RT Journal Article
SR Electronic
A1 Kovacova, Slavomira
A1 Hnilicova, Petra
A1 Grossmann, Jan
A1 Hajas, Gabriel
A1 Kantorova, Ema
A1 Kurca, Egon
T1 Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) syndrome:  Treatment approach depends on disease course
JF Biomedical papers
YR 2023
VO 167
IS 3
SP 303
OP 308
DO 10.5507/bp.2022.013
UL https://biomed.papers.upol.cz/artkey/bio-202303-0012.php
AB 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.