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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。
Objective To investigate the pathogenesis, clinical, imaging and pathological features and prognosis of Hashimoto’s encephalopathy (HE). Methods The clinical data, treatment and prognosis of one case of clinically and pathologically confirmed HE patients were retrospectively analyzed. Results The patient presented with stroke-like episodes, with rapid progression of cognitive dysfunction and epilepsy. Serum anti-thyroid antibodies were significantly increased. Head MRI showed bilateral medial temporal lobe, hippocampus and left basal ganglia, , Long T2 and FLAIR high signal, white matter-based, enhanced scan no strengthening effect. MRS indicated that Cho peak in lesion area slightly increased, NAA peak decreased slightly, Lac peak and Lip peak increased. Brain histopathology showed reactive glial cell proliferation, small perivascular lymphocyte infiltration, the presence of vacuolar and myelin disintegration changes. The impact of corticosteroids and oral maintenance treatment, symptoms and imaging improved significantly. Conclusion HE is an encephalopathy syndrome with elevated thyroid antibody level, diverse clinical manifestations and effective hormone therapy. Imaging abnormal cortical or subcortical lesions with no specific pathological changes.