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Multifocal VEP provide electrophysiological evidence of predominant dysfunction of the optic nerve fibers derived from the central retina in Leber’s hereditary optic neuropathy

  • Neurophthalmology
  • Published:
Graefe's Archive for Clinical and Experimental Ophthalmology Aims and scope Submit manuscript

Abstract

Purpose

To differentiate the bioelectrical cortical responses driven by axons from central and mid-peripheral retina in Leber’s hereditary optic neuropathy (LHON) by using multifocal visual evoked potentials (mfVEP).

Methods

Seventeen genetically confirmed LHON patients (33.35 ± 8.4 years, 17 eyes) and 22 age-matched controls (C) (38.2 ± 6.0 years, 22 eyes) were studied by mfVEP and optical coherence tomography. MfVEP P1 implicit time (P1 IT, ms) and response amplitude density of the N1-P1 components (N1-P1 RAD, nV/deg2) of the second order binary kernel were measured for five concentric retinal areas between the fovea and mid-periphery: 0–20 degrees (R1 to R5).

Results

Mean mfVEP P1 ITs and N1-P1 RADs at all five foveal eccentricities were significantly different (p < 0.01) in LHON when compared to controls. In both groups, mean mfVEP responses obtained from R1 to R5 showed a progressive shortening of P1 ITs (linear fitting, LHON: r  = −0.95; C: r = −0.98) and decrease of N1-P1 RADs (exponential fitting, LHON: r 2 = 0.94; C: r 2 = 0.93). The slope of the linear fitting between mean mfVEP P1 ITs in the two groups was about three times greater in LHON than in controls (LHON: y = −13.33x +182.03; C: y = −4.528x +108.1). MfVEP P1 ITs detected in R1 and R2 (0–5 degrees) were significantly correlated (p < 0.01) with the reduction of retinal nerve fiber layer thickness of the temporal quadrant.

Conclusions

MfVEP identifies abnormal neural conduction along the visual pathways in LHON, discriminating a predominant involvement of axons driving responses from the central retina when compared to those serving the mid-peripheral retina.

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Acknowledgments

Research for this paper was financially supported partially by the Italian Ministry of Health (grant number: 2006 RF-FGB-2006-368547) and partially by Fondazione Roma. The authors acknowledge Dr. Valter Valli Fiore for technical help in electrophysiological evaluations.

Conflict of interest

Each author states that he/she has no proprietary interest in the development or marketing of the instruments used in the present study, and no conflict of interest.

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Authors and Affiliations

  1. Neurophthalmology Unit, Fondazione G.B. Bietti- IRCCS, Via Livenza 3, 00198, Rome, Italy

    Lucia Ziccardi & Vincenzo Parisi

  2. Department of Statistical Sciences, “La Sapienza” University, Piazzale Aldo Moro 5, 00185, Rome, Italy

    Daniela Giannini

  3. Ospedale San Giovanni Evangelista, Via Parrozzani 3, 00019, Tivoli, Italy

    Federico Sadun

  4. Azienda San Camillo-Forlanini, Circonvallazione Gianicolense 87, 00152, Rome, Italy

    Anna Maria De Negri

  5. Studio oculistico d’Azeglio, Via D’Azeglio 5, 40123, Bologna, Italy

    Piero Barboni

  6. IRCCS Istituto Scientifico San Raffaele, Via Olgettina 60, 20132, Milan, Italy

    Piero Barboni

  7. Bellaria Hospital, IRCCS Istituto delle Scienze Neurologiche di Bologna, Via Altura 3, 40139, Bologna, Italy

    Chiara La Morgia & Valerio Carelli

  8. Dipartimento di Scienze Biomediche e Neuromotorie (DIBINEM), Neurology Unit, University of Bologna, Via Ugo Foscolo 7, 40123, Bologna, Italy

    Chiara La Morgia & Valerio Carelli

  9. Doheny Eye Institute, UCLA, 1450 San Pablo St, Los Angeles, CA, 90033, USA

    Alfedo A. Sadun

Authors
  1. Lucia Ziccardi
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  2. Vincenzo Parisi
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  3. Daniela Giannini
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  4. Federico Sadun
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  5. Anna Maria De Negri
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  6. Piero Barboni
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  7. Chiara La Morgia
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  8. Alfedo A. Sadun
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  9. Valerio Carelli
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Corresponding author

Correspondence to Lucia Ziccardi.

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Ziccardi, L., Parisi, V., Giannini, D. et al. Multifocal VEP provide electrophysiological evidence of predominant dysfunction of the optic nerve fibers derived from the central retina in Leber’s hereditary optic neuropathy. Graefes Arch Clin Exp Ophthalmol 253, 1591–1600 (2015). https://doi.org/10.1007/s00417-015-2979-1

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  • DOI: https://doi.org/10.1007/s00417-015-2979-1

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