Longitudinal Imaging in Marchiafava-Bignami disease. (P6.025)

OBJECTIVE: To present a case of Marchiafava-Bignami disease BACKGROUND: Marchiafava-Bignami disease is rare disorder characterized by demyelination and necrosis of corpus callosum secondary to chronic alcoholism. The clinical features include cognitive impairment, dysathria, UMN signs, symptoms of interhemispheric disconnection. In pre-imaging era, the diagnosis was established by autopsy. With the advancement in imaging techniques, computed tomography (CT) and magnetic resonant imaging (MRI) provide opportunity of a reliable in vivo diagnosis. DESIGN/METHODS: This is a case report. We searched Pubmed without time limitation for words Marchiafava-Bignami disease, corpus callosum demyelation, radiologic features and compared our case with the reported cases. RESULTS: Our patient is a 42 year old African American woman with history of chronic alcohol abuse who presented with 2 day history of difficulty in speech, confusion and hallucinations. Patient reported alcohol abuse for past 22 years and used to drink 1-1.5 pint of liquor everyday. Neurological exam was significant for cognitive impairment, dysarthric speech, spasticity of all 4 limbs, hyperreflexia with bilateral ankle clonus, upgoing toes and spastic gait. Brain MRI showed T2 hyperintensities restricted to the genu and splenium of the corpus callosum on FLAIR. A DTI was done to evaluate the integrity of fiber tracts of corpus callosum. It showed corresponding fiber tract disruption in the corpus callosum. The repeat imaging done 18 months later, showed markedly reduced signal abnormality. CONCLUSIONS: Marchiafava-Bignami disease is a rare disorder mainly seen in men with chronic alcoholism. Clinical features described in the literature mainly are coma, seizure, cognitive impairment, dysarthria and signs of interhemispheric disconnection. Our patient mainly had neuropsychiatric features, cognitive impairment, dysathria and pyramidal signs on the exam and radiologic features of corpus callosum fiber disruption and demyelination, which significantly improved in next 18 months. Study Supported by: Department of Neurology and Neuroscience, NJMS, Newark, NJ Disclosure: Dr. Bhat has nothing to disclose. Dr. Raval has nothing to disclose. Dr. Marks has nothing to disclose.