TY - JOUR
T1 - Visual function of a patient with advanced adrenoleukodystrophy
T2 - Comparison of luminance and color contrast sensitivities
AU - Sakai, Shinya
AU - Hirayama, Kazumi
AU - Ogura, Kaeko
AU - Sakai, Naoko
AU - Sudoh, Mutsuko
AU - Murata, Naoya
AU - Iwasaki, Syoichi
PY - 2008/1
Y1 - 2008/1
N2 - 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.
AB - 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.
KW - Adrenoleukodystrophy
KW - Color
KW - Contrast sensitivity
KW - Luminance
KW - Residual visual capacity
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U2 - 10.1016/j.braindev.2007.06.002
DO - 10.1016/j.braindev.2007.06.002
M3 - Article
C2 - 17624708
AN - SCOPUS:37349018023
VL - 30
SP - 68
EP - 72
JO - Brain and Development
JF - Brain and Development
SN - 0387-7604
IS - 1
ER -