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SynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferences
Emergency: requires immediate attention
Marchiafava-Bignami disease
Other Resources UpToDate PubMed
Emergency: requires immediate attention

Marchiafava-Bignami disease

Contributors: Carolyn Zyloney MD, Richard L. Barbano MD, PhD
Other Resources UpToDate PubMed

Synopsis

Marchiafava-Bignami disease is a rare, toxic, neurodegenerative disorder associated with alcohol use disorder and malnutrition and manifesting with neurological symptoms and findings on brain MRI. Cortical laminar necrosis and myelin destruction of the corpus callosum are the most common imaging findings. Patients can present with an acute onset of neurological symptoms or with a subacute to chronic decline. Signs and symptoms may include decline in mental function, memory loss, and unsteady gait. Advanced stage of decline includes delirium, disturbed gait, convulsions, and coma.

Onset is mostly in middle-aged and older men with a history of alcohol use disorder, but it can also occur in malnourished patients who lack a history of alcohol use. Therapies include thiamine supplementation and steroids, with the greatest negative outcome related to a delayed start of thiamine treatment.

Codes

ICD10CM:
G37.1 – Central demyelination of corpus callosum

SNOMEDCT:
386766007 – Marchiafava-Bignami disease

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Best Tests

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Management Pearls

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Therapy

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References

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Last Reviewed:04/04/2019
Last Updated:05/16/2019
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Emergency: requires immediate attention
Marchiafava-Bignami disease
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A medical illustration showing key findings of Marchiafava-Bignami disease : Seizures, Alcohol use, Ataxia, Coma, Delirium, Gait disturbance, Personality changes, Malnutrition, Memory loss, Slurred speech, Impaired cognition
Copyright © 2022 VisualDx®. All rights reserved.