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


Journal

Biomedical papers of the Medical Faculty of the University Palacky, Olomouc, Czechoslovakia
ISSN: 1804-7521
Titre abrégé: Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub
Pays: Czech Republic
ID NLM: 101140142

Informations de publication

Date de publication:
Sep 2023
Historique:
received: 03 01 2022
accepted: 14 03 2022
medline: 3 11 2023
pubmed: 26 3 2022
entrez: 25 3 2022
Statut: ppublish

Résumé

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. 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. These two cases bring attention to this rare, often misdiagnosed but treatable disease.

Identifiants

pubmed: 35332344
doi: 10.5507/bp.2022.013
doi:

Substances chimiques

Steroids 0
Adrenal Cortex Hormones 0

Types de publication

Case Reports

Langues

eng

Sous-ensembles de citation

IM

Pagination

303-308

Déclaration de conflit d'intérêts

The authors report no conflicts of interest in this work.

Références

Pittock SJ, Debruyne J, Krecke KN, Giannini C, van den Ameele J, De Herdt V, McKeon A, Fealey RD, Weinshenker BG, Aksamit AJ, Krueger BR, Shuster EA, Keegan BM. Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Brain 2010;133(9):2626-34.
pubmed: 20639547 doi: 10.1093/brain/awq164
Tobin WO, Guo Y, Krecke KN, Parisi JE, Lucchinetti CF, Pittock SJ, Mandrekar J, Dubey D, Debruyne J, Keegan BM. Diagnostic criteria for chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Brain 2017;140(9):2415-25.
pubmed: 29050399 doi: 10.1093/brain/awx200
Dudesek A, Rimmele F, Tesar S, Kolbaske S, Rommer PS, Benecke R, Zettl UK. CLIPPERS: chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids. Review of an increasingly recognized entity within the spectrum of inflammatory central nervous system disorders. Clin Exp Immunol 2014;175(3):385-96.
pubmed: 24028073 doi: 10.1111/cei.12204
Ferreira RM, Machado G, Souza AS, Lin K, Corrêa-Neto Y. CLIPPERS-like MRI findings in a patient with multiple sclerosis. J Neurol Sci 2013;327(1-2):61-2.
doi: 10.1016/j.jns.2013.01.032
Taieb G, Allou T, Labauge P. Therapeutic Approaches in CLIPPERS. Curr Treat Options Neurol 2017;19(5):17.
pubmed: 28386850 doi: 10.1007/s11940-017-0455-4
Kotlega D, Peda B, Trochanowski T, Golab-Janowska M, Nowacki P. Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) - a case report. Aktualn Neurol 2019;19(3):145-48.
doi: 10.15557/AN.2019.0020
Kamişli Ö, Tecellioğlu M, Erbay MF, Kamişli S, Özcan C. Two New Cases and Literature Review of CLIPPERS Syndrome with Long-Term Follow-up. Noro Psikiyatr Ars 2017;57(2):160-64.
doi: 10.5152/npa.2017.22732
Suer D, Yusifova L, Arsava EM, Ekinci G, Us O, Uluc K. A case report of CLIPPERS (chronic lymphocytic inflammation with pontocerebellar perivascular enhancement responsive to steroids) syndrome. Clin Neuroradiol 2015;25:61-63.
pubmed: 24068102 doi: 10.1007/s00062-013-0260-8
Hou X, Wang X, Xie B, Lin W, Liu J, Ma D, Zhang HL. Horizontal eyeball akinesia as an initial manifestation of CLIPPERS: Case report and review of literature. Medicine (Baltimore) 2016; 95:e4640. doi: 10.1097/MD.0000000000004640
pubmed: 27559963 doi: 10.1097/MD.0000000000004640
Sempere AP, Mola S, Martin-Medina P, Bernabeu A, Khabbaz E, Lopez-Celada S. Response to immunotherapy in CLIPPERS: clinical, MRI, and MRS follow-up. J Neuroimaging 2013;23(2):254-5
pubmed: 21848680 doi: 10.1111/j.1552-6569.2011.00631.x
Gabilondo I, Saiz A, Graus F, Villoslada P. Response to immunotherapy in CLIPPERS syndrome. J Neurol 2011;258:2090-92.
doi: 10.1007/s00415-011-6068-z
Cipriani VP, Arndt N, Pytel P, Reder AT, Javed A. Effective treatment of CLIPPERS with long-term use of rituximab. Neurol Neuroimmunol Neuroinflamm 2018;5(3):e448. doi: 10.1212/NXI.0000000000000448
pubmed: 29511706 doi: 10.1212/NXI.0000000000000448
Marangi A, Zuliani L, Vitale V, Mansour M, Federle L, Novello M, Stefano E d'Amore G, Stenta G, Perini F. Good response to rituximab in chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids presenting as tumor-like infratentorial lesion. Clin Exp Neuroimmunol 2021;00:1-4. doi: 10.1111/cen3.12657
doi: 10.1111/cen3.12657
Mehta D, Yacoub H, El-Hunjul M, Khaitov D. Is there a role for B cells in chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids? A case report. Clin Exp Neuroimmunol 2019;10(2):142-44.
doi: 10.1111/cen3.12515
Abkur TM, Kearney H, Hennessy MJ. CLIPPERS and the need for long-term immunosuppression. Scott Med J 2017;62(1):28-33.
pubmed: 28110629 doi: 10.1177/0036933016689006

Auteurs

Slavomira Kovacova (S)

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

Petra Hnilicova (P)

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

Jan Grossmann (J)

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

Gabriel Hajas (G)

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

Ema Kantorova (E)

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

Egon Kurca (E)

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

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