Early detection of central visual function decline in cone-rod dystrophy by the use of macular focal cone electroretinogram

Lucia Galli-Resta, Marco Piccardi, Lucia Ziccardi, Antonello Fadda, Angelo Minnella, Dario Marangoni, Giorgio Placidi, Giovanni Resta, Benedetto Falsini

Research output: Contribution to journalArticle

8 Citations (Scopus)

Abstract

PURPOSE. To evaluate macular focal cone ERG (fERG) as a tool for reliable and early detection of central retinal function decay in cone-rod dystrophy (CRD). METHODS. A retrospective study of the time course of fERG amplitude and its relation to visual acuity alterations was performed in 47 CRD patients followed yearly for 6.0 ± 3.1 years. Macular focal cone ERG was evoked by a flickering uniform red field overlaying the central 18° of visual field. RESULTS. Macular focal cone ERG follow-up allowed a clear-cut identification of CRD patients as stationary or progressive, in agreement with visual acuity follow-up. In all progressive patients, fERG declined whenever visual acuity declined, and-in 50% of the cases-fERG loss anticipated acuity loss of several years. CONCLUSIONS. Macular focal cone ERG represents a sensitive assay to detect, categorize, and follow the progression of central retinal dysfunction in CRD. Its use as a diagnostic tool in CRD may help anticipate, for an individual patient, the likelihood and rate of further disease progression before visual acuity loss has occurred.

Original languageEnglish
Pages (from-to)6560-6569
Number of pages10
JournalInvestigative Ophthalmology and Visual Science
Volume54
Issue number10
DOIs
Publication statusPublished - 2013

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Visual Acuity
Visual Fields
Disease Progression
Retrospective Studies
Cone-Rod Dystrophies

Keywords

  • Cone-rod dystrophy
  • Focal ERG
  • Visual acuity

ASJC Scopus subject areas

  • Ophthalmology
  • Sensory Systems
  • Cellular and Molecular Neuroscience

Cite this

Early detection of central visual function decline in cone-rod dystrophy by the use of macular focal cone electroretinogram. / Galli-Resta, Lucia; Piccardi, Marco; Ziccardi, Lucia; Fadda, Antonello; Minnella, Angelo; Marangoni, Dario; Placidi, Giorgio; Resta, Giovanni; Falsini, Benedetto.

In: Investigative Ophthalmology and Visual Science, Vol. 54, No. 10, 2013, p. 6560-6569.

Research output: Contribution to journalArticle

Galli-Resta, Lucia ; Piccardi, Marco ; Ziccardi, Lucia ; Fadda, Antonello ; Minnella, Angelo ; Marangoni, Dario ; Placidi, Giorgio ; Resta, Giovanni ; Falsini, Benedetto. / Early detection of central visual function decline in cone-rod dystrophy by the use of macular focal cone electroretinogram. In: Investigative Ophthalmology and Visual Science. 2013 ; Vol. 54, No. 10. pp. 6560-6569.
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AU - Galli-Resta, Lucia

AU - Piccardi, Marco

AU - Ziccardi, Lucia

AU - Fadda, Antonello

AU - Minnella, Angelo

AU - Marangoni, Dario

AU - Placidi, Giorgio

AU - Resta, Giovanni

AU - Falsini, Benedetto

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N2 - PURPOSE. To evaluate macular focal cone ERG (fERG) as a tool for reliable and early detection of central retinal function decay in cone-rod dystrophy (CRD). METHODS. A retrospective study of the time course of fERG amplitude and its relation to visual acuity alterations was performed in 47 CRD patients followed yearly for 6.0 ± 3.1 years. Macular focal cone ERG was evoked by a flickering uniform red field overlaying the central 18° of visual field. RESULTS. Macular focal cone ERG follow-up allowed a clear-cut identification of CRD patients as stationary or progressive, in agreement with visual acuity follow-up. In all progressive patients, fERG declined whenever visual acuity declined, and-in 50% of the cases-fERG loss anticipated acuity loss of several years. CONCLUSIONS. Macular focal cone ERG represents a sensitive assay to detect, categorize, and follow the progression of central retinal dysfunction in CRD. Its use as a diagnostic tool in CRD may help anticipate, for an individual patient, the likelihood and rate of further disease progression before visual acuity loss has occurred.

AB - PURPOSE. To evaluate macular focal cone ERG (fERG) as a tool for reliable and early detection of central retinal function decay in cone-rod dystrophy (CRD). METHODS. A retrospective study of the time course of fERG amplitude and its relation to visual acuity alterations was performed in 47 CRD patients followed yearly for 6.0 ± 3.1 years. Macular focal cone ERG was evoked by a flickering uniform red field overlaying the central 18° of visual field. RESULTS. Macular focal cone ERG follow-up allowed a clear-cut identification of CRD patients as stationary or progressive, in agreement with visual acuity follow-up. In all progressive patients, fERG declined whenever visual acuity declined, and-in 50% of the cases-fERG loss anticipated acuity loss of several years. CONCLUSIONS. Macular focal cone ERG represents a sensitive assay to detect, categorize, and follow the progression of central retinal dysfunction in CRD. Its use as a diagnostic tool in CRD may help anticipate, for an individual patient, the likelihood and rate of further disease progression before visual acuity loss has occurred.

KW - Cone-rod dystrophy

KW - Focal ERG

KW - Visual acuity

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