{"orpha":231466,"name":"Acute sensory ataxic neuropathy","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["ASAN","Acute sensory ataxic GBS","Acute sensory ataxic Guillain-Barré syndrome"],"definition":"A rare variant of Guillain-Barré syndrome characterized by acute onset monophasic sensory neuropathy with diminished or absent tendon reflexes, loss of proprioception, positive Romberg sign and nerve conduction features of demyelination. It presents several weeks after acute infection with paresthesias, ataxia and neuropathic pain.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-231466/","orphanet_url":"https://www.orpha.net/en/disease/detail/231466","names":{"fr":{"name":"Neuropathie ataxique sensorielle aiguë","synonyms":["ASAN","SGB ataxique sensoriel aigu","Syndrome de Guillain-Barré ataxique sensoriel aigu"],"definition":"Un variant rare du syndrome de Guillain-Barré caractérisé par une neuropathie sensitive monophasique aigüe associée à des réflexes tendineux réduits ou absents, une perte de la proprioception, un signe de Romberg positif et des vitesses de conduction nerveuse évoquant une démyélinisation. La condition se présente plusieurs semaines après une infection aigüe par une paresthésie, une ataxie et une douleur neuropathique."},"de":{"name":"Neuropathie, akute ataxische sensorische","synonyms":["ASAN","Guillain-Barré-Syndrom, akutes ataxische sensorisches"],"definition":"Eine seltene Variante des Guillain-Barré-Syndroms, gekennzeichnet durch eine akut einsetzende monophasische sensorische Neuropathie mit verminderten oder fehlenden Sehnenreflexen, Verlust der Propriozeption, positivem Romberg-Zeichen und Nervenleitungsmerkmalen einer Demyelinisierung. Sie tritt mehrere Wochen nach der akuten Infektion mit Parästhesien, Ataxie und neuropathischen Schmerzen auf."},"es":{"name":"Neuropatía atáxica sensitiva aguda","synonyms":["ASAN","GBS atáxico sensitivo agudo","Síndrome de Guillain-Barré atáxico sensitivo agudo"],"definition":"Es una variante poco frecuente del síndrome de Guillain-Barré caracterizada por una neuropatía sensitiva monofásica de presentación aguda con reflejos tendinosos disminuidos o ausentes, pérdida de propiocepción, signo de Romberg y estudios de conducción nerviosa con características desmielinizantes. Se presenta varias semanas después de la infección aguda con parestesias, ataxia y dolor neuropático."},"it":{"name":"Neuropatia atassica sensoriale acuta","synonyms":["ASAN","GBS atassica sensoriale acuta","Sindrome atassica sensoriale acuta di Guillain-Barré"],"definition":"La neuropatia atassica sensoriale acuta è una variante rara della sindrome di Guillain-Barré, caratterizzata da neuropatia sensoriale monofasica ad esordio acuto, associata ad una riduzione o all'assenza dei riflessi tendinei, alla perdita della propriocezione, al segno di Romberg e a valori di conduzione nervosa indicativi di una demielinizzazione. La malattia esordisce diverse settimane dopo un'infezione acuta con parestesie, atassia e dolore neuropatico."},"nl":{"name":"Acute sensorische ataxische neuropathie","synonyms":["Acuut sensorisch ataxisch syndroom van Guillain-Barré","ASAN","Acuut sensorisch ataxisch GBS"],"definition":"Een zeldzame variant van syndroom van Guillain-Barré, gekarakteriseerd door acuut aanvangende monofasische sensorische neuropathie met verminderde of afwezige peesreflexen, verlies van proprioceptie, positieve test van Romberg, en zenuwgeleiding kenmerkend voor demyelinisatie. De ziekte manifesteert zich enkele weken na acute infectie met paresthesieën, ataxie en neuropathische pijn."},"pt":{"name":"Neuropatia atáxica sensorial aguda"},"pl":{"name":"Ostra ataksja czuciowa","synonyms":["ASAN","Ostra ataksja czuciowa GBS","Ostry czuciowy ataktyczny zespół Guillaina i Barrégo"],"definition":"*Ostra neuropatia czuciowa z ataksją jest rzadkim wariantem zespołu Guillaina i Barrégo charakteryzującym się ostrym początkiem jednofazowej neuropatii czuciowej z osłabionymi odruchami ścięgnistymi lub ich brakiem, utratą czucia głębokiego mięśni, dodatnim objawem Romberga i objawami zaburzonego przewodnictwa nerwowego w wyniku demielinizacji. Występuje kilka tygodni po ostrej infekcji pod postacią parastezji, ataksji i bólu neuropatycznego."},"cs":{"name":"Akutní senzorická ataxická neuropatie","synonyms":["ASAN","Akutní senzorický ataxický GBS","Akutní senzorický ataxický Guillainův-Barrého syndrom","Akutní senzorický ataxický Guillain-Barrého syndrom"]},"uk":{"name":"Гостра сенсорна атаксична нейропатія","synonyms":["Гострий сенсорний атаксичний GBS","Гострий сенсорний атаксичний синдром Гійєна-Барре","ASAN"],"definition":"A rare variant of Guillain-Barré syndrome characterized by acute onset monophasic sensory neuropathy with diminished or absent tendon reflexes, loss of proprioception, positive Romberg sign and nerve conduction features of demyelination. It presents several weeks after acute infection with paresthesias, ataxia and neuropathic pain."},"zh":{"name":"急性感觉共济失调性神经病","synonyms":["急性感觉共济失调性吉兰-巴雷综合征"],"definition":"一种罕见的吉兰-巴雷综合征，其特征为急性发作的单相感觉神经病变，伴有腱反射减弱或缺失、本体感觉丧失、Romberg征阳性和脱髓鞘神经传导特征。在急性感染数周后出现感觉异常、共济失调和神经病理性疼痛症状。"},"tr":{"name":"Akut duyusal ataksik nöropati","synonyms":["ASAN","Akut duyusal ataksik GBS","Akut duyusal ataksik Guillain-Barré sendromu"]}},"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":"1951716831"},{"source":"MONDO","code":"0016500","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4707661","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}