Palmo-Plantar hyperkeratosis, intellectual disability, and spastic paraplegia in two maternal half brothers: further evidence for an X-linked inheritance

Am J Med Genet A. 2013 Jun;161A(6):1390-3. doi: 10.1002/ajmg.a.35871. Epub 2013 Apr 23.

Abstract

In 1983, Fitzsimmons et al. reported four brothers with an unrecognized disorder characterized by intellectual disability, spastic paraplegia, and palmo-plantar hyperkeratosis (OMIM 309500). In this report, we describe a family in which two males, maternal half-brothers, had learning disabilities. Both patients also showed spasticity in the lower limbs and palmo-plantar hyperkeratosis. The mother of the affected boys had learning difficulties but did not show any dermatological symptoms. This report confirms that the association of features reported by Fitzsimmons et al. is a distinct entity and further suggests an X-linked mode of inheritance.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Child
  • Comparative Genomic Hybridization
  • Female
  • Genes, X-Linked / genetics*
  • Genetic Association Studies
  • Humans
  • Intellectual Disability / diagnosis
  • Intellectual Disability / genetics*
  • Keratoderma, Palmoplantar / diagnosis
  • Keratoderma, Palmoplantar / genetics*
  • Learning Disabilities / diagnosis
  • Learning Disabilities / genetics
  • Male
  • Pedigree
  • Siblings
  • Spastic Paraplegia, Hereditary / diagnosis
  • Spastic Paraplegia, Hereditary / genetics*

Associated data

  • OMIM/309500