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-responsive
2、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 the mes
3、ial 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 hyperintense signals o
4、ver 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;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