Battaglia F, Attane F, Robinson A, Martini L, Siboni J, Tannier C
Service de Neurologie, Centre Hospitalier A. Gayraud, Carcassonne.
Rev Neurol (Paris). 2005 Sep;161(8-9):844-7. doi: 10.1016/s0035-3787(05)85146-x.
Miller-Fisher syndrome (MFS) is a rare auto-immune post-infectious syndrome, characterized by an ataxia, an ophthalmoplegia and a generalized areflexia. It is considered as a clinical variant of Guillain-Barré syndrome (GBS). MFS is correlated with the presence of anti-GQ1b antibodies, elevated cerebrospinal fluid (CSF) protein levels, presence of mostly sensitive electrophysiological abnormalities and for some authors central involvement with increased signal intensity of brainstem and cerebellum on MRI. Recurrent MFS is extremely rare with only 21 cases since the first description in 1970.
A 54-year-old women presented MFS with two episodes in 19 years. Clinically, the first episode was a "classical" MFS, and the second an extensive MFS with tetraparesis and respiratory failure. CSF protein levels and cerebral MRI were normal. Anti-GQ1b antibodies were strongly positive and anti-GM1, anti-GM2 antibodies were slightly positive, campylobacter jejuni serology was negative. Electromyography showed isolated sensory abnormalities in median nerves territory.
We report a new case of recurrent MFS with unusual clinical, biological and electrophysiological features.
米勒-费希尔综合征（MFS）是一种罕见的自身免疫性感染后综合征，其特征为共济失调、眼肌麻痹和全身性反射消失。它被认为是吉兰-巴雷综合征（GBS）的一种临床变异型。MFS与抗GQ1b抗体的存在、脑脊液（CSF）蛋白水平升高、大多为敏感的电生理异常的存在相关，并且对于一些作者而言，还与MRI上脑干和小脑信号强度增加的中枢受累有关。复发性MFS极为罕见，自1970年首次描述以来仅有21例。
一名54岁女性在19年中出现了两次MFS发作。临床上，第一次发作是“典型”的MFS，第二次是伴有四肢轻瘫和呼吸衰竭的广泛性MFS。CSF蛋白水平和脑部MRI正常。抗GQ1b抗体呈强阳性，抗GM1、抗GM2抗体呈弱阳性，空肠弯曲菌血清学检查为阴性。肌电图显示正中神经区域存在孤立的感觉异常。
我们报告了一例具有不寻常临床、生物学和电生理特征的复发性MFS新病例。
