桥本氏脑病经典幻灯

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; 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、