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29.01.2024 | Review

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): contemporary advances and current controversies

verfasst von: Er-Chuang Li, Qi-Lun Lai, Meng-Ting Cai, Gao-Li Fang, Wei Fang, Yang Zheng, Ye Du, Bing-Qing Du, Chun-Hong Shen, Song Qiao, Mei-Ping Ding, Yin-Xi Zhang

Erschienen in: Journal of Neurology | Ausgabe 4/2024

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Abstract

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an inflammatory syndrome with characteristic clinical, radiological, and pathological features, and can be effectively treated with corticosteroid-based immunotherapies. The exact pathogenesis of CLIPPERS remains unclear, and specific diagnostic biomarkers are not available. According to the 2017 diagnostic criteria, probable CLIPPERS should be considered in middle-aged patients with subacute onset of pontocerebellar symptoms and typical punctuate and curvilinear gadolinium enhancement lesions (“salt-and-pepper” appearance) located in the hindbrain (especially pons) on magnetic resonance imaging. In addition, CLIPPERS-mimics, such as central nervous system (CNS) lymphoma, and several antibody-associated autoimmune CNS diseases (e.g., myelin oligodendrocyte glycoprotein antibody-associated disease, autoimmune glial fibrillary acidic protein astrocytopathy, and anti-N-methyl-d-aspartate receptor encephalitis), should be extensively excluded. The prerequisite for definite CLIPPERS is the perivascular T-cell-predominant inflammatory infiltration observed on pathological analysis. A biopsy is strongly suggested when clinical/radiological red flags are present. Most patients with CLIPPERS respond well to corticosteroids and have a good prognosis. Long-term low-dose corticosteroid maintenance therapy or corticosteroids coupled with immunosuppressants are recommended to prevent the recurrence of the syndrome. The potential progression of CLIPPERS to lymphoma has been suggested in some cases; therefore, at least 2-year clinical and radiological follow-up is essential. Here, we critically review the recent developments and provided an update on the clinical characteristics, diagnostic criteria, differential diagnoses, and therapeutic management of CLIPPERS. We also discuss the current controversies in this context that can be resolved in future research studies.

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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 (2010) Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Brain 133(9):2626–2634. https://doi.org/10.1093/brain/awq164CrossRefPubMed

Simon NG, Parratt JD, Barnett MH, Buckland ME, Gupta R, Hayes MW, Masters LT, Reddel SW (2012) Expanding the clinical, radiological and neuropathological phenotype of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). J Neurol Neurosurg Psychiatry 83(1):15–22. https://doi.org/10.1136/jnnp-2011-301054CrossRefPubMed

Taieb G, Duflos C, Renard D, Audoin B, Kaphan E, Pelletier J, Limousin N, Tranchant C, Kremer S, de Sèze J, Lefaucheur R, Maltête D, Brassat D, Clanet M, Desbordes P, Thouvenot E, Magy L, Vincent T, Faillie JL, de Champfleur N, Castelnovo G, Eimer S, Branger DF, Uro-Coste E, Labauge P (2012) Long-term outcomes of CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) in a consecutive series of 12 patients. Arch Neurol 69(7):847–855. https://doi.org/10.1001/archneurol.2012.122CrossRefPubMed

Tobin WO, Guo Y, Krecke KN, Parisi JE, Lucchinetti CF, Pittock SJ, Mandrekar J, Dubey D, Debruyne J, Keegan BM (2017) Diagnostic criteria for chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Brain 140(9):2415–2425. https://doi.org/10.1093/brain/awx200CrossRefPubMed

Al-Chalabi M, DelCimmuto NR, Beran A, Devarasetty PP, Mhanna A, Mahfooz N, Sheikh A (2022) Clinical characteristics, management, and outcomes of CLIPPERS: a comprehensive systematic review of 140 patients from 100 studies. Mult Scler Relat Disord 68:104112. https://doi.org/10.1016/j.msard.2022.104112CrossRefPubMed

Guo J, Bu Y, Liu W (2022) Case report: a case with mogad and anti-nmdar encephalitis overlapping syndrome mimicing radiological characteristics of CLIPPERS. Front Immunol 13:832084. https://doi.org/10.3389/fimmu.2022.832084CrossRefPubMedPubMedCentral

Galazky I, Büntjen L, Voges J, Sandalcioglu IE, Mawrin C, Haghikia A (2022) Brain biopsy in patients with CLIPPERS syndrome: why and when. Ther Adv Neurol Disord 15:17562864211062820. https://doi.org/10.1177/17562864211062821CrossRefPubMedPubMedCentral

Picarelli H, Yamaki VN, Casal YR, de Brito MH, de Lima L, Ayres AS, de Oliveira MCB (2021) CLIPPERS with exclusive extra pontine involvement. Neurol Neuroimmunol Neuroinflamm 8(3):e989. https://doi.org/10.1212/nxi.0000000000000989CrossRefPubMedPubMedCentral

Smith A, Matthews Y, Kossard S, Turner J, Buckland ME, Parratt J (2014) Neurotropic T-cell lymphocytosis: a cutaneous expression of CLIPPERS. J Cutan Pathol 41(8):657–662. https://doi.org/10.1111/cup.12344CrossRefPubMed

10.

Pessoa MSL, M de Lima FEM, A Uhoa LR, Torres DM, Coimbra PP, F Távora DG, (2022) Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) with extrapontine and pulmonary involvement: case report of a rare presentation and review of literature. Neurol India 70(1):405–408. https://doi.org/10.4103/0028-3886.338665CrossRefPubMed

11.

Vola E, Russo C, Macera A, Del Vecchio W, Puoti G, Caranci F, Elefante A (2019) Unusual CLIPPERS presentation and role of MRI examination in the proper diagnostic assessment: a case report. Eur J Radiol Open 6:212–214. https://doi.org/10.1016/j.ejro.2019.05.005CrossRefPubMedPubMedCentral

12.

Nakamura R, Ueno Y, Ando J, Matsuda H, Masuda A, Iiduka K, Shingai N, Takanashi M, Yokoyama K, Komatsu N, Hattori N (2016) Clinical and radiological CLIPPERS features after complete remission of peripheral T-cell lymphoma, not otherwise specified. J Neurol Sci 364:6–8. https://doi.org/10.1016/j.jns.2016.02.057CrossRefPubMed

13.

Doubrovinskaia S, Sahm F, Thier MC, Bendszus M, Wick W, Seliger C, Kaulen LD (2021) Primary CNS lymphoma after CLIPPERS: a case series. J Neurol Neurosurg Psychiatry 92(12):1348–1349. https://doi.org/10.1136/jnnp-2020-325759CrossRefPubMed

14.

Ferilli MAN, Papi C, Sabatelli M, Colosimo C, Iorio R (2022) MOG autoimmunity mimicking CLIPPERS syndrome: case report and literature review. J Neuroimmunol 367:577875. https://doi.org/10.1016/j.jneuroim.2022.577875CrossRefPubMed

15.

Wang L, Holthaus EA, Jimenez XF, Tavee J, Li Y (2015) MRI evolution of CLIPPERS syndrome following herpes zoster infection. J Neurol Sci 348(1–2):277–278. https://doi.org/10.1016/j.jns.2014.11.022CrossRefPubMed

16.

Ahn JW, Jeong JY, Hwang SK, Shin HY, Park JS (2021) A case of diffuse large B cell lymphoma initially presenting as CLIPPERS: possible role of the Epstein-Barr virus. Neurol Sci 42(3):1199–1202. https://doi.org/10.1007/s10072-020-04750-6CrossRefPubMed

17.

De Graaff HJ, Wattjes MP, Rozemuller-Kwakkel AJ, Petzold A, Killestein J (2013) Fatal B-cell lymphoma following chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids. JAMA Neurol 70(7):915–918. https://doi.org/10.1001/jamaneurol.2013.2016CrossRefPubMed

18.

Ma Y, Sun X, Li W, Li Y, Kang T, Yang X, Jiang W (2016) Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) with intracranial Epstein-Barr virus infection: a case report. Medicine (Baltimore) 95(46):e5377. https://doi.org/10.1097/md.0000000000005377CrossRefPubMed

19.

Weng CF, Chan DC, Chen YF, Liu FC, Liou HH (2015) Chronic hepatitis B infection presenting with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): a case report. J Med Case Rep 9:266. https://doi.org/10.1186/s13256-015-0750-1CrossRefPubMedPubMedCentral

20.

Montini F, Martinelli V, Sangalli F, Callea M, Anzalone N, Filippi M (2021) Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) after SARS-CoV-2 pneumonia. Neurol Sci 42(10):4373–4375. https://doi.org/10.1007/s10072-021-05472-zCrossRefPubMedPubMedCentral

21.

Hillesheim PB, Parker JR, Parker JC Jr, Escott E, Berger JR (2012) Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids following influenza vaccination. Arch Pathol Lab Med 136(6):681–685. https://doi.org/10.5858/arpa.2011-0428-CRCrossRefPubMed

22.

Ortega MR, Usmani N, Parra-Herran C, Adams DJ, Steingo B, Rammohan KW (2012) CLIPPERS complicating multiple sclerosis causing concerns of CNS lymphoma. Neurology 79(7):715–716. https://doi.org/10.1212/WNL.0b013e3182648b77CrossRefPubMed

23.

Zalewski NL (2017) Tobin WO (2017) CLIPPERS. Curr Neurol Neurosci Rep 17(9):65. https://doi.org/10.1007/s11910-017-0773-7CrossRefPubMed

24.

Dudesek A, Rimmele F, Tesar S, Kolbaske S, Rommer PS, Benecke R, Zettl UK (2014) 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 175(3):385–396CrossRefPubMedPubMedCentral

25.

Wang X, Huang D, Huang X, Zhang J, Ran Y, Lou X, Gui Q, Yu S (2017) Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): a lymphocytic reactive response of the central nervous system? A case report. J Neuroimmunol 305:68–71. https://doi.org/10.1016/j.jneuroim.2017.01.014CrossRefPubMed

26.

Deng Y, Zhao B, Wei B, Zhang S, Liu M (2021) Parotid carcinoma following chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids: a case report. BMC Neurol 21(1):110. https://doi.org/10.1186/s12883-021-02135-6CrossRefPubMedPubMedCentral

27.

McKeon A, Pittock SJ (2011) Paraneoplastic encephalomyelopathies: pathology and mechanisms. Acta Neuropathol 122(4):381–400. https://doi.org/10.1007/s00401-011-0876-1CrossRefPubMed

28.

Li EC, Lai QL, Cai MT, Zheng Y, Fang GL, Fang W, Du BQ, Shen CH, Ding MP, Zhang YX (2022) Kelch-like protein 11 antibody-associated paraneoplastic neurological syndrome: a state-of-the-art review. Clin Immunol 241:109074. https://doi.org/10.1016/j.clim.2022.109074CrossRefPubMed

29.

Ganaraja VH, Rezk M, Dubey D (2022) Paraneoplastic neurological syndrome: growing spectrum and relevance. Neurol Sci 43(6):3583–3594. https://doi.org/10.1007/s10072-022-06083-yCrossRefPubMed

30.

Pelosof LC, Gerber DE (2010) Paraneoplastic syndromes: an approach to diagnosis and treatment. Mayo Clin Proc 85(9):838–854. https://doi.org/10.4065/mcp.2010.0099CrossRefPubMedPubMedCentral

31.

Shahrizaila N, Yuki N (2013) Bickerstaff brainstem encephalitis and Fisher syndrome: anti-GQ1b antibody syndrome. J Neurol Neurosurg Psychiatry 84(5):576–583. https://doi.org/10.1136/jnnp-2012-302824CrossRefPubMed

32.

Al-Araji A, Kidd DP (2009) Neuro-Behçet’s disease: epidemiology, clinical characteristics, and management. Lancet Neurol 8(2):192–204. https://doi.org/10.1016/s1474-4422(09)70015-8CrossRefPubMed

33.

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 (2018) Omics-based approach reveals complement-mediated inflammation in chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Front Immunol 9:741. https://doi.org/10.3389/fimmu.2018.00741CrossRefPubMedPubMedCentral

34.

Mélé N, Guiraud V, Labauge P, Oppenheim C, Mas JL, Taieb G (2014) Effective antituberculous therapy in a patient with CLIPPERS: New insights into CLIPPERS pathogenesis. Neurol Neuroimmunol Neuroinflamm 1(1):e6. https://doi.org/10.1212/nxi.0000000000000006CrossRefPubMedPubMedCentral

35.

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 (2019) CLIPPERS and its mimics: evaluation of new criteria for the diagnosis of CLIPPERS. J Neurol Neurosurg Psychiatry 90(9):1027–1038. https://doi.org/10.1136/jnnp-2018-318957CrossRefPubMed

36.

Kastrup O, van de Nes J, Gasser T, Keyvani K (2011) Three cases of CLIPPERS: a serial clinical, laboratory and MRI follow-up study. J Neurol 258(12):2140–2146. https://doi.org/10.1007/s00415-011-6071-4CrossRefPubMed

37.

Zhang YX, Hu HT, Ding XY, Chen LH, Du Y, Shen CH, Guo Y, Ding MP (2016) CLIPPERS with diffuse white matter and longitudinally extensive spinal cord involvement. Neurology 86(1):103–105. https://doi.org/10.1212/wnl.0000000000002212CrossRefPubMed

38.

Zhang W, Cui L, Dong M, Tian Z, Jiao Y, Jiao J (2020) Area postrema syndrome: a rare feature of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids. Front Neurol 11:730. https://doi.org/10.3389/fneur.2020.00730CrossRefPubMedPubMedCentral

39.

Korostyshevskaya AM, Stankevich JA, Vasilkiv LM, Bogomyakova OB, Korobko DS, Gornostaeva AM, Tulupov A (2023) CLIPPERS: multiparametric and quantitative MRI features. Radiol Case Rep 18(1):368–376. https://doi.org/10.1016/j.radcr.2022.10.043CrossRefPubMed

40.

Corral MAP, Gavito-Higuera J, O’Neill T, Chennan SRE (2021) CLIPPERS associated with hypersensitivity pneumonitis: a case report. Radiol Case Rep 16(9):2411–2415. https://doi.org/10.1016/j.radcr.2021.05.077CrossRefPubMedPubMedCentral

41.

Anand S, Das A, Choudhury SS (2019) Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) in limited cutaneous sclerosis: a rare disease combination. BMJ Case Rep 12(1):e226259. https://doi.org/10.1136/bcr-2018-226259CrossRefPubMedPubMedCentral

42.

Ohta Y, Nomura E, Tsunoda K, Yamashita T, Takahashi Y, Sato K, Takemoto M, Hishikawa N, Abe K (2017) chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) with limbic encephalitis. Intern Med 56(18):2513–2518. https://doi.org/10.2169/internalmedicine.8533-16CrossRefPubMedPubMedCentral

43.

Yiannopoulou KG, Papanagiotou P, Anastasiou AI, Papadimitriou DA (2020) Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) with simultaneous presentation of Hashimoto thyroiditis. Acta Neurol Belg 120(5):1237–1239. https://doi.org/10.1007/s13760-020-01371-wCrossRefPubMed

44.

Song Y, Bai M, Chen H, Liu S, Zhao Y, Liu K (2021) Co-occurrence of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids and posterior reversible encephalopathy syndrome: a case report. Mult Scler Relat Disord 49:102791. https://doi.org/10.1016/j.msard.2021.102791CrossRefPubMed

45.

Berry B, Joppa S, Labin E, Puram V, McCleary K, Clark HB, Nelson F (2021) Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids may extend above and below pons and is associated with other autoimmune diseases. Life (Basel) 11(11):1120. https://doi.org/10.3390/life11111120CrossRefPubMed

46.

Taieb G, Duran-Peña A, de Chamfleur NM, Moulignier A, Thouvenot E, Allou T, Lacour A, Hoang-Xuan K, Pelletier J, Labauge P (2016) Punctate and curvilinear gadolinium enhancing lesions in the brain: a practical approach. Neuroradiology 58(3):221–235. https://doi.org/10.1007/s00234-015-1629-yCrossRefPubMed

47.

Yang B, Liu F, Li J, Wen Y (2021) Increased number of perivascular CD20-positive B lymphocytes in the neuropathology of clippers: findings of 6 patients from Mainland China. Eur Neurol 84(1):22–30. https://doi.org/10.1159/000512406CrossRefPubMed

48.

Pesaresi I, Sabato M, Desideri I, Puglioli M, Moretti P, Cosottini M (2013) 3.0T MR investigation of CLIPPERS: role of susceptibility weighted and perfusion weighted imaging. Magn Reson Imaging 31(9):1640–1642CrossRefPubMed

49.

Morassi M, Cobelli M, Ghiselli E, Costa P, Bagatto D, Pievani M, Bnà C (2019) Diffuse white matter alteration in CLIPPERS: advanced MRI findings from two cases. J Neurol Sci 402:40–47. https://doi.org/10.1016/j.jns.2019.04.041CrossRefPubMed

50.

Rössling R, Pehl D, Lingnau M, Prüss H (2018) A case of CLIPPERS challenging the new diagnostic criteria. Brain 141(2):e12. https://doi.org/10.1093/brain/awx335CrossRefPubMed

51.

Rauschenbach L, Kebir S, Radbruch A, Darkwah Oppong M, Gembruch O, Teuber-Hanselmann S, Gielen GH, Scheffler B, Glas M, Sure U, Lemonas E (2020) Challenging implications of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids syndrome with an atypical presentation: report of two cases. World Neurosurg 143:507-512.e501. https://doi.org/10.1016/j.wneu.2020.07.123CrossRefPubMed

52.

Tohge R, Nagao M, Yagishita A, Matsubara S (2012) A case of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) in East Asia. Intern Med 51(9):1115–1119. https://doi.org/10.2169/internalmedicine.51.7040CrossRefPubMed

53.

Gabilondo I, Saiz A, Graus F, Villoslada P (2011) Response to immunotherapy in CLIPPERS syndrome. J Neurol 258(11):2090–2092. https://doi.org/10.1007/s00415-011-6068-zCrossRefPubMed

54.

Gul M, Chaudhry AA, Chaudhry AA, Sheikh MA, Carsons S (2015) Atypical presentation of CLIPPERS syndrome: a new entity in the differential diagnosis of central nervous system rheumatologic diseases. J Clin Rheumatol 21(3):144–148. https://doi.org/10.1097/rhu.0000000000000238CrossRefPubMed

55.

Blaabjerg M, Ruprecht K, Sinnecker T, Kondziella D, Niendorf T, Kerrn-Jespersen BM, Lindelof M, Lassmann H, Kristensen BW, Paul F, Illes Z (2016) Widespread inflammation in CLIPPERS syndrome indicated by autopsy and ultra-high-field 7T MRI. Neurol Neuroimmunol Neuroinflamm 3(3):e226. https://doi.org/10.1212/nxi.0000000000000226CrossRefPubMedPubMedCentral

56.

Mubasher M, Sukik A, El Beltagi AH, Rahil A (2017) Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids, with cranial and caudal extension. Case Rep Neurol Med 2017:2593096. https://doi.org/10.1155/2017/2593096CrossRefPubMedPubMedCentral

57.

Kerrn-Jespersen BM, Lindelof M, Illes Z, Blaabjerg M, Lund EL, Klausen C, Christiansen I, Sellebjerg F, Kondziella D (2014) CLIPPERS among patients diagnosed with non-specific CNS neuroinflammatory diseases. J Neurol Sci 343(1–2):224–227. https://doi.org/10.1016/j.jns.2014.06.002CrossRefPubMed

58.

Symmonds M, Waters PJ, Küker W, Leite MI, Schulz UG (2015) Anti-MOG antibodies with longitudinally extensive transverse myelitis preceded by CLIPPERS. Neurology 84(11):1177–1179. https://doi.org/10.1212/wnl.0000000000001370CrossRefPubMedPubMedCentral

59.

Taieb G, Wacongne A, Renard D, Figarella-Branger D, Castelnovo G, Labauge P (2011) A new case of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids with initial normal magnetic resonance imaging. Brain 134(Pt 8):e182. https://doi.org/10.1093/brain/awq390CrossRefPubMed

60.

Roosendaal SD, Geurts JJ, Vrenken H, Hulst HE, Cover KS, Castelijns JA, Pouwels PJ, Barkhof F (2009) Regional DTI differences in multiple sclerosis patients. Neuroimage 44(4):1397–1403. https://doi.org/10.1016/j.neuroimage.2008.10.026CrossRefPubMed

61.

Pache F, Zimmermann H, Finke C, Lacheta A, Papazoglou S, Kuchling J, Wuerfel J, Hamm B, Ruprecht K, Paul F, Brandt AU, Scheel M (2016) Brain parenchymal damage in neuromyelitis optica spectrum disorder—A multimodal MRI study. Eur Radiol 26(12):4413–4422. https://doi.org/10.1007/s00330-016-4282-xCrossRefPubMed

62.

Zhang L, Liu XH, Jin F, Liu MX, Zhang M, Zhang Y, Zhou DB, Zhang W (2019) 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. J Clin Neurosci 66:156–164. https://doi.org/10.1016/j.jocn.2019.04.022CrossRefPubMed

63.

Alsherbini K, Beinlich B, Salamat MS (2014) Diffusely infiltrating central nervous system lymphoma involving the brainstem in an immune-competent patient. JAMA Neurol 71(1):110–111. https://doi.org/10.1001/jamaneurol.2013.1578CrossRefPubMed

64.

Zhang YX, Ding MP, Zhang T, Tang YL, Guo Y, Fan J, Chen LR, Chen Q (2013) Lymphomatoid granulomatosis with CNS involvement can lead to spontaneous remission: case study. CNS Neurosci Ther 19(7):536–538. https://doi.org/10.1111/cns.12109CrossRefPubMedPubMedCentral

65.

Fujisawa N, Oya S, Mori H, Matsui T (2015) Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids with a significant elevation of β-2 microglobulin levels. J Korean Neurosurg Soc 58(5):487–490. https://doi.org/10.3340/jkns.2015.58.5.487CrossRefPubMedPubMedCentral

66.

Martin T, Duke S, Kumar V, Fadhil A, Ibrahim R, Palmer K, Yazdani S, Devereaux M, Gunzler S, Serra A, Abboud H (2021) NMDAR antibody encephalitis overlapping with CLIPPERS syndrome in a psoriasis patient on adalimumab. J Neurol 268(2):714–716. https://doi.org/10.1007/s00415-020-10341-4CrossRefPubMed

67.

Reindl M, Di Pauli F, Rostásy K, Berger T (2013) The spectrum of MOG autoantibody-associated demyelinating diseases. Nat Rev Neurol 9(8):455–461. https://doi.org/10.1038/nrneurol.2013.118CrossRefPubMed

68.

Fang B, McKeon A, Hinson SR, Kryzer TJ, Pittock SJ, Aksamit AJ, Lennon VA (2016) Autoimmune glial fibrillary acidic protein astrocytopathy: a novel meningoencephalomyelitis. JAMA Neurol 73(11):1297–1307. https://doi.org/10.1001/jamaneurol.2016.2549CrossRefPubMed

69.

Bradshaw MJ, Pawate S, Koth LL, Cho TA, Gelfand JM (2021) Neurosarcoidosis: pathophysiology, diagnosis, and treatment. Neurol Neuroimmunol Neuroinflamm 8(6):e1084. https://doi.org/10.1212/nxi.0000000000001084CrossRefPubMedPubMedCentral

70.

Tian D, Zhu X, Xue R, Zhao P, Yao Y (2018) Case 259: primary central nervous system lymphomatoid granulomatosis mimicking chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Radiology 289(2):572–577. https://doi.org/10.1148/radiol.2018161475CrossRefPubMed

71.

Purysko CP, Purysko AS (2015) Lymphomatoid granulomatosis. J Urol 194(6):1759–1760. https://doi.org/10.1016/j.juro.2015.09.063CrossRefPubMed

72.

Berkman J, Ford C, Johnson E, Malow BA, Aulino JM (2018) Misdiagnosis: CNS Erdheim-Chester disease mimicking CLIPPERS. Neuroradiol J 31(4):399–402. https://doi.org/10.1177/1971400917710251CrossRefPubMed

73.

Cohen Aubart F, Idbaih A, Emile JF, Amoura Z, Abdel-Wahab O, Durham BH, Haroche J, Diamond EL (2021) Histiocytosis and the nervous system: from diagnosis to targeted therapies. Neuro Oncol 23(9):1433–1446. https://doi.org/10.1093/neuonc/noab107CrossRefPubMedPubMedCentral

74.

Davis JW, Auerbach A, Crothers BA, Lewin E, Lynch DT, Teschan NJ, Schmieg JJ (2022) Intravascular large B-cell lymphoma. Arch Pathol Lab Med 146(9):1160–1167. https://doi.org/10.5858/arpa.2021-0165-RACrossRefPubMed

75.

Limousin N, Praline J, Motica O, Cottier JP, Rousselot-Denis C, Mokhtari K, Gonzalez-Aguilar A, De Toffol B (2012) Brain biopsy is required in steroid-resistant patients with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). J Neurooncol 107(1):223–224. https://doi.org/10.1007/s11060-011-0724-0CrossRefPubMed

76.

Moreira I, Cruto C, Correia C, Alves JE, Taipa R, Pires MM (2015) Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): postmortem findings. J Neuropathol Exp Neurol 74(2):186–190. https://doi.org/10.1097/nen.0000000000000162CrossRefPubMed

77.

Taieb G, Labauge P (2015) Anti-MOG antibodies with longitudinally extensive transverse myelitis preceded by CLIPPERS. Neurology 85(14):1262–1263. https://doi.org/10.1212/01.wnl.0000472753.44905.98CrossRefPubMed

78.

Buttmann M, Metz I, Brecht I, Brück W, Warmuth-Metz M (2013) Atypical chronic lymphocytic inflammation with pontocerebellar perivascular enhancement responsive to steroids (CLIPPERS), primary angiitis of the CNS mimicking CLIPPERS or overlap syndrome? A case report J Neurol Sci 324(1–2):183–186. https://doi.org/10.1016/j.jns.2012.10.017CrossRefPubMed

79.

Taieb G, Labauge P (2016) CLIPPERS features before, during and after lymphoma. J Neurol Sci 366:251–252. https://doi.org/10.1016/j.jns.2016.04.052CrossRefPubMed

80.

Taieb G, Uro-Coste E, Clanet M, Lassmann H, Benouaich-Amiel A, Laurent C, Delisle MB, Labauge P, Brassat D (2014) A central nervous system B-cell lymphoma arising two years after initial diagnosis of CLIPPERS. J Neurol Sci 344(1–2):224–226. https://doi.org/10.1016/j.jns.2014.06.015CrossRefPubMed

81.

Lin AW, Das S, Fraser JA, Ang LC, Florendo-Cumbermack A, Jenkins ME, Tay KY (2014) Emergence of primary CNS lymphoma in a patient with findings of CLIPPERS. Can J Neurol Sci 41(4):528–529. https://doi.org/10.1017/s0317167100018631CrossRefPubMed

82.

Yi F, Tian Y, Chen XY, Zhou Y, Hu YC, Sun Q, Guo JF, Tang B, Zhou L, Li J, Xu HW (2018) Two cases of CLIPPERS with increased number of perivascular CD20-positive B lymphocytes. Brain 141(10):e75. https://doi.org/10.1093/brain/awy234CrossRefPubMed

83.

Yuen CA, Rezania K, Park DM, Reder AT (2021) Asymptomatic brainstem lesions and pachymeningeal enhancement after anti-PD-1 therapy. Immunotherapy 13(1):11–17. https://doi.org/10.2217/imt-2020-0186CrossRefPubMed

84.

Keegan BM, Pittock SJ (2012) Cutting-edge questions about CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids). Arch Neurol 69(7):819–820. https://doi.org/10.1001/archneurol.2012.1015CrossRefPubMed

85.

Taieb G, Allou T, Labauge P (2017) Therapeutic Approaches in CLIPPERS. Curr Treat Options Neurol 19(5):17. https://doi.org/10.1007/s11940-017-0455-4CrossRefPubMed

86.

Kovacova S, Hnilicova P, Grossmann J, Hajas G, Kantorova E, Kurca E (2022) Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) syndrome: treatment approach depends on disease course. Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub 167(3):303–308. https://doi.org/10.5507/bp.2022.013CrossRefPubMed

87.

Veerapandiyan A, Chaudhari A, Deo P, Ming X (2017) Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): a pediatric case report with six year follow-up. Mult Scler Relat Disord 17:95–98. https://doi.org/10.1016/j.msard.2017.07.012CrossRefPubMed

88.

Rempe T, Becktepe JS, Metz I, Brück W, Stürner KH, Deuschl G, Berg D, Baron R, Zeuner R, Leypoldt F (2019) A case of CLIPPERS syndrome responsive to tocilizumab. Neurol Neuroimmunol Neuroinflamm 6(3):e545. https://doi.org/10.1212/nxi.0000000000000545CrossRefPubMedPubMedCentral

89.

Didier PJ, Adrián ML, Paola SA, Mauricio FS, Omar CS, Liset RY, Luis RJ (2018) CLIPPERS syndrome responsive to Leflunomide: a case report. Mult Scler Relat Disord 25:265–267. https://doi.org/10.1016/j.msard.2018.07.048CrossRefPubMed

90.

Thebault S, Bergman H, Atkins HL, Freedman MS, Brooks J (2023) CLIPPERS responsive to cladribine as a durable steroid-sparing agent. Neurol Neuroimmunol Neuroinflamm 10(1):e200060. https://doi.org/10.1212/nxi.0000000000200060CrossRefPubMed

91.

Vattoth S, Aker L, Abdelhady M, El Beltagi AH (2022) Case 302: supratentorial lymphocytic inflammation with parenchymal perivascular enhancement responsive to steroids. Radiology 303(3):726–730. https://doi.org/10.1148/radiol.204423CrossRefPubMed

92.

Tsibonakis A, Theodorou A, Korkolopoulou P, Tsivgoulis G (2023) Supratentorial lymphocytic inflammation with parenchymal perivascular enhancement responsive to steroids: a potentially overlooked diagnosis. Ann Neurol. https://doi.org/10.1002/ana.26821CrossRefPubMed

93.

Lv C, Zhu F, Chen C, Wang Y, Guo D, Zhang Z (2022) A rare case report of primary Sjögren’s syndrome with clinical characteristics similar to those of CLIPPERS. BMC Neurol 22(1):414. https://doi.org/10.1186/s12883-022-02945-2CrossRefPubMedPubMedCentral

94.

Debinski C, Goergen S, McLean C, Buckland ME, Kumar B, Tiller G, Cole T, Ojaimi S, Fahey M (2021) Exploring the Intersection of Isolated-CNS hemophagocytic lymphohistiocytosis and pediatric chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids. J Child Neurol 36(11):935–942. https://doi.org/10.1177/08830738211009654CrossRefPubMed

95.

Taieb G, Kaphan E, Duflos C, Lebrun-Frénay C, Rigau V, Thouvenot E, Duhin-Gand E, Lefaucheur R, Hoang-Xuan K, Coulette S, Ouallet JC, Menjot de Champfleur N, Tranchant C, Picard C, Fusaro M, Sepulveda FE, Labauge P, de Saint BG (2021) Hemophagocytic lymphohistiocytosis gene mutations in adult patients presenting With CLIPPERS-Like Syndrome. Neurol Neuroimmunol Neuroinflamm 8(3):e970. https://doi.org/10.1212/nxi.0000000000000970CrossRefPubMedPubMedCentral

96.

Ponnatt TS, Lilley CM, Mirza KM (2022) Hemophagocytic lymphohistiocytosis. Arch Pathol Lab Med 146(4):507–519. https://doi.org/10.5858/arpa.2020-0802-RACrossRefPubMed

97.

Al-Samkari H, Berliner N (2018) Hemophagocytic lymphohistiocytosis. Annu Rev Pathol 13:27–49. https://doi.org/10.1146/annurev-pathol-020117-043625CrossRefPubMed

98.

Yin HX, Zhou Y, Xu Y, Li ML, Zhang Z, Zhou LX, Zhang Y, Zhu YC, Peng B, Cui LY (2020) A case report of autoimmune glial fibrillary acidic protein astrocytopathy diagnosed after long term diagnosis of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids. Front Neurol 11:598650. https://doi.org/10.3389/fneur.2020.598650CrossRefPubMedPubMedCentral

99.

Law LY, Riminton DS, Nguyen M, Barnett MH, Reddel SW, Hardy TA (2019) The spectrum of immune-mediated and inflammatory lesions of the brainstem: clues to diagnosis. Neurology 93(9):390–405. https://doi.org/10.1212/wnl.0000000000008015CrossRefPubMed

Titel: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): contemporary advances and current controversies
verfasst von: Er-Chuang Li
Qi-Lun Lai
Meng-Ting Cai
Gao-Li Fang
Wei Fang
Yang Zheng
Ye Du
Bing-Qing Du
Chun-Hong Shen
Song Qiao
Mei-Ping Ding
Yin-Xi Zhang
Publikationsdatum: 29.01.2024
Verlag: Springer Berlin Heidelberg
Erschienen in: Journal of Neurology / Ausgabe 4/2024
Print ISSN: 0340-5354
Elektronische ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-024-12189-4

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