Pediatric CLIPPERS: Expanding the Neuroimaging Spectrum of a Rare Steroid-Responsive Neuroinflammatory Syndrome-A Systematic Review
Abstract
Background: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an uncommon inflammatory disorder of the central nervous system that is exceptionally rare during childhood. Although pontocerebellar punctate enhancement is considered its neuroradiological hallmark, the full spectrum of pediatric clinical and imaging manifestations remain poorly characterized because available evidence is limited to isolated case reports and small case series. We conducted a systematic review to characterize the clinical presentation, neuroimaging phenotype, diagnostic challenges, therapeutic strategies, and outcomes of pediatric CLIPPERS. Methods: A systematic literature search was conducted in PubMed/MEDLINE and Scopus from database inception to December 2025, following PRISMA guidelines. Studies reporting patients younger than 18 years with a diagnosis of CLIPPERS were eligible. Clinical, neuroradiological, laboratory, histopathological, therapeutic, and outcome data were extracted using a standardized form. Diagnostic certainty was assessed according to the Tobin criteria, and methodological quality was evaluated using the Joanna Briggs Institute (JBI) critical appraisal tools. Results: Ten studies comprising 13 pediatric patients fulfilled the inclusion criteria. Median age at presentation was 13 years (range 3-16 years), with a slight male predominance. Ataxia was the most common presenting manifestation (84.6%), followed by cranial…
How strong is the evidence?
Evidence Hub reads this as a systematic review, done in people. Its evidence level is 1 of 5 (Highest · pooled evidence). Pooled evidence from several studies sits at the top of the evidence pyramid. Reported size: 13 participants or records.
Read the paper
Related studies
- A Survey on Knowledge Graphs: Representation, Acquisition, and Applications
- Toxicity of nanomaterials
- The Human Gene Mutation Database: towards a comprehensive repository of inherited mutation data for medical research, genetic diagnosis and next-generation sequencing studies
- Halogen bonding (X‐bonding): A biological perspective
- Biofilm formation at the air–liquid interface by the Pseudomonas fluorescens SBW25 wrinkly spreader requires an acetylated form of cellulose
- Opioid Activation of Toll-Like Receptor 4 Contributes to Drug Reinforcement
Go deeper in Evidence Hub
Evidence Hub adds a study data sheet (design, sample size, evidence level, key numbers), an AI summary and a chat grounded in the paper's text.