Preview

Bulletin of Siberian Medicine

Advanced search

CLIPPERS. Three clinical cases and review

https://doi.org/10.20538/1682-0363-2019-4-256-265

Abstract

CLIPPERS (Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) is a rare inflammatory disease of the central nervous system, during which the pons of the brain is damaged. This disease was described for the first time in 2010 by S.J. Pittock et.al. At present, there have been around 50 described cases of the disease. Up to the present moment, there are difficulties diagnosing this disease. In the article, a literature review and three clinical cases are presented. Furthermore, the necessity of further research is shown for improving the accuracy and specificity of the diagnostic criteria, as well as for defining biomarkers and developing algorithms of effective therapy.

About the Authors

L. N. Prakhova
N.P. Bechtereva Institute of the Human Brain of the Russian Academy of Sciences (RAS)
Russian Federation

DM, Head of the Neurorehabilitation Laboratory,

9, Akademika Pavlova Str., St. Petersburg, 197376



A. S. Parfyonova
N.P. Bechtereva Institute of the Human Brain of the Russian Academy of Sciences (RAS)
Russian Federation

Resident, Department of Neurology,

9, Akademika Pavlova Str., St. Petersburg, 197376



Zh. I. Savintseva
N.P. Bechtereva Institute of the Human Brain of the Russian Academy of Sciences (RAS)
Russian Federation

PhD, Rentgenologist, Radiology Department,

9, Akademika Pavlova Str., St. Petersburg, 197376



A. G. Ilves
N.P. Bechtereva Institute of the Human Brain of the Russian Academy of Sciences (RAS)
Russian Federation

PhD, Senior Researcher, Neuroimmunology Laboratory,

9, Akademika Pavlova Str., St. Petersburg, 197376



E. V. Bubnova
Academician I.P. Pavlov First St. Petersburg State Medical University
Russian Federation

PhD, Associate Professor,

6/8, Lva Tolstogo Str., St. Petersburg, 197022



V. S. Krasnov
Academician I.P. Pavlov First St. Petersburg State Medical University
Russian Federation

PhD, Associate Professor, Department of Neurology,

6/8, Lva Tolstogo Str., St. Petersburg, 197022



A. M. Tarasova
Academician I.P. Pavlov First St. Petersburg State Medical University
Russian Federation

Neurologist,

6/8, Lva Tolstogo Str., St. Petersburg, 197022



N. A. Totolyan
Academician I.P. Pavlov First St. Petersburg State Medical University
Russian Federation

DM, Professor, Department of Neurology,

6/8, Lva Tolstogo Str., St. Petersburg, 197022



References

1. Pittock S.J., Debruyne J., Krecke K.N., Giannini C., van den Ameele J., de Herdt V., McKeon A., Fealey R.D., Weinshenker B.G., Aksamit A.J., Krueger B.R., Shuster E.A., Keegan B.M. Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Brain. 2010; 133 (9): 2626–2634. DOI: 10.1093/brain/awq164.

2. Dudesek A., Rimmele F., Tesar S., Kolbaske S., Rommer P.S., Benecke R., Zettl U.K. CLIPPERS: chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids. Review of an increasingly reñognized entity within the spectrum of inflammatory central nervous system disorders. Clin. Exp. Immunol. 2014; 175 (3): 385–396. DOI: 10.1111/cei.12204.

3. Taieb G., Duflos C., Renard D., Audoin B., Kaphan E., elletier 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 J.L., de Champfleur N., Castelnovo G., Eimer S., Branger D.F., Uro-Coste E., Labauge P. Long-term outcomes of CLIPPERS (chronic lymphocytic inflammation with pontine perivascularenhancement responsive to steroids) in a consecutive series of 12 patients. Arch. Neurol. 2012; 69 (7): 847–855. DOI: 10.1001/archneurol.2012.122.

4. Simon N.G., Parratt J.D., Barnett M.H., Buckland M.E., Gupta R., Hayes M.W., Masters L.T., Reddel S.W. Expanding the clinical, radiological and neuropathological phenotype of chronic lymphocyticinflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). J. Neurol. Neurosurg. Psychiatry. 2012; 83 (1): 15–22. DOI: 10.1136/jnnp-2011-301054.

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

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

7. Taieb G., Wacongne A., Renard D., Figarella-Branger D., Castelnovo G., Labauge P. A new case of chronic lymphocytic inflammation with pontine perivascular enhancement resposive to steroids with initial normal magnetic resonance imaging. Brain. 2011; 134 (Pt 8): e182; author reply e183. DOI: 10.1093/brain/awq390.

8. Gabilondo I., Saiz A., Graus F., Villoslada P. Re sponse to immunotherapy in CLIPPERS syndrîme. J. Neurol. 2011; 258 (11): 2090–2092. DOI: 10.1007/s00415-011-6068-z.

9. Duprez T.P., Sindic C.J. Contrast-enhanced magnetic resonance imaging and perfusion-weighted imaging for monitoring features in severe CLIPPERS. Brain. 2011; 134 (Pt 8): e184. DOI: 10.1093/brain/awr034.

10. Sczesni K.C., Alekseyev A., Schlegel U., Skodda S. Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids. Nervenarzt. 2012; 83 (6): 775–781 (in Germ.). DOI: 10.1007/s00115-011-3430-4.

11. Sempere A.P., 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): 254–255. DOI: 10.1111/j.1552-6569.2011.00631.x.

12. Ortega M.R., Usmani N., Parra-Herran C., Adams D.J., Steingo B., Rammohan K.W. CLIPPERS complicating multiple sclerosis causing concerns of CNS lymphoma. Neurology. 2012; 79 (7): 715–716. DOI: 10.1212/wnl.0b013e3182648b77.

13. Hillesheim P.B., Parker J.R., Parker J.C.Jr, Escott E., Berger J.R. Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids following influenza vaccination. Arch. Pathol. Lab. Med. 2012; 136 (6): 681–685. DOI: 10.5858/arpa.2011-0428-cr.

14. List J., Lesemann A., Wiener E., Walter G., Hopmann D., Schreiber S., Ruprecht K. A new case of chronic lymphocytic inflamma tion with pontine perivascular enhancement re sponsive to steroids. Brain. 2011; 134 (Pt. 8): e185. DOI: 10.1093/brain/awr035.

15. Jones J.L., Dean A.F., Antoun N., Scoffings D.J., Burnet N.G., Coles A.J. ‘Radiologically compatible CLIPPERS’ may conceal a number of pathologies. Brain. 2011; 134 (Pt. 8): e187. DOI: 10.1093/brain/awr134.

16. Limousin N., Praline J., Motica O., Cottier J.P., Rousselot-Denis C., Mokhtari K., Gonzalez-Aguilar A., De Toffol B. Brain biopsy is required in steroid-re sistant patients with chronic lymphocytic in flammation with pontineperivascular enhancement responsive to steroids (CLIPPERS). J. Neu rooncol. 2012; 107 (1): 223–224. DOI: 10.1007/s11060-011-0724-0.

17. De Graaff H.J., Wattjes M.P., Rozemuller-Kwakkel A.J., Petzold A., Killestein J. Fatal B-cell lymphoma following chronic lymphocytic inflammation with pontine perivascularenhancement responsive to steroids. JAMA Neu rol. 2013; 70 (7): 915–918. DOI: 10.1001/jamaneurol.2013.2016.

18. Buttmann M., Metz I., Brecht I., Brück W., WarmuthMetz M. 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. 2013; 324 (1–2): 183–186. DOI: 10.1016/j.jns.2012.10.017.

19. Ferreira R.M., Machado G., Souza A.S., Lin K., Corrêa-Neto Y. CLIPPERS-like MRI findings in a patient with multiple sclerosis. J. Neurol. Sci. 2013; 327 (1–2): 61–62. DOI: 10.1016/j.jns.2013.01.032.

20. Keegan B.M., Pittock S.J. Cutting-edge questions about CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids). Arch. Neu rol. 2012; 69 (7): 819–820. DOI: 10.1001/archneurol.2012.1015.

21. Weng C.F., Chan D.C., Chen Y.F., Liu F.C., Liou H.H. Chronic hepatitis B infection presenting with chronic lymphocytic inflammation with pon-tineperivascular enhancement responsive to steroids (CLIPPERS): a case report. J. Med. Case Rep. 2015; 9: 266. DOI: 10.1186/s13256-015-0750-1.

22. Wang L., Holthaus E.A., Jimenez X.F., Tavee J., Li Y. MRI evolution of CLIPPERS syndrome fol-lowing herpes zoster infection. J. Neurol. Sci. 2015; 348 (1–2): 277–278. DOI: 10.1016/j.jns.2014.11.022.

23. Biotti D., Deschamps R., Shotar E., Maillart E., Obadia M., Mari I., Savatovsky J., Gout O. CLIPPERS: chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids. Pract. Neurol. 2011; 11 (6): 349–351. DOI: 10.1136/practneurol-2011-000043.

24. Wijntjes J., Wouda E.J., Siegert C.E., Karas G.B., Vlaar A.M. Need for prolonged immunosupressive therapy in CLIPPERS – a case report. BMC Neurol. 2013; 13: 49. DOI: 10.1186/1471-2377-13-49.

25. Tan B.L., Agzarian M., Schultz D.W. CLIPPERS: Induction and maintenance of re-mission using hydroxychloroquine. Neurol. Neuroimmunol. Neuroinflamm. 2015; 2 (1): e56. DOI: 10.1212/nxi.0000000000000056.

26. Rico M., Villafani J., Tuñón A., Mateos V., Oliva-Nacarino P. IFN beta 1a as glucocorticoids-sparing therapy in a patient with CLIPPERS. Am. J. Case Rep. 2016; 17: 47–50. DOI: 10.12659/ajcr.896102.


Review

For citations:


Prakhova L.N., Parfyonova A.S., Savintseva Zh.I., Ilves A.G., Bubnova E.V., Krasnov V.S., Tarasova A.M., Totolyan N.A. CLIPPERS. Three clinical cases and review. Bulletin of Siberian Medicine. 2019;18(4):256-265. (In Russ.) https://doi.org/10.20538/1682-0363-2019-4-256-265

Views: 674


Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 License.


ISSN 1682-0363 (Print)
ISSN 1819-3684 (Online)