Marchiafava-Bignami Disease with Cortical Involvement.

Abstract

Marchiafava-Bignami disease (MBD) is a neurological degenerative disorder with a pathognomonic hallmark of symmetric demyelination in the corpus callosum (CC). Most reported cases were chronic alcoholics and some showed cortical lesions related to poor clinical prognosis. Herein we report a case of a chronic alcoholic who presented with confusion and generalized weakness. Nerve fiber integrity and metabolic changes were evaluated with Magnetic resonance imaging (MRI) sequences including diffusion tensor imaging (DTI) and MRS. MRI revealed the typical callosal lesions of MBD with bilateral frontoparietal cortical lesions. DTI and MRS showed both impaired myelin integrity and axonal density in the CC. The cortical lesions partly disappeared after intravenous administration of high-dose multivitamins and corticosteroids. The patient regained consciousness 3 months later while dysarthria and quadriplegia persisted. Three years later, the patient can interact occasionally with people and the functional activities of both upper and lower limbs have no improvement. To our knowledge, this is the first report of DTI together with MRS assisting in evaluating the prognosis of MBD.