Walker-Dyson Syndrome
| Classification according to ICD-10 | |
|---|---|
| Q13.1 | Lack of iris (congenital) |
| ICD-10 online (WHO version 2019) | |
The Walker-Dyson syndrome is a very rare congenital disease with the main features of aniridia and mental retardation .
Synonyms are: aniridia - mental retardation
The name refers to the authors of the first description from 1974 by FA Walker and C. Dyson.
distribution
The frequency is given as less than 1 in 1,000,000, inheritance is autosomal dominant . So far the syndrome has been described in one family. The cause is not yet known.
Clinical manifestations
Clinical criteria are:
- slight intellectual disability
- Aniridia
- Lens dislocation
- Optic nerve hypoplasia
- cataract
Individual evidence
- ↑ a b c Aniridia - mental retardation. In: Orphanet (Rare Disease Database).
- ↑ FA Walker, C. Dyson: Dominantly inherited aniridia associated with mental retardation and other eye abnormalities. In: Birth defects original article series. Volume 10, Number 7, 1974, pp. 147-149, PMID 4422267 .
