[Diagnostic imaging of marchiafava-bignami disease]

Marchiafava-Bignami disease (MBD) is a rare alchol related disorder characterized by demyelination of the corpus callosum. Clinical features include impaired consciousness, seizure, dysarthria, limb hypertonus, frontal lobe symptoms in the acute stage and interhemispheric disconnection syndromes in the chronic stage. While autopsy was historically necessary for a definitive diagnosis of MBD, imaging systems such as X-ray computed tomography and magnetic resonance imaging have enabled in vivo diagnosis. We reviewed 91 reported MBD cases additional to our 9 own cases focusing on their imaging. The corpus callosum in MBD has irreversible necrosis secondary to edema and demyelination, often complicated by bleeding in the subacute stage and it becomes atrophic in the chronic stage. The observed patterns of corpus callosal lesions were highly variable, sometimes complicated by extra-callosal lesions in the frontal cortex, white matter, and putamen. Moreover, these lesions are sometimes detected in non-alcoholic patients. Although the underlying causes of MBD are still unknown, investigating its imaging characteristics together with clinical and pathological features will contribute to elucidating its etiology. Furthermore, MBD as an alcohol related disease is linked with neurological emergency presentation. Thus, early diagnosis and treatment are essential.