Chan Derrick W S, Lim C C Tchoyoson, Tay Stacey K H, Choong Chew-Thye, Phuah Huan Kee
Pediatric Neurology Service, Department of Pediatric Medicine, KK Women's and Children's Hospital, Singapore.
Pediatr Neurol. 2007 Jun;36(6):414-7. doi: 10.1016/j.pediatrneurol.2007.02.005.
Both the syndrome of mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS syndrome) and Hashimoto's encephalopathy can present with nonspecific encephalopathy. Hashimoto's encephalopathy is an association of steroid-responsive encephalopathy with elevated thyroid peroxidase antibodies. Steroid-responsive encephalopathy, however, is not characteristic of the MELAS syndrome, which typically presents with stroke-like episodes and lactic acidosis in cerebrospinal fluid and blood. Here, a patient is described with goiter, recurrent encephalopathy and elevated thyroid peroxidase antibodies who apparently responded to steroid therapy; however, magnetic resonance imaging was atypical for Hashimoto's encephalopathy, and she was diagnosed with MELAS syndrome. This syndrome can present with apparent steroid-responsive encephalopathy and elevated thyroid peroxidase antibodies, mimicking Hashimoto's encephalopathy, and should be suspected if lactic acidosis is present and typical features are detected on magnetic resonance imaging.
线粒体脑肌病伴乳酸血症和卒中样发作综合征（MELAS综合征）和桥本脑病均可表现为非特异性脑病。桥本脑病是一种与甲状腺过氧化物酶抗体升高相关的类固醇反应性脑病。然而，类固醇反应性脑病并非MELAS综合征的特征，MELAS综合征通常表现为卒中样发作以及脑脊液和血液中的乳酸酸中毒。本文描述了一名患有甲状腺肿、复发性脑病且甲状腺过氧化物酶抗体升高的患者，该患者对类固醇治疗有明显反应；然而，磁共振成像表现不符合桥本脑病，她被诊断为MELAS综合征。该综合征可表现出明显的类固醇反应性脑病和甲状腺过氧化物酶抗体升高，酷似桥本脑病，若存在乳酸酸中毒且磁共振成像检测到典型特征，则应怀疑MELAS综合征。
