{"orpha":447881,"name":"Idiopathic dropped head syndrome","type":"Clinical syndrome","group":"Disorder","status":"active","flags":[],"synonyms":["Isolated neck extensor myopathy"],"definition":"A rare acquired skeletal muscle disease characterized by severe weakness of the neck extensor muscles causing progressive reducible kyphosis of the cervical spine and the inability to hold the head up, in the absence of a known cause. Histological studies reveal a non-inflammatory myopathic picture. The clinical course is relatively benign, although cervical myelopathy may develop.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-447881/","orphanet_url":"https://www.orpha.net/en/disease/detail/447881","names":{"fr":{"name":"Syndrome de la tête tombante idiopathique","synonyms":["Myopathie idiopathique cervicale"],"definition":"Maladie acquise rare du muscle squelettique caractérisée par une faiblesse sévère des muscles extenseurs du cou provoquant une cyphose réductible d'évolution progressive de la colonne cervicale et une incapacité à maintenir la tête en position verticale, en l'absence de toute cause connue. Les études histologiques révèlent un tableau clinique myopathique non inflammatoire. L'évolution clinique est relativement bénigne, malgré le risque de développement d'une myélopathie cervicale."},"de":{"name":"Idiopathisches Dropped-Head-Syndrom","synonyms":["Isolierte Myopathie der Kopfextensoren"],"definition":"Eine seltene erworbene Skelettmuskelerkrankung, die durch eine schwere Schwäche der Nackenstreckmuskeln gekennzeichnet ist, die eine fortschreitende, reduzierbare Kyphose der Halswirbelsäule und die Unfähigkeit, den Kopf hochzuhalten, verursacht, ohne dass eine Ursache bekannt ist. Histologische Untersuchungen zeigen ein nicht-entzündliches myopathisches Bild. Der klinische Verlauf ist relativ gutartig, obwohl sich eine zervikale Myelopathie entwickeln kann."},"es":{"name":"Síndrome idiopático de cabeza caída","synonyms":["Miopatía idiopática de los extensores del cuello"],"definition":"Es una enfermedad adquirida y poco frecuente del músculo esquelético caracterizada por debilidad grave de los músculos extensores del cuello ocasionando cifosis progresiva y reductible de la columna cervical, así como por una incapacidad para mantener la cabeza erguida, en ausencia de una causa conocida. Los estudios histológicos revelan un cuadro miopático no inflamatorio. El curso clínico es relativamente benigno, aunque puede desarrollarse una mielopatía cervical."},"it":{"name":"Sindrome idiopatica della testa cadente","synonyms":["Miopatia isolata dei muscoli estensori del collo"],"definition":"Si tratta di una malattia muscolo-scheletrica acquisita rara, caratterizzata da marcata debolezza dei muscoli estensori del collo, che causa una cifosi riducibile progressiva del tratto cervicale della colonna e l'incapacità del paziente di tenere la testa diritta. L'eziologia non è nota. L'esame istologico evidenzia una miopatia non infiammatoria. Il decorso clinico è relativamente benigno, ma i pazienti possono sviluppare una mielopatia cervicale."},"nl":{"name":"Idiopathisch hangend hoofd-syndroom","synonyms":["Geïsoleerde nekextensormyopathie"],"definition":"Een zeldzame, verworven aandoening van skeletspier, gekarakteriseerd door ernstige zwakte van de strekspieren van de nek, met als gevolg progressieve reponibele kyfose van de cervicale wervelkolom en het onvermogen om het hoofd rechtop te houden, zonder een gekende oorzaak. Histologische onderzoeken tonen een niet-inflammatoir myopathisch beeld. Het klinische verloop is relatief goedaardig, hoewel cervicale myelopathie kan ontwikkelen."},"pt":{"name":"Síndrome idiopático da cabeça caída"},"pl":{"name":"Idiopatyczny zespół opadania głowy","synonyms":["Izolowana miopatia prostownika grzbietu"]},"cs":{"name":"Idiopatický syndrom padající hlavy","synonyms":["Izolovaná myopatie extenzorů šíje"]},"uk":{"name":"Ідіопатичний синдром опущеної голови","synonyms":["Ізольована міопатія розгиначів шиї"],"definition":"A rare acquired skeletal muscle disease characterized by severe weakness of the neck extensor muscles causing progressive reducible kyphosis of the cervical spine and the inability to hold the head up, in the absence of a known cause. Histological studies reveal a non-inflammatory myopathic picture. The clinical course is relatively benign, although cervical myelopathy may develop."},"zh":{"name":"特发性歪头综合征","synonyms":["特发性颈伸肌肌病"],"definition":"一种罕见的获得性骨骼肌疾病，其特征为颈部伸肌严重无力，导致进行性减弱的颈椎后凸畸形，不明原因的无法抬头。组织学研究显示出非炎症性肌病。尽管可能会发展为脊髓型颈椎病，但临床进程相对较温和。"},"tr":{"name":"İdiyopatik düşük baş sendromu","synonyms":["İzole boyun ekstansör miyopatisi"]}},"xrefs":[{"source":"ICD-10","code":"G72.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0018654","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C3266164","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98006,"name":"Rare neurologic disease"},"parents":[{"orpha":206638,"name":"Acquired skeletal muscle disease","classifications":[181]}],"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":null}