Broch Line, Amthor Karl-Friedrich
Nevrologisk avdeling, Sykehuset Buskerud, 3004 Drammen, Norway.
Tidsskr Nor Laegeforen. 2010 May 20;130(10):1041-3. doi: 10.4045/tidsskr.09.0697.
Hashimoto's encephalopathy, also called steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), is a rare syndrome with an unknown pathogenesis. Clinical onset is insidious or acute with variable neurological symptoms. Spinal protein is elevated in most cases, but findings are non-specific in routine blood tests and on cerebral CT and EEG. The most important diagnostic clue is elevated levels of thyroid antibodies, especially anti-thyroid peroxidase antibody (anti-TPO). Administration of corticosteroids is the treatment of choice and usually results in complete recovery.
A 66-year-old woman had experienced episodic neurological symptoms over a few weeks. On admission she demonstrated moderate aphasia, tremor and myoclonus, but no other focal neurological signs. A cerebral CT scan and routine blood tests did not provide diagnostic clues. The cerebrospinal fluid protein level was markedly elevated, and treatment with antibiotic and antiviral agents against possible encephalitis was started. However, the clinical condition did not improve. Further testing showed elevated levels of anti thyroxine peroxidase (aTPO) antibodies. Based on this finding, and after exclusion of other causes, the patient was diagnosed with Hashimoto's encephalitis. On treatment with systemic steroids, the clinical condition improved over a few days.
Hashimoto's encephalitis is a rare syndrome. Still, it is important to diagnose patients correctly. Treatment with steroids induces marked clinical improvement and usually confers a good prognosis.
桥本脑病，也称为自身免疫性甲状腺炎相关性类固醇反应性脑病（SREAT），是一种发病机制不明的罕见综合征。临床起病隐匿或急性起病，伴有多种神经症状。大多数病例脊髓蛋白升高，但常规血液检查、脑部CT和脑电图检查结果无特异性。最重要的诊断线索是甲状腺抗体水平升高，尤其是抗甲状腺过氧化物酶抗体（抗TPO）。使用皮质类固醇是首选治疗方法，通常可完全康复。
一名66岁女性在几周内出现发作性神经症状。入院时，她表现为中度失语、震颤和肌阵挛，但无其他局灶性神经体征。脑部CT扫描和常规血液检查未提供诊断线索。脑脊液蛋白水平明显升高，开始使用抗生素和抗病毒药物治疗可能的脑炎。然而，临床症状并未改善。进一步检查显示抗甲状腺过氧化物酶（aTPO）抗体水平升高。基于这一发现，并排除其他病因后，该患者被诊断为桥本脑炎。经全身类固醇治疗后，临床症状在数天内得到改善。
桥本脑炎是一种罕见综合征。尽管如此，正确诊断患者很重要。类固醇治疗可使临床症状明显改善，通常预后良好。
