Electroretinographic oscillatory potentials in Leber hereditary optic neuropathy.

Autor: Barboni MTS; Department of Ophthalmology, Semmelweis University, Budapest, Hungary., Sustar Habjan M; Eye Hospital, University Medical Centre Ljubljana, Grablovičeva 46, 1000, Ljubljana, Slovenia., Petrovic Pajic S; Eye Hospital, University Medical Centre Ljubljana, Grablovičeva 46, 1000, Ljubljana, Slovenia.; Clinic for Eye Diseases, University Clinical Center of Serbia, Belgrade, Serbia., Hawlina M; Eye Hospital, University Medical Centre Ljubljana, Grablovičeva 46, 1000, Ljubljana, Slovenia. marko.hawlina@gmail.com.; Medical Faculty, Department of Ophthalmology, University of Ljubljana, Grablovičeva 46, 1000, Ljubljana, Slovenia. marko.hawlina@gmail.com.
Jazyk: angličtina
Zdroj: Documenta ophthalmologica. Advances in ophthalmology [Doc Ophthalmol] 2024 Jun; Vol. 148 (3), pp. 133-143. Date of Electronic Publication: 2024 Mar 07.
DOI: 10.1007/s10633-024-09968-9
Abstrakt: Purpose: Leber 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.
Methods: Seven 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.
Results: ERG 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.
Conclusions: Reduced 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.
(© 2024. The Author(s).)
Databáze: MEDLINE
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