{"orpha":95613,"name":"Pituitary apoplexy","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Pituitary tumor apoplexy"],"definition":"A rare pituitary disease characterized by hemorrhagic or non-hemorrhagic necrosis of the pituitary gland. Clinical manifestations typically comprise sudden and severe headache (often with nausea and vomiting), visual disturbances (visual-field defects, loss of visual acuity), oculomotor palsies, and variable degrees of altered consciousness, ranging from lethargy to coma. Acute endocrine dysfunction may also be present, most commonly corticotropic deficiency with severe hypotension and hyponatremia as well as secondary adrenal failure, but also thyrotropic and gonadotropic deficiency.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-95613/","orphanet_url":"https://www.orpha.net/en/disease/detail/95613","names":{"fr":{"name":"Apoplexie hypophysaire","synonyms":["Apoplexie tumorale hypophysaire"],"definition":"Maladie hypophysaire rare caractérisée par une nécrose hémorragique ou non hémorragique de l'hypophyse. Les signes cliniques incluent généralement des céphalées soudaines et sévères (souvent accompagnées de nausées et de vomissements), des troubles visuels (défauts du champ visuel, perte d'acuité visuelle), des paralysies oculomotrices et des degrés variables d'altération de la conscience, allant de la léthargie au coma. On observe également un dysfonctionnement endocrinien aigu, le plus souvent un déficit corticotrope avec une hypotension et une hyponatrémie sévères ainsi qu'une insuffisance surrénale secondaire, mais aussi une insuffisance thyréotrope et gonadotrope."},"de":{"name":"Hypophyseninfarkt","synonyms":["Hypophysentumor-Infarkt"],"definition":"Eine seltene Hypophysenerkrankung, die durch eine hämorrhagische oder nicht-hämorrhagische Nekrose der Hypophyse gekennzeichnet ist. Zu den typischen klinischen Manifestationen gehören plötzliche und starke Kopfschmerzen (oft mit Übelkeit und Erbrechen), Sehstörungen (Gesichtsfelddefekte, Verlust der Sehschärfe), okulomotorische Lähmungen und unterschiedliche Grade von Bewusstseinsstörungen, die von Lethargie bis zum Koma reichen. Es kann auch eine akute endokrine Dysfunktion vorliegen, am häufigsten ein kortikotroper Mangel mit schwerer Hypotonie und Hyponatriämie sowie sekundärem Nebennierenversagen, aber auch ein thyreotroper und gonadotroper Mangel."},"es":{"name":"Apoplejía hipofisaria","synonyms":["Apoplejía hipofisaria tumoral"],"definition":"Es una enfermedad hipofisaria poco frecuente caracterizada por necrosis hemorrágica o no hemorrágica de la hipófisis. Las manifestaciones clínicas comprenden típicamente cefalea repentina e intensa (a menudo con náusea y vómitos), alteraciones visuales (defectos del campo visual, pérdida de agudeza visual), parálisis oculomotora y grados variables de alteración del estado de conciencia, que van desde letargia hasta coma. También puede haber disfunción endocrina aguda, más comúnmente deficiencia corticotrópica con hipotensión e hiponatremia graves, así como insuficiencia suprarrenal secundaria y deficiencia tirotrópica y gonadotrópica."},"it":{"name":"Apoplessia ipofisaria","definition":"Si tratta di una malattia rara caratterizzata dalla necrosi emorragica o non emorragica dell'ipofisi. I segni clinici comprendono la cefalea improvvisa e grave (spesso associata alla nausea e al vomito), le alterazioni della vista (difetti del campo visivo, perdita dell'acuità visiva), le paralisi oculomotorie e l'alterazione dello stato di coscienza, di gravità variabile, che comprende la letargia fino al coma. I pazienti possono presentare anche una disfunzione endocrina acuta, di solito un deficit corticotropo con ipotensione grave, iponatriemia e insufficienza surrenalica secondaria, ma anche un deficit tireotropo e gonadotropo."},"nl":{"name":"Apoplexie van hypofyse","synonyms":["Hypofysetumor-apoplexie"],"definition":"Een zeldzame aandoening van hypofyse, gekarakteriseerd door hemorragische of niet-hemorragische necrose van hypofyse. Gangbare klinische manifestaties zijn plotse en ernstige hoofdpijn (vaak met misselijkheid en braken), visuele stoornissen (gezichtsvelddefecten, verlies van gezichtsscherpte), oculomotorische verlammingen, en variabele gradaties van gewijzigd bewustzijn, gaande van lethargie tot coma. Acute endocriene disfunctie kan ook aanwezig zijn, meestal deficiëntie van corticotropine met ernstige hypotensie en hyponatriëmie en secundaire bijnierschorsinsufficiëntie, maar ook deficiëntie van thyrotropine en gonadotropine."},"pt":{"name":"Apoplexia da pituitária"},"pl":{"name":"Apopleksja przysadki","synonyms":["Pituitary tumor apoplexy"]},"cs":{"name":"Pituitární apoplexie","synonyms":["Pituitary tumor apoplexy"]},"uk":{"name":"Апоплексія гіпофіза","synonyms":["Апоплексія пухлини гіпофіза"],"definition":"A rare pituitary disease characterized by hemorrhagic or non-hemorrhagic necrosis of the pituitary gland. Clinical manifestations typically comprise sudden and severe headache (often with nausea and vomiting), visual disturbances (visual-field defects, loss of visual acuity), oculomotor palsies, and variable degrees of altered consciousness, ranging from lethargy to coma. Acute endocrine dysfunction may also be present, most commonly corticotropic deficiency with severe hypotension and hyponatremia as well as secondary adrenal failure, but also thyrotropic and gonadotropic deficiency."},"zh":{"name":"垂体卒中","synonyms":["垂体瘤卒中"],"definition":"一种罕见的垂体疾病，以垂体出血性或非出血性坏死为特征。临床表现通常包括突然且严重的头痛（通常伴有恶心和呕吐）、视觉障碍（视野缺损、视力丧失）、动眼神经麻痹和不同程度的意识改变（从昏睡到昏迷）。可出现急性内分泌功能障碍，最常见的是促肾上腺皮质激素缺乏，伴有严重低血压和低钠血症以及继发性肾上腺功能衰竭，但也可表现为促甲状腺激素和促性腺激素缺乏。"},"tr":{"name":"Hipofiz apopleksi","synonyms":["Hipofiz tümörü apopleksi"]}},"xrefs":[{"source":"ICD-10","code":"E23.6","qualifier":"NTBT","icd_relation":"index","validated":true},{"source":"ICD-11","code":"5A61.0","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1938573221"},{"source":"MONDO","code":"0006908","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D010899","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0032001","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10056447","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97978,"name":"Rare endocrine disease"},"parents":[{"orpha":95502,"name":"Acquired pituitary hormone deficiency","classifications":[193]}],"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":"2017-03-20","source":"26414232[PMID]_21044119[PMID]_26812964[PMID]_25859804[PMID]_2436600[PMID]_22491696[PMID]_20842779[PMID]_15879908[PMID]_12546370[PMID]_11357158[PMID]_10684913[PMID]_9596029[PMID]_7608291[PMID]_2878231[PMID]","terms":[{"hpo":"HP:0030521","label":"Bitemporal hemianopia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0030907","label":"Thunderclap headache","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0030591","label":"Abnormal kinetic perimetry test","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030595","label":"Abnormal static automated perimetry test","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011748","label":"Adrenocorticotropic hormone deficiency","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0006824","label":"Cranial nerve paralysis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000651","label":"Diplopia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002315","label":"Headache","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000815","label":"Hypergonadotropic hypogonadism","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000822","label":"Hypertension","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001943","label":"Hypoglycemia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002902","label":"Hyponatremia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002615","label":"Hypotension","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000802","label":"Impotence","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002017","label":"Nausea and vomiting","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000876","label":"Oligomenorrhea","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002893","label":"Pituitary adenoma","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0008245","label":"Pituitary hypothyroidism","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0008202","label":"Reduced circulating prolactin concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002921","label":"Abnormality of the cerebrospinal fluid","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000622","label":"Blurred vision","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001259","label":"Coma","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000824","label":"Decreased response to growth hormone stimulation test","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:0100829","label":"Galactorrhea","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0040075","label":"Hypopituitarism","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000870","label":"Increased circulating prolactin concentration","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011499","label":"Mydriasis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000980","label":"Pallor","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000613","label":"Photophobia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000508","label":"Ptosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0007663","label":"Reduced visual acuity","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002339","label":"Abnormal caudate nucleus morphology","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000863","label":"Central diabetes insipidus","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001289","label":"Confusion","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000845","label":"Elevated circulating growth hormone concentration","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001262","label":"Excessive daytime somnolence","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0003118","label":"Increased circulating cortisol level","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001895","label":"Normochromic anemia","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0100661","label":"Trigeminal neuralgia","frequency":"Very rare (<4-1%)","criteria":null}]}}