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[1例与髓鞘相关糖蛋白神经病变相关的多发性硬化症]

[A case of multiple sclerosis associated with myelin associated glycoprotein neuropathy].

作者信息

Nakane S, Furuya T, Shirabe S, Nakamura T, Yoshimura T

机构信息

First Department of Internal Medicine, Nagasaki University School of Medicine.

出版信息

Rinsho Shinkeigaku. 1998 Dec;38(12):1042-8.

Abstract

A 28-year-old woman had developed chronic, recurrent, visual disturbance (bilateral), and girdle sensation at Th 5-6. She was admitted to our hospital because of left visual disturbance, distal limb weakness on right side, and numbness of four extremities. The neurological examination revealed decreased visual acuity of the left eye with abnormality of the optic disk, moderate muscle weakness of the right upper and lower extremities, absent tendon reflexes and paresthesia on distal portions of the four limbs. Laboratory examinations disclosed the titration of anti-myelin associated glycoprotein (MAG) antibody (IgM) and CSF protein was elevated (104 mg/dl). Motor nerve conduction studies revealed conduction block in more than one nerve. The conduction velocities in the upper and lower extremities were all diminished. P100 latency was prolonged by flash visual evoked potential (VEP) studies. N13-N20 interpeak latency of somatosensory evoked potential (SEP) of median nerve was also prolonged. She was treated with steroid pulse therapy, followed by an oral dose of 30 mg/day of prednisolone. Her symptoms resolved completely three months later, and multifocal conduction block subsided on electrophysiological study. There are some cases of multiple sclerosis with multifocal conduction block, but such a case is very rare in Japan. We discussed the pathogenic mechanisms of these conditions, and we conclude that we must take notice of demyelinating neuropathy in multiple sclerosis and that nerve conduction studies are useful for detecting them.

摘要

一名28岁女性出现慢性复发性双侧视觉障碍及胸5-6水平束带感。因左眼视觉障碍、右侧肢体远端无力及四肢麻木入住我院。神经系统检查发现左眼视力下降、视盘异常、右侧上下肢中度肌无力、腱反射消失及四肢远端感觉异常。实验室检查显示抗髓鞘相关糖蛋白(MAG)抗体(IgM)滴度升高,脑脊液蛋白升高(104mg/dl)。运动神经传导研究显示多条神经存在传导阻滞。上下肢传导速度均减慢。闪光视觉诱发电位(VEP)研究显示P100潜伏期延长。正中神经体感诱发电位(SEP)的N13-N20峰间潜伏期也延长。给予类固醇冲击治疗,随后口服泼尼松龙30mg/天。三个月后症状完全缓解,电生理研究显示多灶性传导阻滞消失。有多发性硬化伴多灶性传导阻滞的病例,但在日本这种病例非常罕见。我们讨论了这些情况的发病机制,得出结论,在多发性硬化中必须注意脱髓鞘性神经病,神经传导研究有助于检测它们。

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