A Case Report of CLIPPERS (Chronic Lymphocytic Inflammation with Pontocerebellar Perivascular Enhancement Responsive to Steroids) Syndrome

Excerpt

Chronic lymphocytic inflammation with pontocerebellar perivascular enhancement responsive to steroids (CLIPPERS) syndrome is a recently described central nervous system (CNS) disorder characterized by distinctive radiological and clinical features [1]. It is a pontine-centric disorder [1] with typical imaging features consisting of symmetric curvilinear gadolinium enhancement peppering the pons, cerebellum, and adjacent structures [1‐3]. Almost all patients with CLIPPERS syndrome have clinical symptoms related to the involvement of brainstem and cerebellum [1‐3]. Subacute gait ataxia, diplopia, and cerebellar dysarthria are the cardinal clinical features in the majority of cases with CLIPPERS [1‐3]. Immunopathological studies of patients with CLIPPERS syndrome have shown a characteristic, but nonspecific, severe perivascular T-cell-predominant infiltrate, without apparent pathological findings of other known infectious, inflammatory, or demyelinating diseases [1, 2]. There is an ongoing discussion whether CLIPPERS is a syndrome or an entity, because no specific biomarker for CLIPPERS has been identified yet, and a body of increasing evidence show that this disorder overlaps with other syndromes [4, 5]. Although the exact pathophysiological mechanism of this syndrome has not been fully elucidated, neuropathological findings and the clinical response to immunosuppressant agents, especially steroids, suggest an autoimmune or other inflammation mediated pathogenesis [1]. Herein, we present a new case with the clinical and radiological findings consistent with CLIPPERS syndrome. …