PurposeLeber hereditary optic neuropathy (LHON) affects retinal ganglion cells causing
severe vision loss. Pattern electroretinogram and photopic negative response (PhNR)
of the light-adapted (LA) full-field electroretinogram (ERG) are typically affected
in LHON. In the present study, we evaluated dark-adapted (DA) and LA oscillatory potentials
(OPs) of the flash ERG in genetically characterized LHON patients to dissociate slow
from fast components of the response.MethodsSeven adult patients (mean age = 28.4
+/- 5.6) in whom genetic diagnosis confirmed LHON with mtDNA or nuclear DNAJC30 (arLHON)
pathogenic variants were compared to 12 healthy volunteers (mean age = 35.0 +/- 12.1).
Full-field ERGs were recorded from both eyes. Offline digital filters at 50, 75 and
100 Hz low cutoff frequencies were applied to isolate high-frequency components from
the original ERG signals.ResultsERG a-waves and b-waves were comparable between LHON
patients and controls, while PhNR was significantly reduced (p = 0.009) in LHON patients
compared to controls, as expected. OPs derived from DA signals (75 Hz low cutoff frequency)
showed reduced peak amplitude for OP2 (p = 0.019). LA OP differences between LHON
and controls became significant (OP2: p = 0.047, OP3: p = 0.039 and OP4: p = 0.013)
when the 100 Hz low-cutoff frequency filter was applied.ConclusionsReduced OPs in
LHON patients may represent disturbed neuronal interactions in the inner retina with
preserved photoreceptoral (a-wave) to bipolar cell (b-wave) activation. Reduced DA
OP2 and high-cutoff LA OP alterations may be further explored as functional measures
to characterize LHON status and progression.
