{"orpha":284448,"name":"CLIPPERS","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids"],"definition":"CLIPPERS is a rare neuroinflammatory disorder characterized by brainstem-predominant encephalomyelitis which typically presents with cerebellar and cranial nerve manifestations (gait ataxia, dysarthria, visual disorders, parasthesias), as well as brainstem, myelopathy and cognitive findings, that respond to steroid treatment. Punctate curvilinear post-gadolinium contrast enhancement predominantly in the pons and cerebellum is observed on brain MRI and prominent, perivascular, CD3+ T-cell predominantly lymphocytic inflammation in neuropathology.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-284448/","orphanet_url":"https://www.orpha.net/en/disease/detail/284448","names":{"fr":{"name":"CLIPPERS","synonyms":["Inflammation chronique lymphocytaire avec rehaussement périvasculaire pontique cortico-sensible","Inflammation chronique lymphocytaire avec rehaussement périvasculaire pontique corticosensible"],"definition":"Maladie neuro-inflammatoire rare caractérisée par une encéphalomyélite affectant principalement le tronc cérébral, se déclarant généralement par des signes cliniques d'atteinte du cervelet et des nerfs crâniens (démarche ataxique, dysarthrie, troubles visuels, paresthésies), ainsi que du tronc cérébral, associés à des symptômes cognitifs évocateurs d'une myélopathie, qui répondent au traitement par stéroïdes. L'IRM cérébrale révèle un rehaussement ponctué et curviligne de contraste au gadolinium, principalement dans le pons et le cervelet, tandis que l'examen histologique révèle une inflammation lymphocytaire d'architecture périvasculaire à prédominance de lymphocytes T CD3+."},"de":{"name":"CLIPPERS"},"es":{"name":"CLIPPERS","synonyms":["Inflamación linfocítica crónica con mejora perivascular pontina sensible a esteroides"],"definition":"CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) es un trastorno neuroinflamatorio poco frecuente, caracterizado por encefalomielitis de predominio en el tronco encefálico. Por lo general, se presenta con manifestaciones cerebelosas y de pares craneales (marcha atáxica, disartria, trastornos visuales, parestesias), así como signos de tronco cerebral, mielopatía y cognitivos. Responde al tratamiento con esteroides. En la RM cerebral tras administración de gadolinio se observa captación de contraste puntiforme curvilíneo predominantemente en la protuberancia cerebral y el cerebelo, así como inflamación linfocitaria perivascular con predominio de células T CD3+ patente en el estudio neuropatológico."},"it":{"name":"CLIPPERS","synonyms":["Infiammazione linfocitica cronica con rialzamento perivascolare pontino sensibile agli steroidi"],"definition":"La CLIPPERS è una malattia neuro-infiammatoria rara, caratterizzata da un'encefalomielite, che interessa prevalentemente il tronco encefalico, e di solito esordisce con sintomi clinici cerebellari e a livello dei nervi cranici (andatura atassica, disartria, disturbi della vista, parestesie) e del tronco encefalico, associati a sintomi cognitivi suggestivi di una mielopatia, che rispondono al trattamento con steroidi. La RMN evidenzia un rinforzo puntato curvilineo dopo la somministrazione di un mezzo di contrasto contenente gadolinio, prevalentemente nel ponte e nel cervelletto, mentre l'esame istologico evidenzia una marcata infiammazione linfocitaria perivascolare dei linfociti T CD3+."},"nl":{"name":"CLIPPERS","synonyms":["Chronische lymfocytische ontsteking met pontiene perivasculaire verbetering als respons op steroïden"],"definition":"CLIPPERS is een zeldzame neuro-inflammatoire stoornis, en wordt gekarakteriseerd door encefalomyelitis van voornamelijk de hersenstam die zich doorgaans presenteert met manifestaties aan cerebellum en hersenzenuwen (gangataxie, dysartrie, visuele stoornissen, paresthesieën), alsook bevindingen van de hersenstam, myelopathie en cognitieve bevindingen die reageren op behandeling met steroïden. Puntvormige curvilineaire contrastversterking na toediening van gadolinium wordt voornamelijk in de pons en het cerebellum waargenomen bij MRI van de hersenen, en neuropathologie toont uitgesproken perivasculaire, voornamelijk CD3+ T-cel lymfocytaire inflammatie."},"pt":{"name":"CLIPPERS"},"pl":{"name":"CLIPPERS","synonyms":["Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids"]},"cs":{"name":"CLIPPERS","synonyms":["Chronický lymfocytární zánět s pontinním perivaskulárním zvýrazněním, odpovídající na steroidy"]},"uk":{"name":"CLIPPERS","synonyms":["Хронічне лімфоцитарне запалення з понтинним периваскулярним посиленням, що реагує на стероїди"],"definition":"CLIPPERS is a rare neuroinflammatory disorder characterized by brainstem-predominant encephalomyelitis which typically presents with cerebellar and cranial nerve manifestations (gait ataxia, dysarthria, visual disorders, parasthesias), as well as brainstem, myelopathy and cognitive findings, that respond to steroid treatment. Punctate curvilinear post-gadolinium contrast enhancement predominantly in the pons and cerebellum is observed on brain MRI and prominent, perivascular, CD3+ T-cell predominantly lymphocytic inflammation in neuropathology."},"zh":{"name":"慢性淋巴细胞性炎症伴脑桥血管周围强化对类固醇的反应","synonyms":["慢性淋巴细胞性炎症伴脑桥周围血管类固醇敏感"],"definition":"CLIPPERS是一种罕见的神经炎症，其特征是主要累及脑干的脑脊髓炎，通常有小脑和颅神经表现（步态共济失调，构音障碍，视觉障碍，感觉异常），以及对类固醇治疗有效的脑干、脊髓病和认知障碍。在脑MRI上可见脑桥和小脑的点状曲线增强，在神经病理学中可见血管周围以CD3+T细胞为主的淋巴细胞炎症。"},"tr":{"name":"CLIPPERS","synonyms":["Steroidlere yanıt veren pontin perivasküler gelişim ile seyreden kronik lenfositik inflamasyon"]}},"xrefs":[{"source":"ICD-10","code":"G04.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"8E4A.0","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"913443626"},{"source":"MONDO","code":"0017297","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C3854437","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"10779","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10075197","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98006,"name":"Rare neurologic disease"},"parents":[{"orpha":182064,"name":"Rare neuroinflammatory or neuroimmunological disease","classifications":[181]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":50.0,"area":"Worldwide","status":"Validated","source":"24028073[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}