{"orpha":2942,"name":"Postpoliomyelitis syndrome","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Postpolio syndrome","Postpoliomyelitic syndrome"],"definition":"Postpoliomyelitis syndrome (PPS) is a neurologic disorder characterized by the development of new neuromuscular symptoms such as progressive muscular weakness or abnormal muscle fatigability occurring in survivors of the acute paralytic form of poliomyelitis, 15-40 years after recovery from the disease, and that is unexplained by other medical causes. Other manifestations that can occur gradually include generalized fatigue, muscle atrophy, muscle and joint pain, intolerance to cold, and difficulties sleeping, swallowing or breathing.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-2942/","orphanet_url":"https://www.orpha.net/en/disease/detail/2942","names":{"fr":{"name":"Syndrome post-poliomyélite","synonyms":["Syndrome post-polio"],"definition":"Le syndrome post-poliomyélitique (SPP) est un trouble neurologique non infectieux caractérisé par le développement de nouveaux symptômes neuromusculaires, tels qu'une faiblesse musculaire progressive ou une fatigabilité anormale survenant chez les survivants de la forme paralysante aigüe de la poliomyélite 15 à 40 ans après la récupération de la maladie, et sans lien avec d'autres causes médicales. D'autres manifestations pouvant survenir graduellement incluent une atrophie musculaire, des myalgies, des arthralgies, une intolérance au froid, des difficultés pour dormir, une dysphagie ou une dyspnée."},"de":{"name":"Post-Poliomyelitis-Syndrom","synonyms":["PPS","Post-Polio-Syndrom"],"definition":"Das Postpoliomyelitis-Syndrom (PPS) ist eine neurologische Erkrankung, die durch die Entwicklung neuer neuromuskulärer Symptome wie fortschreitende Muskelschwäche oder abnormale Muskelermüdbarkeit gekennzeichnet ist, die bei Überlebenden der akuten paralytischen Form der Poliomyelitis (siehe diesen Begriff) 15 bis 40 Jahre nach der Genesung von der Krankheit auftreten und nicht durch andere medizinische Ursachen erklärt werden können. Zu den weiteren Symptomen, die allmählich auftreten können, gehören allgemeine Erschöpfung, Muskelatrophie, Muskel- und Gelenkschmerzen, Kälteintoleranz und Schlaf-, Schluck- oder Atemprobleme."},"es":{"name":"Síndrome post-poliomielitis","synonyms":["Síndrome post-polio","Síndrome post-poliomielítico"],"definition":"El síndrome postpoliomielítico es un trastorno neurológico caracterizado por el desarrollo de nuevos síntomas neuromusculares tales como debilidad muscular progresiva o la propensión anómala a la fatiga muscular que se da entre los supervivientes de la forma paralítica aguda de la poliomielitis (véase este término), entre 15 y 40 años después de la curación de la enfermedad, y que no se explica por ninguna otra causa médica. Otras manifestaciones que pueden aparecer de forma gradual son la fatiga generalizada, atrofia muscular, dolor articular y muscular, intolerancia al frío, y dificultades para dormir, deglutir o respirar."},"it":{"name":"Sindrome post-poliomielite","synonyms":["Sindrome post-poliomielitica","Sindrome post-polio"],"definition":"La sindrome post-poliomielitica (PPS) è una malattia neurologica caratterizzata dallo sviluppo di nuovi sintomi neuromuscolari, come la debolezza muscolare progressiva o l'affaticamento muscolare anomalo, nei pazienti sopravvissuti alla forma paralitica acuta della poliomielite (si veda questo termine), 15-40 anni dopo la remissione dalla malattia, e che non è riconducibile ad altre cause mediche. Si possono manifestare gradualmente l'affaticamento generalizzato, l'atrofia muscolare, il dolore muscolare e articolare, l'intolleranza al freddo, i disturbi del sonno, le difficoltà nella deglutizione e nella respirazione."},"nl":{"name":"Postpoliomyelitissyndroom","synonyms":["Postpoliosyndroom"],"definition":"Een neurologische aandoening, gekarakteriseerd door ontwikkeling van nieuwe neuromusculaire symptomen zoals progressieve spierzwakte of abnormale spiermoeheid bij overlevenden van de acute paralytische vorm van poliomyelitis, 15-40 jaar na hun herstel van de ziekte, die niet kunnen verklaard worden door een andere medische oorzaak. Andere manifestaties die gradueel kunnen voorkomen, zijn onder meer gegeneraliseerde vermoeidheid, spieratrofie, spier- en gewrichtspijn, intolerantie voor koude, en moeilijkheden met slapen, slikken of ademen."},"pt":{"name":"Síndrome pós-poliomielite","synonyms":["Sequela pós-polio","Sequela pós-poliomielíte","Síndrome pós-polio","Síndrome pós-poliomielítico"]},"pl":{"name":"Zespół Post-Polio","synonyms":["Zespół poporażenny"]},"cs":{"name":"Postpoliomyelitický syndrom","synonyms":["Postpolio syndrom","Následky postpoliomyelitidy","Následky dětské obrny","Syndrom po infekční obrně"]},"uk":{"name":"Постполіомієлітний синдром","synonyms":["Наслідки постполіомієліту","Синдром постполіомієліту","Постполіомієлітичний синдром","Наслідки постполіомієліту"],"definition":"Postpoliomyelitis syndrome (PPS) is a neurologic disorder characterized by the development of new neuromuscular symptoms such as progressive muscular weakness or abnormal muscle fatigability occurring in survivors of the acute paralytic form of poliomyelitis (see this term), 15-40 years after recovery from the disease, and that is unexplained by other medical causes. Other manifestations that can occur gradually include generalized fatigue, muscle atrophy, muscle and joint pain, intolerance to cold, and difficulties sleeping, swallowing or breathing."},"zh":{"name":"脊髓灰质炎后综合征","synonyms":["脊髓灰质炎后综合征"]},"tr":{"name":"Postpoliomiyelit sendromu","synonyms":["Postpolio sekeller","Postpolio sendromu","Postpoliomiyelitik sendrom","Postpoliomiyelit sekeli"]}},"xrefs":[{"source":"ICD-10","code":"G14","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"8B62","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"2018885243"},{"source":"MONDO","code":"0017416","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D016262","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0080040","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"4454","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10036239","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98006,"name":"Rare neurologic disease"},"parents":[{"orpha":98506,"name":"Acquired motor neuron disease","classifications":[181]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adolescent","Adult","Elderly"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2018-01-15","source":"15599928[PMID]","terms":[{"hpo":"HP:0001324","label":"Muscle weakness","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012531","label":"Pain","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0011446","label":"Abnormality of higher mental function","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002829","label":"Arthralgia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003551","label":"Difficulty climbing stairs","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012378","label":"Fatigue","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100786","label":"Hypersomnia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003326","label":"Myalgia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001367","label":"Abnormal joint morphology","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001260","label":"Dysarthria","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002015","label":"Dysphagia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002380","label":"Fasciculations","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003394","label":"Muscle spasm","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002093","label":"Respiratory insufficiency","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003202","label":"Skeletal muscle atrophy","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002360","label":"Sleep abnormality","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002791","label":"Hypoventilation","frequency":"Very rare (<4-1%)","criteria":null}]}}