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Abstract Details

Machiava Bignami: A Case of a 62 Year Old Woman with Malnutrition Who Presents with Altered Mental Status, Vision Loss with Acute Restricted Diffusion of the Splenium of the Corpus Callosum
General Neurology
P1 - Poster Session 1 (12:00 PM-1:00 PM)
6-005

To highlight a rare differential of altered mental status, vision loss, and MRI findings initially suggestive of stroke.

Marchiafava-Bignami is a rare disorder of demyelination or necrosis of the corpus callosum and adjacent subcortical white matter typically seen in patients who are chronic alcoholics, although there are reports in non-alcoholic, chronically malnourished patients as well.  Symptoms include mental status changes, memory loss, dysarthria, and ataxia.  Some patients could become stuporous, comatose or even experienced death. Head Computer tomography shows hypodense lesions of corpus callosum and Brain magnetic resonance (MRI) shows T1 hypointensities, and T2 hyperintensities.  Thinning of the corpus callosum or necrosis could be seen.

62 year old woman with a history of hypertension, diabetes, smoking, but no history of alcoholism presented with altered mental status. At baseline, she was independent but now disoriented and not following commands.  For four months prior to presentation, she had experienced severe, unexplained persistent vomiting that resulted in weight loss of 90 pounds, despite extensive gastroenterology work up.

She was found to have normocytic anemia with hemoglobin of 7.3g/dl and hypoalbuminemia 2.2g/dl.  An MRI of the brain showed restricted diffusion in the splenium of the corpus callosum. During hospitalization, she developed acute painless, bilateral vision loss. Given history and constellation of symptoms, she was empirically treated with intravenous Thiamine because of malnutrition and concern for Wernicke’s encephalopathy.  Within a day of starting IV thiamine, she regained her vision, encephalopathy resolved within a day or two after initiation of treatment.  Thiamine level was 15 (78-185nmol/L). 

The restricted diffusion noted on MRI involving the splenium of the corpus callosum subsequently improved on repeated MRIs which was consistent with Marchiafava Bignami. This case highlights the importance of recognizing how thiamine deficiency could cause similar MRI findings as seen in stroke, especially in patients with severe weight loss.
Authors/Disclosures
Ephrem Teklemariam, MD (Cooper University Hospital)
PRESENTER
Dr. Teklemariam has nothing to disclose.
Ryna Then, MD, FAAN (Jefferson Einstein Philadelphia Hospital) Dr. Then has nothing to disclose.