Marchiafava–Bignami Syndrome (MBS) is a rare and severe neurological disorder, first described in 1903 by Italian pathologists Ettore Marchiafava and Amico Bignami.
It is most commonly seen in chronic heavy alcoholics 🍷, particularly in association with malnutrition and vitamin deficiencies.
Pathologically, it is characterized by demyelination and necrosis of the corpus callosum, the major tract connecting the two cerebral hemispheres 🧠.

🧬 Aetiology & Risk Factors

Long-term, severe alcohol abuse 🍺.
Malnutrition & vitamin deficiencies, especially thiamine (B1), also niacin and folate.
Occasionally reported in non-alcoholic malnourished states (e.g. post-bariatric surgery, eating disorders).
Exact mechanism thought to involve metabolic stress → toxic–nutritional insult → callosal demyelination and necrosis.

📊 Types

Type	Corpus Callosum Involvement	Clinical Picture
Type A	Diffuse, severe, entire corpus callosum	Coma, stupor, seizures, pyramidal signs, poor prognosis ⚠️
Type B	Partial / less extensive involvement	Milder symptoms, confusion, gait disturbance, better prognosis 🙂

🩺 Clinical Features

Onset may be acute, subacute, or chronic.
Neuropsychiatric: Depression, paranoia, psychosis, progressive dementia-like syndrome.
Neurological: Seizures ⚡, hemiparesis, aphasia, ataxia, abnormal involuntary movements.
Severe cases: Rapid progression to stupor, coma, or death if untreated.

🔍 Investigations

Bloods: Look for malnutrition, low thiamine, electrolyte abnormalities.
CT Scan: May show hypodense corpus callosum lesions.
MRI (gold standard):
- T1 hypointensity, T2/FLAIR hyperintensity of corpus callosum.
- Often involves genu and splenium; can extend to adjacent white matter.
Rule out mimics: Wernicke’s encephalopathy, multiple sclerosis, lymphoma, toxic/metabolic encephalopathies.

💊 Management

🚫 Absolute alcohol cessation.
🍽️ Correct malnutrition: high-dose thiamine (IV), plus folate and other vitamins.
Supportive care: hydration, electrolyte correction, seizure control (antiepileptics).
Monitor for complications: aspiration, infections, encephalopathy.
Prognosis: Variable. Type A often poor; Type B may partially recover if treated early.

🧠 Key Learning Points

Think of MBS in a chronic alcoholic with seizures + confusion + callosal MRI changes.
Shares overlap with Wernicke’s, but MRI shows corpus callosum involvement.
High suspicion + early thiamine = better outcomes.

📚 References

Marchiafava–Bignami Syndrome (PubMed)
Review on Marchiafava–Bignami Syndrome
Adams & Victor’s Principles of Neurology – Callosal and alcohol-related disorders

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Marchiafava Bignami syndrome

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