Department of Medicine, Greater Baltimore Medical Center, Baltimore, MD 21204, USA.
Seizure. 2011 Nov;20(9):717-9. doi: 10.1016/j.seizure.2011.04.011.
Hashimoto's encephalopathy is an under-recognized and rare disease that presents a diagnostic conundrum since many features are suggestive of infectious etiologies and yet treatment is immunosuppressive medications. We describe a case of a male with a history of recurrent seizures who presented with a subacute onset of confusion, persistent leukocytosis and fever while on immunosuppressant therapy, whose response to high dose steroids was incomplete and who achieved a complete remission of all symptoms when plasmapheresis was initiated. A negative cerebrospinal fluid (CSF) and serum microbiological testing in a patient with an abnormal EEG, increased CSF protein, and elevated thyroid antibodies should prompt consideration of Hashimoto's encephalopathy. If after treating with high dose steroids, there is a sub-optimal clinical response, plasmapheresis should be considered.
桥本脑病是一种未被充分认识且罕见的疾病,由于其许多特征提示感染性病因,而治疗却采用免疫抑制药物,这给诊断带来了难题。我们描述了一例有复发性癫痫病史的男性患者,其亚急性起病,表现为意识混乱,持续性白细胞增多和发热,此时正在接受免疫抑制剂治疗,大剂量类固醇治疗反应不完全,而当开始血浆置换时,所有症状完全缓解。对于脑电图异常、脑脊液蛋白升高和甲状腺抗体升高的患者,如果脑脊液和血清微生物学检测为阴性,应考虑桥本脑病。如果大剂量类固醇治疗后临床反应不佳,应考虑血浆置换。