{"orpha":83600,"name":"Encephalitis lethargica","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Von Economo encephalitis"],"definition":"A rare brain inflammatory disease characterized by acute or subacute encephalitis with involvement of the midbrain and basal ganglia occurring in children as well as adults. Initial symptoms are pharyngitis and fever, followed by progressive lethargy, sleep disturbances, extrapyramidal symptoms (parkinsonism, chorea, dystonia), neuropsychiatric manifestations (obsessive-compulsive behavior, mutism, catatonia), and ocular features (oculogyric crises). Autoantibodies against human basal ganglia are often positive. Survivors may develop post-encephalitic syndromes, most prominently parkinsonism.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-83600/","orphanet_url":"https://www.orpha.net/en/disease/detail/83600","names":{"fr":{"name":"Encéphalite léthargique","synonyms":["Encéphalite de von Economo"],"definition":"Maladie neuro-inflammatoire rare caractérisée par une encéphalite aiguë ou subaiguë avec une atteinte du mésencéphale et des ganglions de la base, survenant aussi bien chez les enfants que chez les adultes. Les premiers signes cliniques sont une pharyngite et une fièvre, suivies d'une léthargie progressive, de troubles du sommeil, d'une atteinte du système extrapyramidal (parkinsonisme, chorée, dystonie), des atteintes neuropsychiatriques (comportement obsessionnel-compulsif, mutisme, catatonie) et des troubles oculaires (crises oculogyres). Les auto-anticorps dirigés contre les cellules de ganglions de la base sont souvent positifs. Les patients qui recouvrent la santé peuvent développer des syndromes post-encéphaliques, notamment le parkinsonisme."},"de":{"name":"Encephalitis lethargica","synonyms":["Von Economo-Enzephalitis"],"definition":"Eine seltene entzündliche Erkrankung des Gehirns, die durch eine akute oder subakute Enzephalitis mit Beteiligung des Mittelhirns und der Basalganglien gekennzeichnet ist und sowohl bei Kindern als auch bei Erwachsenen auftritt. Erste Symptome sind Pharyngitis und Fieber, gefolgt von fortschreitender Lethargie, Schlafstörungen, extrapyramidalen Symptomen (Parkinsonismus, Chorea, Dystonie), neuropsychiatrischen Manifestationen (zwanghaftes Verhalten, Mutismus, Katatonie) und okulären Erscheinungen (okulogyrische Krisen). Autoantikörper gegen menschliche Basalganglien sind häufig positiv. Überlebende können post-enzephalitische Syndrome entwickeln, vor allem Parkinsonismus."},"es":{"name":"Encefalitis letárgica","synonyms":["Encefalitis de Von Economo"],"definition":"Es una enfermedad inflamatoria del cerebro poco frecuente caracterizada por una encefalitis aguda o subaguda con afectación del mesencéfalo y de los ganglios basales que se presenta tanto en niños como en adultos. Los síntomas iniciales son faringitis y fiebre, seguidos de letargia progresiva, trastornos del sueño, síntomas extrapiramidales (parkinsonismo, corea, distonía), manifestaciones neuropsiquiátricas (comportamiento obsesivo compulsivo, mutismo, catatonia) y hallazgos oculares (crisis oculógiras). Con frecuencia se detectan autoanticuerpos contra los ganglios basales humanos. Los pacientes que sobreviven pueden desarrollar síndrome postencefalítico, principalmente parkinsonismo."},"it":{"name":"Encefalite letargica","synonyms":["Encefalite di von Economo"],"definition":"Si tratta di una malattia infiammatoria cerebrale rara, caratterizzata da encefalite acuta o subacuta con interessamento del mesencefalo e dei gangli basali, che colpisce sia i bambini che gli adulti. I sintomi d'esordio comprendono la faringite e la febbre, seguite dalla letargia progressiva, dai disturbi del sonno, dai sintomi extrapiramidali (parkinsonismo, corea, distonia), dai sintomi neuropsichiatrici (disturbo ossessivo-compulsivo, mutismo, catatonia) e dai segni oculari (crisi oculogire). Spesso i pazienti sono positivi per gli autoanticorpi diretti contro i gangli basali. I pazienti che sopravvivono alla malattia possono sviluppare sindromi post-encefalitiche, in particolare il parkinsonismo."},"nl":{"name":"Encephalitis lethargica","synonyms":["Encefalitis, von Economo-type"],"definition":"Een zeldzame inflammatoire aandoening van hersenen, gekarakteriseerd door acute of subacute encefalitis met betrokkenheid van middenhersenen en basale ganglia die voorkomt bij zowel kinderen als volwassenen. Initiële symptomen zijn faryngitis en koorts, gevolgd door progressieve lethargie, slaapstoornissen, extrapiramidale symptomen (parkinsonisme, chorea, dystonie), neuropsychiatrische manifestaties (obsessief-compulsief gedrag (dwanghandelen), mutisme, katatonie), en oculaire kenmerken (oculogyrische crisissen). Auto-antilichamen tegen humane basale ganglia zijn vaak positief. Overlevers ontwikkelen mogelijk postencefalitische syndromen, vooral parkinsonisme."},"pt":{"name":"Encefalite letárgica"},"pl":{"name":"Śpiączkowe zapalenie mózgu","synonyms":["Zapalenie mózgu Von Economo"]},"cs":{"name":"Encephalitis lethargica","synonyms":["Von Economova encefalitida","Ekonomova encefalitida"]},"uk":{"name":"Летаргічний енцефаліт","synonyms":["Енцефаліт фон Економо"],"definition":"A rare brain inflammatory disease characterized by acute or subacute encephalitis with involvement of the midbrain and basal ganglia occurring in children as well as adults. Initial symptoms are pharyngitis and fever, followed by progressive lethargy, sleep disturbances, extrapyramidal symptoms (parkinsonism, chorea, dystonia), neuropsychiatric manifestations (obsessive-compulsive behavior, mutism, catatonia), and ocular features (oculogyric crises). Autoantibodies against human basal ganglia are often positive. Survivors may develop post-encephalitic syndromes, most prominently parkinsonism."},"zh":{"name":"昏睡性脑炎","synonyms":["埃科诺莫脑炎"]},"tr":{"name":"Ensefalit letarjika","synonyms":["Von Economo ensefaliti"]}},"xrefs":[{"source":"ICD-10","code":"A85.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"1C80","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1777779617"},{"source":"MONDO","code":"0019384","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0014040","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"6332","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10052369","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":306666,"name":"Rare parkinsonian syndrome due to neurodegenerative disease","classifications":[181]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Europe","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2018-03-24","source":"14570817[PMID]_10563641[PMID]_25129855[PMID]_3801849[PMID]_6126720[PMID]_6039103[PMID]","terms":[{"hpo":"HP:0001298","label":"Encephalopathy","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002360","label":"Sleep abnormality","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012547","label":"Abnormal involuntary eye movements","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002960","label":"Autoimmunity","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0007146","label":"Bilateral basal ganglia lesions","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000651","label":"Diplopia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100660","label":"Dyskinesia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002315","label":"Headache","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002922","label":"Increased CSF protein concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0010702","label":"Increased circulating immunoglobulin concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001254","label":"Lethargy","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0005986","label":"Limitation of neck motion","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001268","label":"Mental deterioration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003326","label":"Myalgia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001300","label":"Parkinsonism","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0025439","label":"Pharyngitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0004429","label":"Recurrent viral infections","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001337","label":"Tremor","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003484","label":"Upper limb muscle weakness","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002607","label":"Bowel incontinence","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001662","label":"Bradycardia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001259","label":"Coma","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002883","label":"Hyperventilation","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0009763","label":"Limb pain","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000613","label":"Photophobia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000709","label":"Psychosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001250","label":"Seizure","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0025258","label":"Stiff neck","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000020","label":"Urinary incontinence","frequency":"Occasional (29-5%)","criteria":null}]}}