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Dentici-Novelli neurodevelopmental syndrome

Summary

Dentici-Novelli neurodevelopmental syndrome (DENNED) is an autosomal recessive disorder characterized by global developmental delay with impaired intellectual development apparent from infancy. The severity of the phenotype is highly variable: more severely affected individuals have axial hypotonia, peripheral spasticity, microcephaly, early-onset seizures, brain imaging abnormalities, and are unable to walk or speak. Those with a less severe phenotype may achieve some developmental goals and show less severe intellectual disability (Dentici et al., 2022). [from OMIM]

Genes See tests for all associated and related genes

  • Also known as: DENNED, ZNF526
    Summary: zinc finger protein 526

Clinical features

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Imported from Human Phenotype Ontology (HPO)

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