
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发表时间：
2001-09-01
影响因子：
6
通讯作者：
Tonali, P
中科院分区：
文献类型：
--
作者：
Sabatelli, M;Madia, F;Tonali, P
我们描述了四名患有慢性炎症性脱髓鞘多神经病(CIDP)的纯运动型患者。无感觉症状，神经学检查感觉正常，感觉纤维电生理检查和腓肠神经活检结果正常，提示运动纤维受累。发病年龄较小(3-29岁)，临床病程为复发-缓解期。在1.5~14年的随访期内，定期的临床和电生理检查显示选择性累及运动神经仍然是一种恒定的特征。肌电图和神经传导研究继续显示为单纯的脱髓鞘神经病，没有轴突损伤的迹象。所有患者对类固醇无反应，但在接受免疫球蛋白治疗后显著改善。2例患者接受了干扰素α治疗，疗效良好。总而言之，在我们的四名患者中，单纯运动受累的发生在很长一段时间内多次复发，这表明纯粹的运动性CIDP可能代表了特定的免疫过程的结果，而不是炎症在周围神经中随机分布的结果。
We describe four patients affected by chronic inflammatory demyelinating polyneuropathy (CIDP) in a pure motor form. Selective involvement of motor fibers was suggested by the absence of sensory symptoms, normal sensation at neurological examination and normal findings on electrophysiological testing of sensory fibres and sural nerve biopsy. The onset of the disease occurred at a young age (3-29 years) and the clinical course was relapsing-remitting. Over a followup periode of 1.5-14 years, periodical clinical and electrophysiological examinations showed that selective involvement of motor fibers remained a constant feature. Electromyography and nerve conduction studies continued to show a purely demyelinating neuropathy without signs of axonal impairment. All patients were steroid-unresponsive, whereas they considerably improved after being treated with immunoglobulins. Two patients were treated with interferon alpha and showed a good response. In conclusion, the occurrence in our four patients of pure motor involvement over a long period of time during which several relapses occurred, suggests that pure motor CIDP may represent the result of a specific immunological process rather than of a random distribution of inflammation throughout peripheral nerves.