{"orpha":231426,"name":"Pharyngeal-cervical-brachial variant of Guillain-Barré syndrome","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["PCB variant of GBS","PCB variant of Guillain-Barré syndrome","Pharyngeal-cervical-brachial weakness","Pharyngo-cervico-brachial variant of GBS","Pharyngo-cervico-brachial variant of Guillain-Barré syndrome"],"definition":"Pharyngeal-cervical-brachial variant of Guillain-Barré syndrome is a rare, acquired peripheral neuropathy disease characterized by rapidly progressive oropharyngeal (facial palsy, dysarthria) and cervicobrachial weakness, associated with upper limb weakness and hypo/areflexia, in the absence of ophthalmoplegia, ataxia, altered consciousness, and prominent lower limb weakness. The presence of monospecific IgG anti-GT1a antibodies is associated.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-231426/","orphanet_url":"https://www.orpha.net/en/disease/detail/231426","names":{"fr":{"name":"Variant pharyngo-cervico-brachial du syndrome de Guillain-Barré","synonyms":["Faiblesse pharyngo-cervico-brachiale","Variant PCB du SGB","Variant PCB du syndrome de Guillain-Barré","Variant pharyngo-cervico-brachial du SGB"],"definition":"Neuropathie périphérique acquise rare caractérisée par une faiblesse oropharyngée (paralysie faciale, dysarthrie) et cervicobrachiale rapidement progressive, associée à une faiblesse des membres supérieurs et à une hypo/aréflexie, en l'absence d'ophtalmoplégie, d'ataxie, d'altération de la conscience, ainsi qu'à une faiblesse importante des membres inférieurs. La présence d'anticorps IgG monospécifiques anti-GT1a est associée."},"de":{"name":"Guillain-Barré-Syndrom, pharyngeal-zervikal-brachiale Variante"},"es":{"name":"Variante faringo-cérvico-braquial del síndrome de Guillain-Barré","definition":"Es una neuropatía periférica adquirida poco frecuente caracterizada por debilidad orofaringea (parálisis facial, disartria) y cervicobraquial rápidamente progresiva, asociada a debilidad de las extremidades superiores e hipo- / arreflexia, en ausencia de oftalmoplejia, ataxia, alteración de la conciencia y notable debilidad en las extremidades inferiores. Está asociada a la presencia de anticuerpos IgG anti-GT1a monoespecíficos."},"it":{"name":"Variante faringea-cervicale-brachiale della sindrome di Guillain-Barré","definition":"La variante faringea-cervicale-brachiale della sindrome di Guillain-Barré è una neuropatia periferica acquisita rara, caratterizzata da debolezza orofaringea (paralisi facciale, disartria) e cervicobrachiale a progressione rapida, associate a debolezza degli arti superiori e ipo/areflessia, in assenza di oftalmoplegia, atassia, alterazioni dello stato di coscienza ed evidente debolezza degli arti inferiori. Sono presenti anticorpi IgG anti-GT1a monospecifici."},"nl":{"name":"Faryngeale-cervicale-brachiale variant van syndroom van Guillain-Barré","synonyms":["PCB variant van syndroom van Guillain-Barré","Faryngeale-cervicale-brachiale zwakte","Faryngo-cervico-brachiale variant van GBS","Faryngo-cervico-brachiale variant van syndroom van Guillain-Barré","PCB variant van GBS"],"definition":"Faryngeale-cervicale-brachiale variant van syndroom van Guillain-Barré is een zeldzame, verworven, perifere neuropathie die gekarakteriseerd wordt door snel progressieve orofaryngeale (aangezichtsverlamming, dysartrie) en cervicobrachiale zwakheid, geassocieerd met zwakte en hypo-/areflexie van de bovenste ledematen, in afwezigheid van oftalmoplegie, ataxie, gewijzigd bewustzijn, en aanzienlijke zwakte van de onderste ledematen. De aanwezigheid van monospecifieke IgG anti-GT1a antilichamen is geassocieerd."},"pt":{"name":"Variante faringea-cervical-braqueal da síndrome de Guillain-Barré"},"pl":{"name":"Gardłowo-szyjno-ramienny wariant zespołu Guillaina i Barrégo","synonyms":["Gardłowo-szyjno-ramienny wariant GBS","Gardłowo-szyjno-ramienny wariant zespołu Guillaina i Barrégo","PCB wariant GBS","PCB wariant zespołu Guillaina i Barrégo","Słabość gardłowo-szyjno-ramienna"]},"cs":{"name":"Faryngo-cerviko-brachiální varianta Guillainova-Barrého syndromu","synonyms":["PCB varianta GBS","PCB varianta Guillainova-Barrého syndromu","Faryngo-cerviko-brachiální varianta Guillain-Barrého syndromu","Faryngo-cerviko-brachiální slabost","Faryngo-cerviko-brachiální varianta GBS","PCB varianta Guillain-Barrého syndromu"]},"uk":{"name":"Глотково-шийно-плечовий варіант синдрому Гійєна-Барре","synonyms":["Варіант друкованої плати GBS","ПХБ-варіант синдрому Гійєна-Барре","Глотково-шийно-плечова слабкість","Фаринго-цервіко-брахіальний варіант GBS","Фаринго-цервіко-брахіальний варіант синдрому Гійєна-Барре"],"definition":"Pharyngeal-cervical-brachial variant of Guillain-Barré syndrome is a rare, acquired peripheral neuropathy disease characterized by rapidly progressive oropharyngeal (facial palsy, dysarthria) and cervicobrachial weakness, associated with upper limb weakness and hypo/areflexia, in the absence of ophthalmoplegia, ataxia, altered consciousness, and prominent lower limb weakness. The presence of monospecific IgG anti-GT1a antibodies is associated."},"zh":{"name":"吉兰-巴雷综合征咽-颈-臂变体","synonyms":["吉兰-巴雷综合征咽-颈-臂变体"],"definition":"吉兰-巴雷综合征的咽宫颈肱变异是一种罕见的获得性周围神经病变，其特征为快速进展的口咽部肌无力（面瘫、构音障碍）和颈臂无力，伴有上肢无力和反射减退/消失，无眼肌麻痹、共济失调、意识改变和明显下肢肌无力。单特异性IgG抗GT1a抗体的存在与此相关。"},"tr":{"name":"Guillain-Barrè sendromunun faringeal-servikal-brakiyal varyantı","synonyms":["GBS'nin PCB varyantı","Faringeal-servikal-brakiyal zayıflık","Guillain-Barré sendromunun PCB varyantı","GBS'nin faringo-serviko-brakiyal varyantı","Guillain-Barré sendromunun faringo-serviko-brakiyal varyantı"]}},"xrefs":[{"source":"ICD-10","code":"G61.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"8C01.0","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"517146728"},{"source":"MONDO","code":"0016496","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5190783","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98006,"name":"Rare neurologic disease"},"parents":[{"orpha":231416,"name":"Regional variant of Guillain-Barré syndrome","classifications":[181]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Multigenic/multifactorial","Not applicable"],"onset":["All ages"],"prevalence":[],"phenotypes":null}