{"orpha":94091,"name":"Mills syndrome","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare, acquired motor neuron disease characterized by a slowly progressive, unilateral, ascending or descending hemiplegia, associated to unilateral or asymmetrical pyramidal signs and no sensory loss. It is a diagnosis of exclusion and controversy exists regarding whether the presence of bulbar symptoms, sphincter disturbances, fasciculations or cognitive manifestations characterize the disease.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-94091/","orphanet_url":"https://www.orpha.net/en/disease/detail/94091","names":{"fr":{"name":"Syndrome de Mills","definition":"Maladie acquise rare du motoneurone, caractérisée par une hémiplégie lentement progressive, unilatérale, ascendante ou descendante, associée à des signes d'atteinte pyramidale unilatérale ou asymétrique, sans perte sensorielle. Il s'agit d'un diagnostic d'exclusion et la présence de symptômes d'atteinte bulbaire, de troubles sphinctériens, de fasciculations ou de manifestations cognitives comme caractéristiques de la maladie reste controversée."},"de":{"name":"Mills-Syndrom","definition":"Eine seltene, erworbene Motoneuronenerkrankung, die durch eine langsam fortschreitende, einseitige, auf- oder absteigende Hemiplegie gekennzeichnet ist, die mit einseitigen oder asymmetrischen pyramidalen Zeichen ohne sensorischen Verlust einhergeht. Es handelt sich um eine Ausschlussdiagnose, und es besteht Uneinigkeit darüber, ob das Vorhandensein von bulbären Symptomen, Sphinkterstörungen, Faszikulationen oder kognitiven Manifestationen die Krankheit kennzeichnet."},"es":{"name":"Síndrome de Mills","definition":"Es una enfermedad adquirida poco frecuente de la motoneurona, caracterizada por hemiplejia unilateral, ascendente o descendente, lentamente progresiva, asociada a signos piramidales unilaterales o asimétricos, sin afectación sensitiva. Es un diagnóstico por exclusión y existe controversia sobre si la presencia de síntomas bulbares, alteraciones esfinterianas, fasciculaciones o manifestaciones cognitivas son características de la enfermedad."},"it":{"name":"Sindrome di Mills","synonyms":["Sclerosi laterale amiotrofica, tipo emiplegico"],"definition":"È una malattia acquisita rara dei motoneuroni, caratterizzata da emiplegia monolaterale, ascendente o discendente, a progressione lenta, associata a segni piramidali monolaterali o asimmetrici, senza perdita sensoriale. La diagnosi si basa sull'esclusione di altre malattie; è al momento dibattuto se i sintomi bulbari, le disfunzioni sfinteriche, le fascicolazioni e i sintomi cognitivi siano distintivi della malattia."},"nl":{"name":"Syndroom van Mills","definition":"Een zeldzame, verworven motorneuronziekte gekarakteriseerd door een traag progressieve, unilaterale, stijgende of dalende hemiplegie, geassocieerd met unilaterale of asymmetrische piramidale tekenen en geen zintuiglijk verlies. Het gaat om een uitsluitingsdiagnose en er bestaat controverse over hoe de aanwezigheid van bulbaire symptomen, stoornissen van de sluitspier, fasciculaties of cognitieve manifestaties de ziekte kenmerkt."},"pt":{"name":"Síndrome de Mills"},"pl":{"name":"Zespół Millsa","synonyms":["Stwardnienie zanikowe boczne, typu porażenie połowicze"]},"cs":{"name":"Millsův syndrom","synonyms":["Amyotrofická laterální skleróza, hemiplegický typ"]},"uk":{"name":"Синдром Міллса","definition":"A rare, acquired motor neuron disease characterized by a slowly progressive, unilateral, ascending or descending hemiplegia, associated to unilateral or asymmetrical pyramidal signs and no sensory loss. It is a diagnosis of exclusion and controversy exists regarding whether the presence of bulbar symptoms, sphincter disturbances, fasciculations or cognitive manifestations characterize the disease."},"zh":{"name":"Mills综合征","definition":"一种罕见的获得性运动神经元疾病，特征是缓慢进行性、单侧、上升性或下降性偏瘫，伴有单侧或不对称的锥体束征，无感觉丧失。该病为排除性诊断，关于延髓症状、括约肌功能紊乱、肌束震颤、认知障碍等症状是否是该病的特征，还存在争议。"},"tr":{"name":"Mills sendromu"}},"xrefs":[{"source":"ICD-10","code":"G12.2","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"8B60.Y","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0035892","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5191669","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98006,"name":"Rare neurologic disease"},"parents":[{"orpha":98506,"name":"Acquired motor neuron disease","classifications":[181]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Adolescent","Adult","Childhood","Elderly"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.12,"area":"United Kingdom","status":"Validated","source":"30631918[PMID]"}],"phenotypes":null}