Rapid Recovery from Marchiafava-Bignami Disease after Intravenous Corticosteroid Administration

Abstract

Background: Marchiafava-Bignami disease (MBD) is a rare disorder characterized by acute demyelination of the corpus callosum in people with chronic alcoholism. Early thiamine treatment can reduce neurological complications of MBD. However, the outcome of MBD is usually unfavorable. Here we report a case of MBD responded to intravenous corticosteroid administration. Case Report: 46-year-old woman with chronic alcoholism presented to our emergency department with two days of dysarthria, dysphagia, extremity weakness, and decreased consciousness. Diffusion-weighted image and fluid attenuated inversion recovery revealed high signal intensities of the left frontal cortex and the entire corpus callosum. After receiving high dose intravenous thiamine and multivitamin, she did not improve. At the third day of admission, intravenous steroid was started. Her mental status and weakness were rapidly improved. At 2 weeks later, she could walk independently. Follow-up MRI revealed only a small lesion in the splenium of corpus callosum. Conclusion: The MBD in our case is associated with thiamine deficiency with main pathology of edema and demyelination of corpus callosum. Corticosteroid stabilized blood brain barrier and reduced inflammation and edema. Therefore, corticosteroid therapy might have beneficial effects as an alternative therapy for MBD. Key Words: Marchiafava-Bignami disease; Corticosteroid; Extracallosal involvement