Vietnamese Journal of Neurology

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

Marchiafava-Bignami disease: A rare clinical case

Published: July 17, 2026
Lượt đọc: 88

Abstract

Objectives: To report a rare case of Marchiafava-Bignami disease (MBD)—a demyelinating/necrotic disorder of the corpus callosum primarily associated with heavy alcohol use—to raise clinical awareness of this frequently misdiagnosed condition. Subjects and methods: Case description of a 53-year-old man with a history of over 10 years of heavy alcohol consumption, admitted with progressively worsening consciousness disturbance over 3 weeks, accompanied by communication difficulties, inability to name objects, impaired bimanual coordination, and limb weakness. The patient had previously been misdiagnosed with stroke and treated as an outpatient with aspirin/statin without improvement. Results: On admission, the patient had a GCS of 9, quadriparesis (muscle strength 1/5), hyperreflexia, and bilateral positive Babinski sign. Brain MRI revealed characteristic lesions in the middle layer of the corpus callosum with diffusion restriction on DWI, corresponding ADC signal reduction, and the "sandwich sign" on T1W imaging—diagnostic hallmarks of MBD. Blood and cerebrospinal fluid studies were within normal limits, excluding other causes (CNS infection, metabolic disturbances, Wernicke's encephalopathy, vitamin B12/folate deficiency). The patient was treated with high-dose thiamine (1500mg/day IV) combined with B-complex vitamins; however, his consciousness continued to deteriorate, hospital-acquired sepsis developed, and he died of septic shock on day 10 after admission. Conclusion: MBD is an extremely rare disorder with diverse, nonspecific clinical presentations that are easily confused with motor neuron disease or acute stroke. Interhemispheric disconnection signs (such as inability to name objects held in the left hand) are suggestive but not diagnostic. Brain MRI showing characteristic middle-layer corpus callosum lesions remains the gold standard for diagnosis. Given the unclear pathophysiology, no specific treatment currently exists; early diagnosis and treatment remain critical determinants of prognosis.

Keywords
Marchiafava-Bignami disease corpus callosum sandwich sign alcoholism demyelination

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 Bệnh viện Quân y 175 Đoàn Văn Anh Vũ Bệnh viện Quân y 175 Mai Trọng Duy Bệnh viện Quân y 175 Trương Minh Thương Bệnh viện Quân y 175 Hoàng Tiến Trọng Nghĩa Bệnh viện Quân y 175
Marchiafava-Bignami disease: A rare clinical case

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Section Ca lâm sàng
Category Nghiên cứu
Pages 54-56
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