17p13.1 microdeletion: genetic and clinical findings in a new patient with epilepsy and comparison with literature

Am J Med Genet A. 2014 Jan;164A(1):225-30. doi: 10.1002/ajmg.a.36225.

Abstract

Array comparative genomic hybridization is now a powerful tool to investigate patients with multiple congenital abnormalities and intellectual/motor impairment, and genomic imbalances are identified in a growing number of children with intellectual disability. Deletions in the 17p13.1 region have been reported in patients with dysmorphic features and developmental delay but a consistent phenotype has yet to emerge. Here, we report on the diagnosis of a 17p13.1 microdeletion of 829 kb in an 8-year-old girl presenting with profound cognitive disability, psychomotor delay, facial dysmorphisms, and refractory epilepsy. This deletion comprises 44 genes, including 8 OMIM morbid genes. We discuss genetic, clinical, and epileptic features comparing our patient with those previously reported in the literature.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Abnormalities, Multiple / diagnosis*
  • Abnormalities, Multiple / genetics*
  • Child
  • Chromosome Deletion*
  • Chromosomes, Human, Pair 17*
  • Comparative Genomic Hybridization
  • Electroencephalography
  • Facies
  • Female
  • Humans
  • In Situ Hybridization, Fluorescence
  • Phenotype