July 27, 2023
A 56-year-old man presents with cognitive complaints and aggressive behavior. He reports consuming heavy amounts of alcohol and tobacco.
Marchiafava-Bignami Disease
Background:
- Ethanol directly induces the production of reactive oxygen species and indirectly impairs the absorption of thiamine, contributing to oxidative stress. Rarely, and usually in the setting of chronic alcohol use and malnourishment, this can lead to demyelination or necrosis of the corpus callosum and subsequent atrophy. This condition is called Marchiafava-Bignami disease.
- The atrophy appears to be more pronounced in the posterior callosal regions (splenium), where it may be associated with the formation of cavitary lesions.
Clinical Presentation:
- Cognitive impairment, apraxia, spasticity, dysarthria, seizures
Key Diagnostic Features:
- Atrophy of the corpus callosum; symmetric T2 and T2/FLAIR hyperintensities; cavitary formations secondary to necrosis of the central layers may occur
Differential Diagnoses:
- Ischemia: infarctions of the corpus callosum can cause cavitary formations.
- Demyelination: conditions such as multiple sclerosis or neuromyelitis optica spectrum disorder may originate T2 hyperintense callosal lesions.
- Malignancy: gliomas or primary SNC lymphomas may rarely occur in this region.
- Metabolic: conditions such as hemolytic-uremic syndrome, hypoglycemia, Wilson disease, or drug toxicity may cause similar cytotoxic lesions of the corpus callosum.
Treatment:
- No treatment option is currently available.
