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Biallelic CACNA2D2 variants in epileptic encephalopathy and cerebellar atrophy.

Jaya PunethaEnder KaracaAlper GezdiriciRyan E LamontDavut PehlivanDana MarafiJuan Pablo AppendinoJill V HunterZeynep C AkdemirJawid M FatihShalini N JhangianiRichard A GibbsA Micheil InnesJennifer E PoseyJames R Lupski
Published in: Annals of clinical and translational neurology (2019)
Our study supports the candidacy of CACNA2D2 as a disease gene associated with a phenotypic spectrum of neurological disease that include features of developmental and epileptic encephalopathy, ataxia, and cerebellar atrophy. Age at presentation may affect apparent penetrance of neurogenetic trait manifestations and of a particular clinical neurological endophenotype, for example, seizures or ataxia.
Keyphrases
  • early onset
  • copy number
  • genome wide
  • intellectual disability
  • gene expression
  • magnetic resonance imaging
  • dna methylation
  • cerebral ischemia
  • computed tomography
  • brain injury