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Related Experiment Videos

Therapeutic Approaches in CLIPPERS.

Guillaume Taieb1, Thibaut Allou2, Pierre Labauge2

  • 1Department of Neurology, CHU Montpellier, Hopital Guy de Chauliac, 80 avenue Augustin Fliche, 34295, Montpellier Cedex 5, France. taiebguillaume@gmail.com.

Current Treatment Options in Neurology
|April 8, 2017
PubMed
Summary

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare brainstem disease. Methotrexate appears most promising among steroid-sparing agents for managing CLIPPERS, but further trials are needed.

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Area of Science:

  • Neurology
  • Neuroimmunology
  • Inflammatory Diseases

Background:

  • Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare, steroid-sensitive brainstem inflammatory disease.
  • Approximately 60 cases reported globally since 2010, with a mean onset age of 50 years, predominantly affecting men.
  • The disease follows a relapsing-remitting course without chronic progression, but severe attacks can lead to disability and atrophy.

Purpose of the Study:

  • To review the literature on CLIPPERS management and evaluate the efficacy of steroid-sparing therapies.
  • To identify potential therapeutic recommendations in the absence of controlled studies.

Main Methods:

  • Systematic analysis of PubMed articles using keywords "chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids" and "CLIPPERS".
Keywords:
CLIPPERS mimicsClippersCorticosteroid-sparing therapyImmunosuppressive therapyTh17-mediated disease

Related Experiment Videos

  • Evaluation of corticosteroid-sparing agent efficacy based on a relapse-free period of ≥24 months without concomitant corticosteroid therapy.

    • Main Results:

    • High-dose corticosteroids induce clinical and radiological improvement during attacks, but long-term remission is key to preventing sequelae.
    • No relapses occurred with chronic corticosteroid therapy above 20 mg/day, but side effects necessitate steroid-sparing options.
    • Methotrexate, cyclophosphamide, and hydroxychloroquine showed "probable" efficacy as steroid-sparing agents, with methotrexate being most suitable in two cases.

    Conclusions:

    • Maintaining CLIPPERS remission is crucial for preventing long-term disability.
    • Methotrexate is suggested as the most suitable steroid-sparing agent for CLIPPERS, considering risk-benefit.
    • Randomized controlled trials are essential to establish definitive therapeutic recommendations for CLIPPERS.