MR Imaging Findings in Xp21.2 Duplication Syndrome

J Radiol Case Rep. 2016 May 31;10(5):9-14. doi: 10.3941/jrcr.v10i5.2563. eCollection 2016 May.

Abstract

Xp21.2 duplication syndrome is a rare genetic disorder of undetermined prevalence and clinical relevance. As the use of chromosomal microarray has become first line for the work-up of childhood developmental delay, more gene deletions and duplications have been recognized. To the best of our knowledge, the imaging findings of Xp21.2 duplication syndrome have not been reported. We report a case of a 33 month-old male referred for developmental delay that was found to have an Xp21.2 duplication containing IL1RAPL1 and multiple midline brain malformations.

Keywords: Xq21.2 duplication; corpus callosum; fornix; hypothalamus; olfactory; vermis.

Publication types

  • Case Reports

MeSH terms

  • Abnormalities, Multiple
  • Brain / abnormalities*
  • Child, Preschool
  • Chromosomes, Human, Pair 21 / genetics
  • Chromosomes, Human, X / genetics*
  • Diagnosis, Differential
  • Gene Duplication*
  • Genes, Duplicate / genetics
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Syndrome