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X-linked intellectual disability, Najm type

ORPHA:163937· ICD-10 Q04.3

Definition

Najm type X-linked intellectual deficit is a rare cerebellar dysgenesis syndrome characterized by variable clinical manifestations ranging from mild intellectual deficit with or without congenital nystagmus, to severe cognitive impairment associated with cerebellar and pontine hypoplasia/atrophy and abnormalities of cortical development.

Prevalence
<1 / 1 000 000
Inheritance
X-linked dominant
Age of onset
Infancy, Neonatal