Marchiafava-Bignami disease presenting as acute dysarthria and ataxia.
نویسنده
چکیده
Dear editor, This is a case of a 68-year-old right-handed man admitted to the hospital with 2 days of acute onset of slurred speech and unsteady gait. He denied any other neurological complaints. His past medical history was significant for alcohol dependence with daily alcohol use and 1 day a week of binge drinking for >10 years. He denied any history of alcohol-related seizures, DTs, psychiatric diagnosis or alcohol-related legal or interpersonal problems. The patient was oriented, able to name, read, repeat and follow commands. There was impaired shortterm memory. He had severe dysarthria. Cranial nerves were intact. There was decreased vibration at toes with hyporreflexia at ankles; the remaining reflexes and motor exam were normal. There was dysmetria in all limbs and a wide-based ataxic gait. The differential diagnosis was a vascular event, alcohol-related cerebellar degeneration, Wernicke’s encephalopathy, Marchiafava–Bignami disease, paraneoplastic cerebellar degeneration or a posterior fossa mass. The baseline CBC and comprehensive metabolic panel were within normal limits except for an elevated white blood cell count and mildly elevated AST. Baseline vitamin levels were not obtained. The MRI-brain showed abnormal high DWI (diffusion-weighted imaging) and T2 signal in the splenium of the corpus callosum with no mass effect or edema and scattered T2 signal hyperintensities within the bilateral hemispheric white matter (Fig. 1). A diagnosis of Marchiafava–Bignami disease (MBD) was made. The patient was started on high-dose thiamine intravenously for 5 days (500 mg three times a day) and oral vitamin B
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Marchiafava-Bignami Disease: Clinical and MRI Findings in Two Patients
Marchiafava-Bignami disease (MBD) is a rare disorder characterized by the degeneration of the corpus callosum. Before the era of magnetic resonance imaging (MRI), the diagnosis of MBD was usually made on autopsy because of the wide spectrum of clinical manifestation (dementia, frontal lobe releasing signs, inability to walk, hemiparesis, dysarthria, dysphagia, spasticity, and altered consciousn...
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3. Gimeno MJ, Lasierra R, Pina JI. Marchiafava—Bignami disease. Four case reports. Rev Neurol. 2002;35:596—8. 4. Vázquez C, Salamano R, Legnani C, Cardinal P. Marchiafava— Bignami disease in Uruguay. Neurologia. 2008;23:322—8. 5. Rusche-Skolarus LE, Lucey BP, Vo KD, Snider BJ. Transient encephalopathy in a postoperative non-alcoholic female with Marchiafava—Bignami disease. Clin Neurol Neurosur...
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ورودعنوان ژورنال:
- Alcohol and alcoholism
دوره 50 2 شماره
صفحات -
تاریخ انتشار 2015