Marchiafava–Bignami syndrome presenting with cognitive and behavioral impairment in a chronic alcoholic: A case report

Dear Editor, Marchiafava–Bignami syndrome (MBS) is a rare toxic–nutritional encephalopathy characterized by demyelination and necrosis of the corpus callosum, primarily linked to chronic alcoholism and resulting from thiamine deficiency.[1,2] We report a case to highlight the critical role of neuroimaging and timely intervention in managing the chronic cognitive variant of this condition. A 40-year-old chronic alcoholic male, working as a food safety officer, presented with a 3-month history of progressive forgetfulness and short-term memory deficits, severely impacting his functional and occupational status. His alcohol consumption was substantial (one to two pouches three times daily over the preceding 18 months), accompanied by withdrawal symptoms. The patient belonged to an adequate socioeconomic background, was not malnourished, and had no evidence of comorbid illness or target organ damage. Routine initial investigations were normal. However, brain MRI revealed abnormal signal intensity (demyelination) in the genu and splenium of the corpus callosum, highly suggestive of Marchiafava–Bignami syndrome (MBS) [Figure 1]. Other differential diagnoses such as Wernicke–Korsakoff syndrome, multiple sclerosis, and encephalitis were considered and ruled out primarily based on characteristic MRI findings and clinical presentation. Following a relapse, he was readmitted after 2 months, and cognitive assessment indicated mild cognitive impairment (MMSE 21/30). A repeat MRI performed at this time demonstrated progressive demyelination and necrosis involving the genu, body, and splenium of the corpus callosum [Figure 2]. The patient was managed with high-dose intramuscular thiamine (300 mg four times daily), along with multivitamins. Lorazepam was administered for alcohol withdrawal symptoms at a dose of 2 mg four times daily (maximum up to 14 mg/day) and was gradually tapered. Supportive nutritional therapy and alcohol cessation counselling were provided. Gradual improvement in withdrawal symptoms and cognitive functions was observed over 2 weeks.Figure 1: Brain MRI indicating abnormal signal intensity in the corpus callosum (genu and splenium), suggestive of demyelinationFigure 2: Repeat MRI suggesting progressive demyelination and necrosisMBS typically involves the genu and splenium of the corpus callosum. Clinically, it can manifest acutely with coma or seizures, or chronically with cognitive decline, disorientation, and apathy, consistent with this patient’s presentation of memory loss.[3,4] MRI is the diagnostic gold standard, revealing T2/FLAIR hyperintensities suggestive of demyelination. This case underscores that although rare, MBS must be considered in chronic alcohol users presenting with cognitive or psychiatric complaints. Prompt thiamine administration and sustained alcohol abstinence are crucial to prevent progression and are associated with a better prognosis.[5] Timely diagnosis using neuroimaging and comprehensive management, including thiamine therapy and psychiatric intervention, can enhance overall outcomes and prevent irreversible neurological damage. Author contributions Conceptualization, data collection, manuscript drafting: DB. Literature review and critical revision of the manuscript: SS. Final manuscript approval: all authors. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.