Vietnamese Journal of Neurology

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Articles Issue: Tạp chí thần kinh học số 38 Ca lâm sàng

Marchiafava-Bignami disease: A rare clinical case

Published: May 26, 2024
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Abstract

Marchiafava-Bignami disease (MBD) is a rare disorder characterized by demyelination or necrosis of the corpus callosum and adjacent white matter damage. Although the literature acknowledges that most cases of MBD occur in individuals with a history of heavy alcohol consumption, there are also reports of cases in non-alcohol users, suggesting that alcohol may not be the primary cause of the observed lesions.

The clinical manifestations of MBD are diverse and nonspecific regarding the etiology. Symptoms can present acutely, subacutely, or chronically. Acute presentations typically involve sudden loss of consciousness, seizures, while subacute onset may manifest as disconnection syndrome between the hemispheres, dysarthria, apraxia, agnosia, and in chronic expressions, cognitive decline, behavioral disturbances, psychosis, and symptoms of disconnection syndrome between the hemispheres. Diagnosis relies on typical radiological features, particularly lesions in the corpus callosum, with MRI of the brain considered the gold standard for evaluating MBD lesions.

Due to the unclear pathogenesis of MBD, specific treatment is currently lacking. Current management primarily focuses on supportive care such as vitamin B supplementation, treatment of comorbidities, and cessation of alcohol in heavy alcohol users. Prognosis is therefore cautious, but early diagnosis and intervention are crucial factors in aiding better patient recovery.

References

1.
Tian TY, Pescador Ruschel MA, Park S, Liang JW. Marchiafava-Bignami Disease. 2023 Jan 27. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan–. PMID: 30252263.
2.
Wenz H, Eisele P, Artemis D, Förster A, Brockmann MA. Acute Marchiafava-Bignami disease with extensive diffusion restriction and early recovery: case report and review of the literature. J Neuroimaging. 2014 Jul-Aug;24(4):421-4
3.
Lhermitte F, Marteau R, Serdaru M, Chedru F: Signs of interhemispheric disconnection in marchiafava-bignami disease. Arch Neurol. 1977, 34: 254-10.1001/archneur.1977.00500160068015.

Authors

  • Nguyễn Xuân Diệu
  • Doan Van Anh Vu
  • Mai Trong Duy
  • Truong Minh Thuong
  • Hoang Tien Trong Nghia

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Section Articles
Category Ca lâm sàng