AUTOIMMUNE BRAINSTEM ENCEPHALITIS (CLIPPERS-SYNDROM): A CASE REPORT
https://doi.org/10.25207/1608-6228-2019-26-3-18-24
Abstract
Aim. In this paper, we aim to analyse our own clinical observation of a patient with CLIPPERS syndrome on the basis of available literature data.
Materials and methods. The medical record of a patient treated at the Neurology Unit of the S.V. Ochapovsky Regional Clinical Hospital No. 1 (Kradnodarsy Krai) was investigated, along with available national and foreign literary sources.
Results. The patient was diagnosed with autoimmune brainstem encephalitis (CLIPPERS syndrome) on the basis of the diagnostic CLIPPERS syndrome criteria modified and adapted by W. Tobin and co-authors in 2017. Among them are: 1) subacute development of symptoms for the pons and cerebellum lesions along with other signs of CNS damage (onset is characterised by diplopia and ataxia); 2) good response to glucocorticosteroid (GCS) therapy; 3) lack of involvement of the peripheral nervous system; 4) enhanced MRI lesions homogeneously accumulating contrast agents without a mass effect; 5) a significant decrease in the accumulation of contrast material during GCS therapy; 6) absence of myelin loss.
Conclusion. Considering that the GCS therapy produces a rapid and significant effect, it is important to focus on early detection and treatment of this disease through the development of more accurate markers and evaluation criteria for its diagnosis.
About the Authors
Marianna A. BarabanovaRussian Federation
Dr. Sci. (Med.), Prof., Department for Nervous System Diseases and Neurosurgery with a course in Nervous System Diseases and Neurosurgery, Faculty of Advanced Training and Occupational Retraining, Kuban State Medical University, Ministry of Healthcare of the Russian Federation,
Mitrofanа Sedinа str., 4, Krasnodar, 350063;
Head of Neurology Unit, Research Institute — Regional Clinical Hospital 1 n.a. Prof. S.V. Ochapovsky, Ministry of Healthcare of Krasnodar Krai,
1 Maya str., 167, Krasnodar, 350086
Elena V. Krivomlina
Russian Federation
Neurologist, Neurology Unit,
1 Maya str., 167, Krasnodar, 350086
Maria A. Berezhnaya
Russian Federation
6th year student, Paediatric Faculty,
Mitrofanа Sedinа str., 4, Krasnodar, 350063;
tel.: +7 (938) 512-60-40; Ayvazovsky str., 100–58, Krasnodar, 350063
Elizaveta A. Teplyakova
Russian Federation
6th year student, Paediatric Faculty,
Mitrofanа Sedinа str., 4, Krasnodar, 350063
References
1. Pittock S., Debruyne J., Krecke K., 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. Likhachev S.A., Astapenko A.V., Pleshko I.V., Korbut T.V., Antonenko A.I., Naumenko D.V., Pavlovskaya T.S., Zavodskaya O.V. Autoimmune brainstem enhcephalitis — CLIPPERS syndrome (cases reports). Nevrologicheskii Zhurnal. 2015; 20(6): 15–21 (In Russ., English abstract). DOI: 10.18821/1560-9545-2015-20-6-15-21
3. Ruina E.A., Smirnov A.A., Komshina K.S., Parshina E.V. CLIPPERS syndrome: clinical case and literature review. Meditsinskii Al’manakh. 2016; 5(45): 146–149 (In Russ., English abstract). DOI: 10.21145/2499-9954-2016-5-146-149
4. Sivertseva S.A., Sivertsev M.Yu., Bazhukhin D.V., Vorob’ev D.P., Boiko A.N. Syndrome CLIPPERS (literature review and a case report). Zhurnal Nevrologii i Psikhiatrii im. S.S. Korsakova. 2017; 117(10-2): 24–34 (In Russ., English abstract). DOI: 10.17116/jnevro201711710224-34
5. Shmidt T.E., Pronin I.N., Kazantsev K.Yu., Voskresenskaya O.N., Damulin I.V., Aleksandrov A.A., Yakhno N.N. CLIPPERS syndrome. Nevrologiya, Neiropsikhiatriya, Psikhosomatika. 2018; 10(2): 76–82 (In Russ., English abstract). DOI: 10.14412/2074-2711-2018-2-76-82
6. Tobin W., Guo Y., Krecke K., Parisi J.E., Lucchinetti C.F., Pittock S.J., Mandrekar J., Dubey D., Debruyne J., Keegan B.M. Diagnostic criteria for chronic lymphocytic inflammation with pontine perivascular enhancement responsive to sreroids (CLIPPERS). Brain. 2017; 140(9): 2415–2425. DOI: 10.1093/brain/awx200
7. Simon N., Parratt J., Barnett M., Buckland M.E., Gupta R., Hayes M.W., Masters L.T., Reddel S.W. Expanding the clinical, radiological and neuropathological phenotype of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to sreroids (CLIPPERS). J. Neurol. Neurosurg. Psychiatry. 2012; 83(1): 15–22. DOI: 10.1136/jnnp-2011-301054
8. Smith A., Matthews Y., Kossard S., Turner J., Buckland M.E., Parratt J. Neurotropic T-cell lymphocytosis: a cutaneous expression of CLIPPERS. J. Cutan.Pathol. 2014; 41(8): 657–662. DOI: 10.1111/cup.12344
9. 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 J.L., de Champfleur N., Castelnovo G., Eimer S., Branger D.F., Uro-Coste E., Labauge P. Longterm of CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to sreroids) in a consecutive series of 12 patients. Arch. Neurol. 2012; 69(7): 847–855. DOI: 10.1001/archneurol.2012.122
10. 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 recognized entity within the spectrum of inflammatory central nervous system disorders. Clin. Exp. Immunol. 2014; 175(3): 385–396. DOI: 10.1111/cei.12204
11. Buttmann M., Metz I., Brecht I., Bruck W., Warmuth-Metz 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
12. List J., Lesemann A., Wiener E., Walter G., Hopmann D., Schreiber S., Ruprecht K. A new case of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids. Brain. 2011; 134(Pt 8): е185. DOI: 10.1093/brain/awr035
13. Shmidt T.E. Rare demyelinating diseases of central nervous system. Nevrologicheskii Zhurnal. 2016; 21(5): 252–264. (In Russ., English abstract). DOI: 10.18821/1560-9545-2016-21-5-252-264
14. Duprez T.P., Sindic C.J.M. Contrast-enhanced magnetic resonance imaging and perfusion-weighted imaging for monitoring features in severe CLIPPERS. Brain. 2011; 134(8): e184. DOI: 10.1093/brain/awr034
15. 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
Review
For citations:
Barabanova M.A., Krivomlina E.V., Berezhnaya M.A., Teplyakova E.A. AUTOIMMUNE BRAINSTEM ENCEPHALITIS (CLIPPERS-SYNDROM): A CASE REPORT. Kuban Scientific Medical Bulletin. 2019;26(3):18-24. (In Russ.) https://doi.org/10.25207/1608-6228-2019-26-3-18-24