Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids

Abstract

In this chapter we describe a case of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) in which a 16-year-old female presented with a protracted course of gait instability and sensory changes which progressed over 5 years to loss of ambulation. MRI with contrast revealed pathognomonic curvilinear and punctate lesions peppering the brainstem and spinal cord. Together, the combination of gait ataxia, spinal cord localizing focal neurological deficits, and characteristic imaging findings led to the diagnosis of CLIPPERS. In this chapter, the clinical presentation, diagnostic criteria, and necessary workup for excluding other processes that may mimic this disease are described. Furthermore, therapeutic interventions are discussed, and the relevance and possible necessity of brain biopsy are examined.