ORPHA:2932
Chronic inflammatory demyelinating polyneuropathy
Synonyms: CIDP · Chronic inflammatory demyelinating polyradiculoneuropathy
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Childhood, Elderly
- Status
- active
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.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Chronic inflammatory demyelinating polyneuropathy | CIDP · Chronic inflammatory demyelinating polyradiculoneuropathy |
| fr | Polyradiculonévrite inflammatoire démyélinisante chronique | |
| de | Chronische inflammatorische demyelinisierende Polyneuropathie | CIDP · Chronische demyelinisierende inflammatorische Polyradikuloneuropathie |
| es | Polineuropatía desmielinizante inflamatoria crónica | CIDP · Polirradiculoneuropatía desmielinizante inflamatoria crónica |
| it | Polineuropatia demielinizzante infiammatoria cronica | CIDP · Poliradiculoneuropatia demielinizzante infiammatoria cronica |
| nl | Chronische inflammatoire demyeliniserende polyneuropathie | CIDP · Chronische inflammatoire demyeliniserende polyradiculoneuropathie |
| pt | Polineuropatia desmielinizante inflamatória crónica | |
| pl | Przewlekła zapalna polineuropatia demielinizacyjna | CIDP · Przewlekła zapalna poliradikuloneuropatia demielinizacyjna |
| cs | Chronická zánětlivá demyelinizační neuropatie | CIDP · Chronická zánětlivá demyelinizační polyradikuloneuropatie |
| uk | Хронічна запальна демієлінізуюча полінейропатія | Хронічна запальна демієлінізуюча полірадикулонейропатія · CIDP |
| zh | 慢性炎症性脱髓鞘性多发性神经病 | 慢性感染性多发性神经根性脱髓鞘性神经病 |
| tr | Kronik inflamatuar demiyelinizan polinöropati | CIDP · Kronik inflamatuar demiyelinizan poliradikülonöropati |
Definition in:
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Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | G61.8 | NTBT | attributed | validated |
| ICD-11 | 8C01.3 | E | specific | validated |
| MONDO | 0006702 | E | validated | |
| MeSH | D020277 | E | validated | |
| UMLS | C0393819 | E | validated | |
| GARD | 6102 | E | validated | |
| MedDRA | 10057645 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (2):
- ORPHA:208978Chronic polyradiculoneuropathy
rare neurological diseases - ORPHA:182064Rare neuroinflammatory or neuroimmunological disease
rare neurological diseases
Children (1):
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Genes
0 directOrphanet records no gene directly on this code.
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Prevalence and incidence
10 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 1-9 / 100 000 | 3.7 / 100 000 | Europe | Value and class | Not yet validated | ORPHANET |
| Annual incidence | 1-9 / 1 000 000 | 0.36 / 100 000 | Italy | Value and class | Validated | 17494979[PMID] |
| Point prevalence | 1-9 / 100 000 | 2.6 / 100 000 | United Kingdom | Value and class | Not yet validated | 23679015[PMID]_10209187[PMID]_19260065[PMID] |
| Point prevalence | 1-9 / 100 000 | 3.86 / 100 000 | Italy | Value and class | Validated | 17494979[PMID] |
| Point prevalence | 1-9 / 100 000 | 7.7 / 100 000 | Norway | Value and class | Validated | 11284994[PMID] |
| Point prevalence | 1-5 / 10 000 | 12 / 100 000 | Egypt | Value and class | Validated | 23146298[PMID] |
| Annual incidence | 1-9 / 1 000 000 | 0.48 / 100 000 | Japan | Value and class | Validated | 18223015[PMID] |
| Point prevalence | 1-9 / 100 000 | 1.61 / 100 000 | Japan | Value and class | Validated | 18223015[PMID] |
| Annual incidence | 1-9 / 1 000 000 | 0.15 / 100 000 | Australia | Value and class | Validated | 10589544[PMID] |
| Point prevalence | 1-9 / 100 000 | 1.9 / 100 000 | Australia | Value and class | Validated | 10589544[PMID] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
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HPO phenotypes
17 annotations · validated 2017-07-10| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0040129 | Abnormal nerve conduction velocity | Diagnostic criterion |
| HP:0001284 | Areflexia | |
| HP:0000762 | Decreased nerve conduction velocity | Diagnostic criterion |
| HP:0030200 | Fatiguable weakness of proximal limb muscles | Diagnostic criterion |
| HP:0012078 | Motor conduction block | |
| HP:0003401 | Paresthesia | |
| HP:0011096 | Peripheral demyelination | |
| HP:0009830 | Peripheral neuropathy | |
| HP:0003481 | Segmental peripheral demyelination/remyelination | |
| HP:0010871 | Sensory ataxia | |
| HP:0003474 | Somatic sensory dysfunction | |
| HP:0002317 | Unsteady gait | |
| Frequent (79-30%) | ||
| HP:0003551 | Difficulty climbing stairs | |
| HP:0002527 | Falls | |
| HP:0001288 | Gait disturbance | |
| HP:0030237 | Hand muscle weakness | |
| Occasional (29-5%) | ||
| HP:0010833 | Spontaneous pain sensation | |
Run the differential on the 12 obligate / very frequent terms
Annotation source: 27902997[PMID]
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/2932
- This record as JSON: /data/d/2932.json