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Punctate pontine perivascular enhancement

MedGen UID:
1053984
•Concept ID:
CN378387
•
Finding
HPO: HP:6001037

Definition

Multiple punctate (speckled, patchy spot-like) and/or curvilinear areas of gadolinium enhancement distributed around blood vessels in the pons. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPunctate pontine perivascular enhancement

Professional guidelines

PubMed

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

Recent clinical studies

Etiology

CLIPPERS Responsive to Cladribine as a Durable Steroid-Sparing Agent.
Thebault S, Bergman H, Atkins HL, Freedman MS, Brooks J
Neurol Neuroimmunol Neuroinflamm 2023 Jan;10(1) Epub 2022 Nov 17 doi: 10.1212/NXI.0000000000200060. PMID: 36396449Free PMC Article
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
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): A pediatric case report with six year follow-up.
Veerapandiyan A, Chaudhari A, Deo P, Ming X
Mult Scler Relat Disord 2017 Oct;17:95-98. Epub 2017 Jul 8 doi: 10.1016/j.msard.2017.07.012. PMID: 29055484
Response to immunotherapy in CLIPPERS: clinical, MRI, and MRS follow-up.
Sempere AP, Mola S, Martin-Medina P, Bernabeu A, Khabbaz E, Lopez-Celada S
J Neuroimaging 2013 Apr;23(2):254-5. Epub 2011 Aug 17 doi: 10.1111/j.1552-6569.2011.00631.x. PMID: 21848680

Diagnosis

Clinicoradiological features of probable chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) syndrome.
John DS, Kankara SR, Palasamudram Kumaran S, Hussain AS
BMJ Case Rep 2024 Apr 16;17(4) doi: 10.1136/bcr-2023-258067. PMID: 38627057Free PMC Article
CLIPPERS Responsive to Cladribine as a Durable Steroid-Sparing Agent.
Thebault S, Bergman H, Atkins HL, Freedman MS, Brooks J
Neurol Neuroimmunol Neuroinflamm 2023 Jan;10(1) Epub 2022 Nov 17 doi: 10.1212/NXI.0000000000200060. PMID: 36396449Free PMC Article
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids, a mimicker of malignancy: a case report and review of the literature.
Zhuang E, Shane L, Ramezan N, Ismail AF, Vora NL
J Med Case Rep 2021 May 18;15(1):246. doi: 10.1186/s13256-021-02814-5. PMID: 34001259Free PMC Article
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
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

Clinicoradiological features of probable chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) syndrome.
John DS, Kankara SR, Palasamudram Kumaran S, Hussain AS
BMJ Case Rep 2024 Apr 16;17(4) doi: 10.1136/bcr-2023-258067. PMID: 38627057Free PMC Article
CLIPPERS Responsive to Cladribine as a Durable Steroid-Sparing Agent.
Thebault S, Bergman H, Atkins HL, Freedman MS, Brooks J
Neurol Neuroimmunol Neuroinflamm 2023 Jan;10(1) Epub 2022 Nov 17 doi: 10.1212/NXI.0000000000200060. PMID: 36396449Free PMC Article
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids, a mimicker of malignancy: a case report and review of the literature.
Zhuang E, Shane L, Ramezan N, Ismail AF, Vora NL
J Med Case Rep 2021 May 18;15(1):246. doi: 10.1186/s13256-021-02814-5. PMID: 34001259Free PMC Article
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
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

Prognosis

A case report of CLIPPERS syndrome and literature review.
Zhang L, Zhao D, Li J, Feng J, Zhang Q, Liu L, Liu J, Chen J
Medicine (Baltimore) 2021 Jun 4;100(22):e26090. doi: 10.1097/MD.0000000000026090. PMID: 34087853Free PMC Article
Peripheral T cell lymphoma after chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): a case report.
Liu XH, Jin F, Zhang M, Liu MX, Wang T, Pan BJ, Zhang L
BMC Neurol 2019 Nov 4;19(1):266. doi: 10.1186/s12883-019-1507-z. PMID: 31684908Free PMC Article
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): A pediatric case report with six year follow-up.
Veerapandiyan A, Chaudhari A, Deo P, Ming X
Mult Scler Relat Disord 2017 Oct;17:95-98. Epub 2017 Jul 8 doi: 10.1016/j.msard.2017.07.012. PMID: 29055484
Horizontal eyeball akinesia as an initial manifestation of CLIPPERS: Case report and review of literature.
Hou X, Wang X, Xie B, Lin W, Liu J, Ma D, Zhang HL
Medicine (Baltimore) 2016 Aug;95(34):e4640. doi: 10.1097/MD.0000000000004640. PMID: 27559963Free PMC Article
Response to immunotherapy in CLIPPERS: clinical, MRI, and MRS follow-up.
Sempere AP, Mola S, Martin-Medina P, Bernabeu A, Khabbaz E, Lopez-Celada S
J Neuroimaging 2013 Apr;23(2):254-5. Epub 2011 Aug 17 doi: 10.1111/j.1552-6569.2011.00631.x. PMID: 21848680

Clinical prediction guides

Olivary hypertrophy improved by steroid treatment: Two case reports with unique presentations.
Ohara M, Sanjo N, Hattori T, Oyama J, Hamada M, Ozaki K, Yokota T
J Neuroimmunol 2019 Sep 15;334:577003. Epub 2019 Jul 5 doi: 10.1016/j.jneuroim.2019.577003. PMID: 31306854

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