[Marchiafava-Bignami disease]

Abstract

Introduction: This paper reviews the literature about Marchiafava-Bignami disease (MBD).

Development: Recognized since 1903, MBD is a rare condition mainly observed in hardened drinkers and resulting in demyelinization and necrosis of the corpus callosum and, occasionally, adjacent subcortical white matter. The symptomatology is diverse and with magnetic resonance imaging (MRI) is now possible to diagnose this disease on its early stages. The clinical course of MBD is varied and it may be acute, subacute or chronic. Although numerous etiological hypotheses have been proposed the pathophysiology of MBD remains unknown. A few cases have been described in nonalcoholics, demonstrating that ethanol alone is not responsible for the lesions. Neuropsychological deficits (interhemispheric disconnection syndrome) due to the callosal lesion are also frequently observed in MBD.

Conclusion: Since its initial description, a remarkable advance in our knowledge of MBD has been achieved.