A case report of acute Marchiafava-Bignami disease: a rare clinical entity in chronic alcoholism
Marchiafava‑Bignami disease (MBD) is a rare neurological disease often associated with a chronic consumption of alcohol and malnutrition, which is characterized by a demyelination and necrosis of the corpus callosum. We present a case of a 21-year-old male with chronic alcoholism who presented with an acute altered sensorium and seizure, which were initially treated as meningoencephalitis. His persistent poor Glasgow coma scale score and ideomotor recovery with encephalitic changes on his electroencephalogram prompted urgent magnetic resonance imaging (MRI) of his brain, which revealed extensive symmetrical hyperintensities in the corpus callosum. The diagnosis of MBD was made because of the typical MRI findings and after the exclusion of other possible diagnosis. The patient was promptly treated with a parenteral thiamine and showed a good recovery at 3 months follow-up. This case highlights the importance of diagnosing and recognizing MBD in a patient with chronic alcoholism as prompt treatment could prevent irreversible damage, which could carry high morbidity.
Tung CS, Wu SL, Tsou JC, Hsu SP, Kuo HC, Tsui HW. Marchiafava-Bignami disease with widespread lesions and complete recovery. AJNR Am J Neuroradiol. 2010;31(8):1506-7. https://doi.org/10.3174/ajnr.A1897
DeDios-Stern S, Gotra MY, Soble JR. Comprehensive neuropsychological findings in a case of Marchiafava-Bignami disease. Clin Neuropsychol. 2020;1-12. https://doi.org/10.1080/13854046.2020.1731608
Marchiafava E, Bignami A. Sopra un alterazione del corpo calloso osservata in soggetti alcoolisti. Riv Patol Nerv Ment. 1903;8:544-9. Italian.
Heinrich A, Runge U, Khaw AV. Clinicoradiologic subtypes of Marchiafava-Bignami disease. J Neurol. 2004;251(9):1050-9. https://doi.org/10.1007/s00415-004-0566-1
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(2):168-73. https://doi.org/10.1136/jnnp-2013-305979
Filippi M, Rocca MA, Ciccarelli O, De Stefano N, Evangelou N, Kappos L, et al. MRI criteria for the diagnosis of multiple sclerosis: MAGNIMS consensus guidelines. Lancet Neurol. 2016;15(3):292-303. https://doi.org/10.1016/S1474-4422(15)00393-2
Gultekin SH, Rosenfeld MR, Voltz R, Eichen J, Posner JB, Dalmau J. Paraneoplastic limbic encephalitis: neurological symptoms, immunological findings and tumour association in 50 patients. Brain. 2000;123 (Pt 7):1481-94. https://doi.org/10.1093/brain/123.7.1481
Ruzek KA, Campeau NG, Miller GM. Early Diagnosis of central pontine myelinolysis with diffusion-weighted imaging. AJNR Am J Neuroradiol. 2004;25(2):210-3.
Naeije R, Franken L, Jacobovitz D, Flament-Durand J. Morel's laminar sclerosis. Eur Neurol. 1978;17(3):155-9. https://doi.org/10.1159/000114939
Berger JR, Aksamit AJ, Clifford DB, Davis L, Koralnik IJ, Sejvar JJ, et al. PML diagnostic criteria: consensus statement from the AAN neuroinfectious disease section. Neurology. 2013;80(15):1430-8. https://doi.org/10.1212/WNL.0b013e31828c2fa1
Fernandes LMP, Bezerra FR, Monteiro MC, Silva ML, de Oliveira FR, Lima RR, et al. Thiamine deficiency, oxidative metabolic pathways and ethanol-induced neurotoxicity: How poor nutrition contributes to the alcoholic syndrome, as Marchiafava-Bignami disease. Eur J Clin Nutr. 2017;71(5):580-6. https://doi.org/10.1038/ejcn.2016.267
Cui Y, Zheng L, Wang X, Zhang W, Yuan D, Wei Y. Marchiafava-Bignami disease with rare etiology: a case report. Exp Ther Med. 2015;9(4):1515-7. https://doi.org/10.3892/etm.2015.2263
Caulo M, Briganti C, Notturno F, Committeri G, Mattei PA, Tartaro A, et al. Non-alcoholic partially reversible Marchiafava-Bignami disease: review and relation with reversible splenial lesions. A case report and literature review Neuroradiol J. 2009;22(1):35-40. https://doi.org/10.1177/197140090902200106
Boutboul D, Lidove O, Aguilar C, Klein I, Papo T. Marchiafava-Bognami disease complicating SC hemoglobin disease and Plasmodium Falciparum infection. Presse Med. 2010;39(9):990-3. https://doi.org/10.1016/j.lpm.2010.02.052
Carrilho PEM, Santos MBM Dos, Piasecki L, Jorge AC. Marchiafava-Bignami disease: a rare entity with a poor outcome. Rev Bras Ter intensiva. 2013;25(1):68-72. https://doi.org/10.1590/S0103-507X2013000100013
Mkrtchyan G, Aleshin V, Parkhomenko Y, Kaehne T, Di Salvo ML, Parroni A, et al. Molecular mechanisms of the non-coenzyme action of thiamin in brain: biochemical, structural and pathway analysis. Sci Rep. 2015;5:12583. https://doi.org/10.1038/srep12583
July J, Adeline, Tjandrawidjaja G, Siahaan YMT, Widysanto A, Horiyanto H, et al. A case of nonalcoholic Wernicke's encephalopathy due to an extreme diet based on magnetic resonance imaging features. Med J Indones. 2019;28(3):276-9. https://doi.org/10.13181/mji.v28i3.2629
Brion S. Marchiafava-Bignami disease. In: Vinken PJ, Bruyn GW, editors. Handbook of clinical neurology. Amsterdam: North Holland Publishing Company; 1977. p. 317-29.
Marjama J, Yoshino MT, Reese C. Marchiafava-Bignami disease. Premortem diagnosis of an acute case utilizing magnetic resonance imaging. J Neuroimaging. 1994;4(2):106-9. https://doi.org/10.1111/jon199442106
Ikeda A, Antoku Y, Abe T, Nishimura H, Iwashita H. Marchiafava-Bignami disease: consecutive observation at acute stage by magnetic resonance imaging and computerized tomography. Jpn J Med. 1989;28(6):740-3. https://doi.org/10.2169/internalmedicine1962.28.740
Kohler CG, Ances BM, Coleman AR, Ragland JD, Lazarev M, Gur RC. Marchiafava-Bignami disease: literature review and case report. Neuropsychiatry Neuropsychol Behav Neurol. 2000;13(1):67-76.
Chen X, Wang Y, Wang Y, Tong D, Zhu B. Alcoholic and non-alcoholic Marchiafava-Bignami disease: a comparative study. Neurosci Med. 2016;7:99-105. https://doi.org/10.4236/nm.2016.73011
Kikkawa Y, Takaya Y, Niwa N. [A case of Marchiafava-Bignami disease that responded to high-dose intravenous corticosteroid administration]. Rinsho Shinkeigaku. 2000;40(11):1122-5. Japanese.
Tao H, Kitagawa N, Kako Y, Yamanaka H, Ito K, Denda K, et al. A case of anorexia nervosa with Marchiafava-Bignami disease that responded to high-dose intravenous corticosteroid administration. Psychiatry Res. 2007;156(2):181-4. https://doi.org/10.1016/j.pscychresns.2007.04.013
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