Subacute inflammatory demyelinating polyneuropathy
Synonyms: Subacute inflammatory demyelinating polyradiculoneuropathy
- Type
- Disease
- Group
- Disorder
- Age of onset
- All ages
- Status
- active
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).
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Subacute inflammatory demyelinating polyneuropathy | Subacute inflammatory demyelinating polyradiculoneuropathy |
| fr | Polyradiculonévrite dysimmunitaire subaiguë | |
| de | Polyneuropathie, demyelinisierende inflammatorische subakute | Polyradikuloneuropathie, demyelinisierende entzündliche subakute |
| es | Polineuropatía desmielinizante inflamatoria subaguda | Polirradiculoneuropatía desmielinizante inflamatoria subaguda |
| it | Polineuropatia infiammatoria demielinizzante subacuta | Poliradicoloneuropatia infiammatoria demielinizzante subacuta |
| nl | Subacute inflammatoire demyeliniserende polyneuropathie | Subacute inflammatoire demyeliniserende polyradiculoneuropathie |
| pt | Polineuropatia inflamatória desmielinizante subaguda | |
| pl | Podostra zapalna polineuropatia demielinizacyjna | Podostra zapalna poliradikuloneuropatia demielinizacyjna |
| cs | Subakutní zánětlivá demyelinizační polyneuropatie | Subakutní zánětlivá demyelinizační polyradikuloneuropatie |
| uk | Підгостра запальна демієлінізуюча полінейропатія | Підгостра запальна демієлінізуюча полірадикулонейропатія |
| zh | 亚急性炎性脱髓鞘性多发性神经病 | 亚急性炎性脱髓鞘性多发性神经根神经病 |
| tr | Subakut inflamatuar demiyelinizan polinöropati | Subakut inflamatuar demiyelinizan poliradikülonöropati |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | G61.8 | NTBT | attributed | validated |
| ICD-11 | 8C01.2 | E | specific | validated |
| MONDO | 0016102 | E | validated | |
| UMLS | C0456517 | E | validated | |
| MedDRA | 10081726 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:207038Acute and subacute inflammatory demyelinating polyneuropathy
rare neurological diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
40 annotations · validated 2021-12-07| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0007377 | Abnormality of somatosensory evoked potentials | |
| HP:0003431 | Decreased motor nerve conduction velocity | |
| HP:0000762 | Decreased nerve conduction velocity | |
| HP:0002922 | Increased CSF protein concentration | |
| HP:0001324 | Muscle weakness | |
| Frequent (79-30%) | ||
| HP:0001284 | Areflexia | |
| HP:0003447 | Axonal loss | |
| HP:0007078 | Decreased amplitude of sensory action potentials | |
| HP:0007220 | Demyelinating motor neuropathy | |
| HP:0003551 | Difficulty climbing stairs | |
| HP:0006881 | Diffuse peripheral demyelination | |
| HP:0003565 | Elevated erythrocyte sedimentation rate | |
| HP:0002359 | Frequent falls | |
| HP:0004302 | Functional motor deficit | |
| HP:0001288 | Gait disturbance | |
| HP:0001974 | Increased total leukocyte count | |
| HP:0012531 | Pain | |
| HP:0011096 | Peripheral demyelination | |
| HP:0007141 | Sensorimotor neuropathy | |
| HP:0003474 | Somatic sensory dysfunction | |
| HP:0007262 | Symmetric peripheral demyelination | |
| Occasional (29-5%) | ||
| HP:0001430 | Abnormality of the calf musculature | |
| HP:0006844 | Absent patellar reflexes | |
| HP:0001266 | Choreoathetosis | |
| HP:0007230 | Decreased distal sensory nerve action potential | |
| HP:0003448 | Decreased sensory nerve conduction velocity | |
| HP:0002460 | Distal muscle weakness | |
| HP:0002936 | Distal sensory impairment | |
| HP:0003237 | Increased circulating IgG level | |
| HP:0010505 | Limitation of movement at ankles | |
| HP:0006376 | Limited elbow flexion | |
| HP:0008800 | Limited hip movement | |
| HP:0012078 | Motor conduction block | |
| HP:0002403 | Positive Romberg sign | |
| HP:0032169 | Severe infection | |
| HP:0003376 | Steppage gait | |
| HP:0001337 | Tremor | |
| Very rare (<4-1%) | ||
| HP:0002270 | Abnormality of the autonomic nervous system | |
| HP:0002086 | Abnormality of the respiratory system | |
| HP:0006824 | Cranial nerve paralysis | |
Run the differential on the 5 obligate / very frequent terms
Annotation source: 25299280[PMID]_12929187[PMID]_22005162[PMID]_15911813[PMID]_31307604[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/206594
- This record as JSON: /data/d/206594.json