2q23 de novo microdeletion involving the MBD5 gene in a patient with developmental delay, postnatal microcephaly and distinct facial features

Am J Med Genet A. 2011 Feb;155A(2):424-9. doi: 10.1002/ajmg.a.33821.

Abstract

We report on a female patient with a de novo interstitial deletion of chromosome region 2q23.1-23.3 identified by array-CGH. She had significant global delay with developmental regression at age 6 years. She developed seizures at age 3 years with progressive difficulties with balance, loss of fine motor skills and aggressive behavior. She had short stature, microcephaly, and distinct facial features. Her speech was dysarthric, and she demonstrated repetitive hand movements. In this article, we compare the clinical features of our patient with previously reported cases with a 2q23.1 deletion.

Publication types

  • Case Reports

MeSH terms

  • Abnormalities, Multiple / genetics
  • Abnormalities, Multiple / pathology*
  • Child
  • Chromosome Deletion*
  • Chromosomes, Human, Pair 2 / genetics*
  • Comparative Genomic Hybridization
  • DNA-Binding Proteins / genetics*
  • Developmental Disabilities / genetics
  • Developmental Disabilities / pathology*
  • Face / abnormalities
  • Female
  • Humans
  • Microcephaly / genetics
  • Microcephaly / pathology*

Substances

  • DNA-Binding Proteins
  • MBD5 protein, human