桥本氏脑病的临床特点

Clinical features of Hashimoto's encephalopathy

目的探讨桥本氏脑病(HE)的临床特点。方法对本院收治的11例和文献报道的17例HE患者的临床资料进行回顾性分析。结果本组男5例,女23例;年龄21～81岁;伴有甲状腺功能亢进3例,亚临床甲状腺功能亢进4例,亚临床甲状腺功能减低1例。首发症状为智能下降、癫痫、精神症状、头痛、卒中样发作、行走不稳、行动迟缓、构音和吞咽障碍。主要临床症状依次为智能下降23例(82.1%),癫痫发作14例(50.0%),精神症状14例(50.0%),意识障碍7例(25.0%),头痛7例(25.0%),行动迟缓4例(14.3%),发热3例(10.7%),卒中样发作3例(10.7%),构音/吞咽障碍2例(7.1%),复视1例,伴有心脏受累2例,多发大动脉炎及雷诺病各1例。26例患者查血甲状腺球蛋白抗体及27例查血甲状腺过氧化物酶抗体均显著升高。21例患者行脑电图检查,17例异常,均为全脑的广泛慢波。26例头颅MRI检查17例异常,主要累及皮质和皮质下白质,大多呈边界模糊的斑片状长T1、长T2;T2Flair呈高信号,部分为混杂信号。糖皮质激素治疗均有明显的疗效,1例死亡,其余预后良好。结论 HE多见于中年以上的女性,首发及主要症状以双侧脑皮质或皮质下白质损害症状为主,甲状腺相关抗体指标显著升高;MRI可见分布广泛的斑片状长T1、长T2信号病灶。早期糖皮质激素治疗预后较好。

Objective To investigate the clinical features of Hashimoto＇s encephalopathy （HE）. Methods The clinical data of 11 HE patients who treated in our hospital and other 17 HE patients from literatures were analyzed retrospectively. Results There were 5 males and 23 females in this group, aged ranged from 21 - 81. Hyperthyroidism was in 3 cases, subclinical hyperthyroidism in 4 cases, and subclinic hypothyrodism in 1 case. The first symptoms were decrease of intelligence, seizures, psychiatric symptom, headache, stroke-like episodes, unstable gait, motor retardation and articulation/deglutition disorders. The main clinical symptoms were in sequence of decrease of intelligence in 23 cases （82.1%）, seizure disorder in 14 cases （50.0%）, psychiatric symptom in 14 cases （50.0%） , consciousness disorders in 7 cases （25.0%） , headeach in 7 cases （25.0%） , motor retardation in 4 cases （ 14.3% ）, fever in 3 cases （ 10.7% ）, stroke-like episodes in 3 cases （ 10.7% ）, articulation/deglutition disorders in 2 cases （7. 1% ） , diplopia in 1 case and accompanied with heart involvement in 2 cases, takayasu artritis and raynaud disease in 1 case separately. There were significantly higher in thyroglobulin antibody measured in 26 cases and thyroid peroxidase measured in 27 cases. Electrocardiogram examination was checked in 21 cases, it showed that 17 cases （80.95%） were abnormal, and there was widely slow wave in whole brain. MRI was checked in 26 cases, and 17 cases were abnormal, and it mainly involved in the cortex and subcortical white. Blurred boundary was plaque-like, and it showed high T,, high T2, high T2 Flair signal and mixed signal partially. Corticosteroids treatment was effective. One case was died, the other had good prognosis. Conclusions HE is mainly seen in in the middle-aged women. First and mainly clinical symptoms are bilateral cortex and subcortical white＇s symptoms. Thyroid-related antibodies are significantly increased. MRI showed plaque-like lesions widely, and it is high T1, high T2. Early corticosteroids therapy would be better effective.