A Case of Marchiafava-Bignami Disease with Personality Change

Kim, Hyoung Jun; Lee, Sang Yeol; Park, Min Cheol

Abstract

Marchiafava-Bignami Disease is a rare disorder characterized pathologically by demyelination of the corpus callosum. This disease could be related with chronic alcoholism although a proven etiology has not been reported. Marchiafava-Bignami Disease (MBD) could cause the fatal acute phase symptoms (seizure, coma, and death), various neurological symptoms (tremor, dysarthria, gait disturbance, apraxia), and cognitive impairments (memory impairment, disorientation). It is also reported that MBD causes a dementia. Approximately 250 cases have been reported regarding the MBD since it was first reported in 1903. However, only 20 cases have revealed a favorable prognosis. We found a case of MBD with personality change and chronic alcoholism subsequent to the repeated improvement and aggravation for 4 years. This case is demonstrated a atrophy of splenium of corpus callosum by Magnetic Resonance Imaging. The case also is diagnosed the personality changes, such as emotional irritability, impulsivity, and indignation-expose due to general medical condition, as described in Diagnostic and Statistic Manual of Mental Disorder 4th edition (DSM-IV). However, in early phase this diagnosis did not revealed. The patient was improved in impulse control and behavior by treatment with the Carbamazepine. We investigate the etiology, pathogenesis, symptom &sign, and treatment regarding the MBD in neuropsychiatric aspect.