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Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids

MedGen UID:
1433964
•Concept ID:
C3854437
•
Disease or Syndrome
Synonym: CLIPPERS
SNOMED CT: CLIPPERS - chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (773688007); Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (773688007)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0017297
Orphanet: ORPHA284448

Definition

A rare neuroinflammatory disorder with characteristics of brainstem-predominant encephalomyelitis, which typically presents with cerebellar and cranial nerve manifestations (gait ataxia, dysarthria, visual disorders, parasthesia), as well as brainstem, myelopathy and cognitive findings that respond to steroid treatment. Punctate curvilinear post-gadolinium contrast enhancement predominantly in the pons and cerebellum is observed on brain MRI and prominent, perivascular, CD3+ T-cell predominantly lymphocytic inflammation in neuropathology. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids

Professional guidelines

PubMed

Clinical characteristics, management, and outcomes of CLIPPERS: A comprehensive systematic review of 140 patients from 100 studies.
Al-Chalabi M, DelCimmuto NR, Beran A, Devarasetty PP, Mhanna A, Mahfooz N, Sheikh A
Mult Scler Relat Disord 2022 Dec;68:104112. Epub 2022 Aug 18 doi: 10.1016/j.msard.2022.104112. PMID: 36029706
CLIPPERS and its mimics: evaluation of new criteria for the diagnosis of CLIPPERS.
Taieb G, Mulero P, Psimaras D, van Oosten BW, Seebach JD, Marignier R, Pico F, Rigau V, Ueno Y, Duflos C, Fominykh V, Guiraud V, Lebrun-Frénay C, Camdessanché JP, Kerschen P, Ahle G, Téllez N, Rovira A, Hoang-Xuan K, Pelletier J, Labauge P; in cooperation with the French CLIPPERS group
J Neurol Neurosurg Psychiatry 2019 Sep;90(9):1027-1038. Epub 2019 May 9 doi: 10.1136/jnnp-2018-318957. PMID: 31072955
Diagnostic criteria for chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS).
Tobin WO, Guo Y, Krecke KN, Parisi JE, Lucchinetti CF, Pittock SJ, Mandrekar J, Dubey D, Debruyne J, Keegan BM
Brain 2017 Sep 1;140(9):2415-2425. doi: 10.1093/brain/awx200. PMID: 29050399

Recent clinical studies

Etiology

Clinical characteristics, management, and outcomes of CLIPPERS: A comprehensive systematic review of 140 patients from 100 studies.
Al-Chalabi M, DelCimmuto NR, Beran A, Devarasetty PP, Mhanna A, Mahfooz N, Sheikh A
Mult Scler Relat Disord 2022 Dec;68:104112. Epub 2022 Aug 18 doi: 10.1016/j.msard.2022.104112. PMID: 36029706
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) associated with or without lymphoma: Comparison of clinical features and risk factors suggestive of underlying lymphomas.
Zhang L, Liu XH, Jin F, Liu MX, Zhang M, Zhang Y, Zhou DB, Zhang W
J Clin Neurosci 2019 Aug;66:156-164. Epub 2019 May 11 doi: 10.1016/j.jocn.2019.04.022. PMID: 31088767
Diagnostic criteria for chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS).
Tobin WO, Guo Y, Krecke KN, Parisi JE, Lucchinetti CF, Pittock SJ, Mandrekar J, Dubey D, Debruyne J, Keegan BM
Brain 2017 Sep 1;140(9):2415-2425. doi: 10.1093/brain/awx200. PMID: 29050399
Immune-mediated disorders.
Höftberger R, Lassmann H
Handb Clin Neurol 2017;145:285-299. doi: 10.1016/B978-0-12-802395-2.00020-1. PMID: 28987176
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS).
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
Brain 2010 Sep;133(9):2626-34. Epub 2010 Jul 17 doi: 10.1093/brain/awq164. PMID: 20639547

Diagnosis

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): contemporary advances and current controversies.
Li EC, Lai QL, Cai MT, Fang GL, Fang W, Zheng Y, Du Y, Du BQ, Shen CH, Qiao S, Ding MP, Zhang YX
J Neurol 2024 Apr;271(4):1747-1766. Epub 2024 Jan 29 doi: 10.1007/s00415-024-12189-4. PMID: 38286842
Diagnostic criteria for chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS).
Tobin WO, Guo Y, Krecke KN, Parisi JE, Lucchinetti CF, Pittock SJ, Mandrekar J, Dubey D, Debruyne J, Keegan BM
Brain 2017 Sep 1;140(9):2415-2425. doi: 10.1093/brain/awx200. PMID: 29050399
Immune-mediated disorders.
Höftberger R, Lassmann H
Handb Clin Neurol 2017;145:285-299. doi: 10.1016/B978-0-12-802395-2.00020-1. PMID: 28987176
CLIPPERS.
Zalewski NL, Tobin WO
Curr Neurol Neurosci Rep 2017 Sep;17(9):65. doi: 10.1007/s11910-017-0773-7. PMID: 28726127
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.
Dudesek A, Rimmele F, Tesar S, Kolbaske S, Rommer PS, Benecke R, Zettl UK
Clin Exp Immunol 2014 Mar;175(3):385-96. doi: 10.1111/cei.12204. PMID: 24028073Free PMC Article

Therapy

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): contemporary advances and current controversies.
Li EC, Lai QL, Cai MT, Fang GL, Fang W, Zheng Y, Du Y, Du BQ, Shen CH, Qiao S, Ding MP, Zhang YX
J Neurol 2024 Apr;271(4):1747-1766. Epub 2024 Jan 29 doi: 10.1007/s00415-024-12189-4. PMID: 38286842
Diagnostic criteria for chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS).
Tobin WO, Guo Y, Krecke KN, Parisi JE, Lucchinetti CF, Pittock SJ, Mandrekar J, Dubey D, Debruyne J, Keegan BM
Brain 2017 Sep 1;140(9):2415-2425. doi: 10.1093/brain/awx200. PMID: 29050399
CLIPPERS.
Zalewski NL, Tobin WO
Curr Neurol Neurosci Rep 2017 Sep;17(9):65. doi: 10.1007/s11910-017-0773-7. PMID: 28726127
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.
Dudesek A, Rimmele F, Tesar S, Kolbaske S, Rommer PS, Benecke R, Zettl UK
Clin Exp Immunol 2014 Mar;175(3):385-96. doi: 10.1111/cei.12204. PMID: 24028073Free PMC Article
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS).
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
Brain 2010 Sep;133(9):2626-34. Epub 2010 Jul 17 doi: 10.1093/brain/awq164. PMID: 20639547

Prognosis

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): contemporary advances and current controversies.
Li EC, Lai QL, Cai MT, Fang GL, Fang W, Zheng Y, Du Y, Du BQ, Shen CH, Qiao S, Ding MP, Zhang YX
J Neurol 2024 Apr;271(4):1747-1766. Epub 2024 Jan 29 doi: 10.1007/s00415-024-12189-4. PMID: 38286842
Spectrum of Autoimmune Diseases-Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS)-Clinical Case.
Meller A, Pawlukowska W, Machowska-Sempruch K, Marta M
Medicina (Kaunas) 2023 Mar 11;59(3) doi: 10.3390/medicina59030549. PMID: 36984550Free PMC Article
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) syndrome: Treatment approach depends on disease course.
Kovacova S, Hnilicova P, Grossmann J, Hajas G, Kantorova E, Kurca E
Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub 2023 Sep;167(3):303-308. Epub 2022 Mar 21 doi: 10.5507/bp.2022.013. PMID: 35332344
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) associated with or without lymphoma: Comparison of clinical features and risk factors suggestive of underlying lymphomas.
Zhang L, Liu XH, Jin F, Liu MX, Zhang M, Zhang Y, Zhou DB, Zhang W
J Clin Neurosci 2019 Aug;66:156-164. Epub 2019 May 11 doi: 10.1016/j.jocn.2019.04.022. PMID: 31088767
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS).
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
Brain 2010 Sep;133(9):2626-34. Epub 2010 Jul 17 doi: 10.1093/brain/awq164. PMID: 20639547

Clinical prediction guides

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): contemporary advances and current controversies.
Li EC, Lai QL, Cai MT, Fang GL, Fang W, Zheng Y, Du Y, Du BQ, Shen CH, Qiao S, Ding MP, Zhang YX
J Neurol 2024 Apr;271(4):1747-1766. Epub 2024 Jan 29 doi: 10.1007/s00415-024-12189-4. PMID: 38286842
The pathogenesis hypothesis and research progress of CLIPPERS: A literature review.
Cao L, Liu M, Guo L, Li M, Xu M, Wang R
Medicine (Baltimore) 2023 Mar 17;102(11):e33211. doi: 10.1097/MD.0000000000033211. PMID: 36930124Free PMC Article
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) associated with or without lymphoma: Comparison of clinical features and risk factors suggestive of underlying lymphomas.
Zhang L, Liu XH, Jin F, Liu MX, Zhang M, Zhang Y, Zhou DB, Zhang W
J Clin Neurosci 2019 Aug;66:156-164. Epub 2019 May 11 doi: 10.1016/j.jocn.2019.04.022. PMID: 31088767
Omics-Based Approach Reveals Complement-Mediated Inflammation in Chronic Lymphocytic Inflammation With Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS).
Blaabjerg M, Hemdrup AL, Drici L, Ruprecht K, Garred P, Höftberger R, Kristensen BW, Kondziella D, Sejbaek T, Hansen SW, Nielsen HH, Jensen P, Meyer M, Paul F, Lassmann H, Larsen MR, Illes Z
Front Immunol 2018;9:741. Epub 2018 Apr 23 doi: 10.3389/fimmu.2018.00741. PMID: 29740431Free PMC Article
Diagnostic criteria for chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS).
Tobin WO, Guo Y, Krecke KN, Parisi JE, Lucchinetti CF, Pittock SJ, Mandrekar J, Dubey D, Debruyne J, Keegan BM
Brain 2017 Sep 1;140(9):2415-2425. doi: 10.1093/brain/awx200. PMID: 29050399

Recent systematic reviews

Clinical characteristics, management, and outcomes of CLIPPERS: A comprehensive systematic review of 140 patients from 100 studies.
Al-Chalabi M, DelCimmuto NR, Beran A, Devarasetty PP, Mhanna A, Mahfooz N, Sheikh A
Mult Scler Relat Disord 2022 Dec;68:104112. Epub 2022 Aug 18 doi: 10.1016/j.msard.2022.104112. PMID: 36029706
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) associated with or without lymphoma: Comparison of clinical features and risk factors suggestive of underlying lymphomas.
Zhang L, Liu XH, Jin F, Liu MX, Zhang M, Zhang Y, Zhou DB, Zhang W
J Clin Neurosci 2019 Aug;66:156-164. Epub 2019 May 11 doi: 10.1016/j.jocn.2019.04.022. PMID: 31088767

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