Multifocal electroretinograms in X-linked retinoschisis.
OBJECTIVE
To study local retinal cone function in patients with X-linked retinoschisis (XLRS) by multifocal ERGs (mfERGs).


METHODS
mfERGs were recorded from seven eyes of seven patients with XLRS (mean age +/- SD, 22.1 +/- 3.2 years; range, 18 to 25 years). Five eyes had microcystic changes in the macula and two eyes had nonspecific macular degeneration. Two eyes had peripheral retinoschisis, and some of the stimuli fell on this area. The stimulus array consisted of 103 hexagons and the total recording time was set at approximately 4 minutes. The amplitudes and implicit times of both focal and summed responses for the first- and second-order kernels were analyzed.


RESULTS
The amplitudes of the first-order kernel were markedly reduced in the central retina in all eyes. A large variation was observed in the amplitudes outside the fovea. The amplitudes of the focal cone ERGs at the peripheral retinoschisis did not differ from those recorded from adjacent retinal loci without the retinoschisis. The implicit times of the first-order kernel were significantly delayed, and the amplitudes of the second-order kernels were more affected than the first-order kernels across the whole field in all XLRS eyes.


CONCLUSIONS
The cone-mediated retinal responses were more impaired in the central than peripheral retina in eyes with XLRS. Delayed implicit times of the first-order kernel and reduced second-order kernel across the whole testing field in all XLRS eyes suggest that there is widespread cone-system dysfunction in XLRS.
Piao
Chang-Hua
CH
Department of Ophthalmology, Nagoya University School of Medicine, Showa-ku, Nagoya, Japan.
Kondo
Mineo
M
Nakamura
Makoto
M
Terasaki
Hiroko
H
Miyake
Yozo
Y
D000293:Adolescent; D000328:Adult; D004596:Electroretinography / Q000379:methods*; D040181:Genetic Diseases, X-Linked / Q000235:genetics / Q000503:physiopathology*; D006801:Humans; D017949:Retinal Cone Photoreceptor Cells / Q000502:physiology*; D041441:Retinoschisis / Q000235:genetics* / Q000503:physiopathology*; D014794:Visual Fields