Yu Hang, Ding Man, Cao Qian, Zhou Rumeng, Yao Jiajia, Fu Rong, Liu Yue, Xiao Zheman, Lu Zuneng
Department of Neurology, Renmin Hospital of Wuhan University, Wuhan, China.
J Clin Neurol. 2023 Nov;19(6):589-596. doi: 10.3988/jcn.2022.0370. Epub 2023 Jul 13.
We aimed to determine the clinical features of Miller Fisher syndrome (MFS) in southern China and compare them with those presenting in other countries.
We collected the medical records of patients diagnosed with MFS during 2013-2016. We analyzed the age, sex, onset season, precursor events, clinical symptoms and signs, findings of nerve conduction studies (NCS), cerebrospinal fluid (CSF), therapeutic remedies, nadir time, and length of hospital stay of patients with MFS in southern China. We concurrently compared the differences between urban and rural areas and between patients with incomplete ophthalmoplegia (IO) and complete ophthalmoplegia (CO).
The study enrolled 72 patients: 36 from rural areas and 36 from urban areas, and 50 males and 22 females. The mean age at onset was 47.72 years, and 30 (41.7%) and 21 (29.2%) patients developed MFS in spring and winter, respectively. The typical triad of ophthalmoplegia, ataxia, and areflexia was observed in 50 (69.4%) patients. A history of upper respiratory tract infection 1 week before onset was found in 52.8% of the patients, while 5.6% experienced gastrointestinal infections and 48 (73.8%) exhibited albuminocytological dissociation in the CSF study. Only 26 (36.1%) patients presented abnormalities in NCS. Moreover, restricted outward eyeball movement presented in 83.5% of the patients with classic MFS and acute ophthalmoplegia, and bilateral symmetrical ophthalmoplegia presented in 64.2%. With the exception of the higher proportion of NCS abnormalities in urban areas (47.2% vs. 25.0%), urban and rural differences were insignificant regarding sex ratio, age at onset, high-incidence season, precursor events, disease characteristics, and albuminocytological dissociation in the CSF. Furthermore, patients with CO were older than those with IO (64.53±7.69 vs. 43.19±14.40 years [mean±standard deviation], <0.001).
The patients with MFS were mostly male and middle-aged, and most presented in winter and (especially) spring. More than half of the patients had clear precursor events, most of which were classic MFS with the typical triad. More than 70% of the patients presented albuminocytological dissociation in the CSF. NCS abnormalities were uncommon in MFS. The age at onset was lower in patients with IO than in patients with CO; bilateral symmetrical extraocular muscle paralysis was the most common symptom, and the external rectus was the most frequently involved muscle.
我们旨在确定中国南方米勒费雪综合征(MFS)的临床特征,并与其他国家的病例进行比较。
我们收集了2013年至2016年期间诊断为MFS的患者的病历。分析了中国南方MFS患者的年龄、性别、发病季节、前驱事件、临床症状和体征、神经传导研究(NCS)结果、脑脊液(CSF)情况、治疗方法、最低点时间和住院时间。同时比较了城乡之间以及不完全性眼肌麻痹(IO)和完全性眼肌麻痹(CO)患者之间的差异。
该研究共纳入72例患者,其中36例来自农村地区,36例来自城市地区,男性50例,女性22例。发病时的平均年龄为47.72岁,分别有30例(41.7%)和21例(29.2%)患者在春季和冬季患MFS。50例(69.4%)患者出现了典型的眼肌麻痹、共济失调和腱反射消失三联征。52.8%的患者在发病前1周有上呼吸道感染史,5.6%的患者有胃肠道感染史,48例(73.8%)患者的脑脊液检查显示蛋白细胞分离。只有26例(36.1%)患者的NCS检查出现异常。此外,83.5%的典型MFS和急性眼肌麻痹患者出现眼球外展受限,64.2%的患者出现双侧对称性眼肌麻痹。除城市地区NCS异常比例较高外(47.2%对25.0%),城乡在性别比例、发病年龄、高发季节、前驱事件、疾病特征和脑脊液蛋白细胞分离方面差异不显著。此外,CO患者的年龄比IO患者大(平均±标准差,64.53±7.69岁对43.19±14.40岁,P<0.001)。
MFS患者以男性和中年为主,多数在冬季和(尤其是)春季发病。超过半数的患者有明确的前驱事件,大多数为具有典型三联征的经典MFS。超过70%的患者脑脊液检查显示蛋白细胞分离。MFS患者NCS异常并不常见。IO患者的发病年龄低于CO患者;双侧对称性眼外肌麻痹是最常见的症状,外直肌是最常受累的肌肉。