M a r c h i a f a v a B i g n a m i d i s e a s e ( M B D )… Click to show full abstract
M a r c h i a f a v a B i g n a m i d i s e a s e ( M B D ) i s a r a r e neuropsychiatr ic disease that i s most frequently encountered in people with alcoholism, but also in people who are malnourished (1,2). MBD is pathologically characterized by symmetrical demyelination, necrosis, cystic degeneration, and atrophy of the corpus callosum (CC), usually in its central parts; these characteristics can also be accompanied by extracallosal lesions (1,2). Although the etiology and mechanisms behind MBD remain obscure, its relationships with alcohol abuse and malnutrition suggest that the underlying cause may be metabolic (3). One hypothesis is that alcohol neurotoxicity or nutrient deficiency may contribute to cerebral hypometabolism and acute demyelination of the CC and, in severe cases, lead to cell necrosis (4). Patients with MBD frequently present with unconsciousness, confusion, dysarthria, impaired walking, mutism, and seizures (1). Intracerebral hemorrhage (ICH) occurs primarily due to substandard management of hypertension or, less frequently, rupture of a vascular malformation or cerebral amyloid angiopathy (CAA). It is occasionally caused by a hemorrhagic diathesis or cocaine or alcohol abuse (5). Microhemorrhages have been found in the CC, cortex, and subcortex of several patients with MBD (6-10). To our knowledge, ICH has not been observed in a patient with MBD to date. This case report presents the first case of a patient with a history of chronic alcoholism presenting with MBD and concurrent ICH in the left external capsule.
               
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