Periodic synchronous dischargeを呈しCreutzfeldt-Jakob病との鑑別を要した橋本脳症の1例 [in Japanese] Hashimoto's encephalopathy presenting periodic synchronous discharge, as a differential diagnosis for Creutzfeldt-Jakob disease [in Japanese]
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症例は57歳男性である．精神発達遅滞を有する．1年4ヵ月前より性格変化が出現し，無言・無動症となった．痙攣重積状態となり入院した．脳波でperiodic synchronous dischargeをみとめ，脳MRI拡散強調画像で両前頭葉皮質・視床・右島に高信号域をみとめたため，孤発性Creutzfeldt-Jakob病（CJD）がうたがわれた．しかし，甲状腺自己抗体および抗N末端α-enolase抗体が陽性であり，ステロイドにて臨床症状，脳波・MRI所見の明らかな改善をみとめたため橋本脳症と診断した．CJDをうたがう症例では，橋本脳症も必ず鑑別することが重要と考えられた．
Here, we report a case of Hashimoto's encephalopathy (HE) mimicking Creutzfeldt-Jakob disease (CJD). A 57-year-old man was admitted to our hospital for status epilepticus. He had gradually presented personality change over the last two years. On admission, he was in state of akinetic mutism. He exhibited seizures on the left side of his body, including the face, and intermittent myoclonic movement. Routine laboratory tests showed no abnormalities, including thyroid functions. An EEG study showed typical periodic synchronous discharge (PSD). Brain MRI showed high-intensity areas in the bilateral frontal cortex, thalamus, and right insula on diffusion-weighted imaging (DWI). So, initially, sporadic CJD was suspected. However, there were no abnormalities in the caudate or putamen on MRI. Anti-TG and anti-TPO antibodies, as well as anti-NAE antibody were all positive. He was administered methylpredonisolone pulse therapy. Subsequently, his consciousness levels and EEG and MRI findings markedly improved. So, he was finally diagnosed with HE. HE should be considerd in patients with PSD on EEG, even if the patients have typical MRI abnormalities of CJD. Anti-thyroid antibodies should be examined in such patients.
- Rinsho Shinkeigaku
Rinsho Shinkeigaku 53(9), 716-720, 2013
Societas Neurologica Japonica