Objective:

Background:

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an inflammatory disorder that presents with MRI enhancing lesions of the pons with corresponding clinical symptoms. To date there have been few published cases of CLIPPERS with supratentorial lesions which have also been steroid responsive with immediate clinical and radiographic worsening upon cessation of steroid therapy. Two patients with supratentorial, steroid responsive lesions are presented to support the growing literature for SLIPPERS as its own clinical entity.

Design/Methods:

Patients received care at tertiary medical center. Two patients were followed for >1 year and >10 years. Patient A initially presented with confusion and fatigue, sleeping >20 hours per day and patient B presented with clumsiness, falls and significant fatigue.

Results:

Both initial MRIs revealed enhancing supratentorial lesions corresponding to clinical presentations. Evaluation included CSF testing for inflammatory, autoimmune and infectious etiologies. Patient A underwent brain biopsy with confirmation of perivascular T-cell lymphocytic infiltration and gliosis with GFAP staining. Patient A was initiated on steroid therapy with rapid clinical and radiographic improvement with immediate clinical worsening upon cessation. Patient A has since been maintained on immunosuppression for almost one year. Patient B did not undergo biopsy but was exquisitely steroid responsive and subsequently switched to steroid sparing immunosuppression with disease remission for >10 years.

Conclusions:

We present tow cases of a disease pattern clinically and radiographically consistent with SLIPPERS with disease activity in the supratentorial region.