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customerservice@lww.com. From the *Department of Medicine, Winthrop University Hospital, Mineola; †Department of Radiology Stony Brook University Medical Center, Stony Brook; and ‡Department of Rheumatology, Clinical Immunology and Allergy, Winthrop University Hospital, Mineola, NY. Academician N.N. 2015 Jul–Sep;18(3):345-7. doi: 10.4103/09722327.152085. Sempere A, Mola S, Martin-Medina P, et al. Brain. 2010 Sep;133(9):2626-34. doi: 10.1093/brain/awq164. 4. During this period, different variants of clinical diagnosis have been considered. CLIPPERS has a basic entry at the NIH Office of Rare Diseases Research (this has been updated in 2013). Nevrologicheskii zhurnal. CLIPPERS typically manifests between 13 and 78 years of age (13,30). Please enable scripts and reload this page. Epub 2011 Aug 17. The authors declare no conflict of interest. Schmidt T.E., Pronin I.N., Kazantsev K.Yu., Voskresenskaya O.N., Damulin I.V., Aleksandrov A.A., Yakhno N.N. 13. Our case is unique in that this is the first case which demonstrates involvement of the supratentorial perivascular spaces in addition to the classic infratentorial involvement as initially described by Pittock et al (Brain. Correspondence: Maryam Gul, MD, Department of Medicine, Winthrop University Hospital, 222 Station Plaza North, Suite 509, Mineola, NY 11501. This website uses cookies. Wolters Kluwer Health Copyright © 2020 Elsevier B.V. or its licensors or contributors. CLIPPERS syndrome. By continuing you agree to the use of cookies. 9. Sechenov First Moscow State Medical University (Sechenov University), Ministry of Health of Russia Russian Federation, A. Sechenov First Moscow State Medical University (Sechenov University), Ministry of Health of Russia, Academician N.N. I.M. In this review article, we aim to explore different CNS manifestations of rheumatologic diseases with emphasis on the utility of imaging and cerebrospinal fluid findings. No definite sex predilection is seen ( 13 , 24 , 30 ). Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): A case report and review of literature. You may search for similar articles that contain these same keywords or you may 2018;10(2):76-82. Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS) was recently described in 2010, therefore the long-term prognosis is uncertain and there is no consensus on which steroid-sparing treatment is the most indicated. By continuing to browse this web-site you agree to use cookies and the above service. JCR: Journal of Clinical Rheumatology21(3):144-148, April 2015. 1. For immediate assistance, contact Customer Service: Jones J, Dean A, Antoun N, et al. Registered users can save articles, searches, and manage email alerts. Registered users can save articles, searches, and manage email alerts. access full text with Ovid®. Gabilondo I, Saiz A, Graus F, Villoslada P. Response to immunotherapy in CLIPPERS syndrome. Neurology, Neuropsychiatry, Psychosomatics, I.M. Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an inflammatory disorder consisting of (1) subacute clinical brainstem signs and symptoms, (2) characteristic punctate and curvilinear perivascular peppercorn-like gadolinium enhancement of the pons, brachium pontis, and cerebellum in MRI, (3) dense perivascular CD4+ T cell … doi: 10.1093/brain/awr034. Epub 2011 Mar 7. CLIPPERS syndrome responsive to Leflunomide: A case report. 14. Epub 2011 Nov 5. I was recently contacted by someone with a close relative who had been diagnosed with possible "SLIPPERS" syndrome. 800-638-3030 (within USA), 301-223-2300 (international). P. 2-34. Radiologically compatible CLIPPERS may conceal a numbers of pathologies. Autoimmune stem encephalitis syndrome CLIPPERS. Kozhevnikov Clinic of Nervous System Diseases. 7. For information on cookies and how you can disable them visit our Privacy and Cookie Policy. For more information, please refer to our Privacy Policy. Some error has occurred while processing your request. Numerous autoimmune diseases can affect the central nervous system (CNS), and variable clinical presentations confound the differential diagnosis. The typical clinical presentation consists of a subacute course of progressive gait disorders, ataxia, and dysarthria, as in this patient ( 28 ). You may be trying to access this site from a secured browser on the server. Epub 2011 May 8. CLIPPERS syndrome, PACNS, neurosarcoid, neuro-Behçet, neuro-Sjögren syndrome, neuropsychiatric systemic lupus erythematosus. 2. Burdenko National Medical Research Center for Neurosurgery, Ministry of Health of Russia, Vol 9, No 1S (2017): SPECIAL ISSUE: EPILEPSY, Дисциркуляторная энцефалопатия и сосудистые когнитивные расстройства, Vol 5, No 2S (2013): Special issue "Stroke", https://doi.org/10.14412/2074-2711-2018-2-76-82, Creative Commons Attribution 4.0 License, Russian Register of Pregnancy and Epilepsy, Attention deficit hyperactivity disorder in children, Dissection of internal carotid and vertebral arteries: clinical presentation, diagnosis, and treatment, Postherpetic neuralgia: Therapeutic and prophylactic aspects and pregabalin therapy. I.M. Longterm of CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to sreroids) in a consecutive series of 12 patients. J Neurol Neurosurg Psychiatry. 2010;133:2626–2634). Неврологический журнал. E-mail: maryamgul@gmail.com. Data is temporarily unavailable. J Neurol. Your message has been successfully sent to your colleague. Steroid-sparing cost-effective therapies are a recommended option in CLIPPERS. 6 The beneficial effect of tocilizumab in CLIPPERS could therefore potentially be explained by its effect on the differentiation of T cells into effector or regulatory T ... 2018. The paper describes CLIPPERS syndrome in a patient who has been followed up in a clinic for 10 years. 2016;(5):252-64. 800-638-3030 (within USA), 301-223-2300 (international) Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to sreroids. We review the classic physical examination findings, characteristic imaging features, cerebrospinal fluid results, and serum biomarkers. 2011 Sep 6;77(10):e57-8. Kazantsev Keywords Limousin N, Praline J, Motica O, et al. Epub 2011 Oct 4. We use cookies to help provide and enhance our service and tailor content and ads. Volume 25, October 2018, Pages 265-267. 2011 Nov;258(11):2090-2. doi: 10.1007/s00415-011-6068-z. I started a forum page (2013) on this blog for people who want to share experiences or ask for help and there are other more general forums on the web (e.g. In addition, we also present a unique case of newly described autoimmune entity CLIPPERS syndrome. https://doi.org/10.1016/j.msard.2018.07.048. As far as we have documented, this is the first case of CLIPPERS kept under control with leflunomide in the medium-term with a good relative prognosis. Eventually after the suspension of the steroid-sparing treatment the patient recurred. Case report. A. Aleksandrov J Neurooncol. Initially proposed by Dr Armand in 2015 this condition differs from CLIPPERS in which part of the brain is predominantly affected. Teaching neuroimages: punctuate and curvilinear enhancement peppering the pons responsive to sreroids. A 43-year-old man with progressive symptoms of pontocerebellar dysfunction and radiological findings consistent with CLIPPERS was admitted to our clinic and treated with steroids in the acute phase, then he continue leflunomide for one year, showing sustained clinical and radiological remission. Sechenov First Moscow State Medical University (Sechenov University), Ministry of Health of Russia Russian Federation, N. N. Yakhno 2014 Mar;73(3): 262-7. doi: 10.1097/NEN.0000000000000050. 3. Sechenov First Moscow State Medical University (Sechenov University), Ministry of Health of Russia Russian Federation, I. N. Pronin Lippincott Journals Subscribers, use your username or email along with your password to log in. 11. Berlin: Springer-Verlag; 1978. Log in to view full text. In addition, we also present a unique case of newly described autoimmune entity CLIPPERS syndrome. doi: 10.1212/WNL.0b013e31822cfa4a. 2011 Aug;134(Pt 8):e187. I initially suspected a typo but then realised that this was a CLIPPERS variant which had passed me by. Most countries seem to have some kind of organisation devoted to rare diseases. A.Ya. 15. This case shows the usefulness of leflunomide in the treatment of CLIPPERS syndrome. 2013 Apr;23(2):254-5. doi: 10.1111/j.1552-6569.2011.00631.x. 2012 Jan;83(1):15-22. doi: 10.1136/jnnp-2011-301054. Neurology, Neuropsychiatry, Psychosomatics. 2012 Mar;107(1):223-4. doi: 10.1007/s11060-011-0724-0. https://doi.org/10.14412/2074-2711-2018-2-76-82, T. E. Schmidt The utility of azathioprine as a cost-effective option is reinforced. Kleinschmidt-DeMasters B, West M. CLIPPERS with chronic small vessel damage more often overlap small vessel vasculitis?. 6. Simon N, Parratt J, Barnett M, et al. Brain. Search for Similar Articles 2010;133:2626–2634). Duprez T, Sindic C. Contrast-enhanced magnetic resonance imaging and perfusionweighted imaging for monitoring features in severe CLIPPERS. If you're not a subscriber, you can: You can read the full text of this article if you: Institutional members The challenging task of properly characterizing various CNS autoimmune diseases enables patients to be rapidly triaged and appropriately treated. A Randomized Clinical Trial, Systemic Sclerosis: Commonly Asked Questions by Rheumatologists, Osteonecrosis of Multiple Joints in a Patient With Limited Scleroderma/CREST Syndrome. I.M. All registration fields are required. Responsive to immunotherapy in CLIPPERS. (In Russ.) to maintaining your privacy and will not share your personal information without (In Russ.)]. 8. The item(s) has been successfully added to ", This article has been saved into your User Account, in the Favorites area, under the new folder. ScienceDirect ® is a registered trademark of Elsevier B.V. ScienceDirect ® is a registered trademark of Elsevier B.V. CLIPPERS syndrome responsive to Leflunomide: A case report. Wolters Kluwer Health, Inc. All rights reserved. Sechenov First Moscow State Medical University (Sechenov University), Ministry of Health of Russia Russian Federation, I. V. Damulin Tobin W, Guo Y, Krecke K, et al. Burdenko National Medical Research Center for Neurosurgery, Ministry of Health of Russia Russian Federation, 16, Fourth Tverskaya-Yamskaya St., Moscow 125047, K. Yu. Lefaucher N, Bouwyn J, Ahtoy P, et al. Epub 2011 Jun 7. Expanding the clinical, radiological and neuropathological phenotype of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to sreroids (CLIPPERS). 12. 2011 Aug;134 (Pt 8):e184; author reply e186. 2015;(6):15-21. I.M. Please try again soon. Brain biopsy is required in steroid-resitance patients with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to sreroids (CLIPPERS). 10. I.M. I.M. By continuing to use this website you are giving consent to cookies being used.

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