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Medicina Interna
Print version ISSN 0872-671X
Medicina Interna vol.27 no.4 Lisboa Dec. 2020
https://doi.org/10.24950/Imagem/151/20/4/2020
IMAGENS EM MEDICINA /IMAGES IN MEDICINE
Doença de Marchiafava-Bignami: Uma Complicação Rara do Abuso do Álcool
Marchiafava-Bignami Disease: A Rare Complication of Alcohol Abuse
Marinha Silva1 https://orcid.org/0000-0002-5344-4794
Inês Carvalho2 https://orcid.org/0000-0001-8023-842X
Eduardo Freitas2 https://orcid.org/0000-0003-3197-2569
José Nuno Alves2 https://orcid.org/0000-0003-3187-9938
1Serviço de Medicina Interna, Hospital de Santa Maria Maior, Barcelos, Portugal
2Serviço de Neurologia, Hospital de Braga, Braga, Portugal
Palavras-chave: Alcoolismo/ complicações; Corpo Caloso; Doença de Marchiafava-Bignami/ etiologia.
Keywords: Alcoholism/ complications; Corpus Callosum; Marchiafava-Bignami Disease/ etiology.
Introduction
Chronic alcohol abuse is a common disorder. Its effects include neurologic complications through both direct and indirect effects on the central and peripheral nervous systems. Marchiafava-Bignami disease (MBD) is caused by demyelination and necrosis of the corpus callosum and it has long been considered to be of either a toxic or nutritional etiology.1
Case Report
A 43-year-old man, with chronic alcoholism (over 120 g daily), was brought to the emergency department after a two-week period of behavioral changes. There was no history of illicit drug use. On initial assessment his mini mental state scalewas 19/30, he was disorientated in time and place, with reduced speech output, psychomotor retardation, but with no focalsigns. His blood pressure was normal and hehad no fever. Cerebral computerized tomography revealed a callosal hypodensity and global atrophy, with no acute vascular lesions (Fig. 1A). Laboratory studies disclosed mild macrocyticanemia and elevated liver enzymes; renal, thyroid and liver function were normal; systemic inflammatory biomarkers and serumethanol were negative. Abdominal ultrasonography showedsigns of steatosis, with no splenomegaly or ascites. Brain magnetic resonance imaging (MRI) confirmed the callosal lesion, with restricted diffusion (Fig. 2B) and showed T2/FLAIR hyperintensities concerning callosal genu, body and splenium (Fig.s 1A and 2A), suggesting MBD. He was started on several vitamin supplementation (including thiamine), with a good clinical outcome.
Discussion
MBD pathophysiology includes cytotoxic edema, demyelination and callosal necrosis, presumably attributed to the combination of alcohol-induced neurotoxicity and deficiency of B-complex vitamins.2-5 The clinical course is broad and some cases may evolve to rapid mental state deterioration and death.1
There are no treatment guidelines but some reports suggest a favorable response to thiamine, enhancing the synergism between alcohol neurotoxic effects and hypovitaminosis B1 (responsible for Wernicke encephalopathy).4 Our patient improved with thiamine reposition which shows the of early management.
REFERENCES
1. Hillbom M, Saloheimo P, Fujioka S, Wszolek ZK, Juvela S, Leone MA. Diagnosis and management of Marchiafava-Bignami disease: a review of CT/MRI confirmed cases. J Neurol Neurosurg Psychiatry. 2014;85:168-73. doi: 10.1136/jnnp-2013-305979 [ Links ]
2. Murthy SB, Jawaid A, Bock JE, Qureshi SU, Schulz PE. Marchiafava-Big-nami disease (MBD) in a nonalcoholic patient: a case report. Can J Neurol Sci. 2010;37:138-40. doi: 10.1017/s0317167100009823. [ Links ]
3. Boutboul D, Lidove O, Aguilar C, Klein I, Papo T. Marchiafava-Bignami disease complicating SC hemoglobin disease and Plasmodium falciparum infection. Presse Med. 2010;39:990-3. doi: 10.1016/j.lpm.2010.02.052. [ Links ]
4. Loh Y, Watson WD, Verma A, Krapiva P. Restricted diffusion of the splenium in acute Wernicke’s encephalopathy. J Neuroimaging. 2005;15:373-5. doi: 10.1177/1051228405279037.
5. Tavares P, Lizardo RG, Falcão C, Ponte A. Doença de Marchiafava-Big-nami: descrição de um caso: Rev Casos Clin Online. [consultado Jan 2020] Disponível em: http://casosclinicosonline.spmi.pt/artigos_consultar.php?id=2019186.07-01-2020.4 [ Links ]
Responsabilidades Éticas
Conflitos de Interesse: Os autores declaram a inexistência de conflitos de interesse na realização do presente trabalho.
Fontes de Financiamento: Não existiram fontes externas de financiamento para a realização deste artigo.
Confidencialidade dos Dados: Os autores declaram ter seguido os protocolos da sua instituição acerca da publicação dos dados de doentes.
Proteção de Pessoas e Animais: Os autoresdeclaram que os procedimentos seguidos estavam deacordo com os regulamentos estabelecidos pelos responsáveis da Comissão de Investigação Clínica e Ética e de acordo com a Declaração de Helsínquia da Associação Médica Mundial.
Proveniência e Revisão por Pares: Não comissionado; revisão externa por pares.
Ethical Disclosures
Conflicts of interest: The authors have no conflicts of interest to declare.
Financing Support: This work has not received any contribution,grant or scholarship
Confidentiality of Data: The authors declare that they have followed the protocols of their work center on the publication of data from patients.
Protection of Human and Animal Subjects: The authorsdeclare that the procedures followedwere inaccordance with the regulations of the relevant clinical research ethics committee and with those of the Code of Ethics of the World Medical Association (Declaration of Helsinki).
Provenance and Peer Review: Not commissioned; externally peer reviewed.
© Autor (es) (ou seu (s) empregador (es)) e Revista SPMI 2020. Reutilização permitida de acordo com CC BY-NC. Nenhuma reutilização comercial
© Author(s) (or their employer(s)) and SPMI Journal 2020. Re-use permitted under CC BY-NC. No commercial re-use.
Correspondence/ Correspondência:
José Nuno Alves – jose.nuno.aalves@gmail.com
Serviço de Neurologia, Hospital de Braga, Braga, Portugal
Sete Fontes – São Victor, 4710-243, Braga
Received / Recebido: 22/08/2020
Accepted / Aceite: 28/09/2020
Publicado / Published: 18 de Dezembro de 2020