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Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) syndrome: Treatment approach depends on disease course
S. Kovacova, P. Hnilicova, J. Grossmann, G. Hajas, E. Kantorova, E. Kurca
Language English Country Czech Republic
Document type Case Reports
NLK
Directory of Open Access Journals
from 2001
Free Medical Journals
from 1998
Medline Complete (EBSCOhost)
from 2007-06-01
ROAD: Directory of Open Access Scholarly Resources
from 2001
PubMed
35332344
DOI
10.5507/bp.2022.013
Knihovny.cz E-resources
- MeSH
- Adrenal Cortex Hormones therapeutic use MeSH
- Humans MeSH
- Magnetic Resonance Imaging * MeSH
- Central Nervous System Diseases * complications diagnosis drug therapy MeSH
- Pons MeSH
- Disease Progression MeSH
- Steroids therapeutic use MeSH
- Inflammation MeSH
- Check Tag
- Humans MeSH
- Publication type
- Case Reports MeSH
INTRODUCTION: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare inflammatory central nervous system (CNS) disorder, chiefly involving the brainstem, especially the pons. The diagnosis is challenging, requires careful exclusion of alternative diagnoses and a targeted therapeutic approach. CLIPPERS is known to respond well to corticosteroids, but the treatment needs to be long-term and can cause significant side-effects. Moreover, subsequent corticosteroid withdrawal often leads to a relapse. It has been suggested that anti-CD20 molecules could benefit several antibody-mediated CNS inflammatory diseases, including CLIPPERS. CASE REPORT: This paper describes two cases of CLIPPERS. The first demonstrates the benefit of early introduction of corticosteroids with side effects in cases of long-term use. The second demonstrates the efficacy of ocrelizumab (anti-CD20 molecule) in a severe course of CLIPPERS. CONCLUSION: These two cases bring attention to this rare, often misdiagnosed but treatable disease.
Clinic of Neurology Faculty Hospital of Constantine the Philosopher University Nitra Slovak Republic
References provided by Crossref.org
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