Paramyotonia congenita of Von Eulenburg
Paramyotonia congenita of Von Eulenburg
Definition
Paramyotonia congenita of Von Eulenburg is characterized by exercise- or cold-induced myotonia and muscle weakness. Prevalence is unknown. The syndrome is nonprogressive and is transmitted as an autosomal dominant trait. It is caused by mutations in the gene encoding the alpha subunit of the type IV voltage-gated sodium channel (SCN4A; 17q23.3).
Also known as paramyotonia congenita, paramyotonia congenita of Von Eulenburg — per MONDO
Also identified as
- DOID 0111538 per MONDO
- ICD9 359.29 per MONDO
- NCIT C122790 per MONDO
- OMIM 168300 per MONDO
- Orphanet 684 per MONDO
- SCTID 41574007 per MONDO
- UMLS C0221055 per MONDO
Affected Organs
| Organ | Involvement | Source |
|---|---|---|
| Musculature | Disease Has Associated Anatomic Site | NCIT · CC BY 4.0 |
| Musculature | Disease Has Primary Anatomic Site | NCIT · CC BY 4.0 |
| Musculoskeletal system | Disease Has Associated Anatomic Site | NCIT · CC BY 4.0 |
| Musculoskeletal system | Disease Has Primary Anatomic Site | NCIT · CC BY 4.0 |