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GNAO1-related developmental delay-seizures-movement disorder spectrum

ORPHA:592564· ICD-10 G93.4

Definition

A rare genetic neurological disorder characterized by a phenotypic spectrum of mild to severe developmental delay and hypotonia, variably associated with intellectual disability, early-onset seizures, and movement disorders, such as dystonia, ataxia, chorea, and dyskinesia. Brain imaging may show delayed myelination, thin corpus callosum, or cerebral atrophy.

Prevalence
<1 / 1 000 000
Inheritance
Autosomal dominant
Age of onset
Childhood, Infancy, Neonatal