A Case of Recurrent PML Associated with Cortical Intra-Neuronal JC Virus Inclusions in a Woman with HIV (P2.311)

OBJECTIVE: To describe a case of JC Virus spectrum disease in an immunocompromised patient with relapsing course and neuronal involvement. BACKGROUND: PML associated with JCV is a common cause of neurologic deterioration among HIV patients. JCV in HIV patients can have atypical imaging appearance including post-gadolinium enhancement. While PML has long been associated with intranuclear inclusions of JCV in glial cells, recently a novel phenotype has been found associated with productive neuronal infection, JCV Granule Cell Neuronopathy. Repeated survival and recurrence, as seen in our case, is very unusual. DESIGN/METHODS: Case Study RESULTS: A 47 year-old woman with HIV infection on HAART (CD4 count 154, nadir 19, VL 57) and previous PML diagnosis with residual L hemiparesis was referred to our hospital for eye pain and visual field defect. Examination showed a R homonymous hemianopia and L arm and leg contracture with weakness beyond baseline. MRI of brain showed a new 8mm cortically-based enhancing focus in the L occipital lobe. Lumbar puncture demonstrated 2 WBCs, normal protein and glucose, and negative bacterial and viral cultures. CSF cytology was negative for atypical or malignant cells. Due to concern of primary CNS lymphoma, brain biopsy was performed. Pathology showed perivascular inflammatory cell infiltrate. Stains were negative for bacteria, fungi, AFB, toxoplasma, CMV, HSV, and HPV. A papovavirus (SV40) stain was strongly positive consistent with PML. Intraneuronal inclusions consistent with JCV were found. The patient was continued on HAART and mirtazapine was started. CONCLUSIONS: This case highlights the significant workup that can be needed to confirm a diagnosis of PML in HIV patients. While cerebellar granule cell infection is an increasingly recognized variant of JCV associated disease, there have been few reports of intraneuronal JCV infection in the cortex. This case helps broaden the spectrum of disease associated with JCV in immunocompromised patients. Disclosure: Dr. Gutman has nothing to disclose. Dr. Weidenheim has nothing to disclose. Dr. LaSala has nothing to disclose. Dr. Graber has received personal compensation for activities with Stemedica Inc., Novocure Inc., and Biogen Idec.