鳥取臨床科学 1(2): 380-396, 2008

MRI で Marchiafava-Bignami 病が疑われた多発性脳梗塞
われた多発性脳梗塞の多発性脳梗塞の 1 剖検例小西吉裕 1)* 下田光太郎 2) 山本雅司 3) 城戸崎裕介 4) 西村広健 5) 調輝男 6)1) 国立病院機構鳥取医療センター臨床研究部, 2) 同 神経内科
3) 鳥取生協鹿野温泉病院内科, 4) 鳥取生協病院脳神経外科
5) 川崎医科大学病理学教室 1, 6) 川崎医療福祉大学医療技術学部

An autopsy case of multiple cerebral infarctions under an in vivo suspicion ofMarchiafava-Bignami disease based on magnetic resonance imaging
Yoshihiro Konishi1)*, Kotaro Shimoda2), Masashi Yamamoto3), Yusuke Kitozaki4),
Hirotake Nishimura5), Teruo Shirabe6)
1) Department of Clinical Research, National Hospital Organization Tottori Medical Center2) Department of Neurology, National Hospital Organization Tottori Medical Center3) Department of Internal Medicine, Shikano Hot Springs Hospital,Tottori Medical Care and Cooperative Society4) Department of Neurosurgery, Tottori Medical Care and Cooperative Society Hospital5) Department of Pathology, Kawasaki Medical School
6) Faculty of Health Science and Technology, Kawasaki University of Medical Welfare*Correspondence: ykonishi@tottori-iryo.hosp.go.jp
要旨
Marchiafava-Bignami 病（MBD）はアルコール多飲者に生じ, 脳梁に限局した脱髄・壊死病変を来たす疾患である. 核磁気共鳴画像 (MRI) などの画像診断法の進歩により, 脳梁病変が容易にかつ明瞭に描出できるようになって生前診断が可能となり, MBD の臨床例が多く報告されてきている. 今回,73 歳の大酒家男性で, MRI 画像で脳梁萎縮がみられたために MBD が示唆された例を経験した. 飲酒量の増量とともに, 意識障害はなかったが急に横になったまま動かなくなって病院を訪れた. 全般的な精神機能の低下, 右手の強制把握, 強制泣き笑い, 眼球運動障害, 運動失調, パーキンソン症状を呈し,血圧値の変動が激しい高血圧症を有し, 身体各所の動脈硬化と高血圧性心疾患が存在した例である.
MRI にて脳梁の萎縮とその近傍で側脳室前角周囲の大脳深部白質に異常信号域を認め, これは左側では一部脳梁や帯状回白質, 前頭葉皮質下白質にまで及び, その中心には嚢胞性病変が存在した. また,両側の側脳室周囲から半卵円中心に亘る広範な異常信号域のほか, 両側の半卵円中心, 被殻, 視床, および橋に小スポット状の複数個の異常信号域を認めた. 臨床的には多発性脳梗塞と考えられ, 鑑別として MBD が挙げられた. 剖検にて多発性脳梗塞が主病変であり, 脳梁萎縮は MBD ではなく, 左脳梁近傍の前角部周囲白質を中心に存在する大小の多発性脳梗塞病変による神経線維の二次的な変性のためと考えられた. 多発性脳梗塞でも MBD が鑑別診断に挙がってくる MRI 画像を呈し得ることを示す例として重要であり, また MRI に頼り過ぎている MBD の生前診断に警鐘を鳴らす目的で報告した.
鳥取臨床科学 1(2): 380-396, 2008
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Abstract
Marchiafava-Bignami disease（MBD）is rare and has been reported to occur typically in male,middle-aged, malnourished persons, who have a long history of excessive alcoholic consumption. Necrosis
and subsequent demyelination of the corpus callosum are the principal pathological features of MBD.
Advances in diagnostic imaging, particularly magnetic resonance imaging (MRI) techniques have allowed foran early and precise in vivo diagnosis, and several clinical cases of MBD based on the results of MRItechniques were reported in the literature. At the present time, we saw a 71-year-old, right handed man, andconsidered him a case of probable MBD because of his confirmed alcoholism and marked callosal atrophy on
his MRI, although he died of bronchopneumonia at the age of 73. His alcoholic consumption had graduallyincreased. He visited hospital, because he suddenly fell down on the floor and was unable to speak or walkwithout unconsciousness. On examination, he was found to exhibit generalized psychological impairments,
forced grasping of his right hand, forced laughing and crying, disturbance of his ocular movement, ataxia, andparkinsonism. He had hypertension accompanied by very labile blood pressure levels, atherosclerosis
throughout his body, and hypertensive heart disease. Results of an MRI showed marked atrophy of the corpus
callosum, and areas of abnormal signal intensity distributed next to the corpus callosum in the deep white mattersurrounding the anterior horns of the lateral ventricles. Particularly, the aforementioned areas of abnormal
intensity in the left cerebrum extended up to the part of the left corpus callosum, the white matter of thecingulate gyrus, and the subcortical white matter in the left frontal lobe, as well. And cystic lesions were foundin those areas of abnormal high intensity in the white matter. Small spotted areas of abnormal intensity
bilaterally scattered in the centrum semiovale, the putamen, the thalamus and the pons, and diffuse areas ofabnormal intensity were observed in the centrum semiovale surrounding the lateral ventricles. Based on theclinical information and findings, this patient was given a diagnosis of multiple cerebral infarctions, and thediagnosis may have involved differentiation from MBD. The autopsy revealed that multiple cerebral infarctions
were the principal pathological findings in the present case. The marked callosal atrophy was due to multiple
infarct lesions distributed next to the left corpus callosum in the deep white matter surrounding the anterior hornof the left lateral ventricle, but not due to MBD lesions. Those infarct lesions secondarily produced axonal
degeneration, leading to the callosal atrophy. The present case strongly suggests that, even though he really has
a pathological diagnosis of multiple cerebral infarctions, his MRI findings may lead us to consider MBD as anin vivo diagnosis that involves differentiation from the callosal atrophy due to multiple infarctions. At the sametime, this case notifies us to be careful to depend blindly on radiological imaging techniques, including MRI, forin vivo diagnosis of MBD. Tottori J. Clin. Res. 1(2), 380-396, 2008
Key words: Marchiafava-Bignami 病 , 脳 梁 , ア ル コ ー ル 中 毒 , 多 発 性 脳 梗 塞 , 核 磁 気 共 鳴 画 像 ;Marchiafava-Bignami disease, corpus callosum, chronic alcoholism, multiple infarction, magnetic resonanceimaging
はじめにの剖検例が記載され, その後 1903 年に同じくイMarchiafava-Bignami 病（MBD）はアルコールタリア人の Marchiafava と Bignami によって 2多飲者や稀に栄養障害者に生じ, 脳梁に限局し例が追加され, 計 3 例の剖検例が報告されたのた脱髄性もしくは壊死性病変を来たす疾患であが最初である. 従来は剖検でないと確定診断はる. 1898 年, イタリアの Carducci によって最初困難であったが, MRI などの画像診断法の進歩
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