{"orpha":231450,"name":"Acute pure sensory neuropathy","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Acute pure sensory GBS","Acute pure sensory Guillain-Barré syndrome"],"definition":"A rare, acquired, demyelinating neuropathy disease characterized by acute, symmetric, monophasic sensory neuropathy without motor involvement, typically manifesting with numbness in the distal lower limbs which progressively extends to all the limb, tingling sensation in the distal lower limbs, generalized areflexia, and unsteady gait, as well as clumsiness of the upper limbs, pseudoathetosis and loss of vibration sense.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-231450/","orphanet_url":"https://www.orpha.net/en/disease/detail/231450","names":{"fr":{"name":"Neuropathie sensorielle pure aiguë","synonyms":["SGB sensoriel pur aigu","Syndrome de Guillain-Barré sensoriel pur aigu"],"definition":"Neuropathie périphérique dysimmunitaire acquise rare caractérisée par une neuropathie sensorielle aiguë, symétrique et monophasique sans atteinte motrice, se manifestant typiquement par un engourdissement de la partie distale des membres inférieurs qui s'étend progressivement à tout le membre, des picotements dans la partie distale des membres inférieurs, une aréflexie généralisée et une démarche instable, ainsi qu'une maladresse des membres supérieurs, une pseudo-athétose et une perte du sens de la vibration."},"de":{"name":"Neuronopathie, akute reine sensorische","synonyms":["Guillain-Barré-Syndrom, akutes reines sensorisches"]},"es":{"name":"Neuropatía sensitiva pura aguda","synonyms":["GBS sensitivo puro agudo","Síndrome de Guillain-Barré sensitivo puro agudo"],"definition":"Es una neuropatía desmielinizante, adquirida y poco frecuente, caracterizada por neuropatía sensitiva monofásica, aguda y simétrica, sin afectación motora, que se manifiesta generalmente con adormecimiento de los miembros inferiores distales que se extiende progresivamente a todos los miembros, sensación de hormigueo distal en las extremidades inferiores, arreflexia generalizada y marcha inestable, así como torpeza de las extremidades superiores, pseudoatetosis y pérdida de la sensibilidad vibratoria."},"it":{"name":"Neuropatia sensoriale acuta pura","synonyms":["GBS sensoriale pura acuta","Sindrome di Guillain-Barré sensoriale pura acuta"],"definition":"La neuropatia sensoriale acuta pura è una neuropatia demielinizzante acquisita rara, caratterizzata da neuropatia sensoriale monofasica simmetrica acuta, senza interessamento motorio. Di solito esordisce con insensibilità nella porzione distale degli arti inferiori, che si estende progressivamente a tutto l'arto, formicolio nella porzione distale degli arti inferiori, areflessia generalizzata, andatura instabile, goffaggine degli arti superiori, pseudoatetosi e perdita del senso della vibrazione."},"nl":{"name":"Acute zuivere sensorische neuropathie","synonyms":["Acuut zuiver sensorisch GBS","Acuut zuiver sensorisch syndroom van Guillain-Barré"],"definition":"Een zeldzame, verworven, demyeliniserende neuropathie die gekarakteriseerd wort door acute, symmetrische, monofasische sensorische neuropathie zonder motorische betrokkenheid, en zich doorgaans manifesteert met gevoelloosheid in de distale onderste ledematen die progressief uitbreid naar alle ledematen, tintelingen in de distale onderste ledematen, gegeneraliseerde areflexie, en onvaste gang, alsook onhandigheid van de bovenste ledematen, pseudo-athetose en verlies van zintuiglijke waarneming van vibratie."},"pt":{"name":"Neuropatia sensitiva pura aguda","synonyms":["GBS sensitiva pura aguda","Síndrome Guillain-Barré sensitiva pura aguda"]},"pl":{"name":"Ostra izolowana neuropatia czuciowa","synonyms":["Ostry czysty czuciowy GBS","Ostry czysty czuciowy zespół Guillaina i Barrégo"],"definition":"*Ostra izolowana neuropatia czuciowa jest rzadką, nabytą, neuropatią demielinizacyjną, która charakteryzuje się ostrą, symetryczną, jednofazową neuropatią czuciową bez zajęcia włókien ruchowych. Zazwyczaj objawia się drętwieniem w dystalnych odcinkach kończyn dolnych, które stopniowo rozszerza się na całą kończynę, mrowieniem w dystalnych odcinkach kończyn dolnych, uogólnionym brakiem odruchów i chwiejnym chodem, a także niezbornością kończyn górnych, pseudoatetozą i brakiem czucia wibracji."},"cs":{"name":"Akutní čistá sensorická neuropatie","synonyms":["Akutní čistý sensorický GBS","Akutní čistý sensorický Guillainův-Barrého syndrom","Akutní čistý sensorický Guillain-Barrého syndrom"]},"uk":{"name":"Гостра ізольована сенсорна нейропатія","synonyms":["Гострий чисто сенсорний GBS","Гострий чисто сенсорний синдром Гійєна-Барре"],"definition":"A rare, acquired, demyelinating neuropathy disease characterized by acute, symmetric, monophasic sensory neuropathy without motor involvement, typically manifesting with numbness in the distal lower limbs which progressively extends to all the limb, tingling sensation in the distal lower limbs, generalized areflexia, and unsteady gait, as well as clumsiness of the upper limbs, pseudoathetosis and loss of vibration sense."},"zh":{"name":"急性纯感觉神经病","synonyms":["急性纯感觉性吉兰-巴雷综合征"],"definition":"一种罕见的后天性脱髓鞘神经病变，特征为急性对称单相感觉神经病变，无运动受累，典型表现为下肢远端麻木，逐渐延伸至四肢，下肢远端有刺痛感，全身无活动能力，步态不稳，上肢动作笨拙，假性手足徐动，振动感缺失。"},"tr":{"name":"Akut saf duyusal nöropati","synonyms":["Akut saf duyusal GBS","Akut saf duyusal Guillain-Barré sendromu"]}},"xrefs":[{"source":"ICD-10","code":"G61.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0016498","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5190881","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98006,"name":"Rare neurologic disease"},"parents":[{"orpha":231419,"name":"Functional variant of Guillain-Barré syndrome","classifications":[181]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Multigenic/multifactorial","Not applicable"],"onset":["All ages"],"prevalence":[],"phenotypes":null}