Yıldız Özlem Kayım, Balaban Hatice, Özdemir Sibel, Bolayır Ertuğrul, Topaktas Suat
Department of Neurology, Cumhuriyet University School of Medicine, Sivas, Turkey.
Neuroophthalmology. 2011 Jan 16;35(1):40-42. doi: 10.3109/01658107.2010.539761. eCollection 2011.
Miller Fisher syndrome is a rare variant of Guillain-Barré syndrome and it is characterised by ophthalmoplegia, ataxia, and areflexia. Pupillomotor involvement occurs in approximately half of the patients with the disorder. The authors report a patient with acute areflexic mydriasis, external ophthalmoplegia, areflexia, and ataxia. Although the pupils were unreactive to light and near stimuli, administration of 0.1% pilocarpine resulted in marked miosis, suggesting cholinergic supersensitivity. Antibodies against GM1, GD1b, and GQ1b were negative. This is the first report of acute areflexic mydriasis with cholinergic supersensitivity in anti-GQ1b-negative Miller Fisher syndrome.
米勒-费希尔综合征是吉兰-巴雷综合征的一种罕见变异型,其特征为眼肌麻痹、共济失调和腱反射消失。约半数该疾病患者会出现瞳孔运动受累。作者报告了1例患有急性无反射性瞳孔散大、眼球外肌麻痹、腱反射消失和共济失调的患者。尽管瞳孔对光和近刺激无反应,但给予0.1%毛果芸香碱后出现明显瞳孔缩小,提示胆碱能超敏反应。抗GM1、GD1b和GQ1b抗体均为阴性。这是抗GQ1b阴性的米勒-费希尔综合征伴胆碱能超敏反应的急性无反射性瞳孔散大的首例报告。