Iwahashi T, Inoue A, Koh S, Yanagihara N
Department of Internal Medicine, School of Medicine, Shinshu University.
Arerugi. 1995 Sep;44(9):1176-80.
We here report a case of Miller Fisher syndrome (MFS) in which serum anti-cerebellar antibody was detected by Western blot analysis. The 32-year-old male studied suffered from diplopia, gait ataxia and sensory disturbance in the distal portion of the upper limbs preceded by cold-like symptoms. Neurological examination on admission revealed that he had external ophthalmoplegia with bilateral ptosis, cerebellar ataxia and areflexia. A cerebrospinal fluid examination showed albuminocytologic dissociation with a protein concentration of 60 mg/dl. Brain CT and MRI showed no significant abnormalities. The patient was diagnosed as MFS, and treated it with two sessions of immunoadsorption plasmapheresis (IAPP). After receiving IAPP therapy, the patient's neurological symptoms and signs were improved. Western blot analysis showed the existence of antibody directed against mouse cerebellum but not against mouse cerebrum, brain stem, and spinal cord in his serum, the level of which was decreased after the IAPP therapy. Serum anti-GQ1b antibody was also elevated. As far as we are aware, there have been no reports showing the existence of anti-cerebellar antibodies detected by Western blot analysis. Though the pathogenesis of MFS remains unclear, our findings suggest that anti-cerebellar antibody detected by Western blot analysis may be caused by cerebellar ataxia in MFS.
我们在此报告一例通过蛋白质印迹分析检测出血清抗小脑抗体的米勒-费希尔综合征(MFS)病例。该32岁男性患者在出现类似感冒症状后,出现复视、步态共济失调和上肢远端感觉障碍。入院时的神经系统检查发现他有双侧眼睑下垂的眼外肌麻痹、小脑共济失调和无反射。脑脊液检查显示蛋白细胞分离,蛋白浓度为60mg/dl。脑部CT和MRI未显示明显异常。该患者被诊断为MFS,并接受了两次免疫吸附血浆置换(IAPP)治疗。接受IAPP治疗后,患者的神经症状和体征有所改善。蛋白质印迹分析显示其血清中存在针对小鼠小脑而非小鼠大脑、脑干和脊髓的抗体,IAPP治疗后该抗体水平降低。血清抗GQ1b抗体也升高。据我们所知,尚无通过蛋白质印迹分析检测到抗小脑抗体存在的报道。尽管MFS的发病机制仍不清楚,但我们的研究结果表明,通过蛋白质印迹分析检测到的抗小脑抗体可能是MFS中小脑共济失调的原因。