Approach Considerations
In 2017, Hillborn et al. [27] reviewed data from 153 cases of Marchiafava-Bignami disease (MBD) confirmed by brain imaging. They observed a significant trend for a better overall outcome in subjects who were treated with thiamine compared to those who remained untreated. The dose of thiamine should be the same as recommended for Wernicke’s disease, and the therapy should continue for as long as recovery is going on. Multiple studies have shown that early administration of parenteral thiamine is associated with better outcomes, particularly if administered within 2 weeks of symptom onset. [27] Corticosteroids are often used in the treatment of MBD and may reduce brain edema, suppress demyelination, stabilize the blood-brain barrier, and reduce inflammation. However, in their analysis Hillborn et al. could not observe any positive net effect. This may have been because many cases were treated with both steroids and multivitamins, and it was difficult to see which played the major role in recovery. However, they reported no adverse effects associated with steroid treatment.
With regard to more unusual treatments, a case report by Staszewski et al described amantadine given together with thiamine, vitamin B-12, and folate; the patient improved. [16] In another case, reported by Kikkawa et al, administration of high-dose corticosteroids was said to precede clinical improvement. In patients who improved, the CT and MRI scan findings also improved, at least somewhat. [28]
Inpatient care
Patients are usually admitted because they present with stupor, coma, and, frequently, seizures.
Follow-up
Patients who survive should receive rehabilitation and, if appropriate, alcohol and nutritional counseling.
Consultations
Depending on the specific presentation and course of MBD, the patient may require consultation with the following specialists:
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Neurologist - For seizure control
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Critical care specialist - For coma management
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Neuropsychologist - For workup of the dementia
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Neurorehabilitation specialist
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Psychiatrist or psychologist - For treatment of alcoholism
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T2-weighted axial image in a patient with Marchiafava-Bignami disease showing a high-signal lesion in the corpus callosum.
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Sagittal fluid attenuation and inversion recovery image displaying central hypointensity (suggesting cavitation) with surrounding hyperintense rim (active inflammation) (white arrowheads) involving the genu, body, and splenium of corpus callosum. Courtesy of Case Reports in Radiology, Hindawi Publishing Corp.
