Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub. 2023, 167(3):303-308 | DOI: 10.5507/bp.2022.013

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) syndrome: Treatment approach depends on disease course

Slavomira Kovacova¹, Petra Hnilicova², Jan Grossmann¹, Gabriel Hajas³, Ema Kantorova¹, Egon Kurca¹

¹ Clinic of Neurology, Jessenius Faculty of Medicine in Martin, Comenius University Bratislava, Slovak Republic

² Biomedical Center Martin, Jessenius Faculty of Medicine in Martin, Comenius University in Bratislava, Slovak Republic

³ Clinic of Neurology, Faculty Hospital of Constantine the Philosopher University, Nitra, Slovak Republic

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.

Keywords: CLIPPERS, ocrelizumab, corticosteroids, autoimmune disorders, brainstem

Received: January 3, 2022; Revised: March 4, 2022; Accepted: March 14, 2022; Prepublished online: March 21, 2022; Published: September 21, 2023  Show citation