{"orpha":206594,"name":"Subacute inflammatory demyelinating polyneuropathy","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Subacute inflammatory demyelinating polyradiculoneuropathy"],"definition":"A rare neuroinflammatory disease characterized by muscular weakness with impaired sensation, absent or diminished tendon reflexes and elevated cerebrospinal fluid (CSF) protein content. Subacute inflammatory demyelinating polyneuropathy (SIDP) is an intermediate form between Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP).","note":null,"url":"https://raremap.alftech.space/d/ORPHA-206594/","orphanet_url":"https://www.orpha.net/en/disease/detail/206594","names":{"fr":{"name":"Polyradiculonévrite dysimmunitaire subaiguë","definition":"Maladie neuro-inflammatoire rare caractérisée par une faiblesse musculaire accompagnée d'une sensitivité altérée, des réflexes tendineux absents ou diminués et d'un taux de protéines du liquide cérébro-spinal (LCS) élevé. La polyneuropathie dysimmunitaire subaiguë (PDS) est une forme intermédiaire entre le syndrome de Guillain-Barré (SGB) et la polyradiculonévrite inflammatoire démyélinisante chronique (PIDC)."},"de":{"name":"Polyneuropathie, demyelinisierende inflammatorische subakute","synonyms":["Polyradikuloneuropathie, demyelinisierende entzündliche subakute"],"definition":"Die Subakut-entzündliche demyelinisierende Polyneuropathie (SIDP) ist eine subakute progrediente, symmetrische, sensorische und/oder motorische Erkrankung. Charakteristische Symptome sind Muskelschwäche mit eingeschränkter Sensorik, fehlende oder abgeschwächte Sehnenreflexe und erhöhte Eiweißkonzentration in der Zerebrospinalflüssigkeit (CSF). Die SIDP ist eine intermediäre Form zwischen Guillain-Barré-Syndrom (GBS) und Chronisch-entzündlicher demyelinisierender Polyneuropathie (CIDP; s. dort). Die Zeit, zu der die Krankheit den höchsten Schweregrad (Nadir) erreicht und der anschließende Verlauf unterscheidet die SIDP von GBS und CIDP."},"es":{"name":"Polineuropatía desmielinizante inflamatoria subaguda","synonyms":["Polirradiculoneuropatía desmielinizante inflamatoria subaguda"],"definition":"La polineuropatía desmielinizante inflamatoria subaguda (SIDP) es un trastorno sensorial y/o motor subagudo progresivo y simétrico caracterizado por debilidad muscular con falta de sensibilidad, reflejos tendinosos ausentes o disminuidos y elevación del nivel de proteínas en el líquido cefalorraquídeo (LCR). La SIDP es una forma intermedia entre el síndrome de Guillain-Barré (SGB) y la polineuropatía desmielinizante inflamatoria crónica (CIDP; ver estos términos)."},"it":{"name":"Polineuropatia infiammatoria demielinizzante subacuta","synonyms":["Poliradicoloneuropatia infiammatoria demielinizzante subacuta"],"definition":"La polineuropatia demielinizzante infiammatoria subacuta (SIDP) è una malattia motoria e/o sensoriale simmetrica progressiva subacuta, caratterizzata da debolezza muscolare con perdita della sensibilità, assenza o diminuzione dei riflessi tendinei e aumento delle proteine del liquido cerebrospinale (LCS). La SIDP rappresenta una forma intermedia tra la sindrome di Guillain-Barré (GBS) e la polineuropatia demielinizzante infiammatoria cronica (CIDP; si vedano questi termini)."},"nl":{"name":"Subacute inflammatoire demyeliniserende polyneuropathie","synonyms":["Subacute inflammatoire demyeliniserende polyradiculoneuropathie"],"definition":"Subacute inflammatoire demyeliniserende polyneuropathie (SIDP) is een subacute progressieve symmetrische sensorische en/of motorische aandoening, gekenmerkt door spierzwakte met verstoorde sensatie, afwezige of verminderde peesreflexen, en verhoogde eiwitgehaltes in cerebrospinaal vocht (CSV). SIDP is een intermediaire vorm tussen syndroom van Guillain-Barré (GBS) en chronische inflammatoire demyeliniserende polyneuropathie (CIDP)."},"pt":{"name":"Polineuropatia inflamatória desmielinizante subaguda","definition":"A polineuropatia desmielinizante inflamatória subaguda (SIDP) é uma patologia sensorial simétrica progressiva subaguda e/ou motora caracterizada por fraqueza muscular e tacto diminuido, reflexos tendinosos ausentes ou diminuídos e líquido cefalorraquidiano com proteínas elevadas (CSF). A SIDP é uma forma intermédia entre o síndrome de Guillain-Barré (GBS) e polineuropatia desmielinizante inflamatória crónica (CIDP; ver estes termos). O tempo decorrido até atingir a gravidade máxima (nadir) e o curso subsequente permite diferenciar a SIDP do GBS e da CIDP."},"pl":{"name":"Podostra zapalna polineuropatia demielinizacyjna","synonyms":["Podostra zapalna poliradikuloneuropatia demielinizacyjna"]},"cs":{"name":"Subakutní zánětlivá demyelinizační polyneuropatie","synonyms":["Subakutní zánětlivá demyelinizační polyradikuloneuropatie"]},"uk":{"name":"Підгостра запальна демієлінізуюча полінейропатія","synonyms":["Підгостра запальна демієлінізуюча полірадикулонейропатія"],"definition":"Subacute inflammatory demyelinating polyneuropathy (SIDP) is a subacute progressive symmetric sensorial and/or motor disorder characterized by muscular weakness with impaired sensation, absent or diminished tendon reflexes and elevated cerebrospinal fluid (CSF) proteins. SIDP is an intermediate form between Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP; see these terms)."},"zh":{"name":"亚急性炎性脱髓鞘性多发性神经病","synonyms":["亚急性炎性脱髓鞘性多发性神经根神经病"],"definition":"亚急性炎性脱髓鞘性多发性神经病(SIDP)是一种亚急性的进行性对称性感觉障碍和（或）运动障碍，其主要临床表现为肌无力伴感觉减退，肌腱反射减弱或消失，脑脊液(CSF)蛋白升高。SIDP是介于吉兰-巴雷综合征(GBS)和慢性炎性脱髓鞘性多发性神经病(CIDP;见术语)之间。"},"tr":{"name":"Subakut inflamatuar demiyelinizan polinöropati","synonyms":["Subakut inflamatuar demiyelinizan poliradikülonöropati"]}},"xrefs":[{"source":"ICD-10","code":"G61.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"8C01.2","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1692167541"},{"source":"MONDO","code":"0016102","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0456517","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10081726","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98006,"name":"Rare neurologic disease"},"parents":[{"orpha":207038,"name":"Acute and subacute inflammatory demyelinating polyneuropathy","classifications":[181]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2021-12-07","source":"25299280[PMID]_12929187[PMID]_22005162[PMID]_15911813[PMID]_31307604[PMID]","terms":[{"hpo":"HP:0007377","label":"Abnormality of somatosensory evoked potentials","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0003431","label":"Decreased motor nerve conduction velocity","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000762","label":"Decreased nerve conduction velocity","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002922","label":"Increased CSF protein concentration","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001324","label":"Muscle weakness","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001284","label":"Areflexia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003447","label":"Axonal loss","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0007078","label":"Decreased amplitude of sensory action potentials","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0007220","label":"Demyelinating motor neuropathy","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003551","label":"Difficulty climbing stairs","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0006881","label":"Diffuse peripheral demyelination","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003565","label":"Elevated erythrocyte sedimentation rate","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002359","label":"Frequent falls","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0004302","label":"Functional motor deficit","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001288","label":"Gait disturbance","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001974","label":"Increased total leukocyte count","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012531","label":"Pain","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011096","label":"Peripheral demyelination","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0007141","label":"Sensorimotor neuropathy","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003474","label":"Somatic sensory dysfunction","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0007262","label":"Symmetric peripheral demyelination","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001430","label":"Abnormality of the calf musculature","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0006844","label":"Absent patellar reflexes","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001266","label":"Choreoathetosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0007230","label":"Decreased distal sensory nerve action potential","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003448","label":"Decreased sensory nerve conduction velocity","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002460","label":"Distal muscle weakness","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002936","label":"Distal sensory impairment","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003237","label":"Increased circulating IgG level","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0010505","label":"Limitation of movement at ankles","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0006376","label":"Limited elbow flexion","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0008800","label":"Limited hip movement","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012078","label":"Motor conduction block","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002403","label":"Positive Romberg sign","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0032169","label":"Severe infection","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003376","label":"Steppage gait","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001337","label":"Tremor","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002270","label":"Abnormality of the autonomic nervous system","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002086","label":"Abnormality of the respiratory system","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0006824","label":"Cranial nerve paralysis","frequency":"Very rare (<4-1%)","criteria":null}]}}