{"orpha":228165,"name":"Baló concentric sclerosis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Concentric demyelination"],"definition":"A rare multiple sclerosis variant characterized by discrete concentrically layered, ring-like lesions in the cerebral white matter, consisting of alternating layers of myelinated and demyelinated tissue. Patients most commonly present with symptoms of an intracerebral mass lesion, including headache, cognitive abnormalities, behavioral changes, seizures, aphasia, or hemiparesis, among others, although there may also be classic focal symptoms of multiple sclerosis, such as focal weakness, ataxia, sensory disturbance, or diplopia.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-228165/","orphanet_url":"https://www.orpha.net/en/disease/detail/228165","names":{"fr":{"name":"Sclérose concentrique de Baló","synonyms":["Démyélinisation concentrique"],"definition":"Variante rare de la sclérose en plaques caractérisée par des lésions discrètes, disposées en couches concentriques dans la substance blanche cérébrale, alternant des tissus myélinisés et démyélinisés. Les symptômes les plus fréquents sont ceux d'une lésion tumorale intracérébrale, notamment des céphalées, des troubles cognitifs, des changements de comportement, des crises convulsives, une aphasie ou une hémiparésie, mais aussi les symptômes focaux, typiques de la sclérose en plaques, tels qu'une faiblesse focale, une ataxie, des troubles sensoriels ou une diplopie."},"de":{"name":"Konzentrische Sklerose Baló","synonyms":["Baló-Krankheit","Demyelinisierung, konzentrische"],"definition":"Eine seltene Variante der Multiplen Sklerose, die durch diskrete, konzentrisch geschichtete, ringförmige Läsionen in der weißen Hirnsubstanz gekennzeichnet ist, die aus abwechselnden Schichten von myelinisiertem und demyelinisiertem Gewebe bestehen. Die Patienten präsentieren sich meist mit Symptomen einer intrazerebralen Gewebeverdichtung, darunter Kopfschmerzen, kognitive Störungen, Verhaltensänderungen, Krampfanfälle, Aphasie oder Hemiparese, obwohl auch klassische fokale Symptome der Multiplen Sklerose wie fokale Schwäche, Ataxie, Sensibilitätsstörungen oder Diplopie auftreten können."},"es":{"name":"Esclerosis concéntrica de Baló","synonyms":["Desmielinización concéntrica"],"definition":"Es una variante poco frecuente de la esclerosis múltiple caracterizada por lesiones en forma de anillo en capas concéntricas en la sustancia blanca cerebral, consistentes en capas alternas de tejido mielinizado y desmielinizado. Los pacientes se presentan, por lo general, con síntomas de una lesión de masa intracerebral, incluyendo cefalea, alteraciones cognitivas, trastornos de conducta, crisis epilépticas, afasia o hemiparesia, entre otros, aunque también pueden experimentar síntomas focales clásicos de la esclerosis múltiple, tales como debilidad focal, ataxia, trastornos sensitivos o diplopía."},"it":{"name":"Sclerosi concentrica di Baló","synonyms":["Demielinizzazione concentrica"],"definition":"Si tratta di una variante rara della sclerosi multipla, caratterizzata da lesioni discrete simili ad anelli, disposte a strati concentrici, localizzate nella sostanza bianca cerebrale, composte da strati alternati di tessuto mielinizzato e di tessuto demielinizzato. Di solito i pazienti presentano sintomi secondari alla presenza di una massa intracerebrale, che comprendono la cefalea, le anomalie cognitive, le alterazioni del comportamento, le crisi epilettiche, l'afasia e l'emiparesi, che possono associarsi ai sintomi focali classici della sclerosi multipla, compresa la debolezza focale, l'atassia, i deficit sensoriali e la diplopia."},"nl":{"name":"Concentrische sclerose van Baló","synonyms":["Concentrische demyelinisatie"],"definition":"Een zeldzame variant van multipele sclerose, gekarakteriseerd door discrete, concentrisch gelaagde, ringvormige laesies in cerebrale stof, bestaande uit afwisselende lagen van gemyeliniseerd en gedemyeliniseerd weefsel. Doorgaans presenteren patiënten zich met symptomen van een intracerebrale massa, waaronder hoofdpijn, cognitieve anomalieën, gedragsveranderingen, insulten, afasie, en hemiparese, hoewel klassieke focale symptomen van multipele sclerose ook mogelijk zijn, zoals focale zwakte, ataxie, sensorische stoornissen, of diplopie."},"pt":{"name":"Esclerose concêntrica de Balò"},"pl":{"name":"Stwardnienie koncentryczne Baló","synonyms":["Demielinizacja koncentryczna"]},"cs":{"name":"Balóova koncentrická skleróza","synonyms":["Koncentrická demyelinizace"]},"uk":{"name":"Концентричний склероз Бало","synonyms":["Концентрична демієлінізація"],"definition":"A rare multiple sclerosis variant characterized by discrete concentrically layered, ring-like lesions in the cerebral white matter, consisting of alternating layers of myelinated and demyelinated tissue. Patients most commonly present with symptoms of an intracerebral mass lesion, including headache, cognitive abnormalities, behavioral changes, seizures, aphasia, or hemiparesis, among others, although there may also be classic focal symptoms of multiple sclerosis, such as focal weakness, ataxia, sensory disturbance, or diplopia."},"zh":{"name":"同心圆性硬化","synonyms":["同心圆性脱髓鞘"],"definition":"多发性硬化症的一种罕见变异形式，其特征是大脑白质内散在的同心圆层状病变，由有髓组织层和脱髓鞘组织层交替组成。患者常表现出由脑内肿块引起的症状，包括头痛、认知异常、行为改变、癫痫、失语症或偏瘫等，但也可能有典型的多发性硬化症的局灶性症状，如局灶性无力、共济失调、感觉障碍或复视。"},"tr":{"name":"Balo'nun konsantrik skleroz","synonyms":["Konsantrik demiyelinizasyon"]}},"xrefs":[{"source":"ICD-10","code":"G37.5","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"8A40.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1387119224"},{"source":"MONDO","code":"0016430","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0004712","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"5885","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10010252","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}