Unusual case of Marchiafava-Bignami disease presenting as axial hypotonia

Abstract

Introduction. Marchiafava-Bignami disease is a rare disor­der mostly associated with chronic heavy alcohol consump­tion that results in progressive demyelination and necrosis of the corpus callosum. Case report. We reported a 35-year-old woman with a history of alcohol consumption and malnutrition. Neurological examination revealed axial hy­potonia, dysarthric speech and lack of motor coordination. The brain multislice computed tomography imaging dem­onstrated hypodense lesion of the corpus callosum. On the basis of her history, clinical features and imaging studies, the diagnosis of an acute form of Marchiava-Bignami disease was made. Definite diagnosis was confirmed at autopsy. Conclusion. Marchiafava-Bignami disease is of a medical emergency and early recognition and early aggressive treat­ment are critical for a good clinical outcome. To our knowl­edge, this is the first case of Marchiafava-Bignami disease presented with axial hypotonia.