Zakład Patofizjologii Widzenia Obuocznego i Leczenia Zeza I Katedry Chorób Oczu, Uniwersytet Medyczny w Łodzi, Łódź, Polska.
Neurol Neurochir Pol. 2011 May-Jun;45(3):297-300. doi: 10.1016/s0028-3843(14)60083-9.
Total, bilateral ophthalmoplegia is very rare. More than 50% of cases are Miller Fisher (MFS) and Guillain-Barré (GBS) syndromes. There is a correlation of MFS with anti-GQ1b antibodies. High levels of GQ1b gangliosides are found in myelin sheathes of cranial nerves supplying the extraocular muscles. This may explain the association of anti-GQ1b antibodies with ophthalmoplegia. Anti-GQ1b were also found in cases of GBS accompanied by ophthalmoplegia, atypical MFS (MFS without ataxia), MFS/GBS overlap syndromes and Bickerstaff brainstem encephalitis. This has led some authors to classify them as 'anti-GQ1b syndromes'. In this article we describe a diagnostically difficult case of a patient with a very rare, total bilateral paralysis of all ocular muscles, accompanied by bilateral ptosis, diminished tendon reflexes of upper extremities, paresis and hypoesthesia of the left upper extremity.
总的来说，双侧眼肌麻痹非常罕见。超过 50%的病例为米勒费舍尔（Miller Fisher，MFS）和吉兰-巴雷（Guillain-Barré，GBS）综合征。MFS 与抗 GQ1b 抗体有关。高浓度的 GQ1b 神经节苷脂存在于供应眼外肌的颅神经髓鞘中。这可以解释抗 GQ1b 抗体与眼肌麻痹的关联。在伴有眼肌麻痹的 GBS、非典型 MFS（无共济失调的 MFS）、MFS/GBS 重叠综合征和 Bickerstaff 脑干脑炎病例中也发现了抗 GQ1b 抗体。这导致一些作者将其归类为“抗 GQ1b 综合征”。在本文中，我们描述了一例诊断困难的患者，其患有非常罕见的、双侧所有眼肌完全瘫痪，伴有双侧上睑下垂、上肢腱反射减弱、左侧上肢无力和感觉减退。
Zotero 插件