【摘要】 目的探讨桥本脑病(Hashimoto’s encephalopathy,HE)的发病机制、临床、影像学和病理学特点及预后。方法回顾性分析1例经临床和病理证实的HE患者的临床资料及治疗和预后情况。结果该患者表现为卒中样发作,伴快速进展的认知功能障碍和癫痫;血清抗甲状腺抗体显著增高,头颅MRI可见双颞叶内侧、海马及左基底节区、放射冠多发斑片状长T1、长T2以及FLAIR高信号,白质为主,增强扫描无强化效应。MRS提示病灶区Cho峰略升高,NAA峰稍降低,Lac峰和Lip峰升高。脑组织病理结果示反应性胶质细胞增生,小血管周围淋巴细胞浸润,存在类空泡样及髓鞘崩解改变。经糖皮质激素冲击及口服维持治疗,症状及影像学明显好转。结论 HE是一种伴有抗甲状腺抗体水平增高、临床表现多样、激素治疗有效的脑病综合征,影像学可见皮质或皮质下异常病灶,病理学无特异性改变。更多还原

【Abstract】 Objective To investigate the pathogenesis, clinical, imaging, pathological features and prognosis of Hashimoto’s encephalopathy(HE). Methods The clinical data, treatment and prognosis in one patients with HE were analyzed retrospectively. Results The main clinical features were stroke-like episodes, rapidly progressive cognitive dysfunction and epilepsy. The serum levels of anfithyroid antibody were remarkable higher with almost normal function of thyroid. Craniocerebral MRI indicated multiple foci increased signal intensity of Tl, T2 and FLAIR images in bilateral temporal lobe, hippocampus and left basal ganglia region, without any enhancement. MRS showed Cho peak slightly elevated,NAA peak slightly lower, Lac peak and Lip peak increased in lesion area. The pathological examination demonstrated reactive gliosis, angiogenesis and a few infiltration of lyrephocyte, with vacuolal-degeneration of some neurons and Myelin disruption. After glucocorticoids impact and oral maintenance treatment, the abnormal changes of imaging rapidly disappeared with marked improvement of clinical symptoms. Conclusion HE is an encephalopathy with the presence of elevated antithyroid antibodies, the diversity of clinical manifestations, and well response to steroids. The imaging shows multiple foci in cortical and subcortical area of brain, the pathological changes are not special.更多还原