神経学および神経科学ジャーナル

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Marchiafava-Bignami Disease: Uncertain MRI Predictors of Outcome

Fabiola De Marchi, Giacomo Tondo, Claudia Varrasi and Roberto Cantello

Marchiafava-Bignami disease (MBD) is a rare, often fatal, toxic neurological disease mainly characterized by demyelination of the corpus callosum, with or without associated lesions of hemispheric white matter. The clinical picture is severe, and includes both neuropsychiatric features, and signs of interhemispheric disconnection. The disease can have an acute or chronic course. Neuroimaging provides the most useful information for diagnosis and prognosis. We present a 44- year-old patient suffering from subacute onset of MBD, in who diffusion-weighted MRI revealed symmetrical hyperintense lesions in the frontal region of cerebral cortex and in the body/splenium of corpus callosum. Involvement of the corpus callosum was partial; the diagnosis was rather prompt, and the treatment was appropriated. The patient, survived to the acute phase, developed a chronic dementia.