{"orpha":139417,"name":"Acute transverse myelitis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare inflammatory demyelinating disorder of the spinal cord that can be either idiopathic (IATM) or secondary to a known cause (SATM).","note":null,"url":"https://raremap.alftech.space/d/ORPHA-139417/","orphanet_url":"https://www.orpha.net/en/disease/detail/139417","names":{"fr":{"name":"Myélite transverse aiguë","definition":"La myélite aiguë transverse (MAT) est une maladie inflammatoire démyélinisante de la moelle épinière soit idiopathique, soit secondaire à une cause connue."},"de":{"name":"Myelitis, akute transverse","synonyms":["Querschnittsmyelitis"],"definition":"Die akute transverse Myelitis (ATM) ist eine entzündliche demyelinisierende Erkrankung des Rückenmarks, die entweder idiopathisch (IATM; s. dort) oder sekundär, im Gefolge einer bekannten Ursache (SATM; s. dort) auftritt."},"es":{"name":"Mielitis transversa aguda","definition":"Es un trastorno desmielinizante inflamatorio de la médula espinal que puede ser tanto idiopático (MTAI) o secundario a una causa conocida (MTAS)."},"it":{"name":"Mielite trasversa acuta"},"nl":{"name":"Acute transversale myelitis","synonyms":["Acute myelitis transversa"],"definition":"Een zeldzame inflammatoire demyeliniserende aandoening van ruggenmerg, die idiopathisch kan zijn (IATM) of secundair aan een gekende oorzaak (SATM)."},"pt":{"name":"Mielite transversa, aguda","definition":"A mielite transversa aguda (ATM) é uma doença desmielinizante inflamatória da medula espinhal que pode ser idiopática (IATM; ver este termo) ou secundária a uma causa conhecida (SATM; ver este termo)."},"pl":{"name":"Ostre poprzeczne zapalenie rdzenia"},"cs":{"name":"Akutní transverzální myelitida"},"uk":{"name":"Гострий поперечний мієліт","definition":"A rare inflammatory demyelinating disorder of the spinal cord that can be either idiopathic (IATM) or secondary to a known cause (SATM)."},"zh":{"name":"急性横贯性脊髓炎","definition":"一种罕见的脊髓炎性脱髓鞘疾病，可为特发性（IATM）或继发于已知原因（SATM）。"},"tr":{"name":"Akut transvers miyelit"}},"xrefs":[{"source":"ICD-10","code":"G37.3","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"8A41.0","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1141415034"},{"source":"MONDO","code":"0015342","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0270627","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"19913","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[{"type":"Referred to","orpha":139420,"name":"OBSOLETE: Secondary acute transverse myelitis","status":"inactive"}],"preferential_parent":{"orpha":98006,"name":"Rare neurologic disease"},"parents":[{"orpha":102000,"name":"Medullar disease","classifications":[181]},{"orpha":182064,"name":"Rare neuroinflammatory or neuroimmunological disease","classifications":[181]}],"children":[{"orpha":592873,"name":"Acute transverse myelitis with anti-MOG antibodies","type":"Clinical subtype"},{"orpha":139423,"name":"Idiopathic acute transverse myelitis","type":"Clinical subtype"}],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Adolescent","Adult","Childhood","Elderly","Infancy"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":8.6,"area":"United States","status":"Validated","source":"31235660[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":7.9,"area":"United States","status":"Validated","source":"31235660[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":4.72,"area":"Worldwide","status":"Validated","source":"19812117[PMID]_19896453[PMID]_31235660[PMID]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2021-11-18","source":"29375121[PMID]_23186897[PMID]_24099672[PMID]","terms":[{"hpo":"HP:0012332","label":"Abnormal autonomic nervous system physiology","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002071","label":"Abnormality of extrapyramidal motor function","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:5000013","label":"Anti-GFAP antibody","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012229","label":"CSF pleocytosis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002922","label":"Increased CSF protein concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001324","label":"Muscle weakness","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003474","label":"Somatic sensory dysfunction","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0031845","label":"Abnormal libido","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0004370","label":"Abnormality of temperature regulation","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0025615","label":"Abscess","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0032492","label":"Anti-myelin oligodendrocyte glycoprotein antibody positivity","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002960","label":"Autoimmunity","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0005341","label":"Autonomic bladder dysfunction","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003487","label":"Babinski sign","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003418","label":"Back pain","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0007305","label":"CNS demyelination","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002019","label":"Constipation","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011967","label":"Decreased circulating copper concentration","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100502","label":"Decreased circulating vitamin B12 concentration","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0032283","label":"Disseminated nontuberculous mycobacterial infection","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0010835","label":"Dissociated sensory loss","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0009053","label":"Distal lower limb muscle weakness","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012534","label":"Dysesthesia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0032271","label":"Extrapulmonary tuberculosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012378","label":"Fatigue","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001288","label":"Gait disturbance","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002578","label":"Gastroparesis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001347","label":"Hyperreflexia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000822","label":"Hypertension","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011972","label":"Hypoglycorrhachia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002721","label":"Immunodeficiency","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0010831","label":"Impaired proprioception","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002495","label":"Impaired vibratory sensation","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031700","label":"Invasive parasitic infection","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001287","label":"Meningitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0010547","label":"Muscle flaccidity","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031179","label":"Nuchal rigidity","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001278","label":"Orthostatic hypotension","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002590","label":"Paralytic ileus","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002385","label":"Paraparesis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0010550","label":"Paraplegia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003401","label":"Paresthesia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031691","label":"Severe viral infection","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001257","label":"Spasticity","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002138","label":"Subarachnoid hemorrhage","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002725","label":"Systemic lupus erythematosus","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003484","label":"Upper limb muscle weakness","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002493","label":"Upper motor neuron dysfunction","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002839","label":"Urinary bladder sphincter dysfunction","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000020","label":"Urinary incontinence","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000016","label":"Urinary retention","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0200023","label":"Priapism","frequency":"Very rare (<4-1%)","criteria":null}]}}