Doença de Marchiafava-Bignami: uma rara entidade com prognóstico sombrio

Abstract

Marchiafava-Bignami disease is a rare affliction characterized by primary degeneration of the corpus callosum associated with chronic consumption of ethanol. The disease may occasionally occur in patients who are not alcoholics but are chronically malnourished. A complex deficiency of group B vitamins is the main etiopathogenic hypothesis, and many patients improve after the administration of these compounds. However, a good response is not always observed. The definitive diagnosis of Marchiafava-Bignami disease can be problematic and is based on features of neuroimaging studies, especially magnetic resonance imaging. Its treatment is still controversial and shows variable results. Because nutritional factors are implicated, as in Wernicke's encephalopathy, some authors claim that replacement of B vitamins is beneficial. The present article is a case report of a severe acute form of Marchiafava-Bignami disease in an alcohol-dependent male patient who improved after the administration of parenteral B vitamins. As a consequence of his neurological and immunologic conditions, he developed multiple pulmonary infections and had a protracted course in the intensive care unit. He eventually died of sepsis associated with an uncommon fungus, Rhodotorula mucilaginosa. The present article reports the clinical and neuroimaging data from this patient and contains a review of Marchiafava-Bignami disease and Rhodotorula infections in the intensive care unit.