Santa Lucia Foundation, Rome, Italy.
Neurol Sci. 2012 Jun;33(3):653-6. doi: 10.1007/s10072-011-0813-z. Epub 2011 Oct 13.
Hashimoto encephalopathy (HE) is a rare and often reversible neurological syndrome associated with autoimmune thyroiditis and steroid-responsiveness. This syndrome includes behavioral symptoms like delusions and delirium, mood disturbances, epilepsy, progressive cognitive impairment and alteration of vigilance and consciousness with confused state until coma. Two subtypes of clinical presentation are described: ictal onset with seizures and stroke like episode and insidious onset with progressive dementia. The pathogenesis is uncertain; several theories have been proposed: autoimmune, vasculitic and demyelinating. Here, we report the case of a patient with HE who was submitted to exhaustive neuropsychological exams in the premorbid and the acute phase, and following resolution of the acute phase. After the initial confusional state resolved, results of the neuropsychological exams revealed a diffuse pattern of cognitive impairment that eventually evolved toward a selective deficit in executive functions. This pattern of cognitive impairment suggests that, after an initial phase characterized by diffuse brain involvement, our patient was primarily affected by frontal lobe sufferance.
桥本脑病（HE）是一种罕见且常可逆转的神经系统综合征，与自身免疫性甲状腺炎和类固醇反应性相关。该综合征包括行为症状，如妄想和意识混乱、情绪障碍、癫痫、进行性认知障碍以及警觉和意识状态改变，直至昏迷。描述了两种临床表现亚型：发作性癫痫发作和卒中型发作和隐匿性起病伴进行性痴呆。发病机制尚不确定；已经提出了几种理论：自身免疫、血管炎和脱髓鞘。在这里，我们报告了一例 HE 患者的病例，该患者在发病前和急性期接受了详尽的神经心理学检查，并在急性期后进行了检查。在初始意识混乱状态缓解后，神经心理学检查结果显示出弥漫性认知障碍模式，最终发展为执行功能选择性缺陷。这种认知障碍模式表明，在以弥漫性脑受累为特征的初始阶段之后，我们的患者主要受到额叶受累的影响。
