{"orpha":83616,"name":"Rubella panencephalitis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare chronic encephalitis developing up to several years after congenital rubella virus infection or rubella infection in childhood, characterized by slowly progressive, wide-spread neurological symptoms, like cognitive decline, cerebellar ataxia, spasticity, and seizures, amongst others. Progredient deterioration of the neurological disease eventually leads to the death of the patient.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-83616/","orphanet_url":"https://www.orpha.net/en/disease/detail/83616","names":{"fr":{"name":"Panencéphalite rubéoleuse","definition":"Encéphalite chronique rare se développant jusqu'à plusieurs années après une infection congénitale par le virus de la rubéole ou par une infection par le virus de la rubéole dans l'enfance, caractérisée par un large spectre de symptômes neurologiques, lentement progressifs, à savoir déclin cognitif, ataxie cérébelleuse, spasticité et convulsions, entre autres. L'évolution de la maladie neurodégénérative conduit in fine au décès du patient."},"de":{"name":"Röteln-Panenzephalitis","synonyms":["Rubella-Panenzephalitis"],"definition":"Eine chronische Enzephalitis, die sich bis zu mehreren Jahren nach einer kongenitalen Rötelnvirusinfektion oder einer Rötelninfektion im Kindesalter entwickelt und durch langsam fortschreitende, weit verbreitete neurologische Symptome wie u. a. kognitiven Verfall, zerebelläre Ataxie, Spastizität und Krampfanfälle gekennzeichnet ist. Die fortschreitende Verschlechterung der neurologischen Erkrankung führt schließlich zum Tod des Patienten."},"es":{"name":"Panencefalitis por rubéola","definition":"Es una encefalitis crónica poco frecuente que se desarrolla hasta varios años después de la infección congénita por el virus de la rubéola o de la infección por rubéola en la infancia. Está caracterizada por síntomas neurológicos generalizados lentamente progresivos, tales como deterioro cognitivo, ataxia cerebelosa, espasticidad y crisis epilépticas, entre otros. El deterioro neurológico progresivo conlleva con frecuencia el fallecimiento del paciente."},"it":{"name":"Panencefalite da rosolia","definition":"Si tratta di un'encefalite cronica rara, che si sviluppa anche a distanza di alcuni anni dall'infezione congenita del virus della rosolia o dall'infezione da rosolia contratta durante l'infanzia. È caratterizzata da sintomi neurologici diffusi a progressione lenta, che comprendono il declino cognitivo, l'atassia cerebellare, la spasticità e le crisi epilettiche. Il deterioramento neurologico progressivo esita nella morte del paziente."},"nl":{"name":"Rubella-panencefalitis","definition":"Een zeldzame, chronische encefalitis die zich tot enkele jaren na congenitale infectie met rubellavirus of infectie met rubellavirus in de kindertijd ontwikkelt, en die gekarakteriseerd wordt door traag progressieve, wijdverspreide neurologische symptomen, zoals onder meer cognitieve aftakeling, cerebellaire ataxie, spasticiteit, en insulten. Progressieve achteruitgang door de neurologische ziekte leidt uiteindelijk tot overlijden van de patiënt."},"pt":{"name":"Panencefalite por rubéola"},"pl":{"name":"Różyczkowe zapalenie mózgu"},"cs":{"name":"Zarděnková panencefalitida"},"uk":{"name":"Краснушний паненцефаліт","definition":"A rare chronic encephalitis developing up to several years after congenital rubella virus infection or rubella infection in childhood, characterized by slowly progressive, wide-spread neurological symptoms, like cognitive decline, cerebellar ataxia, spasticity, and seizures, amongst others. Progredient deterioration of the neurological disease eventually leads to the death of the patient."},"zh":{"name":"风疹性全脑炎"},"tr":{"name":"Kızamıkçık panensefaliti"}},"xrefs":[{"source":"ICD-10","code":"B06.0+","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"G05.1*","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"1F02.0","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1261684403"},{"source":"MONDO","code":"19386","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0238099","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":68416,"name":"Rare infectious disease"},"parents":[{"orpha":98255,"name":"Chronic encephalitis","classifications":[181]},{"orpha":166490,"name":"Infectious disease with epilepsy","classifications":[181]},{"orpha":98252,"name":"Infectious encephalitis","classifications":[181,203]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Childhood"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":20.0,"area":"Worldwide","status":"Validated","source":"9103895[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}