Postpoliomyelitis syndrome
Synonyms: Postpolio syndrome · Postpoliomyelitic syndrome
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Elderly
- Status
- active
Postpoliomyelitis syndrome (PPS) is a neurologic disorder characterized by the development of new neuromuscular symptoms such as progressive muscular weakness or abnormal muscle fatigability occurring in survivors of the acute paralytic form of poliomyelitis, 15-40 years after recovery from the disease, and that is unexplained by other medical causes. Other manifestations that can occur gradually include generalized fatigue, muscle atrophy, muscle and joint pain, intolerance to cold, and difficulties sleeping, swallowing or breathing.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Postpoliomyelitis syndrome | Postpolio syndrome · Postpoliomyelitic syndrome |
| fr | Syndrome post-poliomyélite | Syndrome post-polio |
| de | Post-Poliomyelitis-Syndrom | PPS · Post-Polio-Syndrom |
| es | Síndrome post-poliomielitis | Síndrome post-polio · Síndrome post-poliomielítico |
| it | Sindrome post-poliomielite | Sindrome post-poliomielitica · Sindrome post-polio |
| nl | Postpoliomyelitissyndroom | Postpoliosyndroom |
| pt | Síndrome pós-poliomielite | Sequela pós-polio · Sequela pós-poliomielíte · Síndrome pós-polio · Síndrome pós-poliomielítico |
| pl | Zespół Post-Polio | Zespół poporażenny |
| cs | Postpoliomyelitický syndrom | Postpolio syndrom · Následky postpoliomyelitidy · Následky dětské obrny · Syndrom po infekční obrně |
| uk | Постполіомієлітний синдром | Наслідки постполіомієліту · Синдром постполіомієліту · Постполіомієлітичний синдром · Наслідки постполіомієліту |
| zh | 脊髓灰质炎后综合征 | 脊髓灰质炎后综合征 |
| tr | Postpoliomiyelit sendromu | Postpolio sekeller · Postpolio sendromu · Postpoliomiyelitik sendrom · Postpoliomiyelit sekeli |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | G14 | E | specific | validated |
| ICD-11 | 8B62 | E | specific | validated |
| MONDO | 0017416 | E | validated | |
| MeSH | D016262 | E | validated | |
| UMLS | C0080040 | E | validated | |
| GARD | 4454 | E | validated | |
| MedDRA | 10036239 | 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:98506Acquired motor neuron disease
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 | 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
17 annotations · validated 2018-01-15| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0001324 | Muscle weakness | |
| HP:0012531 | Pain | |
| Frequent (79-30%) | ||
| HP:0011446 | Abnormality of higher mental function | |
| HP:0002829 | Arthralgia | |
| HP:0003551 | Difficulty climbing stairs | |
| HP:0012378 | Fatigue | |
| HP:0100786 | Hypersomnia | |
| HP:0003326 | Myalgia | |
| Occasional (29-5%) | ||
| HP:0001367 | Abnormal joint morphology | |
| HP:0001260 | Dysarthria | |
| HP:0002015 | Dysphagia | |
| HP:0002380 | Fasciculations | |
| HP:0003394 | Muscle spasm | |
| HP:0002093 | Respiratory insufficiency | |
| HP:0003202 | Skeletal muscle atrophy | |
| HP:0002360 | Sleep abnormality | |
| Very rare (<4-1%) | ||
| HP:0002791 | Hypoventilation | |
Run the differential on the 2 obligate / very frequent terms
Annotation source: 15599928[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/2942
- This record as JSON: /data/d/2942.json