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[临床诊断为皮质基底节变性患者的瞳孔异常]

[Pupillary abnormalities in a patient with clinically diagnosed corticobasal degeneration].

作者信息

Hirabayashi H, Takahashi W, Shinohara Y, Takagi S

机构信息

Department of Neurology, Tokai University School of Medicine.

出版信息

Rinsho Shinkeigaku. 1997 Apr;37(4):344-6.

PMID:9248348
Abstract

We report a 65-year-old woman, who suffered from clumsiness of the left hand for 6 years. Gait disturbance, body balance impairment and rigidity of the left arm appeared progressively. Anti-parkinsonian drugs such as 1-dopa, doroxidopa, amantadine and bromocriptine were ineffective. Magnetic resonance imaging showed brain atrophy, especially in the right fronto-parietal lobes. Positron emission tomography (18F-fluoro-2-deoxy-D-glucose) showed decreased glucose metabolism in the right cerebral cortex, putamen and thalamus. A diagnosis of corticobasal degeneration was made, based on the clinical manifestations, MRI and PET findings. She also showed anisocoria, vertical gaze palsy and abnormal pupillary reactions. Her left pupil showed an oval figure shifted from the center position. The pupils exhibited denervation supersensitivity in the 0.125% pilocarpine test. We consider that her pupillary abnormalities may be related to her midbrain lesion (midbrain corectopia) associated with corticobasal degeneration.

摘要

我们报告一位65岁女性,她左手笨拙已有6年。步态障碍、身体平衡受损和左臂僵硬逐渐出现。左旋多巴、司来吉兰、金刚烷胺和溴隐亭等抗帕金森病药物均无效。磁共振成像显示脑萎缩,尤其是右侧额顶叶。正电子发射断层扫描(18F-氟-2-脱氧-D-葡萄糖)显示右侧大脑皮质、壳核和丘脑葡萄糖代谢降低。根据临床表现、MRI和PET检查结果,诊断为皮质基底节变性。她还出现了瞳孔不等大、垂直凝视麻痹和异常瞳孔反应。她的左瞳孔呈椭圆形,偏离中心位置。在0.125%毛果芸香碱试验中,瞳孔表现出失神经超敏反应。我们认为她的瞳孔异常可能与她与皮质基底节变性相关的中脑病变(中脑异位瞳孔)有关。

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