Marchiafava-Bignami disease with rare Etiology: A Case Report

Abstract

Marchiafava-Bignami disease is a rare disorder of demyelination or necrosis of the corpus callosum and adjacent subcortical white matter that occurs predominantly in malnourished alcoholics[1]. The course of the disease may be acute, subacute, or chronic and is marked by dementia, spasticity, dysarthria, and inability to walk. Patients may lapse into coma and die, survive for many years in a demented condition, or occasionally recover[2]. Lesions appear as hypodense areas in portions of the corpus callosum on computed tomography (CT) and as discrete or confluent areas of decreased T1 signal and increased T2 signal on magnetic resonance imaging (MRI)[3]. Alcohol abusers without liver disease, amnesia, or cognitive dysfunction show thinning of the corpus callosum at autopsy[4] and on MRI[5,6], suggesting that alcohol or malnutrition damages the corpus callosum commonly in the absence of the necrotic lesions of Marchiafava-Bignami disease. These findings raise the possibility that aggressive nutritional supplementation along with a reduction in drinking can prevent the development of Marchiafava-Bignami disease in alcohol abusers. Here, we present a case report of MBD diagnosed in a 60 year-old male who presented with gait instability and slurred speech, in an effort to highlight the importance of obtaining MRI in patients presenting with behavioural disturbance and neurological findings, as well as discuss the relationship between thiamine supplementation and demyelinating diseases in the central nervous system.