Preview

Neurology, Neuropsychiatry, Psychosomatics

Advanced search

CLIPPERS syndrome

https://doi.org/10.14412/2074-2711-2018-2-76-82

Full Text:

Abstract

CLIPPERS syndrome (Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids) is a recently described rare disease affecting the central nervous system. It is characterized by subacute development of symptoms of lesions predominantly in the brain stem and cerebellum, by specific magnetic resonance imaging (MRI) changes, perivascular lymphocytic infiltration in the brain substance and a good response to glucocorticoid (GC) therapy. The paper describes CLIPPERS syndrome in a patient who has been followed up in a clinic for 10 years. During this period, different variants of clinical diagnosis have been considered. The final diagnosis was made only when comparing the clinical course and manifestations of the disease, MRI data, as well as the reaction to GC therapy and its discontinuation. Literature data and diagnostic criteria of this disease are presented.

About the Authors

T. E. Schmidt
I.M. Sechenov First Moscow State Medical University (Sechenov University), Ministry of Health of Russia
Russian Federation

Contact: Tatiana Evgenyevna Shmidt 

A.Ya. Kozhevnikov Clinic of Nervous System Diseases 

11, Rossolimo St., Moscow 119021

 



I. N. Pronin
Academician N.N. Burdenko National Medical Research Center for Neurosurgery, Ministry of Health of Russia
Russian Federation
16, Fourth Tverskaya-Yamskaya St., Moscow 125047


K. Yu. Kazantsev
I.M. Sechenov First Moscow State Medical University (Sechenov University), Ministry of Health of Russia
Russian Federation

A.Ya. Kozhevnikov Clinic of Nervous System Diseases 

11, Rossolimo St., Moscow 119021




O. N. Voskresenskaya
I.M. Sechenov First Moscow State Medical University (Sechenov University), Ministry of Health of Russia
Russian Federation

A.Ya. Kozhevnikov Clinic of Nervous System Diseases 

11, Rossolimo St., Moscow 119021




I. V. Damulin
I.M. Sechenov First Moscow State Medical University (Sechenov University), Ministry of Health of Russia
Russian Federation

A.Ya. Kozhevnikov Clinic of Nervous System Diseases 

11, Rossolimo St., Moscow 119021




A. A. Aleksandrov
I.M. Sechenov First Moscow State Medical University (Sechenov University), Ministry of Health of Russia
Russian Federation

A.Ya. Kozhevnikov Clinic of Nervous System Diseases 

11, Rossolimo St., Moscow 119021




N. N. Yakhno
I.M. Sechenov First Moscow State Medical University (Sechenov University), Ministry of Health of Russia
Russian Federation

A.Ya. Kozhevnikov Clinic of Nervous System Diseases 

11, Rossolimo St., Moscow 119021




References

1. Pittock S, Debruyne J, Krecke K, et al. Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to sreroids. Brain. 2010 Sep;133(9):2626-34. doi: 10.1093/brain/awq164. Epub 2010 Jul 17.

2. Likhachev SA, Astapenko AV, Pleshko IV, et al. Autoimmune stem encephalitis syndrome CLIPPERS. Nevrologicheskii zhurnal. 2015;(6):15-21.(In Russ.)]

3. Taieb G, Duflos C, Renard D, et al. Longterm of CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to sreroids) in a consecutive series of 12 patients. Arch Neurol. 2012 Jul;69(7):847-55. doi: 10.1001/archneurol.2012.122.

4. Tobin W, Guo Y, Krecke K, et al. Diagnostic criteria for chronic lymphocytic inflammation with pontine perivascular enhancement responsive to sreroids (CLIPPERS). Brain. 2017 Sep 1;140(9):2415-2425. doi: 10.1093/brain/awx200.

5. Simon N, Parratt J, Barnett M, et al. Expanding the clinical, radiological and neuropathological phenotype of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to sreroids (CLIPPERS). J Neurol Neurosurg Psychiatry. 2012 Jan;83(1):15-22. doi: 10.1136/jnnp-2011-301054. Epub 2011 Nov 5.

6. Duprez T, Sindic C. Contrast-enhanced magnetic resonance imaging and perfusionweighted imaging for monitoring features in severe CLIPPERS. Brain. 2011 Aug;134 (Pt 8):e184; author reply e186. doi: 10.1093/brain/awr034. Epub 2011 Mar 7.

7. Duvernoy H. Human brainstem vessels. Berlin: Springer-Verlag; 1978. P. 2-34

8. Lefaucher N, Bouwyn J, Ahtoy P, et al. Teaching neuroimages: punctuate and curvilinear enhancement peppering the pons responsive to sreroids. Neurology. 2011 Sep 6;77(10):e57-8. doi: 10.1212/WNL.0b013e31822cfa4a.

9. Jones J, Dean A, Antoun N, et al. Radiologically compatible CLIPPERS may conceal a numbers of pathologies. Brain. 2011 Aug;134(Pt 8):e187. doi: 10.1093/brain/awr134. Epub 2011 Jun 7.

10. Limousin N, Praline J, Motica O, et al. Brain biopsy is required in steroid-resitance patients with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to sreroids (CLIPPERS). J Neurooncol. 2012 Mar;107(1):223-4. doi: 10.1007/s11060-011-0724-0. Epub 2011 Oct 4.

11. Kleinschmidt-DeMasters B, West M. CLIPPERS with chronic small vessel damage more often overlap small vessel vasculitis?. J Neuropathol Exp Neurol. 2014 Mar;73(3): 262-7. doi: 10.1097/NEN.0000000000000050.

12. Шмидт ТЕ. Редкие демиелинизирующие заболевания центральной нервной системы. Неврологический журнал. 2016;(5):252-64.

13. Reddy SM, Lath R, Swain M, Ranjan A. Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): A case report and review of literature. Ann Indian Acad Neurol. 2015 Jul–Sep;18(3):345-7. doi: 10.4103/09722327.152085.

14. Gabilondo I, Saiz A, Graus F, Villoslada P. Response to immunotherapy in CLIPPERS syndrome. J Neurol. 2011 Nov;258(11):2090-2. doi: 10.1007/s00415-011-6068-z. Epub 2011 May 8.

15. Sempere A, Mola S, Martin-Medina P, et al. Responsive to immunotherapy in CLIPPERS. J Neuroimaging. 2013 Apr;23(2):254-5. doi: 10.1111/j.1552-6569.2011.00631.x. Epub 2011 Aug 17.


For citation:


Schmidt T.E., Pronin I.N., Kazantsev K.Y., Voskresenskaya O.N., Damulin I.V., Aleksandrov A.A., Yakhno N.N. CLIPPERS syndrome. Neurology, Neuropsychiatry, Psychosomatics. 2018;10(2):76-82. (In Russ.) https://doi.org/10.14412/2074-2711-2018-2-76-82

Views: 299


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


ISSN 2074-2711 (Print)
ISSN 2310-1342 (Online)