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Hendrik P. N. Scholl, Jan Kremers, Reinhard Vonthein, Karen White, Bernhard H. F. Weber; L- and M-Cone–Driven Electroretinograms in Stargardt’s Macular Dystrophy–Fundus Flavimaculatus. Invest. Ophthalmol. Vis. Sci. 2001;42(6):1380-1389.
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purpose. To study the dynamics of the long (L)- and middle
(M)-wavelength–sensitive cone-driven pathways and their interactions
in patients with Stargardt’s macular dystrophy-fundus flavimaculatus
(SMD-FF) and to correlate them with other clinical parameters and
methods. Forty-seven patients with SMD-FF participated in the study. In addition
to standard 30-Hz flicker electroretinograms (30-Hz fERG), ERG
responses were measured to stimuli that modulated exclusively the L or
the M cones (L/M cones) or the two simultaneously. Blood samples were
screened for mutations in the 50 exons of the ABCA4 gene.
results. Patients with SMD-FF did not show a decrease in the mean
L/M-cone–driven ERG sensitivity, but there was a significant increase
in the interindividual variability. The mean L-/M-cone weighting ratio
was normal. However, the L-cone–driven ERG was significantly phase
delayed, whereas the M-cone–driven ERG was significantly phase
advanced. These phase changes were significantly correlated with
disease duration. The amplitude and implicit time of the standard 30-Hz
fERG both correlated significantly with the L/M-cone–driven ERG
sensitivity and with the phase difference between the L/M-cone–driven
ERGs, indicating the complex origin of the standard 30-Hz fERG.
Probable disease-associated mutations in the ABCA4 gene
were found in 40 of 45 patients, suggesting that they form a
genetically fairly uniform SMD-FF study group. There was no correlation
between the genotype and the L/M-cone–driven ERGs.
conclusions. The changes in L/M-cone–driven ERG sensitivity and phase possibly
represent two independent disease processes. The phase changes are
similar to those found in patients with retinitis pigmentosa and
possibly are a general feature of retinal
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