{"orpha":228157,"name":"Marburg acute multiple sclerosis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Acute multiple sclerosis, Marburg type","Acute multiple sclerosis, Marburg variant"],"definition":"Marburg acute multiple sclerosis is a rare variant of multiple sclerosis characterized by a rapidly progressive, aggressive form of multiple sclerosis with numerous large multifocal demyelinating lesions in deep white matter on cerebral MRI that usually leads to severe disability or death within weeks to months without remission. A relapsing form of multiple sclerosis is observed in surviving patients.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-228157/","orphanet_url":"https://www.orpha.net/en/disease/detail/228157","names":{"fr":{"name":"Sclérose en plaques aiguë de Marburg","synonyms":["SEP aiguë de Marburg","Sclérose de Marburg"],"definition":"Variant rare de la sclérose en plaques caractérisé par une forme agressive et rapidement progressive de la sclérose en plaques et de nombreuses lésions démyélinisantes multifocales dans la substance blanche profonde visibles à l'IRM cérébrale. Elle entraîne généralement à un handicap grave ou au décès en quelques semaines ou quelques mois, sans rémission. Une forme récurrente de la sclérose en plaques est observée chez les patients qui survivent à la maladie."},"de":{"name":"Akute multiple Sklerose Marburg","synonyms":["Marburg-Variante der akuten multiplen Sklerose","Multiple Sklerose, akute, Marburg-Variante"],"definition":"Die akute multiple Sklerose Marburg ist eine seltene Variante der Multiplen Sklerose, die sich durch eine schnell fortschreitende, aggressive Form der Multiplen Sklerose mit zahlreichen großen multifokalen demyelinisierenden Läsionen in der tiefen weißen Substanz im zerebralen MRT auszeichnet und in der Regel innerhalb von Wochen bis Monaten ohne Remission zu schwerer Behinderung oder zum Tod führt. Bei überlebenden Patienten wird eine schubweise Form der Multiplen Sklerose beobachtet."},"es":{"name":"Esclerosis múltiple aguda de Marburg","synonyms":["Esclerosis múltiple aguda tipo Marburg","Esclerosis múltiple aguda variante Marburg"],"definition":"Es una variante poco frecuente de la esclerosis múltiple caracterizada por una forma agresiva y rápidamente progresiva de esclerosis múltiple con numerosas lesiones desmielinizantes de gran tamaño multifocales en la sustancia blanca profunda en la RM cerebral. Por lo general, conduce a una discapacidad grave o muerte en semanas o meses sin remisión. En los afectados que sobreviven la enfermedad, se observa una forma recurrente de esclerosis múltiple."},"it":{"name":"Sclerosi multipla acuta di Marburg","synonyms":["Sclerosi multipla acuta, tipo Marburg","Sclerosi multipla acuta, variante Marburg"],"definition":"La sclerosi multipla acuta di Marburg è una variante rara della sclerosi multipla, aggressiva e a progressione rapida, caratterizzata da lesioni multiple demielinizzanti multifocali di grandi dimensioni, localizzate nella sostanza bianca profonda, evidenti alla RMN, che di solito esitano in una grave disabilità o nel decesso nelle settimane o nei mesi successivi all'esordio, in assenza di remissione. Nei pazienti che sopravvivono il quadro clinico è quello di una forma recidivante di sclerosi multipla."},"nl":{"name":"Acute multipele sclerose van Marburg","synonyms":["Acute multipele sclerose, Marburg-type","Acute multipele sclerose, Marburg-variant"],"definition":"Marburg acute multiple sclerose is een zeldzame variant van multiple sclerose, en wordt gekarakteriseerd door een snel progressieve, agressieve vorm van multiple sclerose die talrijke, grote multifocale demyeliniserende letsels in diepe witte stof vertoont op cerebrale MRI, en die zonder remissie meestal leidt tot ernstige invaliditeit of overlijden binnen enkele weken of maanden. Bij overlevende patiënten wordt een recidiverende vorm van multiple sclerose waargenomen."},"pt":{"name":"Esclerose múltipla aguda de Marburg"},"pl":{"name":"Postać Marburga stwardnienia rozsianego","synonyms":["Postać ostra stwardnienia rozsianego, typ Marburga","Postać ostra stwardnienia rozsianego, wariant Marburga"]},"cs":{"name":"Marburgova akutní roztroušená skleróza","synonyms":["Akutní roztroušená skleróza, Marburgova varianta","Akutní roztroušená skleróza, Marburgův typ"]},"uk":{"name":"Марбурзький гострий розсіяний склероз","synonyms":["Гострий розсіяний склероз, марбурзький тип","Гострий розсіяний склероз, марбурзький варіант"],"definition":"Marburg acute multiple sclerosis is a rare variant of multiple sclerosis characterized by a rapidly progressive, aggressive form of multiple sclerosis with numerous large multifocal demyelinating lesions in deep white matter on cerebral MRI that usually leads to severe disability or death within weeks to months without remission. A relapsing form of multiple sclerosis is observed in surviving patients."},"zh":{"name":"Marburg急性多发性硬化","synonyms":["Marburg变体型急性多发性硬化"],"definition":"马尔堡急性多发性硬化症是多发性硬化症的一种罕见变异形式，其特征是快速进展的多发性硬化症，在脑部核磁共振成像（MRI）上脑白质深部有大量多灶性脱髓鞘病变，通常在数周至数月内导致严重残疾或死亡。在存活的病人中可观察到多发性硬化症的一种复发形式。"},"tr":{"name":"Marburg akut çoklu skleroz","synonyms":["Akut çoklu skleroz, Marburg tipi","Akut çoklu skleroz, Marburg varyantı"]}},"xrefs":[{"source":"ICD-10","code":"G35","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"8A4Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"113448281"},{"source":"MONDO","code":"0016429","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4707723","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98006,"name":"Rare neurologic disease"},"parents":[{"orpha":228145,"name":"Multiple sclerosis variant","classifications":[181]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Multigenic/multifactorial"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}