A case of Baraitser-Winter syndrome with unusual brain MRI findings: pachygyria, subcortical-band heterotopia, and periventricular heterotopia

Brain Dev. 2010 Jun;32(6):502-5. doi: 10.1016/j.braindev.2009.04.015. Epub 2009 May 23.

Abstract

Baraitser-Winter syndrome (BaWS) is characterized by iris coloboma, ptosis, hypertelorism, and mental retardation; it is a rare multiple congenital anomaly or a mental-retardation syndrome of unknown etiology. Patients suffering from this syndrome have been also found to show brain anomalies such as pachygyria, subcortical-band heterotopia (SBH), and hippocampal malformations; therefore, these anomalies have been included in the phenotypic spectrum of this syndrome. We report the case of a Japanese boy suffering from BaWS; the patient's brain magnetic resonance imaging scan revealed pachygyria, SBH, and periventricular heterotopia. However, the results of the genome-wide array comparative genomic hybridization did not reveal any chromosomal rearrangements.

Publication types

  • Case Reports

MeSH terms

  • Abnormalities, Multiple / genetics
  • Abnormalities, Multiple / pathology*
  • Brain / pathology*
  • Comparative Genomic Hybridization
  • Humans
  • Infant
  • Intellectual Disability / genetics
  • Intellectual Disability / pathology*
  • Japan
  • Magnetic Resonance Imaging
  • Male
  • Phenotype
  • Syndrome