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Published on: November 23, 2017
[Marchiafava-Bignami disease. 3 cases with favorable prognosis]
P Diraison1, F Rouhart, P Goas
1Service de neurologie, CHU la Cavale-Blanche, Brest, France.
Introduction:
Marchiafava-Bignami disease is a complication of chronic alcoholism, with acute or subacute demyelination of the corpus callosum. Although subacute and benign forms of the disease have been described since the development of CT scan and MRI, it has usually a poor prognosis.
Exegesis:
We report three cases of Marchiafava-Bignami disease with favorable outcome. One of the patient was comatose upon hospital admission. Interhemispheric dysconnection syndrome was evidenced in two patients. CT scan and MRI showed lesions extending to the callosal white matter in these patients.
Conclusion:
Potential existence of Marchiafava-Bignami disease should be investigated in patients presenting with chronic alcoholism and mental confusion. However, accompanying coma and white matter demyelination should not necessarily be considered of poor prognosis. Clinical evaluation of interhemispheric dysconnection is of value in patients presenting with chronic alcoholism and mental confusion.
Insights
Marchiafava-Bignami disease, a complication of chronic alcoholism, can present with severe symptoms like coma. However, this study shows that even in severe cases, favorable outcomes are possible with appropriate evaluation and care.
Area of Science:
- Neurology
- Neuroscience
- Radiology
Background:
- Marchiafava-Bignami disease (MBD) is a rare neurological complication associated with chronic alcoholism.
- It is characterized by demyelination of the corpus callosum, often leading to a poor prognosis.
Observation:
- This report details three cases of MBD with unexpectedly favorable outcomes.
- One patient presented in a comatose state, and two exhibited interhemispheric dysconnection syndrome.
- Neuroimaging (CT and MRI) revealed extensive white matter lesions in the callosal region.
Findings:
- Despite severe initial symptoms, including coma and significant white matter demyelination, MBD can have a positive prognosis.
- The presence of interhemispheric dysconnection syndrome was noted in some patients.
Implications:
- Clinicians should consider MBD in patients with chronic alcoholism and altered mental status, even with severe neurological signs.
- Coma and extensive demyelination do not automatically predict a poor outcome for MBD.
- Evaluating for interhemispheric dysconnection can be valuable in diagnosing and managing MBD.
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