【摘要】 目的探讨Miller-Fisher综合征(简称Fisher综合征)共济失调的机制和定位问题。方法综合分析9例Fisher综合征患者的临床特征和辅助检查结果,并复习相关文献,探讨Fisher综合征共济失调的机制与定位诊断。结果本组9例患者均有眼外肌麻痹、共济失调和四肢腱反射减弱或消失的Fishen三联征,具有Fisher综合征的典型临床特征。所有9例患者的头颅CT及MRI检查均无异常,其中5例患者行脑电图检查结果正常。检查结果显示这些Fisher综合征患者缺乏因小脑受损导致小脑性共济失调的形态学基础。结论目前尚不能确定Fisher综合征患者发生共济失调的原因为小脑性病变。眼外肌麻痹所致共济失调是否是其机制之一,尚有待于进一步研究明确。更多还原

【Abstract】 Objective To explore the mechanisms and the location of the lesion which result in ataxia in patients with Miller Fisher syndrome (short for Fisher syndrome). Methods The clinical features were analyzed and auxiliary examinations were performed in 9 patients with Miller Fisher syndrome, while the pertinent literature was reviewed to analyze the possible mechanism responsible for ataxia in Fisher syndrome. Results All the nine patients manifested the specific clinical features of Fisher syndrome, including external ophthalmoplegia, ataxia, and attenuated or obsolescent tendon reflexes in all the limbs. 8 patients complained diplopia. The symptoms of internal ophthalmoplegia, mydriasis and loss of pupillary light reflex were found in 8 patients. Peripheral facial paralysis was found in 5 cases, glossopharyngeal nerve and vagus nerve paralysis were found in 1 patient. Cerebrospinal fluid (CSF) examinations were performed in 7 patients and showed increase in protein contemt in 6 patients, but CSF specimens in all patients were devoid of leucoytc. Electromyogram (EMG) indicated the existence of myogenic damages in 5 patients. Electroencephalogram was performed in 5 patients and no abnormity was found. Magnetic resonance imaging (MRI) or computerized tomography (CT) scanning of the brain did not reveal any significant abnormities in the cerebellum in all the patients. All the nine patients, who received dexamethasone and some other routine neuroprotective therapies, had a good prognosis. Conclusions On the basis of present study, no definite evidences to support that cerebellar lesion may cause ataxia in the patients with Fisher syndrome. It is assumed that external ophthalmoplegia may play a role in causing ataxia. Further studies are needed to ascertain the exact mechanisms responsible for ataxia in Fisher syndrome.更多还原