Marchiafava-Bignami disease: a case report.

ABSTRACT

Marchiafava-Bignami Disease (MBD) is a rare, severe and usually fatal neurological disorder associated with chronic alcoholism. Previously, the definite diagnosis was confirmed at the autopsy. After the era of modern imaging technology, diagnosis was based on clinical profiles, history of alcoholism and specific location of pathology in corpus the callosum demonstrated by MRI. The authors reported a case of MBD in a 41 year-old alcoholic Thai male who presented with acute confusion and ataxia. MRI of the brain demonstrated demyelination, edema and necrosis of the corpus callosum with extensive symmetrical subcortical white matter lesions. He had a dramatic recovery after treatment with intravenous thiamine. Follow-up MRI revealed atrophic and cystic changes of the corpus callosum and almost complete resolution of the subcortical lesions. Recently, 15 cases of MBD with specific corpus callosal lesion, demonstrated by MRI, were published in the English literature. All had a favorable outcome after treatment with thiamine. Only one case had extensive extracallosal lesions and this case also had a good recovery after treatment. Now, MBD is not a fatal disease and early diagnosis and treatment are crucial.