X-linked intellectual disability-cerebellar hypoplasia syndrome
ORPHA:137831· ICD-10 Q04.3
Definition
X-linked intellectual deficit-cerebellar hypoplasia, also known as OPHN1 syndrome, is a rare syndromic form of cerebellar dysgenesis characterized by moderate to severe intellectual deficit and cerebellar abnormalities.
- Prevalence
- <1 / 1 000 000
- Inheritance
- X-linked dominant
- Age of onset
- Infancy, Neonatal