2020-10-26T15:52:02-07:00 higgs-boson@gmail.com. Diagnostic criteria for chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). q CLIPPERS is a newly described pontine‐centric inflammatory disorder with distinct clinical and radiological features. Enter and update disclosures at http://submit.nn.neurology.org. 10 0 0 10 82 280.99997 Tm (a) Initial MRI shows foci of gadolinium enhancement with a punctate and curvilinear pattern predominantly in the pons, the cerebellar peduncles and the mesencephalon.
7 has highlighted core features of CLIPPERS including clinical (I), radiological (II), GCS response (III) and histopathological (IV) criteria (Table 3). Among 12 patients, 42 relapses were analyzed. /T1_2 1 Tf CLIPPERS was initially described by Dr. Pittock and colleagues in 2010, based on a case series of 8 patients with similar clinical, radiological, and pathological features and a robust response to corticosteroid treatment (Pittock et al 2010). The enhancement corresponds to the lymphocytic perivascular inflammatory pattern seen histologically and described later in this review.
Anthony Reder: critical revision of the manuscript for intellectual content.
An overview on clinical characteristics described in CLIPPERS patients is shown in Table. The appearance of CLIPPERS on MRI is fairly unique, characterised by multiple punctate, patchy and linear regions of contrast enhancement relatively confined to the pons 1. <>
Pathogenesis of CLIPPERS remains poorly understood, and the nosological position of CLIPPERS has still to be established.
Please enable it to take advantage of the complete set of features! In other series 2, 7, 10 and case reports no increased ACE levels could be found. <>stream 2017 Sep 1;140(9):2415-2425. doi: 10.1093/brain/awx200. A case of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) without relapse after early steroid treatment早期ステロイド・パルス治療により寛解に至り,後療法なしで1年間再発を認めなかったchronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids(CLIPPERS)の37歳男性例. Slightly elevated autoimmune antibodies (elevated ANA, dsDNA or SSB/SSA antibodies): in several cases somewhat elevated anti‐nuclear 1, 7, 17, 26, double‐stranded DNA 17 and Sjögren's syndrome antigen A (SSA) 7 or B (SSB) 1 antibodies were observed. Before the condition was described originally and termed ‘CLIPPERS’ in 2010 1, ‘real’ cases of CLIPPERS may have resulted in being diagnosed as diseases that fitted the given clinico‐radiologial constellations most clearly (e.g. Journal of Neuropathology & Experimental Neurology. ( )Tj 's series also demonstrated neuro‐axonal injury, possibly explaining atrophy and disability that may develop over time 7. Scott Med J.
Med. 72 173 449 56 re (http://nn.neurology.org/misc/about.xhtml#permissions)Tj 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. The disorder usually readily responds to immunosuppression with glucocorticosteroids. Based on previous reports in literature - currently encompassing more than 50 reported cases of CLIPPERS - this review addresses clinical features, diagnostic criteria, differential diagnoses and therapeutic management of this peculiar disorder. Unable to process the form. endobj This site needs JavaScript to work properly. It seems noteworthy, however, that some immunosuppressive agents, given alone without sustained GCS therapy, are obviously not capable of maintaining remission and therefore cannot replace GCS completely. Number of times cited according to CrossRef: Síndrome CLIPPERS. Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). 0 1.00001 TD Clinical and Experimental Immunology 2014, 175: 419–24. Indeed, a few cases in the previous literature have been reported as ‘Bickerstaff encephalitis’ that appear, from the clinical features and images presented, at least partially reminiscent of CLIPPERS 46, 47. ADVERTISEMENT: Radiopaedia is free thanks to our supporters and advertisers. Conclusions: Khanduri S, Yadav H, Pandey N, Krishnam A, Nigam S. Cureus. (Subspecialty Collections)Tj | (Information about reproducing this article in parts \(figures,tables\) o\ r in)Tj MRI revealed numerous punctate, contrast-enhancing T1 lesions in the pons, midbrain, medulla, and cerebellum, as well as the cervical and thoracic spine.
As withdrawal of GCS treatment results commonly in disease exacerbation, long-term immunosuppressive therapy appears to be mandatory for sustained improvement. With the use of GCS, patients usually show early and marked clinical improvement within days, although in many cases the restitution may remain incomplete. Find NCBI SARS-CoV-2 literature, sequence, and clinical content: https://www.ncbi.nlm.nih.gov/sars-cov-2/. Rempe T, Becktepe JS, Metz I, Brück W, Stürner KH, Deuschl G, Berg D, Baron R, Zeuner R, Leypoldt F. Neurol Neuroimmunol Neuroinflamm. Stay timely. 3.0T MR investigation of CLIPPERS: role of susceptibility weighted and perfusion weighted imaging. 2 0 obj B cells and plasma cells are found in lower numbers; neutrophils and eosinophils are normally absent. GCS therapy failure seems to be a very strong indicator for an alternative diagnosis and raises the possibility of a tumour or neuronal degeneration, Unusual clinical findings such as fever, marked B symptoms, extracerebral organ manifestations (such as arthritis, uveitis, sicca syndrome, lymphadenopathy, etc.) She had partial left abducens palsy, right-sided pronator drift, numbness, dysmetria, and dysdiadochokinesia. endobj
Notfälle mit Bewusstseinsstörungen und Koma. 1, who treated 50% of patients without biopsy. Nancy Arndt: acquisition of data.
0 0 1 rg Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids, with Cranial and Caudal Extension. SLE, rheumatoid arthritis), infections (e.g. /T1_0 1 Tf Case 3: with extensive spinal cord involvement, acute disseminated encephalomyelitis (ADEM), long tract signs (e.g. CLIPPERS was diagnosed eventually, at a centre with significant experience and expertise.) MRI findings in CLIPPERS show a very striking and characteristic lesion pattern and a high degree of similarity among affected individuals.
(\240 )Tj Seropositive autoimmune encephalitides (e.g. BT -20.00098 0 Td Two New Cases and Literature Review of CLIPPERS Syndrome with Long-Term Follow-up. Find NCBI SARS-CoV-2 literature, sequence, and clinical content: https://www.ncbi.nlm.nih.gov/sars-cov-2/. Clin. Intern. (C) Posttreatment T1-weighted MRI shows resolution of previously enhancing areas of the brainstem. A case report, A new defined entity among the spectrum of central nervous system inflammation: chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS), CLIPPERS‐like MRI findings in a patient with multiple sclerosis, Cutting‐edge questions about CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids), Serial stereotactic biopsy of brainstem lesions in adults improves diagnostic accuracy compared with MRI only, Brain biopsy in cryptogenic neurological disease, Pathophysiology of CLIPPERS still unclear, A mysterious case: chronic lymphocytic inflammation with pontocerebellar perivascular enhancement responsive to steroids (CLIPPERS) syndrome (Meeting abstract 364, American Federation for Medical Research and Participating Societies Regional Meetings: Southern Regional Program Abstracts, 2013), Requirement for safety monitoring for approved multiple sclerosis therapies: an overview, Monoclonal antibodies in treatment of multiple sclerosis, Disease‐modifying therapy in multiple sclerosis and chronic inflammatory demyelinating polyradiculoneuropathy: common and divergent current and future strategies.
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