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.

Marchiafava-Bignami disease with rare etiology: A case report

“…The present study describes a patient who had undergone cardiac carcinoma surgery three years previously, and who had received chemotherapy three times; the last chemotherapy session was one-and-a-half years prior to admission. The etiology of the patient’s condition may have been similar to that described in a previous study, in which a metabolic disorder of the myelin in the brain of a patient with no long-term alcohol abuse, which was caused by long-term malnutrition involving protein, folic acid and particularly thiamine deficiency, led to demyelinating changes and MBD ( 9 ). The corpus callosum, which is the largest commissural fiber system within the hemisphere, has a relatively high myelin content, and may often cause the degeneration and necrosis of nerve cells in such patients.…”

“…To date, the etiology of MBD is incompletely understood, as it is one of the rare complications of chronic alcoholism ( 1 – 2 ). It is believed that MBD may be closely associated with chronic alcoholism and/or malnutrition caused by long-term alcohol abuse; however, MBD can also occur in patients who have no history of alcohol abuse ( 9 – 11 ). The main hypothesis for MBD pathogenesis is that the condition may be associated with malnutrition or vitamin B deficiency ( 9 ), although it is believed that MBD may have other etiologies.…”

“…It is believed that MBD may be closely associated with chronic alcoholism and/or malnutrition caused by long-term alcohol abuse; however, MBD can also occur in patients who have no history of alcohol abuse ( 9 – 11 ). The main hypothesis for MBD pathogenesis is that the condition may be associated with malnutrition or vitamin B deficiency ( 9 ), although it is believed that MBD may have other etiologies. There are reports on MBD caused by existing cyanide, CO poisoning and sepsis, as well as sickle cell disease and Plasmodium infection ( 3 ).…”

“…It appears that, with alcoholism, the prognosis is worse; however, MBD can occur in patients who have no history of alcohol abuse ( 9 – 11 ). The main hypothesis for the pathogenesis of MBD is that the condition is associated with malnutrition or vitamin B deficiency ( 9 ), although there are reports describing cases of MBD caused by existing cyanide, CO poisoning and sepsis, as well as sickle cell disease and Plasmodium infection ( 3 ). The treatment of MBD in those patients remains controversial; however, the administration of nutritional factors, such as high-dose group B vitamins and folic acid, is believed to be beneficial, and numerous patients have shown improvements following the administration of vitamin B therapy ( 9 , 12 ).…”

Clinical and radiological features of Marchiafava–Bignami disease

“…[ 1 , 2 ] Deficiency of group B vitamins might be the etiology of MBD; it is reported that many patients recover after treatment with B vitamins; however, others do not recover. [ 3 ]…”