A fatal case of acute Marchiafava-Bignami disease complicated by acute abdomen- a case report.

IF 2 Q2 EMERGENCY MEDICINE International Journal of Emergency Medicine Pub Date : 2025-04-08 DOI:10.1186/s12245-025-00873-9
Bence Prohászka, Novák Pál Kaposi, Zsuzsanna Jánosi, Bence Gunda, Ildikó Pákozdy, Szabolcs Gaál-Marschal, Dóra Melicher, Bánk G Fenyves, Csaba Varga
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Abstract

Background: Marchiafava-Bignami Disease (MBD) is a rare disorder characterized by demyelination and necrosis of the corpus callosum, with only 300 documented cases worldwide. Chronic alcohol consumption and vitamin B-complex deficiencies are contributing factors. Acute cases may present with a range of neurological symptoms, including seizures and coma. Subacute and chronic forms can lead to interhemispheric disconnection syndrome and progressive dementia.

Case presentation: We present the case of a young male patient's first hospital admission due to an acute decline in conscious level. A detailed history revealed regular alcohol consumption and substandard living conditions. The deterioration in consciousness was attributed to the diagnosis of MBD based on neurological signs, characteristic brain imaging findings, and a history of alcohol use. In addition, a small bowel perforation was also diagnosed. Supportive therapy and thiamine were initiated, and the patient was transferred to surgery for an operation. After two surgeries, the perforation was covered. The patient's level of consciousness showed slight improvement; however, the psychiatrist noted severe cognitive deficits. Ultimately, the patient entered a septic state and passed away.

Conclusion: Acute MBD can potentially cause altered mental state, coma, and death; however, cases can be complicated by other emergency conditions. This case demonstrates the importance of a multidisciplinary approach.

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急性Marchiafava-Bignami病并发急腹症致死1例。
背景:marchiafva - bignami病(MBD)是一种罕见的疾病,其特征是胼胝体脱髓鞘和坏死,全球仅有300例记录病例。长期饮酒和维生素b复合物缺乏是致病因素。急性病例可能出现一系列神经系统症状,包括癫痫发作和昏迷。亚急性和慢性形式可导致半球间断开综合征和进行性痴呆。病例介绍:我们提出的情况下,一个年轻的男性患者的第一次入院,由于急性下降的意识水平。详细的病史显示经常饮酒和不合标准的生活条件。意识的恶化是由于MBD的诊断是基于神经学症状、特征性脑成像发现和酒精使用史。此外,还诊断为小肠穿孔。开始了支持性治疗和硫胺素,患者被转移到外科手术。经过两次手术,穿孔被盖住了。患者的意识水平略有改善;然而,精神科医生指出了严重的认知缺陷。最终,患者进入脓毒症状态并死亡。结论:急性MBD可导致精神状态改变、昏迷和死亡;然而,其他紧急情况可能使情况复杂化。这个案例说明了多学科方法的重要性。
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来源期刊
CiteScore
4.60
自引率
0.00%
发文量
63
审稿时长
13 weeks
期刊介绍: The aim of the journal is to bring to light the various clinical advancements and research developments attained over the world and thus help the specialty forge ahead. It is directed towards physicians and medical personnel undergoing training or working within the field of Emergency Medicine. Medical students who are interested in pursuing a career in Emergency Medicine will also benefit from the journal. This is particularly useful for trainees in countries where the specialty is still in its infancy. Disciplines covered will include interesting clinical cases, the latest evidence-based practice and research developments in Emergency medicine including emergency pediatrics.
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