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一例伴有可逆性胼胝体病变的马基亚法瓦-比尼亚米病罕见病例。

A Rare Case of Marchiafava-Bignami Disease With Reversible Splenial Lesion.

作者信息

Renuji Baishyak, Jaisukhalal Jaisurya, Nadarajah Jeyaseelan, Balagopal Krishnan

机构信息

Neurology, Malankara Orthodox Syrian Church (MOSC) Medical College Hospital, Ernakulam, IND.

General Internal Medicine, Derriford Hospital, University Hospitals Plymouth NHS Trust, Plymouth, GBR.

出版信息

Cureus. 2025 Apr 7;17(4):e81845. doi: 10.7759/cureus.81845. eCollection 2025 Apr.

Abstract

Individuals with a history of chronic alcohol consumption can develop Marchiafava-Bignami disease (MBD), a rare neurological disorder that is thought to occur secondary to thiamine deficiency and alcohol-induced brain damage. It is characterized by the toxic demyelination and necrosis of the corpus callosum. We report the case of a 42-year-old man who developed acute-onset altered sensorium and irrelevant speech output associated with ataxia. The clinical presentation raised a suspicion of MBD, although it was not a top differential diagnosis initially. Magnetic resonance imaging revealed significant demyelination of the splenium of the corpus callosum, confirming the suspicion and prompting immediate intervention aimed at addressing both the neurological manifestations and the possible underlying thiamine deficiency. Relevant history of significant alcohol consumption further supported this diagnosis. Following the initiation of thiamine supplementation and supportive care, the patient exhibited gradual improvement in neurological function, eventually leading to a complete clinical recovery and resolution of radiological findings, suggestive of the type B spectrum of the disease. This report highlights the importance of clinical evaluation and neuroimaging in the diagnosis, treatment, and prognostic stratification of patients with MBD.

摘要

有长期饮酒史的个体可能会患上马基亚法瓦-比尼亚米病(MBD),这是一种罕见的神经系统疾病,被认为继发于硫胺素缺乏和酒精性脑损伤。其特征是胼胝体的中毒性脱髓鞘和坏死。我们报告了一例42岁男性病例,该患者出现急性起病的意识改变和与共济失调相关的胡言乱语。临床表现引发了对MBD的怀疑,尽管最初它并非首要鉴别诊断。磁共振成像显示胼胝体压部有明显脱髓鞘,证实了怀疑并促使立即采取干预措施,旨在解决神经学表现以及可能潜在的硫胺素缺乏问题。大量饮酒的相关病史进一步支持了这一诊断。在开始补充硫胺素和给予支持性治疗后,患者的神经功能逐渐改善,最终实现了临床完全康复且影像学表现消失,提示为该疾病的B型谱。本报告强调了临床评估和神经影像学在MBD患者诊断、治疗及预后分层中的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/de23/12058231/fd96c346da5a/cureus-0017-00000081845-i01.jpg

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