Inflammatory Variant of Progressive Multifocal Leukoencephalopathy; in a Patient with Early Systemic Lupus Erythrematosus (P07.055)

Objective: Systemic Lupus Erythrematosus (SLE) itself may predispose risk of developing Progressive Multifocal Leukoencephalopathy (PML) Background A case report of a 55 year old female with a four month history of SLE who developed an inflammatory variant of PML in the setting of minimal immunosuppression. Design/Methods: Case report. Results: A 55 year female was recently diagnosed with SLE and placed on low dose Plaquenil and Prednisone. Four months later she developed Herpes Zoster and was subsequently treated. Brain MRI was obtained in the setting neurologic decline and revealed multiple T2 nonenhancing hyperintensities in the deep white matter. Rheumatologic and infectious serologies were significant for positive Lyme IGM Western Blot; all other were negative. CSF revealed JC Virus PCR was positive. She was treated for Lyme with IV Ceftriaxone, Prednisone titration was initiated, and Plaquenil was discontinued. The patient was referred to a PML specialist for further evaluation who felt that the rapid evolution of the symptoms, short exposure to steroids, and lack of profound imunnosuppression made the diagnosis of PML less likely. Four weeks later, the patient continued to have clinical worsening and new MRI enhancement. A brain biopsy was performed which revealed a macrophagic leukoencephalitis and glial intranuclear inclusions characteristic of PML with prominent perivascular inflammation. In situ hybridization for JC virus confirmed the diagnosis. Conclusions: Classically PML is seen in immunosuppressed patients although it has also been identified in patients with rheumatologic diseases, in particular SLE. Patients may have atypical onset of neurological deficits, inflammatory features on neuroimaging and biopsy, despite the lack of a profound immunosuppression or a short duration since initial diagnosis. The assumption that all new deficits are SLE flares and continued treatment with immunosuppressants could be detrimental hence the recognition of PML in the setting of SLE is critical to outcome. Disclosure: Dr. Medin has nothing to disclose. Dr. Coyle has received personal compensation for activities with Acorda Therapeutics, Avanir Pharmaceuticals, Bayer Pharmaceuticals Corporation, Biogen Idec, Genzyme Corporation, Novartis, Questcor, Roche Diagnostics Corporation, Sanofi-Aventis Pharmaceuticals, Inc., and Teva Neuroscience. Dr. Coyle has received personal compensation in an editorial capacity for NEURA. Dr. Coyle has received research support from Serono, Inc., Novartis, and Sanofi-Aventis Pharmaceuticals, Inc. Dr. Seidman has nothing to disclose. Dr. Al-Mufti has nothing to disclose. Dr. Kim has nothing to disclose.