[A case of Marchiafava-Bignami disease that responded to high-dose intravenous corticosteroid administration].

We report a 63-year-old male who presented acute confusional state with prominent rigidity of all extremities. The patient had a long history of alcoholism and was in poor physical condition and malnourished. The diagnosis of Marchiafava-Bignami disease was indicated by T2-weighted MRI demonstrating high signal intensity in the corpus callosum. Because Wernicke's encephalopathy was suspected as a complication, thiamine therapy was applied, but there was no clinical improvement. Immediately after high-dose intravenous corticosteroid administration was started, his symptoms rapidly resolved except for mild cognitive impairment. It is supposed that edematous change in the early stages of Marchiafava-Bignami disease causes impairment of the blood-brain barrier. Steroid therapy may prevent such a process by means of its the anti-edema effect and by normalizing blood-brain barrier. The present case suggests that corticosteroid therapy has beneficial effects for Marchiafava-Bignami disease.