Marchiafava Bignami syndrome

Case contributed by Assoc Prof Frank Gaillard

Presentation

No past medical or psychiatric history. Presented to psychiatry unit 10 months ago due to apathy, withdrawn, loss of appetite and loss of weight; which has occurred within several months. He was diagnosed with major depressive disorder and treated as such. He re-presented 6 months ago with severe loss of weight and withdrawn, his speech output has reduced to minimal and currently can't hold conversation at all. He also appeared very cachectic. Also noted one possible episode of auditory hallucination. Noted this afternoon had stiffness of unilateral lower limb. No fever.

Patient Data

Age: 30 years
Gender: Male
MRI

The corpus callosum is abnormal with extensive high T2 signal and marked volume loss and cavitation. Additional white matter abnormality is seen more extensively throughout both hemispheres with ventriculomegaly. 

Case Discussion

This case illustrates what are probably features of prior Marchiafava Bignami syndrome. Subsequent history obtained from the patient, confirmed the presence of a period of severe binge drinking while depressed.

Prior to this, there was no history of alcohol abuse which would have made Marchiafava Bignami syndrome unlikely. Although predominantly white matter abnormalities, the appearances are not those of a leukodystrophy, CADASIL or MS/ADEM. HIV negative and non-immunosuppressed makes PML unlikely (and again I am not sure it involves the cc to this extent). That there was a vague history of hyponatremia does raise the possibility of extrapontine demyelination, however I am not aware of it involving the body of the corpus callosum (rather the splenium). 

Other possibilities include a leukoclastic vasculitis or encephalitis illness e.g. SLE, systemic sclerosis, etc.

PlayAdd to Share

Case information

rID: 16974
Published: 6th Mar 2012
Last edited: 17th Jun 2021
Inclusion in quiz mode: Included

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.