About
- Rare neurological disease seen mostly in alcoholics
- Discovered in 1903 by Italian pathologists Ettore Marchiafava and Amico Bignami.
Aetiology
- Corpus callosum demyelination and necrosis
- Severe and chronic alcoholics in their middle or late adult life.
Types
- A: coma, stupor, pyramidal tract signs. Involves the entire corpus callosum
- B: Less severe with partial damage to the corpus callosum
Clinical
- Can be acute, subacute, or chronic
- Depression, paranoia, psychosis, or dementia.
- Seizures are common
- Hemiparesis, aphasia, abnormal movements
- Ataxia which may progress to coma and/or death.
Investigations
- CT/MRI: Lesions can appear as hypodense regions of the corpus callosum on tomography, and as areas of diminished T1 signal and increased T2 signal on magnetic resonance.
Management
- Largely supportive. Stop alcohol. Optimize nutrition
- Treat any Thiamine deficiency and withdrawal