{"orpha":83467,"name":"Morvan syndrome","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Limbic encephalitis-neuromyotonia-hyperhidrosis-polyneuropathy syndrome","Morvan fibrillary chorea"],"definition":"Morvan syndrome is a rare, life-threatening, acquired neurologic disease characterized by neuromyotonia, dysautonomia and encephalopathy with severe insomnia. Signs involving central (e.g. hallucinations, confusion, amnesia, myoclonus), autonomic (e.g. variations in blood pressure, hyperhidrosis) and peripheral (e.g. painful cramps, myokymia) hyperactivity, as well as systemic manifestations (such as weight loss, pruritus, fever), are reported. Thymoma is present in some cases.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-83467/","orphanet_url":"https://www.orpha.net/en/disease/detail/83467","names":{"fr":{"name":"Syndrome de Morvan","synonyms":["Chorée fibrillaire de Morvan","Syndrome d'encéphalite limbique-neuromyotonie-hyperhidrose-polyneuropathie"],"definition":"Le syndrome de Morvan est un trouble neurologique rare acquis, mettant en jeu le pronostic vital. Il se caractérise par une neuromyotonie, une dysautonomie et une encéphalopathie avec une insomnie sévère. Les signes impliquent une hyperactivité centrale (hallucinations, confusion, amnésie, myoclonus), autonomique (variations de la pression sanguine, hyperhidrose) et périphérique (crampes douloureuses, myokymie). Des manifestations systémiques (telles qu'une perte de poids, un prurit, une fièvre) sont aussi rapportées. Un thymome est présent dans certains cas."},"de":{"name":"Morvan-Syndrom","synonyms":["Fibrilläre Chorea Morvan","Syndrom der limbischen Enzephalitis mit Neuromyotonie, Hyperhidrose und Polyneuropathie"],"definition":"Das Morvan-Syndrom ist eine seltene, lebensbedrohliche, erworbene neurologische Erkrankung, die durch Neuromyotonie, Dysautonomie und Enzephalopathie mit schwerer Schlaflosigkeit gekennzeichnet ist. Es wird über zentrale (z. B. Halluzinationen, Verwirrtheit, Amnesie, Myoklonus), autonome (z. B. Blutdruckschwankungen, Hyperhidrose) und periphere (z. B. schmerzhafte Krämpfe, Myokymie) Hyperaktivität sowie systemische Manifestationen (wie Gewichtsverlust, Pruritus, Fieber) berichtet. In einigen Fällen liegt ein Thymom vor."},"es":{"name":"Síndrome de Morvan","synonyms":["Corea fibrilar de Morvan","Encefalitis límbica-neuromiotonía-hiperhidrosis-polineuropatía","Síndrome de encefalitis límbica-neuromiotonía-hiperhidrosis-polineuropatía"],"definition":"El síndrome de Morvan es una enfermedad neurológica poco frecuente potencialmente mortal, adquirida, caracterizada por neuromiotonía, disautonomía y encefalopatía con insomnio grave. Se ha descrito la presencia de signos de afectación del sistema central (por ejemplo, alucinaciones, confusión, amnesia, mioclono), autónomo (por ejemplo, variaciones en la presión arterial, hiperhidrosis) y periférico (por ejemplo, calambres dolorosos, mioquimia) e hiperactividad, así como manifestaciones sistémicas (como pérdida de peso, prurito, fiebre). En algunos casos puede asociarse un timoma."},"it":{"name":"Sindrome di Morvan","synonyms":["Corea fibrillare di Morvan","Sindrome da encefalite limbica, neuromiotonia, iperidrosi e polineuropatia"],"definition":"La sindrome di Morvan è una malattia neurologica acquisita rara e letale, caratterizzata da neuromiotonia, disautonomia ed encefalopatia con insonnia grave. Sono stati descritti segni di iperattività del sistema nervoso centrale (allucinazioni, confusione, amnesia, mioclono), alterazioni del sistema autonomo (variazioni della pressione sanguigna, iperidrosi) e periferico (crampi dolorosi, miochimia) e segni sistemici (perdita di peso, prurito, febbre). Inoltre, sono stati descritti casi di timoma."},"nl":{"name":"Syndroom van Morvan","synonyms":["Fibrillaire chorea van Morvan","Limbische encefalitis - neuromyotonie - hyperhidrose - polyneuropathie-syndroom"],"definition":"Syndroom van Morvan is een zeldzame, levensbedreigende, verworven neurologische ziekte die wordt gekarakteriseerd door neuromyotonie, dysautonomie en encefalopathie met ernstige insomnia. Tekenen van centrale (e.g. hallucinaties, verwardheid, amnesie, myoclonus), autonome (e.g. variaties in bloeddruk, hyperhidrose) en perifere (e.g. pijnlijke krampen, myokymie) hyperactiviteit, alsook systemische manifestaties (zoals gewichtsverlies, pruritus, koorts), werden gerapporteerd. Thymoom is aanwezig in sommige gevallen."},"pt":{"name":"Síndrome Morvan","synonyms":["Coreia fibrilhar Morvan","Síndrome de encefalite límbica-neuromiotonia-hiperhidrose-polineuropatia"]},"pl":{"name":"Zespół Morvana","synonyms":["Limbiczne zapalenie mózgu - neuromiotonia - hiperhydroza - polineuropatia","Włókienkowa pląsawiaca Morvana"]},"cs":{"name":"Morvanův syndrom","synonyms":["Syndrom zahrnující limbickou encefalitidu, neuromyotonii, hyperhidrózu a polyneuropatii","Morvanova fibrilární chorea"]},"uk":{"name":"Синдром Морвана","synonyms":["Синдром лімбічного енцефаліту-нейроміотонії-гіпергідрозу-полінейропатії","Фібрилярна хорея Морвана"],"definition":"Morvan syndrome is a rare, life-threatening, acquired neurologic disease characterized by neuromyotonia, dysautonomia and encephalopathy with severe insomnia. Signs involving central (e.g. hallucinations, confusion, amnesia, myoclonus), autonomic (e.g. variations in blood pressure, hyperhidrosis) and peripheral (e.g. painful cramps, myokymia) hyperactivity, as well as systemic manifestations (such as weight loss, pruritus, fever), are reported. Thymoma is present in some cases."},"zh":{"name":"Morvan综合征","synonyms":["边缘叶脑炎-神经性肌强直-多汗症-多发性神经病综合征"]},"tr":{"name":"Morvan sendromu","synonyms":["Morvan fibriler kore","Limbik ensefalit-nöromiyotoni-hiperhidroz-polinöropati sendromu"]}},"xrefs":[{"source":"ICD-10","code":"G60.8","qualifier":"NTBT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"8E4A.3","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1599858362"},{"source":"MONDO","code":"0008718","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C3854373","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"9766","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10075006","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98006,"name":"Rare neurologic disease"},"parents":[{"orpha":221114,"name":"Acquired peripheral movement disorder","classifications":[181]},{"orpha":98750,"name":"Autoimmune neurological channelopathy","classifications":[181]},{"orpha":166484,"name":"Inflammatory and autoimmune disease with epilepsy","classifications":[181]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Adult"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":60.0,"area":"Worldwide","status":"Validated","source":"27698847[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET_27698847[PMID]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2024-03-04","source":"32651251[PMID]_29561731[PMID]_32783406[PMID]","terms":[{"hpo":"HP:5000005","label":"Anti-CASPR2","frequency":"Frequent (79-30%)","criteria":null}]}}