MARCHIAFAVA-BIGNAMI DISEASE

Course overview

MARCHIAFAVA-BIGNAMI DISEASE (MBD) IS A VERY RARE DISORDER CHARACTERIZED BY DEMYELINATION AND NECROSIS OF THE CORPUS CALLOSUM. THE ADJACENT SUBCORTICAL WHITE MATTER MAY ALSO BE INVOLVED. THIS ACTIVITY DESCRIBES THE PATHOPHYSIOLOGY, ETIOLOGY, PRESENTATION, AND MANAGEMENT OF MBD. ALTHOUGH CLINICAL FEATURES MAY BE QUITE VARIABLE AND NONSPECIFIC, MARCHIAFAVA-BIGNAMI DISEASE SHOULD BE CONSIDERED IN PATIENTS WITH CHRONIC ALCOHOL ABUSE OR MALNUTRITION WHO PRESENT WITH CERTAIN NEUROLOGICAL SYMPTOMS. THESE SYMPTOMS INCLUDE DEMENTIA, ALTERED MENTAL STATUS, SPASTICITY, DYSARTHRIA, ATAXIA, GAIT ABNORMALITIES, AND SEIZURES. OTHER REPORTED CLINICAL FINDINGS INCLUDE DESYNCHRONIZATION OF BILATERAL SYMMETRIC MOVEMENTS, AND UNILATERAL SIGNS OF AGRAPHIA, APRAXIA, DYSPRAXIA, AND ANOMIA. THIS ACTIVITY ILLUSTRATES THE EVALUATION AND MANAGEMENT OF MARCHIAFAVA-BIGNAMI DISEASE AND REVIEWS THE ROLE OF THE INTERPROFESSIONAL TEAM IN IMPROVING CARE FOR PATIENTS WITH THIS CONDITION.