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

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...

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Main Authors: Bence Prohászka, Novák Pál Kaposi, Zsuzsanna Jánosi, Bence Gunda, Ildikó Pákozdy, Szabolcs Gaál-Marschal, Dóra Melicher, Bánk G. Fenyves, Csaba Varga
Format: Article
Language:English
Published: BMC 2025-04-01
Series:International Journal of Emergency Medicine
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Online Access:https://doi.org/10.1186/s12245-025-00873-9
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author Bence Prohászka
Novák Pál Kaposi
Zsuzsanna Jánosi
Bence Gunda
Ildikó Pákozdy
Szabolcs Gaál-Marschal
Dóra Melicher
Bánk G. Fenyves
Csaba Varga
author_facet Bence Prohászka
Novák Pál Kaposi
Zsuzsanna Jánosi
Bence Gunda
Ildikó Pákozdy
Szabolcs Gaál-Marschal
Dóra Melicher
Bánk G. Fenyves
Csaba Varga
author_sort Bence Prohászka
collection DOAJ
description 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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spelling doaj-art-844601b2e6db419396f354faaf0c49d72025-08-20T02:11:57ZengBMCInternational Journal of Emergency Medicine1865-13802025-04-011811810.1186/s12245-025-00873-9A fatal case of acute Marchiafava-Bignami disease complicated by acute abdomen– a case reportBence Prohászka0Novák Pál Kaposi1Zsuzsanna Jánosi2Bence Gunda3Ildikó Pákozdy4Szabolcs Gaál-Marschal5Dóra Melicher6Bánk G. Fenyves7Csaba Varga8Department of Emergency Medicine, Semmelweis UniversityMedical Imaging Center, Semmelweis UniversityMedical Imaging Center, Semmelweis UniversityDepartment of Neurology, Semmelweis UniversityDepartment of Emergency Medicine, Semmelweis UniversityDepartment of Emergency Medicine, Semmelweis UniversityDepartment of Emergency Medicine, Semmelweis UniversityDepartment of Emergency Medicine, Semmelweis UniversityDepartment of Emergency Medicine, Semmelweis UniversityAbstract 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.https://doi.org/10.1186/s12245-025-00873-9Chronic alcohol consumptionEthanol toxicityAltered mental stateEmergency department
spellingShingle Bence Prohászka
Novák Pál Kaposi
Zsuzsanna Jánosi
Bence Gunda
Ildikó Pákozdy
Szabolcs Gaál-Marschal
Dóra Melicher
Bánk G. Fenyves
Csaba Varga
A fatal case of acute Marchiafava-Bignami disease complicated by acute abdomen– a case report
International Journal of Emergency Medicine
Chronic alcohol consumption
Ethanol toxicity
Altered mental state
Emergency department
title A fatal case of acute Marchiafava-Bignami disease complicated by acute abdomen– a case report
title_full A fatal case of acute Marchiafava-Bignami disease complicated by acute abdomen– a case report
title_fullStr A fatal case of acute Marchiafava-Bignami disease complicated by acute abdomen– a case report
title_full_unstemmed A fatal case of acute Marchiafava-Bignami disease complicated by acute abdomen– a case report
title_short A fatal case of acute Marchiafava-Bignami disease complicated by acute abdomen– a case report
title_sort fatal case of acute marchiafava bignami disease complicated by acute abdomen a case report
topic Chronic alcohol consumption
Ethanol toxicity
Altered mental state
Emergency department
url https://doi.org/10.1186/s12245-025-00873-9
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