{"orpha":83601,"name":"Steroid-responsive encephalopathy associated with autoimmune thyroiditis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Hashimoto encephalitis","SREAT"],"definition":"Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT) is a rare, acquired, neurological disease characterized by encephalopathy associated with elevated antithyroid antibodies, in the absence of other causes. Clinical presentation varies from minor cognitive impairment to status epilepticus and coma, and frequently includes seizures, confusion, speech disorder, memory impairment, ataxia and psychiatric manifestations.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-83601/","orphanet_url":"https://www.orpha.net/en/disease/detail/83601","names":{"fr":{"name":"Encéphalopathie sensible aux stéroïdes associée à une thyroïdite auto-immune","synonyms":["Encéphalite de Hashimoto","SREAT"],"definition":"Maladie neurologique rare et acquise, caractérisée par une encéphalopathie associée à un taux élevé d'anticorps antithyroïdiens, en l'absence d'autres causes. Le tableau clinique varie entre trouble cognitif mineur et état épileptique et coma. Par ailleurs, sont fréquemment observés des crises convulsives, une confusion, des troubles de la parole, des troubles de la mémoire, une ataxie et des troubles psychiatriques."},"de":{"name":"Steroid-responsive Enzephalopathie mit assoziierter Autoimmun-Thyroiditis","synonyms":["Hashimoto-Enzephalitis","SREAT"]},"es":{"name":"Encefalopatía sensible a esteroides asociada a tiroiditis autoinmune","synonyms":["Encefalitis de Hashimoto","SREAT"],"definition":"Es una enfermedad neurológica adquirida y poco frecuente caracterizada por encefalopatía asociada a anticuerpos antitiroideos elevados en ausencia de otras causas. La presentación clínica varía desde un deterioro cognitivo leve hasta el estado epiléptico y el coma e incluye con frecuencia convulsiones, confusión, trastorno del habla, deterioro de la memoria, ataxia y manifestaciones psiquiátricas."},"it":{"name":"Encefalopatia sensibile agli steroidi associata alla tiroidite autoimmune","synonyms":["Encefalite di Hashimoto","SREAT"],"definition":"L'encefalopatia sensibile agli steroidi associata alla tiroidite autoimmune (SREAT) è una malattia neurologica rara acquisita, caratterizzata da encefalopatia associata a livelli elevati di anticorpi antitiroidei, in assenza di altre cause. Il quadro clinico è variabile e va dal deficit cognitivo lieve allo stato epilettico e al coma; spesso fanno parte del quadro clinico le crisi epilettiche, la confusione, i disturbi del linguaggio, i deficit mnemonici, l'atassia e i sintomi psichiatrici."},"nl":{"name":"Steroïdresponsieve encefalopathie geassocieerd met auto-immuunthyreoïditis","synonyms":["Hashimoto-encefalitis","SREAT"],"definition":"Steroïderesponsieve encefalopathie geassocieerd met auto-immune thyreoïditis (SREAT) is een zeldzame, verworven, neurologische ziekte die gekarakteriseerd wordt door encefalopathie geassocieerd met een verhoogd gehalte van antithyroïde antilichamen, in afwezigheid van andere oorzaken. Het klinisch beeld varieert van geringe cognitieve stoornis tot status epilepticus en coma, en omvat vaak insulten, verwardheid, spraakstoornis, geheugenstoornis, ataxie en psychiatrische manifestaties."},"pt":{"name":"Encefalite sensível aos esteróides associada a tiroidite autoimune","synonyms":["Encefalite de Hashimoto"]},"pl":{"name":"Encefalopatia wrażliwa na steroidy z towarzyszącym autoimmunologicznym zapaleniem tarczycy","synonyms":["SREAT","Zapalenie mózgu Hashimoto"]},"cs":{"name":"Encefalopatie asociovaná s autoimunitní thyroiditidou odpovídající na steroidy","synonyms":["Hashimotova encefalitida","SREAT"]},"uk":{"name":"Стероїдозалежна енцефалопатія, повязана з аутоімунним тиреоїдитом","synonyms":["Енцефаліт Хашимото","SREAT"],"definition":"Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT) is a rare, acquired, neurological disease characterized by encephalopathy associated with elevated antithyroid antibodies, in the absence of other causes. Clinical presentation varies from minor cognitive impairment to status epilepticus and coma, and frequently includes seizures, confusion, speech disorder, memory impairment, ataxia and psychiatric manifestations."},"zh":{"name":"自身免疫性甲状腺炎相关激素敏感性脑炎","synonyms":["桥本脑炎"]},"tr":{"name":"Otoimmün tiroidit-ilişkili steroide duyarlı ensefalopati","synonyms":["SREAT","Hashimoto ensefaliti"]}},"xrefs":[{"source":"ICD-10","code":"G04.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"8E4A.0","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1445382314"},{"source":"MONDO","code":"0019385","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"C535841","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0393639","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"8570","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10069432","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98006,"name":"Rare neurologic disease"},"parents":[{"orpha":247242,"name":"Acquired ataxia","classifications":[181]},{"orpha":622014,"name":"Autoimmune encephalitis","classifications":[181]},{"orpha":177101,"name":"Rare adult hypothyroidism","classifications":[193]},{"orpha":98033,"name":"Rare neurologic disease with psychiatric involvement","classifications":[181]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"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":"2016-11-02","source":"25540320[PMID]_11158076[PMID]_11835965[PMID]_21511782[PMID]_12949305[PMID]_16476807[PMID]","terms":[{"hpo":"HP:0006846","label":"Acute encephalopathy","frequency":"Obligate (100%)","criteria":null},{"hpo":"HP:0005318","label":"Cerebral vasculitis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000872","label":"Hashimoto thyroiditis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002500","label":"Abnormal cerebral white matter morphology","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001289","label":"Confusion","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000853","label":"Goiter","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002902","label":"Hyponatremia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000821","label":"Hypothyroidism","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003470","label":"Paralysis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012332","label":"Abnormal autonomic nervous system physiology","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0009102","label":"Anterior open-bite malocclusion","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000739","label":"Anxiety","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000716","label":"Depression","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0007359","label":"Focal-onset seizure","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002197","label":"Generalized-onset seizure","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002315","label":"Headache","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002721","label":"Immunodeficiency","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001974","label":"Increased total leukocyte count","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002017","label":"Nausea and vomiting","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000709","label":"Psychosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002133","label":"Status epilepticus","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001873","label":"Thrombocytopenia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0005991","label":"Limited neck flexion","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002181","label":"Cerebral edema","frequency":"Excluded (0%)","criteria":null}]}}