Electroretinographic oscillatory potentials in Leber hereditary optic neuropathy

Barboni, Mirella T. S. (avtor), Šuštar Habjan, Maja (avtor), Petrović Pajić, Sanja (avtor), Hawlina, Marko (avtor)

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.

• Jezik: Angleški jezik
• Ključne besede: LHON, retina, electroretinogram, photopic negative response, oscillatory potentials
• Vrsta gradiva: Članek v reviji
• Tipologija: 1.01 - Izvirni znanstveni članek
• Organizacija: MF - Medicinska fakulteta
• Status publikacije: Objavljeno
• Različica publikacije: Objavljena publikacija
• Leto izida: 2024
• Št. strani: Str. 133-143
• Številčenje: Vol. 148, iss. 3
• PID: 20.500.12556/RUL-179116
• ISSN pri članku: 1573-2622
• DOI: 10.1007/s10633-024-09968-9
• Datum objave v RUL: 05.02.2026

Gradivo je del revije

• Naslov: Documenta ophthalmologica
• Skrajšan naslov: Doc. ophthalmol.
• Založnik: Springer Nature
• ISSN: 1573-2622
• COBISS.SI-ID: 513144601

Licence

• Licenca: CC BY 4.0, Creative Commons Priznanje avtorstva 4.0 Mednarodna
• Povezava: http://creativecommons.org/licenses/by/4.0/deed.sl
• Opis: To je standardna licenca Creative Commons, ki daje uporabnikom največ možnosti za nadaljnjo uporabo dela, pri čemer morajo navesti avtorja.

Projekti

• Financer: ARRS - Agencija za raziskovalno dejavnost Republike Slovenije
• Številka projekta: P3-0333
• Naslov: Očesne bolezni odraslih in otrok

• Financer: Hungary, National Research, Development, and Innovation Fund
• Program financ.: OTKA-PD
• Številka projekta: 134799