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1例抗GQ1b阳性的不伴有外眼肌麻痹但伴有内眼肌麻痹的非典型费舍尔综合征

[A case of anti-GQ1b-positive atypical Fisher syndrome with internal ophthalmoplegia but without external ophthalmoplegia].

作者信息

Mori M, Takeshima T, Yuki N, Nakashima K

机构信息

Division of Neurology, Institute of Neurological Sciences, Faculty of Medicine, Tottori University.

出版信息

Rinsho Shinkeigaku. 2000 Sep;40(9):887-90.

Abstract

We report a 21-year-old man who developed an atypical form of Fisher syndrome. One week after having a common cold, he was admitted to our hospital because of a gait disturbance. Neurological examination revealed a somnolent state, cerebellar ataxia, areflexia, limb muscle weakness, and numbness in a glove and stocking like distribution. The patient had internal ophthalmoplegia but did not have external ophthalmoplegia. Brain MRI showed no abnormality in the orbital and the pretegmental brain regions. The protein level in the cerebrospinal fluid was 57 mg/dl and the cell count was 5 mononuclear cells/mm3. His serum titer of anti-GQ1b IgG antibody was markedly elevated. There have been only two previous reports of isolated internal ophthalmoplegia with elevated anti-GQ1b antibody. The present case suggests that anti-GQ1b antibody play an important role in the pathogenesis of patients who present with internal ophthalmoplegia but without external ophthalmoplegia.

摘要

我们报告了一名21岁的男性,他患上了一种非典型形式的费舍尔综合征。在患普通感冒一周后,他因步态障碍入住我院。神经系统检查发现嗜睡状态、小脑共济失调、反射消失、肢体肌肉无力以及手套和袜套样分布的麻木感。该患者有眼内肌麻痹,但没有眼外肌麻痹。脑部磁共振成像显示眼眶和中脑顶盖前区无异常。脑脊液中的蛋白质水平为57mg/dl,细胞计数为5个单核细胞/mm³。他的抗GQ1b IgG抗体血清滴度显著升高。此前仅有两篇关于抗GQ1b抗体升高伴孤立性眼内肌麻痹的报告。本病例提示抗GQ1b抗体在表现为眼内肌麻痹但无眼外肌麻痹的患者发病机制中起重要作用。

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