{"orpha":83597,"name":"Acute disseminated encephalomyelitis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["ADEM","Acute demyelinating encephalomyelitis","Acute disseminated encephalitis"],"definition":"A demyelinating disorder of the central nervous system.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-83597/","orphanet_url":"https://www.orpha.net/en/disease/detail/83597","names":{"fr":{"name":"Encéphalomyélite aiguë disséminée","synonyms":["Encéphalite aiguë disséminée","Encéphalomyélite démyélinisante aiguë"]},"de":{"name":"Enzephalomyelitis, akute disseminierte","synonyms":["ADEM","Akute demyelinisierende Enzephalomyelitis","Enzephalitis, akute disseminierte"],"definition":"Eine demyelinisierende Erkrankung des zentralen Nervensystems."},"es":{"name":"Encefalomielitis aguda diseminada","synonyms":["ADEM","EMAD","Encefalitis aguda diseminada","Encefalomielitis aguda desmielinizante"],"definition":"Es un trastorno desmielinizante del sistema nervioso central."},"it":{"name":"Encefalomielite disseminata acuta","synonyms":["Encefalomielite demielinizzante acuta","ADEM","Encefalite disseminata acuta"]},"nl":{"name":"Acute gedissemineerde encefalomyelitis","synonyms":["ADEM","Acute demyeliniserende encefalomyelitis","Acute gedissemineerde encefalitis"],"definition":"Een demyeliniserende aandoening van centraal zenuwstelsel."},"pt":{"name":"Encefalite disseminada aguda","synonyms":["ADEM","Encefalomielite disseminada aguda"]},"pl":{"name":"Ostre rozsiane zapalenie mózgu i rdzemoa kręgowego","synonyms":["ADEM","Ostre rozsiane zapalenie mózgu"]},"cs":{"name":"Akutní diseminovaná encefalomyelitida","synonyms":["ADEM","Akutní diseminovaná encefalitida"]},"uk":{"name":"Гострий розсіяний енцефаломієліт","synonyms":["Гострий розсіяний енцефаліт","ADEM"],"definition":"A demyelinating disorder of the central nervous system."},"zh":{"name":"急性播散性脑脊髓炎","synonyms":["急性播散性脑炎"]},"tr":{"name":"Akut dissemine ensefalomiyelit","synonyms":["ADEM","Akut yaygın ensefalit"]}},"xrefs":[{"source":"ICD-10","code":"G04.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"8A42","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1390433308"},{"source":"MONDO","code":"0019383","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D004673","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0014059","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"8639","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10000709","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98006,"name":"Rare neurologic disease"},"parents":[{"orpha":622014,"name":"Autoimmune encephalitis","classifications":[181]},{"orpha":499047,"name":"Autoimmune/inflammatory optic neuropathy","classifications":[189]},{"orpha":166490,"name":"Infectious disease with epilepsy","classifications":[181]}],"children":[{"orpha":592894,"name":"Acute disseminated encephalomyelitis with anti-MOG antibodies","type":"Clinical subtype"},{"orpha":592900,"name":"Acute disseminated encephalomyelitis without anti-MOG antibodies","type":"Clinical subtype"}],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.6,"area":"Europe","status":"Validated","source":"[EXPERT]"},{"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":"2021-02-04","source":"31340401[PMID]_31371120[PMID]","terms":[{"hpo":"HP:0001298","label":"Encephalopathy","frequency":"Obligate (100%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0007305","label":"CNS demyelination","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0007204","label":"Diffuse white matter abnormalities","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0010845","label":"EEG with generalized slow activity","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002134","label":"Abnormal basal ganglia morphology","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012747","label":"Abnormal brainstem MRI signal intensity","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001317","label":"Abnormal cerebellum morphology","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002518","label":"Abnormal periventricular white matter morphology","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0007256","label":"Abnormal pyramidal sign","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012696","label":"Abnormal thalamic MRI signal intensity","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002143","label":"Abnormality of the spinal cord","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0032492","label":"Anti-myelin oligodendrocyte glycoprotein antibody positivity","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001251","label":"Ataxia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000708","label":"Atypical behavior","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0200149","label":"CSF lymphocytic pleiocytosis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002181","label":"Cerebral edema","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0031696","label":"Disseminated viral infection","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:0100786","label":"Hypersomnia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002922","label":"Increased CSF protein concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0025373","label":"Interictal EEG abnormality","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000737","label":"Irritability","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001268","label":"Mental deterioration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012486","label":"Myelitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002018","label":"Nausea","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001250","label":"Seizure","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002167","label":"Abnormality of speech or vocalization","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000718","label":"Aggressive behavior","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001259","label":"Coma","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001289","label":"Confusion","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0006824","label":"Cranial nerve paralysis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0010843","label":"EEG with focal slow activity","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011193","label":"EEG with focal spikes","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030857","label":"Eye movement-induced pain","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001269","label":"Hemiparesis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001324","label":"Muscle weakness","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031179","label":"Nuchal rigidity","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011151","label":"Obtundation status","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100653","label":"Optic neuritis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0004887","label":"Respiratory failure requiring assisted ventilation","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003474","label":"Somatic sensory dysfunction","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000572","label":"Visual loss","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002013","label":"Vomiting","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0006597","label":"Diaphragmatic paralysis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0012302","label":"Herpes simplex encephalitis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0007103","label":"Hypointensity of cerebral white matter on MRI","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0004305","label":"Involuntary movements","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0020088","label":"Post-vaccination measles","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0020089","label":"Post-vaccination rubella","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0031693","label":"Severe Epstein Barr virus infection","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0031695","label":"Severe parainfluenza infection","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0006562","label":"Viral hepatitis","frequency":"Very rare (<4-1%)","criteria":null}]}}