Electroretinogram and visual evoked potential in two siblings with adult form galactosialidosis.
We recorded flash electroretinograms (ERGs), flash visual evoked potentials (VEPs), pattern ERGs and pattern VEPs in two siblings with adult form galactosialidosis. The amplitude of b-wave was decreased in flash ERGs. The peak latency of flash VEPs was within normal range. The pattern ERGs and VEPs were nonrecordable. These results suggested the damage of retinal ganglion cell and inner nuclear layer in these cases.
Usui
T
T
Department of Ophthalmology, Niigata University School of Medicine, Japan.
Abe
H
H
Takagi
M
M
Yoshizawa
T
T
Hasegawa
S
S
Iwata
K
K
D000328:Adult; D003241:Consanguinity; D004596:Electroretinography; D005074:Evoked Potentials, Visual; D005260:Female; D006801:Humans; D016464:Lysosomal Storage Diseases / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D009439:Neuraminidase / Q000172:deficiency*; D010364:Pattern Recognition, Visual; D010775:Photic Stimulation; D012160:Retina / Q000503:physiopathology; D012165:Retinal Ganglion Cells / Q000502:physiology*; D001616:beta-Galactosidase / Q000172:deficiency*
D009439:Neuraminidase; D001616:beta-Galactosidase