Case Report: Atypical Presentation of PML in a Patient with Alcoholic Cirrhosis (P2.095)

OBJECTIF: To describe an atypical presentation of PML in a patient with an occult immunosuppresseded state secondary to liver disease. BACKGROUND: Progressive multifocal leukoencephalopathy (PML) is a rare but lethal demyelinating disease of the CNS caused by reactivation of the JC polyomavirus (JCV). PML is classically described in individuals with profound cellular immunosuppression such as patients with AIDS, hematological malignancies, organ transplant recipients or those treated with immunosuppressive or immunomodulatory medications. DESIGN/METHODS: Case report and literature review. RESULTS: We report a 55 year-old male with alcoholic cirrhosis who presented with acute onset left sided weakness. His examination was significant for right gaze preference, left hemiparesis, hyperreflexia in right patella and extensor response to plantar stimulation bilaterally. MRI of the brain showed multiple patchy areas of non-enhancing hyperintense T2 and FLAIR signals in bilateral occipital lobes, splenium, subcortical white matter and frontal gray-white matter junction. A few of these foci demonstrated diffusion restriction. No mass effect was seen. Cerebrospinal fluid was acellular, with normal glucose and protein. PCR analysis showed the presence of JCV DNA in CSF. Blood sample analysis demonstrated lymphopenia with only 180 CD4 and high CD4/CD8 ratio of 7.16. Antibodies against HIV, Hepatitis B and C were negative. An extensive investigation to look for other underlying immune-compromising disorders was negative. Based on MRI abnormalities and CSF JC virus PCR, the patient was diagnosed with PML. CONCLUSION: This is one of the few cases of confirmed PML with occult immunosuppression related to cirrhosis. This case highlights the unrecognized immunosuppressive state of cirrhosis and reiterates that PML should be considered as part of the differential diagnostic in any patient with cirrhosis who present with new-onset neurological symptoms. Timing in these cases is classically sub-acute but can be acute mimicking classic cerebrovascular etiologies. Disclosure: Dr. Baba-Ahmed has nothing to disclose. Dr. Neel has nothing to disclose.