A syndrome of microcephaly and retinal pigmentary abnormalities without mental retardation in a family with coincidental autosomal dominant hyperreflexia

Am J Med Genet. 1984 Mar;17(3):585-94. doi: 10.1002/ajmg.1320170306.

Abstract

We describe two brothers with severe microcephaly, unusual retinal pigmentary anomalies, intellectual function in the average or low average range, and a strong family history of hyperreflexia. The brothers have a previously undescribed syndrome, while the hyperreflexia appears to represent a coincidental autosomal dominant Mendelian trait, perhaps linked to the Kell blood group system.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Abnormalities, Multiple / blood
  • Abnormalities, Multiple / genetics*
  • Adult
  • Child
  • Female
  • Fundus Oculi
  • Genes, Dominant*
  • Genetic Linkage
  • Humans
  • Infant
  • Kell Blood-Group System / genetics
  • Male
  • Microcephaly / blood
  • Microcephaly / genetics*
  • Pedigree
  • Pigment Epithelium of Eye / pathology*
  • Reflex, Abnormal / congenital
  • Reflex, Abnormal / genetics*
  • Retinal Diseases / blood
  • Retinal Diseases / congenital
  • Retinal Diseases / genetics*
  • Syndrome

Substances

  • Kell Blood-Group System