A Rare Case of Marchiafava-Bignami Disease in Taiwan

罕見胼胝體變性病患之病例報告

張乃仁(Nai-Jen Chang) ； 廖嘉威(Eric Kar-Wai Liu) ； 容世明(Shih-Ming Jung) ； 廖正智(Cheng-Chih Liao)

《中華放射線醫學雜誌》 35卷3期 (2010/09) Pp. 185-190

摘要

胼胝體變性(Marchiafava-Bignami disease)是一種慢性酒精濫用造成的罕見疾病，且絕大部分的報告病例都在歐美國家。過去此疾病的診斷均藉由屍體解剖及電腦斷層或核磁共振之影像診斷，認為其病理是因為胼胝體的脫鞘及溶解造成。作者在此報導一位五十三歲台灣男性，在臨床症狀及影像檢查上皆高度懷疑胼胝體變性，在病發十六天後，我們安排電腦斷層導引切片欲做確定診斷，卻意外發現胼胝體的髓鞘是完整的，和過去脫鞘的認知完全相反。據我們所知，此病患是唯－位活體切片之病例，因此這樣特殊的發現值得提出討論做為此疾病往後神經病理機轉探討的參考。

並列摘要

Marchiafava-Bignami disease (MBD) is a rare complication of chronic alcoholism especially among the Asian people. The previous understanding of this disease relied upon roegenographic and post-mortem pathological data and is associated with demyelination and necrosis of the corpus callosum. We report a 53-year-old chronic alcoholic Taiwanese male with clinical symptoms of MBD but presented with an atypical radiological cranial study. Based on the suspicion of possible disseminated infectious or neoplastic brain disease, the patient underwent a computer tomography-guided brain biopsy two weeks after the manifestation of disease. The pathological result did not reveal a classical picture of demyelination in MBD but that of necrosis with relatively well-preserved myelination of associated axons. We present this case study and propose a possible pathophysiology of this rare brain degenerative disease during its early stage of progression.