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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