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[Marchiafava-Bignami disease in Uruguay].
C Vázquez1, R Salamano, C Legnani
1Instituto de Neurología, Hospital de Clínicas, Facultad de Medicina, Universidad de la República, Montevideo, Uruguay.
Neurologia (Barcelona, Spain)
|February 6, 2008
Summary
Marchiafava-Bignami disease (MBD), a rare alcohol-related condition, affects the corpus callosum. Early detection through neuroimaging and neuropsychological assessment is crucial for managing this severe neurological disorder.
Area of Science:
- Neurology
- Radiology
Background:
- Marchiafava-Bignami disease (MBD) is a rare neurological disorder associated with chronic alcoholism.
- It is characterized by demyelination and necrosis of the corpus callosum, often presenting with a poor prognosis.
Observation:
- Two fatal cases of MBD are presented, both with acute onset coma.
- Clinical signs included impaired consciousness, hypertonia, and frontal release signs.
- One patient exhibited optic neuropathy, while the other had neuropsychiatric symptoms and pellagra-like skin lesions prior to coma.
Findings:
- Computed tomography (CT) and magnetic resonance imaging (MRI) revealed white matter lesions predominantly in the corpus callosum.
- Neuropsychological evaluation is essential for identifying prodromal neuropsychiatric symptoms and signs of interhemispheric disconnection.
Implications:
- MBD should be considered in chronic alcoholics presenting with prolonged confusion, stupor, or coma.
- Thorough investigation of the corpus callosum via imaging is vital for diagnosing this potentially underdiagnosed condition.
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