(Kiosk 4) Marchiafava-bignami Disease Leading to Protracted Hospital Course in Liver Transplant Recipient: A Case Report

Case Diagnosis: Marchiafava-Bignami Disease in the setting of recent liver transplant.

Case Description or Program Description: A 55 year old male with a history of alcoholic liver cirrhosis status post liver transplant presented with encephalopathy. Neuroimaging revealed new abnormal diffusion restriction of the corpus callosum. The diagnosis of Marchiafava-Bignami Disease (MBD) was made. Prior to liver transplant, patient was independent with mobility and activities of daily living (ADL) with a current functional status being dependent for transfers, inability to ambulate or propel wheelchair, and dependent to maximal assist for ADLs. Acute inpatient rehabilitation program was complicated by severe dysphagia with associated severe acute malnutrition, persistent metabolic and toxic encephalopathy with associated somnolence, decreased gastrointestinal motility and stage IV decubitus ulcer.

Setting: Inpatient rehabilitation facility.

Assessment/Results: Patient was placed on amantadine, thiamine, vitamin B12, folic acid, reglan, periactin and protein supplementations with daily wound care. Additionally, patient continued with immunosuppressive agents in addition to antimicrobial prophylaxis. Patient experienced minimal progress during rehabilitation course. Diet was eventually advanced to minced and moist, mildly thick liquids. However, he remained dependent with transfers, dependent with wheelchair propulsion 15 meters with maximal to moderate assist with ADLs. Repeat imaging no longer revealed hyperintensities along the corpus callosum.

Discussion (relevance): MBD is a rare disorder of demyelination or necrosis of the corpus callosum and adjacent subcortical white matter. These findings are present in malnourished alcoholics with the course of disease manifesting as dementia, spasticity, dysarthria and inability to walk, as in our patient. Although prognosis is poor, early recognition is imperative as patients may have the potential to recover.

Conclusions: This case report highlights the rare disease of MBD leading to debility in the setting of a recent liver transplant. Clinicians should be aware of this devastating disease in alcoholics in order to provide judicious management with aggressive nutritional supplementation along with a reduction in drinking.