{"orpha":163921,"name":"Posttransplant acute limbic encephalitis","type":"Particular clinical situation in a disease or syndrome","group":"Disorder","status":"active","flags":[],"synonyms":["PALE"],"definition":"Posttransplant acute limbic encephalitis is a rare, acquired, non-paraneoplastic limbic encephalitis disorder, that develops in the setting of treatment-related immunosuppression, typically after allogeneic hemapoietic stem cell transplantation, characterized by onset of confusion, headache, anterograde amnesia, seizures and/or loss of consciousness 2-6 weeks following transplantation. Bilateral, non-enhancing T2 hyperintensities in limbic structures are observed on magnetic resonance imaging. Mild cerebrospinal fluid pleocytosis and syndrome of inappropriate antidiuretic hormone secretion may also be associated.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-163921/","orphanet_url":"https://www.orpha.net/en/disease/detail/163921","names":{"fr":{"name":"Encéphalite limbique aiguë post-transplantation","synonyms":["PALE"],"definition":"Encéphalite limbique rare, acquise et non paranéoplasique, qui se développe dans le cadre d'une immunosuppression liée au traitement, généralement après une greffe allogénique de cellules souches hématopoiétiques, caractérisée par l'apparition d'une confusion, de céphalées, d'une amnésie antérograde, de convulsions et/ou d'une perte de conscience survenant entre 2 et 6 semaines après la greffe. Les images pondérées en T2 de l'imagerie par résonance magnétique (IRM) montrent des lésions hyperintenses bilatérales non renforcées dans les structures du système limbique. Une légère pléocytose du liquide céphalorachidien et un syndrome de sécrétion inappropriée d'hormone anti-diurétique peuvent également être associés."},"de":{"name":"Limbische Enzephalitis, akute, nach Transplantation","synonyms":["PALE"]},"es":{"name":"Encefalitis límbica aguda postrasplante","synonyms":["PALE"],"definition":"La encefalitis límbica aguda postrasplante, es un trastorno adquirido poco frecuente de encefalitis límbica, no paraneoplásica, que se origina tras la inmunosupresión terapéutica después de un trasplante alogénico de células madre hematopoyéticas. Está caracterizado por el inicio de confusión, cefalea, amnesia anterógrada, convulsiones y/o pérdida de conciencia entre 2 y 6 semanas después del trasplante. En las imágenes de resonancia magnética se observan hiperintensidades no potenciadas en T2 en estructuras límbicas bilaterales. También se puede asociar pleocitosis leve en el líquido cefalorraquídeo y síndrome de secreción inadecuada de hormona antidiurética."},"it":{"name":"Encefalite limbica acuta post-trapianto","definition":"L'encefalite limbica acuta post-trapianto è un'encefalite limbica rara, non paraneoplastica, acquisita, che insorge nel quadro di un'immunosoppressione correlata al trattamento, di solito dopo un trapianto allogenico di cellule staminali ematopoietiche. È caratterizzata da confusione, cefalea, amnesia anterograda, crisi epilettiche e/o perdita di coscienza tre le 2 e le 6 settimane dopo il trapianto. La risonanza magnetica cerebrale evidenzia iperintensità bilaterali senza rinforzo in T2 nelle strutture limbiche. I pazienti possono presentare una pleocitosi lieve del liquido cerebrospinale e la sindrome da inappropriata secrezione dell'ormone antidiuretico."},"nl":{"name":"Posttransplantie acute limbische encefalitis","synonyms":["PALE"],"definition":"Posttransplantatie acute limbische encefalitis is een zeldzame, verworven, niet-paraneoplastische limbische encefalitis die zich ontwikkelt in de context van behandelingsgerelateerde immuunsuppressie, doorgaans na allogene hematopoëtische stamceltransplantatie, en die gekarakteriseerd wordt door aanvang van verwardheid, hoofdpijn, anterograde amnesie, insulten en/of bewustzijnsverlies 2-6 weken na de transplantatie. Bij beeldvorming met magnetische resonantie worden bilaterale, niet-versterkte T2-hyperintensiteiten in limbische structuren waargenomen. Milde pleocytose in het cerebrospinaal vocht en syndroom met ongepaste secretie van antidiuretisch hormoon kunnen ook geassocieerd zijn."},"pt":{"name":"Encefalite límbica aguda pós-tranplante"},"pl":{"name":"Potransplantacyjne ostre zapalenie układu limbicznego","synonyms":["PALE"],"definition":"*Potransplantacyjne ostre zapalenie układu limbicznego jest rzadkim, nabytym, nienowotworowym zapaleniem układu limbicznego, które rozwija się w przebiegu leczenia immunosupresyjnego, typowo po allogenicznym przeszczepie hematopoetycznych komórek macierzystych. Objawia się splątaniem, bólem głowy, amnezją następczą, drgawkami i/lub utratą przytomności, które występują 2-6 tygodni po przeszczepie. W obrazowaniu metodą rezonansu magnetycznego (MRI) w obrazach T2-zależnych widoczne są obustronne, nie ulegające wzmocnieniu hiperintensywne zmiany w strukturach układu limbicznego. Może również towarzyszyć łagodna pleocytoza płynu mózgowo-rdzeniowego i zespół nieprawidłowego wydzielania hormonu antydiuretycznego."},"cs":{"name":"Posttransplantační akutní limbická encefalitida","synonyms":["PALE"]},"uk":{"name":"Посттрансплантаційний гострий лімбічний енцефаліт","synonyms":["PALE"],"definition":"Posttransplant acute limbic encephalitis is a rare, acquired, non-paraneoplastic limbic encephalitis disorder, that develops in the setting of treatment-related immunosuppression, typically after allogeneic hemapoietic stem cell transplantation, characterized by onset of confusion, headache, anterograde amnesia, seizures and/or loss of consciousness 2-6 weeks following transplantation. Bilateral, non-enhancing T2 hyperintensities in limbic structures are observed on magnetic resonance imaging. Mild cerebrospinal fluid pleocytosis and syndrome of inappropriate antidiuretic hormone secretion may also be associated."},"zh":{"name":"移植后急性边缘脑炎","synonyms":["PALE"],"definition":"移植后急性边缘叶脑炎是一种罕见的获得性非副肿瘤性边缘叶脑炎疾病，发生于治疗相关性免疫抑制的情况下，通常在异体造血干细胞移植后发生，其特征是移植后2-6周出现意识模糊、头痛、顺行性遗忘、惊厥和/或意识丧失。在磁共振成像上可观察到边缘结构的双侧非强化T2高信号。也可能伴有轻度脑脊液细胞增多症和抗利尿激素分泌失调综合征。"},"tr":{"name":"Nakil sonrası akut limbik ensefalit","synonyms":["SOLUK"]}},"xrefs":[{"source":"ICD-10","code":"G04.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0015595","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4750744","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98006,"name":"Rare neurologic disease"},"parents":[{"orpha":97275,"name":"Encephalitis","classifications":[181]},{"orpha":565779,"name":"Rare disorder potentially indicated for transplant or complication after transplantation","classifications":[233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2021-11-30","source":"33396564[PMID]_33548682[PMID]_30256500[PMID]","terms":[{"hpo":"HP:0200149","label":"CSF lymphocytic pleiocytosis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012756","label":"CSF polymorphonuclear pleocytosis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011185","label":"EEG with focal epileptiform discharges","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0031885","label":"Hyperglycorrhachia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002902","label":"Hyponatremia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002922","label":"Increased CSF protein concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002354","label":"Memory impairment","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001250","label":"Seizure","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100806","label":"Sepsis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0020071","label":"Viremia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012332","label":"Abnormal autonomic nervous system physiology","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0025100","label":"Abnormal hippocampus morphology","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100022","label":"Abnormality of movement","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000739","label":"Anxiety","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001251","label":"Ataxia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000708","label":"Atypical behavior","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100543","label":"Cognitive impairment","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001289","label":"Confusion","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000716","label":"Depression","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001332","label":"Dystonia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011203","label":"EEG with abnormally slow frequencies","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001336","label":"Myoclonus","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000709","label":"Psychosis","frequency":"Occasional (29-5%)","criteria":null}]}}