Objective:

To support brain biopsy in cases with cerebral white matter changes in an “immunocompetent” patient with negative JCV PCR in cerebral spinal fluid.  

Background:

Progressive multifocal leukoencephalopathy (PML) is a rare condition that can lead to demyelination of the central nervous system (CNS). It is frequently linked with the Human Polyoma virus also known as John Cunningham virus (JCV) which targets the oligodendrocytes and astrocytes in the CNS. It is an opportunistic virus that preys principally on the immunosuppressed.  Nevertheless, it can be rarely observed in other patient populations. One such unique population will be discussed in the case below. 

Design/Methods:

N/A

Results:

A 65-year-old female with history of alcoholic cirrhosis, bilateral cryptogenic cerebellar infarcts, chronic hepatitis C, and type 2 diabetes mellitus presented with altered mental status, acute right hemiparesis and right lower extremity numbness. MRI brain showed confluent white matter changes in the left posterior frontoparietal vertex with peripheral diffusion restriction and T2 hyperintensity without post contrast-enhancement.  Additional imaging, serum and cerebral spinal fluid (CSF) studies were largely unexceptional including negative JCV PCR for PML. Brain biopsy was performed due to high clinical suspicion and resulted positive for JC virus DNA. Results were consistent with the diagnosis of PML. 

Conclusions:

Cirrhosis can result in systemic immunosuppression. Brain biopsy should be pursued in cases of hepatic dysfunction with high clinical suspicion for PML. This is indicated even in cases with negative CSF JCV PCR.