Osmanlıoğlu Şeyma, Zerr Inga
Department of Neurology, Georg-August-Universität, Göttingen, Germany.
Department of Gynaecology and Obstetrics, Ankara Medipol University Faculty of Medicine, Ankara, Turkey.
Neurol Neurochir Pol. 2023;57(2):198-205. doi: 10.5603/PJNNS.a2023.0005. Epub 2023 Feb 2.
Steroid-responsive encephalopathy in autoimmune thyroiditis (SREAT) is characterised by a wide range of neuropsychiatric symptoms and elevated thyroid antibodies. SREAT can mimic sporadic Creutzfeldt-Jakob disease (sCJD) and distinguishing between both entities is important because SREAT responds to corticosteroids.
Data of patients reported to the National Reference Centre for the Surveillance of CJD in Göttingen, Germany between August 1994 and October 2008 was retrospectively reviewed. In the case and control groups, 49 patients had SREAT and 48 had sCJD with elevated thyroid antibodies.
Antibodies against thyroid peroxidase were the most common antibodies in both SREAT (86%) and sCJD (88%), followed by antibodies against thyroglobulin (SREAT, 63.3%; sCJD, 39.6%; p = 0.020) and TSH-receptor-antibodies (SREAT, 14.3%; sCJD, 2.1%; p = 0.059). Epileptic seizures were observed more frequently in the SREAT group (SREAT, 44.9%; sCJD, 12.5%; p < 0.001). Dementia (SREAT, 61.2%; sCJD, 100%; p < 0.001), ataxia (SREAT, 44.9%; sCJD, 89.6%; p < 0.001), visual impairment (SREAT, 22.4%; sCJD, 50%; p = 0.005), extrapyramidal disorder (SREAT, 32.7%; sCJD, 60.4%; p = 0.006), myoclonus (SREAT, 38.8%; sCJD, 81.3%; p < 0.001) and akinetic mutism (SREAT, 6.1%; sCJD, 37.5%; p < 0.001) were observed more frequently in sCJD. Cerebrospinal fluid (CSF) pleocytosis was observed more frequently in SREAT patients (SREAT, 33.3%; sCJD, 6.4%; p = 0.001), as was a pathological increase in protein concentration (SREAT, 68.8%; sCJD, 36.2%; p = 0.001).
In a case of encephalopathy, the diagnosis of SREAT should also be considered in suspected cases of CJD so as to be able to start corticosteroid treatment quickly.
自身免疫性甲状腺炎伴类固醇反应性脑病（SREAT）的特征是出现广泛的神经精神症状以及甲状腺抗体升高。SREAT可类似散发性克雅氏病（sCJD），区分这两种疾病很重要，因为SREAT对皮质类固醇有反应。
回顾性分析了1994年8月至2008年10月间向德国哥廷根克雅氏病监测国家参考中心报告的患者数据。病例组和对照组中，49例患者患有SREAT，48例患有甲状腺抗体升高的sCJD。
抗甲状腺过氧化物酶抗体是SREAT（86%）和sCJD（88%）中最常见的抗体，其次是抗甲状腺球蛋白抗体（SREAT，63.3%；sCJD，39.6%；p = 0.020）和促甲状腺激素受体抗体（SREAT，14.3%；sCJD，2.1%；p = 0.059）。癫痫发作在SREAT组中更常见（SREAT，44.9%；sCJD，12.5%；p < 0.001）。痴呆（SREAT，61.2%；sCJD，100%；p < 0.001）、共济失调（SREAT，44.9%；sCJD，89.6%；p < 0.001）、视力障碍（SREAT，22.4%；sCJD，50%；p = 0.005）、锥体外系疾病（SREAT，32.7%；sCJD，60.4%；p = 0.006）、肌阵挛（SREAT，38.8%；sCJD，81.3%；p < 0.001）和运动不能性缄默症（SREAT，6.1%；sCJD，37.5%；p < 0.001）在sCJD中更常见。SREAT患者脑脊液（CSF）细胞增多更常见（SREAT，33.3%；sCJD，6.4%；p = 0.001），蛋白质浓度病理性升高也是如此（SREAT，68.8%；sCJD，36.2%；p = 0.001）。
对于脑病患者，在疑似CJD的病例中也应考虑SREAT的诊断，以便能够迅速开始皮质类固醇治疗。
