目的 探讨伴自身免疫性甲状腺炎的肾上腺皮质激素反应性脑病的发病形式、临床表现、实验室检查以及影像学特点,以期早期明确诊断和及时治疗.方法 回顾分析17例临床诊断明确的伴自身免疫性甲状腺炎的肾上腺皮质激素反应性脑病患者的临床资料,总结其发病特点、诊断及治疗原则.结果 17例患者分别以癫(癎)发作(4例)、精神情绪异常(4例)、记忆力减退(4例)、行走不稳(2例)、头痛(2例)、构音障碍(1例)发病；其中急性发病3例、亚急性发病5例、慢性发病9例.所有患者血清抗甲状腺过氧化物酶抗体水平均升高,15例抗甲状腺球蛋白抗体水平升高；脑脊液蛋白定量轻度升高；脑电图以慢波(5例)或(癎)样放电(2例)为主,MRI表现为颅内多发病灶(11例)；临床免疫学指标及肿瘤标志物均于正常值范围.甲泼尼龙(11例)或地塞米松(3例)治疗有效且预后良好,仅2例复发.结论 伴自身免疫性甲状腺炎的肾上腺皮质激素反应性脑病发病形式多样,以癫(癎)发作、认知功能障碍、情绪改变为主要表现,血清抗甲状腺抗体水平升高,MRI可见灰质和白质多发病变.%Objective To investigate the onset pattern,clinical manifestations,laboratory findings and imaging features of 17 Chinese patients with steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT).Methods The clinical data of 17 SREAT patients were collected.Retrospective analysis of their clinical features,as well as their serum levels of anti-thyroid,cerebrospinal fluid (CSF)biochemical indicators,MRI and therapy was performed.Results The initial symptoms of those patients were seizures (4 cases),psychiatric symptoms (4 cases),hypomnesis (4 cases),walking unsteadiness (2 cases),headache (2 cases) and dysarthria (1 case).Three cases were acute onset,5 cases subacute onset and 9 cases chronic onset.The anti-thyroid peroxidase antibody (anti-TPO) of 17 cases were significantly increased,average (928.63 ± 406.28)× 103 IU/L.The anti-thyroglobulin antibody (anti-TG) of 15 cases was increased,average (601.27 ± 1014.12) × 103 IU/L.The protein in CSF was mildly increased,average (513.75 ± 283.15) mg/L.The EEG of 5 patients presented slow wave and the EEG of 2 patients showed epileptiform discharge.The brain MRI of 11 patients showed multifocal lesions in frontal lobe,temporal lobe,parietal lobe,basal ganglia,centrum ovale,corpus callosum,thalamus,cerebellum,and brain stem.The findings of clinical immunological index and tumor markers were normal.Besides,the prognosis of 11 patients treated with methylprednisolone and 3 patients treated with dexamethasone were good.Recurrence occurred in 2 patients.Conclusion Basically,the clinical features of Chinese SREAT patients present seizures,hypomnesis and psychiatric symptoms associated with increased anti-thyroid and multifocal lesions in gray and white matter of brain.
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