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Chronic Lymphocytic Inflammation With Pontine Perivascular Enhancement Responsive to Steroids Clinical Manifestations

Angel Phan1, Gilbert Handal2, Dale W Quest3

  • 1Department of Pediatrics, Texas Tech Paul L Foster School of Medicine, Texas Tech Health Sciences Center, El Paso, Texas.

Pediatric Neurology
|December 16, 2025
PubMed

Insights

Pediatric Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (C.L.I.P.P.E.R.S.) presents differently in children than adults. Early genetic studies and tailored treatments, including stem cell transplant for genetic cases, are crucial for better outcomes.

Area of Science:

  • Pediatric Neurology
  • Neuroimmunology
  • Radiology

Background:

  • Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (C.L.I.P.P.E.R.S.) is a rare condition affecting children.
  • Pediatric C.L.I.P.P.E.R.S. exhibits distinct clinical and radiological features compared to adult presentations.

Purpose of the Study:

  • To explore the clinical manifestations, radiological findings, associated diseases, treatment strategies, and outcomes of C.L.I.P.P.E.R.S. in pediatric patients.
  • To differentiate pediatric C.L.I.P.P.E.R.S. from adult cases and inform optimized diagnostic and therapeutic approaches.

Main Methods:

  • Case study of an 11-year-old male diagnosed with C.L.I.P.P.E.R.S.
  • Comprehensive literature review on pediatric and adult C.L.I.P.P.E.R.S.

Main Results:

  • Pediatric C.L.I.P.P.E.R.S. has unique clinical, etiological, and prognostic profiles compared to adults.
  • Associated mutations (e.g., PRF1) and delayed steroid response necessitate specific treatment considerations.
  • Potential underlying conditions like EBV B cell lymphoma and HLH require investigation.

Conclusions:

  • C.L.I.P.P.E.R.S. in children is a syndrome with diverse etiologies and manifestations, distinct from adult forms.
  • Genetic studies and workup for primary diseases are essential for accurate diagnosis and management.
  • Treatment requires a tailored approach, considering delayed responses, recurrences, and potential need for hematopoietic stem cell transplant.

Keywords:
C.L.I.P.P.E.R.SGlucocorticosteroidMRIParaneoplastic

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