Visual function of a patient with advanced adrenoleukodystrophy: comparison of luminance and color contrast sensitivities

Brain Dev. 2008 Jan;30(1):68-72. doi: 10.1016/j.braindev.2007.06.002. Epub 2007 Jul 10.

Abstract

We assessed achromatic luminance and isoluminant chromatic contrast sensitivity functions of a 20-year-old male. He showed severe motor and intellectual disabilities after advanced adrenoleukodystrophy, which started at the age of 7. Optokinetic nystagmus (OKN) to drifting gratings was used to assess his contrast sensitivities. Although the achromatic luminance contrast sensitivities were lower over the entire range of spatial frequencies tested than those of the healthy adults, they were preserved to the level comparable to healthy 7-year-old children, except for the frequency of 1 and 4 cycles/degree. In contrast, both of the red-green and blue chromatic contrast sensitivities were remarkably lower in all frequency range compare to healthy children and adults. These results indicate that it is possible for even an advanced case of ALD to show residual visual capacity that was preserved to a remarkable extent.

Publication types

  • Case Reports
  • Comparative Study

MeSH terms

  • Adrenoleukodystrophy / complications*
  • Adrenoleukodystrophy / physiopathology*
  • Adult
  • Age Factors
  • Child
  • Color Perception / physiology
  • Contrast Sensitivity / physiology
  • Developmental Disabilities / diagnosis
  • Developmental Disabilities / etiology
  • Developmental Disabilities / physiopathology
  • Female
  • Humans
  • Luminescence
  • Magnetic Resonance Imaging
  • Male
  • Neurologic Examination
  • Photic Stimulation
  • Predictive Value of Tests
  • Vision Disorders / diagnosis
  • Vision Disorders / etiology*
  • Vision Disorders / physiopathology*
  • Vision Tests
  • Visual Cortex / pathology
  • Visual Cortex / physiopathology*
  • Visual Pathways / pathology
  • Visual Pathways / physiopathology*