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BRAIN and NERVE Volume 60, Issue 5 （May 2008）

BRAIN and NERVE 60巻5号（2008年5月発行）

Japanese English

症例報告

Creutzfeldt-Jakob病と類似の臨床経過を示した，Basedow病を伴った橋本脳症の1例 Case Report of a Patient with Hashimoto's Encephalopathy Associated with Basedow's Disease Mimicking Creutzfeldt-Jakob Disease 櫻井岳郎 ¹ , 田中優司 ¹ , 香村彰宏 ¹ , 林祐一 ¹ , 木村暁夫 ¹ , 保住功 ¹ , 米田誠 ² , 犬塚貴 ¹ Takeo Sakurai ¹ , Yuji Tanaka ¹ , Akihiro Koumura ¹ , Yuichi Hayashi ¹ , Akio Kimura ¹ , Isao Hozumi ¹ , Makoto Yoneda ² , Takashi Inuzuka ¹ ¹岐阜大学大学院医学系研究科神経統御学講座神経内科・老年学分野 ²福井大学医学部第二内科 ¹Department of Neurology and Geriatrics, Division of Neuroscience, Research Field of Medical Sciences, Gifu University Graduate School of Medicine ²Second Department of Internal Medicine, Faculty of Medical Sciences, University of Fukui キーワード： Hashimoto's encephalopathy , Basedow's disease , Creutzfeldt-Jakob disease , anti α-enolase antibodies Keyword: Hashimoto's encephalopathy , Basedow's disease , Creutzfeldt-Jakob disease , anti α-enolase antibodies pp.559-565

発行日 2008年5月1日 Published Date 2008/5/1

DOI https://doi.org/10.11477/mf.1416100281

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Abstract 文献概要
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参考文献 Reference

はじめに

　橋本脳症は甲状腺自己抗体と臨床症状が相関する，自己免疫性の脳症と考えられている。意識障害，ミオクローヌスなど多彩な神経症状を示し，時に脳波でperiodic synchronous discharge（PSD）様の突発異常波を伴うことからCreutzfeldt-Jakob病（CJD）と鑑別を要することがある。橋本脳症では，CJDと異なりステロイド治療などが有効であるため，その鑑別は非常に重要である。近年，橋本脳症では血清抗N末端α-enolase（NAE）抗体が高率に陽性であることから，その診断に有用と報告されている^1，2）。

　また橋本脳症は基礎疾患として一般に橋本病を伴うが，時にBasedow病を伴う報告もある^3－5）。われわれは臨床症候と脳波所見がCJDに類似し，Basedow病を伴った橋本脳症の1例を経験したので，文献的考察を加え報告する。

Abstract

　A 79-year-old female was admitted to our hospital because of unconsciousness and convulsion following mental deterioration.　On admission, she exhibited myoclonic movement of the right side of the face and right fingers in addition to rigospasticity and tremors in the right arm and leg.　Laboratory tests revealed hyperthyroidism with an increased anti-TSH-R antibody titer.　In addition, an echogram indicated excessive blood flow at the thyroid; hence, the patient was diagnosed with Basedow's disease.　Interestingly, the tests also revealed increased titer of anti-TPO antibody, anti-Tg antibody, and anti-NH2 terminal of α-enolase (NAE) antibody; in addition, an EEG showed abnormal findings potentially indicating periodic synchronous discharge.　Brain MRI showed cerebral atrophy, and brain ^99mTc-ECD-SPECT images demonstrated an overall decrease in the accumulation of ^99mTc in the cerebrum.　The abovementioned findings are common to patients with Creutzfeldt-Jakob disease (CJD).　We initiated treatment for hyperthyroidism with thiamazole and lugol, but this did not regain consciousness.　Because she had anti-thyroid antibody was observed, we considered a differential diagnosis of Hashimoto's encephalopathy and, in fact, methylprednisolone pulse therapy alleviated her symptoms and normalized the EEG findings.

　The condition in this case clinically mimicked CJD; therefore, the differentiated diagnosis is important because Hashimoto's encephalopathy is treatable disease.