1、Lord Brain在Lancet上报道了一例患者:男,49岁,主要表现为波动性意识障碍、幻觉 、认知功能损害和卒中样发作。既往桥本甲状腺炎辅助检查:甲功(T3T4)低于正常,血抗甲状腺球蛋白抗体(anti-TG)和抗甲状腺微粒体抗体(anti-M)明显增高;脑脊液蛋白增高。经激素和甲状腺素治疗好转。Brain L. Hashimotos disease and en-cephalopathy. Lancet 1966 Sep 3; 2 (7462): 512-4encephalopathy associated with Hashimotos thyroiditissteroid-respo
2、nsive encephalopathy associated with autoim-mune thyroiditis,SREAT)患病率:2.1/100 000发病年龄:9-86岁(平均45-55岁)男女比例:1:5没有家族性桥本脑病的报道可以合并其它自身免疫病(如SLE、I型糖尿病等)anti-TG)anti-Manti-TPOFigure 2. Axial T2-weighted MRI brain scan through the temporal lobes shows bilateral areas of increased signal intensity involving
3、the mesial temporal lobes and hippocampi (arrows).Figure 1. Sagittal T1-weighted MRI brain (without gadolinium) demonstrates a low signal intensity lesion, with localized swelling of the right mesial temporal lobe (arrow). Fig. 2 MRI one day after admission: axial FLAIR images show widespread hyperi
4、ntense signals over the left temporal and both parieto-occipital lobes in the subcortical white matterDuffey P. Hashimotos encephalopathy: postmortem findings after fatal status epilepticus. Neurology. 2003 Oct 28;61(8):1124-6. The neuropathological features include discrete areas of emyelination (G
5、; areas of pallor on Hematoxylin and Eosin arrowheads, H; Luxol Fast Blue staining) with relative reservation of axons (I; Bielschowsky staining). The lack of erivascular cuffs is evident in the Hematoxylin and Eosin staining (G).临床主要表现为认知功能障碍、意识障碍以及精神症状、癫痫、卒中样发作、肌阵挛和震颤等抗甲状腺抗体特别是高滴度抗TPO抗体对激素敏感,甚至有戏剧性疗效不能用其它疾病解释治疗流程治疗流程