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要旨 十数年来の多量の飲酒歴がある62歳男性。発話障害を主訴に当院を受診した。血中サイアミンは低値を示したが,MRIではWernicke脳症の所見は認めず,FLAIRで脳梁の前半部に高信号を認めMarchiafava-Bignami病(MBD)と診断した。拡散強調画像では脳梁のADC上昇が示唆され,サイアミン投与のみでこの脳梁病変は可逆性であった。MBDはアルコールによる栄養障害と考えられているが,脳梁に限局した脱髄病変を生じる稀な疾患で詳細な病態は未だに不明である。MBDの診断,画像所見,病態,治療法について文献的考察を加えて報告する。
Marchiafava-Bignami disease(MBD)associated with chronic alcoholism is a fatal disorder characterized by demyelination of the corpus callosum. A 62-year-old Japanese man, a heavy drinker for his last over 10 years, was admitted to our hospital because of acute onset of speech disturbance. The first MR images showed abnormal signal intensity of the corpus callosum, which was a typical finding of MBD, but no signal abnormality on diffusion-weighted images. At three days after large doses of thiamine administration, MR studies revealed the disappearance of callosal high signal intensity. His symptom gradually improved, the pathogenesis and therapy of MBD were discussed.
(Received : January 19, 2004)
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