Fact-sheet: Marchiafava Bignami Disease
Updated on 04/18/2017 at 7:03 AM
View all RADEOS cases associated with this fact-sheet
Definition
Described in 1903 by 2 pathologists: E Marchiafava and A Bignami.
Rare complication of chronic alcoholism.
Acute demyelination and necrosis of the corpus callosum and centrum semiovale.
Clinical features
2 forms:
- Acute, rapidly fatal form: impaired consciousness progressing to coma, seizures, ataxia, dysarthria, diffuse hypertonia, pyramidal signs.
- Chronic form: progressive dementia with episodes of impaired consciousness, disconnection syndrome, pyramidal syndrome, and cerebellar syndrome.
CT
Hypodense lesions of the corpus callosum, splenium and genu ++
MRI
Symmetric lesions involving the central layer of the corpus callosum, extracallosal lesions: periventricular ++
Acute stage:
- Diffuse T2 hyperintensity of the corpus callosum reflecting edema
- Diffusion hyperintensity with decreased ADC in the acute stage, increased ADC in the subacute stage indicating likely upcoming evolution toward a necrotic appearance
Subacute and chronic stage:
- Cystic necrotic lesions with T1 hypointensity, diffuse involvement of the corpus callosum or localized to the genu, splenium, or body
- Corpus callosum atrophy
Differential diagnosis
Differential diagnoses of corpus callosum lesions:
- Ischemia: central involvement of the corpus callosum
- Inflammatory lesions: ADEM with associated multiple lesions
- Traumatic lesion
- Chronic hydrocephalus: involvement of the posterior midline portion of the corpus callosum
- Tumor invasion: high-grade glioma, lymphoma