PT  - JOURNAL ARTICLE
AU  - Kovacova, Slavomira
AU  - Hnilicova, Petra
AU  - Grossmann, Jan
AU  - Hajas, Gabriel
AU  - Kantorova, Ema
AU  - Kurca, Egon
TI  - Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) syndrome:  Treatment approach depends on disease course
DP  - 2023 Sep 21
TA  - Biomedical papers
PG  - 303--308
VI  - 167
IP  - 3
AID - 10.5507/bp.2022.013
IS  - 12138118
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.