title: Visual function of a patient with advanced adrenoleukodystrophy: comparison of luminance and color contrast sensitivities.
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.
authors.affiliation: Department of Occupational Therapy, Hokkaido University School of Health Sciences, N-15 W-5 Kita-ku, Sapporo 060-0812, Japan. sakai@cme.hokudai.ac.jp
authors.forename: Shinya
authors.initials: S
authors.lastname: Sakai
authors.forename: Kazumi
authors.initials: K
authors.lastname: Hirayama
authors.forename: Kaeko
authors.initials: K
authors.lastname: Ogura
authors.forename: Naoko
authors.initials: N
authors.lastname: Sakai
authors.forename: Mutsuko
authors.initials: M
authors.lastname: Sudoh
authors.forename: Naoya
authors.initials: N
authors.lastname: Murata
authors.forename: Syoichi
authors.initials: S
authors.lastname: Iwasaki
mesh_terms: D000326:Adrenoleukodystrophy / Q000150:complications* / Q000503:physiopathology*; D000328:Adult; D000367:Age Factors; D002648:Child; D003118:Color Perception / Q000502:physiology; D015350:Contrast Sensitivity / Q000502:physiology; D002658:Developmental Disabilities / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology; D005260:Female; D006801:Humans; D049449:Luminescence; D008279:Magnetic Resonance Imaging; D008297:Male; D009460:Neurologic Examination; D010775:Photic Stimulation; D011237:Predictive Value of Tests; D014786:Vision Disorders / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology*; D014787:Vision Tests; D014793:Visual Cortex / Q000473:pathology / Q000503:physiopathology*; D014795:Visual Pathways / Q000473:pathology / Q000503:physiopathology*