Chromosome 17q21.31 duplication syndrome: Description of a new familiar case and further delineation of the clinical spectrum

Eur J Paediatr Neurol. 2016 Jan;20(1):183-7. doi: 10.1016/j.ejpn.2015.09.010. Epub 2015 Oct 22.

Abstract

Introduction: 17q21.31 microduplication syndrome is a recently described condition associated with a broad clinical spectrum, of which psychomotor delay, behavioral disorders and poor social interaction seem to be the most consistent features. Only seven patients have been reported thus far. All have behavioral disorders reminiscent of the autistic spectrum with intellectual skills ranging from normal to mild intellectual deficiency. Other features are variable with no striking common phenotypic features.

Case study: Here we describe the segregation of 17q21.31 duplication in an Italian family.

Discussion: Clinical features and genetic data are reported, and compared with previously reported patients with 17q21.31 microduplication. A comparison of clinical manifestations between deletion and duplication syndromes of the chromosome regione is provided.

Keywords: 17q21.31 microduplication; Autism spectrum disorder; Genetic syndrome; Intellectual disability; KANSL1; Psychomotor delay.

Publication types

  • Case Reports

MeSH terms

  • Chromosome Duplication / genetics*
  • Chromosomes, Human, Pair 17 / genetics*
  • Developmental Disabilities / genetics*
  • Female
  • Humans
  • Italy
  • Male
  • Pedigree
  • Phenotype
  • Syndrome