ローマの神経学者 エットーレ・マルキアファーヴァとマルキアファーヴァ・ビニャミ病（MBD）

Ettore Marchiafava, A Roman Neurologist, and Marchiafava-Bignami Disease (MBD)

本論では，ローマの神経学者マルキアファーヴァ（Ettore Marchiafava）によるマルキアファーヴァ・ビニャミ病（MBD）の研究を取り上げ，筆者自身の研究を中心に最近の知見を概観する。MBDは大量飲酒者に生じる脳梁の左右対称性脱髄性病変を特徴とし，MRIの導入により生前診断が可能となった。急性期には意識障害，失立失歩，抵抗症，速話症様の構音障害がみられ，慢性期には半球間離断症状が出現する。画像診断で示唆されていた亜急性期の一過性出血を，筆者らは病理学的に確認した。病因は未解明であり，治療法の開発が期待される。マルキアファーヴァはマラリア研究でも功績を残し，ローマを愛した微生物学者・臨床家として知られる。

Abstract

This paper explores research on Marchiafava-Bignami Disease (MBD) by the Roman neurologist Ettore Marchiafava. It provides an overview of recent trends in MBD research, primarily focusing on the author's own work, and briefly highlights Marchiafava's role as a microbiologist and clinician, emphasizing his contributions as a notable Roman researcher. MBD is marked by symmetrical demyelinating lesions of the corpus callosum that occur in heavy alcohol consumers. Since 1898, diagnosis has been limited to postmortem findings. By 1985, 149 cases had been reported. Since the 1980s, however, the advent of magnetic resonance imaging (MRI) has enabled ante-mortem diagnosis. Clinical symptoms include astasia-abasia, Gegenhalten, cluttering dysarthria, and impaired consciousness during the acute phase, followed by interhemispheric disconnection symptoms in the chronic phase. Longitudinal X-ray CT and MRI studies have revealed findings suggestive of transient hemorrhage during the subacute phase, which have also been confirmed pathologically. However, the etiology of MBD remains incompletely understood. Ongoing research is needed to elucidate its pathogenesis, and the development of effective treatments is eagerly anticipated. Marchiafava was also a notable researcher of malaria, as well as a microbiologist and clinician with a broad perspective who loved Rome.