{"orpha":2932,"name":"Chronic inflammatory demyelinating polyneuropathy","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["CIDP","Chronic inflammatory demyelinating polyradiculoneuropathy"],"definition":"A rare chronic monophasic progressive or relapsing symmetric sensorimotor disorder characterized by progressive muscular weakness with impaired sensation, absent or diminished tendon reflexes, electrodiagnostic features suggestive of demyelination and occasional elevated cerebrospinal fluid (CSF) protein level.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-2932/","orphanet_url":"https://www.orpha.net/en/disease/detail/2932","names":{"fr":{"name":"Polyradiculonévrite inflammatoire démyélinisante chronique","definition":"Maladie sensorimotrice symétrique rare, chronique, monophasique, évoluant de manière progressive ou par poussées, caractérisée par une faiblesse musculaire progressive accompagnée d'une altération de la sensibilité, d'une absence ou d'une diminution des réflexes tendineux, de signes électrophysiologiques évocateurs d'une démyélinisation et, parfois, d'une élévation du taux de protéines dans le liquide cérébro-spinal (LCS)."},"de":{"name":"Chronische inflammatorische demyelinisierende Polyneuropathie","synonyms":["CIDP","Chronische demyelinisierende inflammatorische Polyradikuloneuropathie"],"definition":"Die Chronisch-entzündliche demyelinisierende Polyneuropathie (CIDP) ist eine chronische, monophasische, progrediente oder rezidivierende, symmetrische sensomotorische Erkrankung. Kennzeichend sind fortschreitende Muskelschwäche mit gestörter Sensorik, fehlende oder reduzierte Sehnenreflexe und erhöhte Eiweißkonzentration in der Zerebrospinalflüssigkeit (CSF)."},"es":{"name":"Polineuropatía desmielinizante inflamatoria crónica","synonyms":["CIDP","Polirradiculoneuropatía desmielinizante inflamatoria crónica"],"definition":"Es una enfermedad sensoriomotora simétrica crónica monofásica, progresiva o recurrente, caracterizada por debilidad muscular progresiva con sensaciones alteradas, reflejos tendinosos ausentes o disminuidos y un nivel elevado de proteínas en el líquido cefalorraquídeo (LCR)."},"it":{"name":"Polineuropatia demielinizzante infiammatoria cronica","synonyms":["CIDP","Poliradiculoneuropatia demielinizzante infiammatoria cronica"]},"nl":{"name":"Chronische inflammatoire demyeliniserende polyneuropathie","synonyms":["CIDP","Chronische inflammatoire demyeliniserende polyradiculoneuropathie"],"definition":"Een chronische, monofasische, progressieve of recidiverende, symmetrische sensorimotorische aandoening, gekarakteriseerd door progressieve musculaire zwakte met verstoorde zintuiglijke gewaarwording, afwezige of verminderde peesreflexen, en toename van eiwitten in cerebrospinaal vocht (CSV)."},"pt":{"name":"Polineuropatia desmielinizante inflamatória crónica","definition":"A polineuropatia desmielinizante inflamatória crónica (PDIC) é uma patologia progressiva monofásica crónica, ou sensório-motora simétrica recidivante, caracterizada por fraqueza muscular progressiva com compromisso da sensibilidade tactil, reflexos ausentes ou diminuídos e proteínas elevadas no líquido cefalorraquidiano (CSF)."},"pl":{"name":"Przewlekła zapalna polineuropatia demielinizacyjna","synonyms":["CIDP","Przewlekła zapalna poliradikuloneuropatia demielinizacyjna"]},"cs":{"name":"Chronická zánětlivá demyelinizační neuropatie","synonyms":["CIDP","Chronická zánětlivá demyelinizační polyradikuloneuropatie"]},"uk":{"name":"Хронічна запальна демієлінізуюча полінейропатія","synonyms":["Хронічна запальна демієлінізуюча полірадикулонейропатія","CIDP"],"definition":"A chronic monophasic, progressive or relapsing symmetric sensorimotor disorder characterized by progressive muscular weakness with impaired sensation, absent or diminished tendon reflexes and elevated cerebrospinal fluid (CSF) proteins."},"zh":{"name":"慢性炎症性脱髓鞘性多发性神经病","synonyms":["慢性感染性多发性神经根性脱髓鞘性神经病"]},"tr":{"name":"Kronik inflamatuar demiyelinizan polinöropati","synonyms":["CIDP","Kronik inflamatuar demiyelinizan poliradikülonöropati"]}},"xrefs":[{"source":"ICD-10","code":"G61.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"8C01.3","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"224318510"},{"source":"MONDO","code":"0006702","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D020277","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0393819","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"6102","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10057645","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98006,"name":"Rare neurologic disease"},"parents":[{"orpha":208978,"name":"Chronic polyradiculoneuropathy","classifications":[181]},{"orpha":182064,"name":"Rare neuroinflammatory or neuroimmunological disease","classifications":[181]}],"children":[{"orpha":48162,"name":"Lewis-Sumner syndrome","type":"Clinical subtype"}],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adolescent","Adult","Childhood","Elderly"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":3.7,"area":"Europe","status":"Not yet validated","source":"ORPHANET"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.36,"area":"Italy","status":"Validated","source":"17494979[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":2.6,"area":"United Kingdom","status":"Not yet validated","source":"23679015[PMID]_10209187[PMID]_19260065[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":3.86,"area":"Italy","status":"Validated","source":"17494979[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":7.7,"area":"Norway","status":"Validated","source":"11284994[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-5 / 10 000","value":12.0,"area":"Egypt","status":"Validated","source":"23146298[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.48,"area":"Japan","status":"Validated","source":"18223015[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":1.61,"area":"Japan","status":"Validated","source":"18223015[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.15,"area":"Australia","status":"Validated","source":"10589544[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":1.9,"area":"Australia","status":"Validated","source":"10589544[PMID]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2017-07-10","source":"27902997[PMID]","terms":[{"hpo":"HP:0040129","label":"Abnormal nerve conduction velocity","frequency":"Very frequent (99-80%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0001284","label":"Areflexia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000762","label":"Decreased nerve conduction velocity","frequency":"Very frequent (99-80%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0030200","label":"Fatiguable weakness of proximal limb muscles","frequency":"Very frequent (99-80%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0012078","label":"Motor conduction block","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0003401","label":"Paresthesia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0011096","label":"Peripheral demyelination","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0009830","label":"Peripheral neuropathy","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0003481","label":"Segmental peripheral demyelination/remyelination","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0010871","label":"Sensory ataxia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0003474","label":"Somatic sensory dysfunction","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002317","label":"Unsteady gait","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0003551","label":"Difficulty climbing stairs","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002527","label":"Falls","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001288","label":"Gait disturbance","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030237","label":"Hand muscle weakness","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0010833","label":"Spontaneous pain sensation","frequency":"Occasional (29-5%)","criteria":null}]}}