{"orpha":297,"name":"Tick-borne encephalitis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["TBE"],"definition":"An infectious disease characterized by central nervous system (CNS) involvement caused by the TBE virus (TBEV) and transmitted to humans principally by the bite of ticks from the <i>Ixodes</i> genus. The symptomatology is often biphasic, with the initial phase being associated with a nonspecific febrile illness and the second phase with meningitis, meningoencephalitis or meningoencephalomyelitis.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-297/","orphanet_url":"https://www.orpha.net/en/disease/detail/297","names":{"fr":{"name":"Encéphalite à tiques","synonyms":["TBE"],"definition":"Maladie infectieuse rare caractérisée par une atteinte du système nerveux central (SNC) causée par le virus TBE (tick-borne encephalitis) (TBEV pour tick-borne encephalitis virus) et transmise à l'homme principalement par la piqûre de tiques du genre <i>Ixodes</i>. L'évolution est souvent biphasique, la phase initiale étant associée à une maladie fébrile non spécifique et la seconde phase à une méningite, une méningo-encéphalite ou une méningo-encéphalomyélite."},"de":{"name":"Zeckenenzephalitis","synonyms":["Lymphadenopathie, zeckenstichassoziierte","TBE"],"definition":"Eine seltene Infektionskrankheit, die durch eine Beteiligung des zentralen Nervensystems (ZNS) gekennzeichnet ist, die durch das FSME-Virus (TBEV) verursacht und hauptsächlich durch den Stich von Zecken der Gattung <i>Ixodes</i> auf den Menschen übertragen wird. Die Symptomatik ist oft zweiphasig, wobei die erste Phase mit einer unspezifischen fieberhaften Erkrankung und die zweite Phase mit Meningitis, Meningoenzephalitis oder Meningoenzephalomyelitis einhergeht."},"es":{"name":"Encefalitis transmitida por garrapatas","synonyms":["ETG"],"definition":"Es una enfermedad infecciosa caracterizada por la afectación del sistema nervioso central (SNC) causada por el virus TBE (TBEV) y transmitida a los humanos principalmente por la picadura de garrapatas del género <i>Ixodes</i>. La sintomatología suele ser bifásica, asociándose la fase inicial a una enfermedad febril inespecífica y la segunda fase a meningitis, meningoencefalitis o meningoencefalomielitis."},"it":{"name":"Encefalite trasmessa da zecche"},"nl":{"name":"Tekenencefalitis","synonyms":["TBE"],"definition":"Een infectieziekte, gekenmerkt door betrokkenheid van centraal zenuwstelsel (CZS), veroorzaakt door TBE-virus (TBEV), en hoofdzakelijk overgedragen op mensen via een beet van een teek van het geslacht <i>Ixodes</i>. De symptomatologie is vaak bifasisch, met een initiële fase die is geassocieerd met aspecifieke febriele ziekte en een tweede fase met meningitis, meningo-encefalitis of meningo-encefalomyelitis."},"pt":{"name":"Encefalite por picada de carraça","synonyms":["TBE"],"definition":"Doença infeciosa caracterizada pelo envolvimento do sistema nervoso central (SNC), causada pelo vírus TBE (TBEV) e transmitida aos humanos principalmente pela picada de carraças do género <i>Ixodes</i>. A sintomatologia é frequentemente bifásica, estando a fase inicial associada a uma doença febril inespecífica e a segunda fase a meningite, meningoencefalite ou meningoencefalomielite."},"pl":{"name":"Kleszczowe zapalenie mózgu","synonyms":["TBE"]},"cs":{"name":"Klíšťová encefalitida","synonyms":["TBE"]},"uk":{"name":"Кліщовий енцефаліт","synonyms":["TBE"],"definition":"An infectious disease characterized by central nervous system (CNS) involvement caused by the TBE virus (TBEV) and transmitted to humans principally by the bite of ticks from the <i>Ixodes</i> genus. The symptomatology is often biphasic, with the initial phase being associated with a nonspecific febrile illness and the second phase with meningitis, meningoencephalitis or meningoencephalomyelitis."},"zh":{"name":"蜱传脑炎","synonyms":["TBE"]},"tr":{"name":"Keneyle geçen ensefalit","synonyms":["TBE"]}},"xrefs":[{"source":"ICD-10","code":"A84.0","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"A84.1","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"A84.8","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"A84.9","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"1C80","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"956664712"},{"source":"MONDO","code":"0017572","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D004675","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0014061","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"5216","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10043847","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":68416,"name":"Rare infectious disease"},"parents":[{"orpha":166490,"name":"Infectious disease with epilepsy","classifications":[181]},{"orpha":98252,"name":"Infectious encephalitis","classifications":[181,203]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"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":"2020-04-10","source":"25984517[PMID]_28928417[PMID]_26601448[PMID]","terms":[{"hpo":"HP:0012229","label":"CSF pleocytosis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0011112","label":"Abnormality of serum cytokine level","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002039","label":"Anorexia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002829","label":"Arthralgia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012378","label":"Fatigue","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002315","label":"Headache","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001882","label":"Leukopenia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003326","label":"Myalgia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002018","label":"Nausea","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:0001291","label":"Abnormal cranial nerve morphology","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:3000047","label":"Abnormal glossopharyngeal nerve morphology","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011392","label":"Abnormality of the vestibular nerve","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000708","label":"Atypical behavior","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003418","label":"Back pain","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100543","label":"Cognitive impairment","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0032044","label":"Decreased vigilance","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000716","label":"Depression","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031987","label":"Diminished ability to concentrate","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002015","label":"Dysphagia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011227","label":"Elevated circulating C-reactive protein concentration","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003565","label":"Elevated erythrocyte sedimentation rate","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001262","label":"Excessive daytime somnolence","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000365","label":"Hearing impairment","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002487","label":"Hyperkinetic movements","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002311","label":"Incoordination","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003237","label":"Increased circulating IgG level","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003496","label":"Increased circulating IgM level","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001974","label":"Increased total leukocyte count","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0009763","label":"Limb pain","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001287","label":"Meningitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000602","label":"Ophthalmoplegia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003470","label":"Paralysis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000751","label":"Personality changes","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000613","label":"Photophobia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0004372","label":"Reduced consciousness","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:0003474","label":"Somatic sensory dysfunction","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011098","label":"Speech apraxia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0025258","label":"Stiff neck","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001873","label":"Thrombocytopenia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000360","label":"Tinnitus","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001308","label":"Tongue fasciculations","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001337","label":"Tremor","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011450","label":"Unusual CNS infection","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002321","label":"Vertigo","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000505","label":"Visual impairment","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002013","label":"Vomiting","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012747","label":"Abnormal brainstem MRI signal intensity","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001637","label":"Abnormal myocardium morphology","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0011441","label":"Abnormality of the medulla oblongata","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001259","label":"Coma","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0031258","label":"Delirium","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002910","label":"Elevated circulating hepatic transaminase concentration","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0010628","label":"Facial palsy","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0030196","label":"Fatigable weakness of respiratory muscles","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0007359","label":"Focal-onset seizure","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002197","label":"Generalized-onset seizure","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0012486","label":"Myelitis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0031003","label":"Polyneuritis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000709","label":"Psychosis","frequency":"Very rare (<4-1%)","criteria":null}]}}