CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) is an extremely rare neurologic inflammatory condition. Fewer than 10 pediatric cases have been described.

Debate persists as to whether it is a distinct disease or a clinical, radiologic, and histologic phenotype evolving into another disorder. We propose that CLIPPERS may be a clinical manifestation of an underlying state of immune-dysregulation.

We describe the case of the youngest known report of CLIPPERS, an 18-month-old infant from Melbourne, Australia. Reviewing the literature for all reported pediatric cases, we identified that robust investigation and whole exome sequencing was underutilized and proposed diagnostic criteria were frequently unmet. Particular focus should be paid to genes known to cause familial hemophagocytic lymphohistiocytosis (HLH), with the CLIPPERS phenotype manifesting as a form of isolated central nervous system (CNS)-HLH in some patients. Curative treatment options such as hematopoietic stem cell transplantation may be appropriate for some patients and should be considered early.

CLIPPERS（慢性淋巴细胞炎症伴脑桥血管周围增强，对类固醇有反应）是一种极为罕见的神经系统炎症。已描述的儿科病例不到 10 例。

关于它是一种独特的疾病还是一种临床、放射学和组织学表型演变成另一种疾病的争论一直存在。我们提出，CLIPPERS 可能是潜在免疫失调状态的临床表现。

我们描述了已知最年轻的 CLIPPERS 报告病例，这是一名来自澳大利亚墨尔本的 18 个月大的婴儿。回顾所有报告的儿科病例的文献，我们发现强有力的调查和全外显子组测序未得到充分利用，并且提出的诊断标准经常未得到满足。应特别关注已知会导致家族性噬血细胞性淋巴组织细胞增多症 (HLH) 的基因，在某些患者中，CLIPPERS 表型表现为一种孤立的中枢神经系统 (CNS)-HLH。造血干细胞移植等治愈性治疗方案可能适合某些患者，应尽早考虑。