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GRIN2B-related developmental delay, intellectual disability and autism spectrum disorder

MedGen UID:
1830118
Concept ID:
C5681638
Disease or Syndrome
Synonyms: Glutamate ionotropic receptor NMDA type subunit 2B-related developmental delay, intellectual disability, autism spectrum disorder; GRIN2B-related developmental delay, intellectual disability, autism spectrum disorder
SNOMED CT: GRIN2B-related developmental delay, intellectual disability, autism spectrum disorder (1260195002); Glutamate ionotropic receptor NMDA type subunit 2B-related developmental delay, intellectual disability, autism spectrum disorder (1260195002)
 
Monarch Initiative: MONDO:0035122
Orphanet: ORPHA589547

Definition

A rare genetic syndromic intellectual disability characterized by infantile or childhood onset of mild to profound developmental delay and intellectual disability in all affected individuals, as well as variable occurrence of epilepsy, autism spectrum disorder/behavioral issues, microcephaly, muscle tone abnormalities such as hypotonia and spasticity, dystonic, dyskinetic or choreiform movement disorder and cortical visual impairment. Brain MRI may reveal abnormal cortical development, hypoplastic corpus callosum, enlarged or dysplastic basal ganglia and hippocampal dysplasia. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • GRIN2B-related developmental delay, intellectual disability and autism spectrum disorder

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