Clinicoradiological features of probable chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) syndrome

BMJ Case Rep. 2024 Apr 16;17(4):e258067. doi: 10.1136/bcr-2023-258067.

Abstract

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a recently described chronic inflammatory central nervous system disease. This case report describes a young female patient presenting with weakness in bilateral upper and lower limbs and tinnitus for 2 months. A neurological examination revealed signs of brainstem and cerebellar involvement. MRI brain showed characteristic features of CLIPPERS, with punctate and nodular enhancement in the pons and cerebellum. Differential diagnoses were systematically considered and excluded. The patient showed significant clinical and radiological improvement with steroid therapy. No clinical or radiological red flags occurred during the follow-up. This case underscores the critical role of integrating clinical and radiological findings to effectively diagnose and manage CLIPPERS. It emphasises the importance of ruling out alternative diagnoses through a thorough evaluation.

Keywords: Brain stem / cerebellum; Immunology; Neuroimaging; Radiology.

Publication types

  • Case Reports

MeSH terms

  • Brain Stem / diagnostic imaging
  • Central Nervous System Diseases* / diagnosis
  • Central Nervous System Diseases* / diagnostic imaging
  • Female
  • Humans
  • Inflammation* / diagnosis
  • Magnetic Resonance Imaging
  • Pons / diagnostic imaging
  • Steroids / therapeutic use

Substances

  • Steroids