Marchiafava–Bignami Disease

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     A 45-year-old man with chronic alcoholism was found at home with altered consciousness. On admission, neurologic examination showed disorientation, perseveration, dysarthria, dyspraxia, peripheral sensory neuropathy, and coarse rigidity of all four limbs. The mean corpuscular volume was 99 μm3, the serum -glutamyltransferase concentration was 133 U per liter (normal range, 5 to 53), and the serum thiamine concentration was 63 nmol per liter (normal range, 70 to 185). Magnetic resonance imaging of the brain showed cerebral and cerebellar atrophy and diffuse swelling of the corpus callosum in the axial view (Panel A, arrow) and in the sagittal view (Panel B, arrows). The findings were consistent with Marchiafava–Bignami disease. There was no decrease of the apparent-diffusion coefficient, and there were no extracallosal lesions. Intravenous thiamine (100 mg) was given, with clinical and radiographic improvement. The patient was discharged and referred to a specialized center for recovery from alcohol dependence.

    Philippe Demaerel, M.D., Ph.D.

    Wim Van Paesschen, M.D., Ph.D.

    Katholieke Universiteit Leuven

    Leuven B-3000, Belgium

    http://www.100md.com/html/DirDu/2006/11/29/30/64/80.htm