Vanderbilt University, Department of Neurology, Nashville, TN 37232, USA.
J Neurol Sci. 2013 Aug 15;331(1-2):67-71. doi: 10.1016/j.jns.2013.05.009. Epub 2013 Jun 5.
To share our experience on clinical presentation and management of patients diagnosed with Hashimoto's Encephalopathy (HE) at Vanderbilt Medical Center between 1999 and 2012.
HE is a rare disorder characterized by encephalopathy and central nervous system (CNS) dysfunction, elevated antithyroid antibodies, the absence of infection or structural abnormalities in the CNS, and a response to treatment with steroids. The relationship between thyroid antibodies and encephalopathy has remained unresolved.
DESIGN/METHODS: Retrospective chart review.
We identified 13 patients who met the criteria for the diagnosis of HE. The median age was 49 years (range, 2-66) and all except one were women. Encephalopathy in the form of altered mental status, stroke-like symptoms or seizures, with prompt resolution of symptoms upon receiving steroids, was the commonest presentation, seen in 7 patients. The second commonest presentation was subacute progressive decrease in cognitive function, which reversed within days to weeks after steroid therapy, seen in 4 patients. Electroencephalogram (EEG) was available in 12 patients and was abnormal in 8, showing nonspecific cerebral dysfunction in all 8 and epileptiform activity in 3. Treatment consisted of steroids in the acute phase for 12 of 13 patients with rapid improvement in symptoms. Maintenance therapy was rituximab in 7 patients, intravenous immunoglobulin (IVIg) in 7, azathioprine in 4, mycophenolate mofetil in 3, and methotrexate in 1 (some patients received sequential therapy with different agents). There was complete or near complete resolution of symptoms in 12 of the 13 patients.
We present a cohort of patients in whom CNS dysfunction was associated with elevated antithyroid antibodies and reversal of disease followed immunomodulatory therapies.
分享我们在范德比尔特医疗中心 1999 年至 2012 年间对诊断为桥本脑病（HE）的患者的临床表现和治疗经验。
HE 是一种罕见的疾病，其特征是脑病和中枢神经系统（CNS）功能障碍、甲状腺自身抗体升高、CNS 无感染或结构异常以及对类固醇治疗有反应。甲状腺抗体与脑病之间的关系仍未解决。
设计/方法：回顾性病历审查。
我们确定了符合 HE 诊断标准的 13 名患者。中位年龄为 49 岁（范围，2-66 岁），除 1 人外均为女性。脑病的表现形式为精神状态改变、类似中风的症状或癫痫发作，在接受类固醇治疗后症状迅速缓解，7 名患者出现这种表现。第二种常见的表现是亚急性认知功能逐渐下降，4 名患者在类固醇治疗后数天至数周内症状逆转。12 名患者中有 12 名进行了脑电图（EEG）检查，8 名异常，8 名均显示非特异性脑功能障碍，3 名显示癫痫样活动。13 名患者中的 12 名在急性期接受类固醇治疗，症状迅速改善。7 名患者接受利妥昔单抗、7 名患者接受静脉注射免疫球蛋白（IVIg）、4 名患者接受硫唑嘌呤、3 名患者接受霉酚酸酯、1 名患者接受甲氨蝶呤（部分患者接受不同药物的序贯治疗）作为维持治疗。13 名患者中的 12 名症状完全或几乎完全缓解。
我们报告了一组患者，其 CNS 功能障碍与甲状腺自身抗体升高有关，并且疾病在免疫调节治疗后逆转。
