SLIPPERS: Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids: A Case Report (P5.118)

Objective: To describe two cases of a supratentorial variant of CLIPPERS we propose to call SLIPPERS Background: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an inflammatory disorder characterized by a combination of clinical symptoms correlating to magnetic resonance gadolinium enhancing lesions of the pons. Rare cases of CLIPPERS with additional supratentorial or spinal lesions have been reported. These inflammatory enhancements improve with steroid therapy, which manifest clinically, but return once steroids are discontinued. We have seen two patients with a similar condition confined to supratentorial locations, analogous to CLIPPERS, which we propose to call SLIPPERS. Methods/Design: The patients were seen at a tertiary medical center. We present two patients followed > 4 years. Patient A initially presented with recurrent seizure episodes; patient B with headache and hemiparesis. Results: In both cases, initial MRI revealed enhancing supratentorial lesions corresponding to initial clinical presentations. Initial work-up revealed negative serum and CSF testing for inflammatory and autoimmune markers as well as infectious etiologies. Both patients underwent brain biopsy: Patient A’s biopsy revealed perivascular inflammation consisting of CD4/CD8 T lymphocytes. Patient B’s biopsy revealed infiltrative CD3 T lymphocytes. Both patients were started on steroid therapy with partial clinical and radiologic response, but repeated recurrence upon tapering steroid therapy. Steroid sparing immunosuppressive therapy seemed effective for chronic suppression of inflammation. Conclusion: We have seen two cases of an entity that is clinically, radiologically and pathologically similar to CLIPPERS, but isolated to the supratentorial compartment, which we propose to call SLIPPERS. Disclosure: Dr. Armand has nothing to disclose. Dr. Graber has received personal compensation for activities with Stemedica Inc., Novocure Inc., and Biogen Idec. Dr. Lado has nothing to disclose. Dr. LaSala has nothing to disclose. Dr. Weidenheim has nothing to disclose.