CLIPPERS

Curr Neurol Neurosci Rep. 2017 Sep;17(9):65. doi: 10.1007/s11910-017-0773-7.

Abstract

Purpose of review: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a recently described treatable, inflammatory, brainstem predominant encephalomyelitis. The diagnosis of CLIPPERS is challenging without a specific biomarker, and thus it is important to consider if both the clinical and radiographic features are consistent with the diagnosis, or rather a disease mimicker.

Recent findings: Many patients with CLIPPERS-like lesions have been described in the literature with follow-up revealing a range of alternative diagnoses, such as malignancies, vasculitis, and other specific inflammatory diseases. As a result, some have proposed that CLIPPERS might represent a pre-malignancy state or simply an initial clinical syndrome of a variety of possible etiologies. We describe the typical clinical, radiographic, and pathological features of CLIPPERS and emphasize consideration for alternative diagnoses when findings are not classic. A recommended diagnostic evaluation and initial treatment plan is provided.

Keywords: Brainstem encephalitis; CLIPPERS; Encephalomyelitis; Lymphoma; PCNSL; Vasculitis.

Publication types

  • Review

MeSH terms

  • Encephalomyelitis / diagnostic imaging*
  • Encephalomyelitis / drug therapy*
  • Humans
  • Inflammation / diagnostic imaging
  • Inflammation / drug therapy
  • Lymphocytes / drug effects
  • Lymphocytes / pathology*
  • Magnetic Resonance Imaging / methods
  • Pons / diagnostic imaging*
  • Pons / drug effects
  • Steroids / pharmacology
  • Steroids / therapeutic use*

Substances

  • Steroids