Marchiafava-Bignami disease: literature review and case report.

Objective: We postulated that disruption of callosal pathways as occurs in Marchiafava-Bignami disease (MBD) is associated with marked impairment in brain functioning as measured by cognitive assessment and neuroimaging.

Background: MBD is considered to be a rare and severe complication of chronic alcoholism. It is characterized by necrosis and subsequent atrophy of the corpus callosum, which is the major brain structure connecting corresponding areas of both hemispheres.

Methods: We review the existing literature on MBD with respect to conceptualization, theories of pathogenesis, forms of the disease, and neuroimaging and neuropsychological findings. We then present the case of a middle-aged man with MBD who underwent extensive clinical, neuropsychological, and neuroimaging studies.

Results: Neuropsychological evaluation revealed a pattern of severe global dementia. Magnetic resonance imaging showed moderate atrophy of anterior callosal regions and severe atrophy of posterior callosal regions in the setting of cortical and subcortical atrophy. Resting metabolism positron emission tomography revealed decreased glucose metabolism most pronounced in subcortical and mesial frontal regions. The differential diagnosis, function of the corpus callosum, and potential limitations of our case study are discussed.

Conclusions: On account of the history, clinical presentation, and results of magnetic resonance imaging of the brain, we diagnosed our patient with chronic MBD.