Long-term Outcomes of CLIPPERS (Chronic Lymphocytic Inflammation With Pontine Perivascular Enhancement Responsive to Steroids) in a Consecutive Series of 12 Patients

Abstract

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a central nervous system (CNS) inflammatory disease defined in 2010 by Pittock and colleagues.¹ They described 8 patients with common clinical, radiological, and pathological features of brainstem involvement that was responsive to and dependent on corticosteroid therapy. Quiz Ref ID The diagnostic criteria for CLIPPERS include the following: (1) episodic brainstem symptoms, (2) characteristic punctuate and curvilinear gadolinium-enhancing lesions peppering the brainstem (mainly in the pons) on magnetic resonance (MR) imaging, and (3) T-lymphocytic infiltrate with perivascular predominance in brain biopsy specimens. Pittock et al¹ suggested that CLIPPERS diagnosis could be made without brain biopsy if clinical and MR imaging features of the disease were present and if alternative diagnoses were excluded. Since then, 15 additional cases of CLIPPERS have been reported.^2-10