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.
Emergency: requires immediate attention
Marchiafava-Bignami disease
Alerts and Notices
Important News & Links
Synopsis
Codes
ICD10CM:
G37.1 – Central demyelination of corpus callosum
SNOMEDCT:
386766007 – Marchiafava-Bignami disease
G37.1 – Central demyelination of corpus callosum
SNOMEDCT:
386766007 – Marchiafava-Bignami disease
Look For
Subscription Required
Diagnostic Pearls
Subscription Required
Differential Diagnosis & Pitfalls
To perform a comparison, select diagnoses from the classic differential
Subscription Required
Best Tests
Subscription Required
Management Pearls
Subscription Required
Therapy
Subscription Required
References
Subscription Required
Last Reviewed:04/04/2019
Last Updated:05/16/2019
Last Updated:05/16/2019