Department of Medical Science, Medical School, Catholic Pontifical University of Sao Paulo, Sorocaba, Brazil.
Department of Neurology, Division of Clinical Medicine, Catholic Pontifical University of Sao Paulo, Sorocaba, Brazil.
J Med Case Rep. 2022 Nov 16;16(1):437. doi: 10.1186/s13256-022-03592-4.
Miller-Fisher Syndrome (MFS) is a variant of Guillain-Barré syndrome (GBS), an acute immune-mediated neuropathy, which manifests as a rapidly evolving areflex motor paralysis. This syndrome presents as a classic triad: ophthalmoplegia, areflexia, and ataxia. MFS is usually benign and self-limited.
A Caucasian patient was admitted to our hospital with the flu, loss of bilateral strength in the lower limbs and upper limbs and sudden-onset ataxia 7 days after receiving a first dose of the Oxford/AstraZeneca COVID-19 vaccine. On neurological examination, the patient had Glasgow Coma Scale score of 15, with absence of meningeal signs; negative Babinski sign; grade 2 strength in the lower limbs and grade 4 strength in the upper limbs; axial and appendicular cerebellar ataxia; and peripheral facial diparesis predominantly on the right, without conjugate gaze deviation. Cerebrospinal fluid (CSF) was collected on admission, and analysis revealed albuminocytological dissociation with CSF protein of 148.9 mg/dL; leukocytes, 1; chlorine, 122; glucose, 65 mg/mL; red cells, 2; and non-reactive venereal disease research laboratory test result. The COVID-19 IgG/IgM rapid immunological test was negative. Electroneuromyography revealed a recent moderate-grade and primarily sensory and motor demyelinating polyneuropathy with associated proximal motor block.
Miller-Fisher Syndrome may be related to events other than infections prior to neuropathy, as in the case reported here. The patient presented strong correlations with findings for MFS reported in the literature, such as the clinical condition, the results of electroneuromyography, and results of the CSF analysis typical for MFS. When treatment was provided as proposed in the literature, the disease evolved with improvement. Ultimately, the diagnosis of incomplete MFS was made, including acute ataxic neuropathy (without ophthalmoplegia).
米勒-费舍尔综合征(MFS)是吉兰-巴雷综合征(GBS)的一种变体,是一种急性免疫介导的神经病,表现为迅速进展的反射消失性运动瘫痪。该综合征表现为经典三联征:眼肌瘫痪、反射消失和共济失调。MFS 通常是良性和自限性的。
一名白人患者因流感入院,在接种牛津/阿斯利康 COVID-19 疫苗第一剂后 7 天出现双侧下肢和上肢无力以及突发性共济失调。神经系统检查时,患者格拉斯哥昏迷量表评分为 15 分,无脑膜刺激征;巴宾斯基征阴性;下肢肌力 2 级,上肢肌力 4 级;轴性和肢体小脑共济失调;右侧周围性面瘫,无共轭眼球偏斜。入院时采集脑脊液(CSF),分析显示蛋白细胞分离,CSF 蛋白 148.9mg/dL;白细胞 1;氯 122;葡萄糖 65mg/mL;红细胞 2;非反应性性病研究实验室检测结果。COVID-19 IgG/IgM 快速免疫检测结果为阴性。神经电生理检查显示近期中度感觉运动脱髓鞘多发性神经病,伴有近端运动阻滞。
米勒-费舍尔综合征可能与神经病发生前的感染以外的事件有关,如本例患者。患者的临床表现、神经电生理检查结果以及 CSF 分析结果均与文献报道的 MFS 相符。根据文献建议进行治疗后,病情改善。最终诊断为不完全性 MFS,包括急性共济失调性神经病(无眼肌瘫痪)。