Marchiafava-Bignami Disease: Case Report and Review of the Literature

Abstract

INTRODUCTION: Marchiafava-Bignami (MB) is a rare disease characterized by the primary degeneration of the callosum mainly associated with chronic consumption of alcohol. However, this condition can be mistaken for many other diseases which cause lesions of the corpus callosum. Even though it has been told that Carducci was the first in reporting a case of MB in 1898, this disease was originally named in honor of two Italian pathologists in 1903, Ettore Machiafava and Amico Bignami, who described the symptoms of an Italian male with excessive consumption of red wine. The majority of patients are male, aged between 40 to 60 years old, with a history of chronic alcoholism and malnutrition. In these cases morbidity and mortality rates are relatively high. In 2004, there were approximately 250 patients described, 200 of them died, 30 were reported with severe dementia and just 20 patients had a positive outcome. The treatment for MB is still controversial and shows changing outcomes. Some authors have proposed some benefits following the administration of vitamin B, corticosteroids and amantadine.

CASE REPORT: A 41-year-old male with a history of 15 years of chronic alcohol abuse drinking whisky and rum; presents disorientation, minor psychomotor agitation; during physical examination the patient presents dysarthry and ataxy. The family member of the patient reports that the patient has presented frequent forgetfulness even causing him labor problems. Nuclear magnetic resonance FLAIR images revealing the presence of demyelination of the corpus callosum, such as a little alteration of the white matter of the brain.