June 2013
Volume 54, Issue 15
Free
ARVO Annual Meeting Abstract  |   June 2013
Long-Term Decay of Central Cone Function in Cone-Rod Dystrophy Evaluated by Focal Electroretinogram
Author Affiliations & Notes
  • Benedetto Falsini
    Ophthalmology, Universita' Cattolica del S. Cuore, Rome, Italy
  • Marco Piccardi
    Ophthalmology, Universita' Cattolica del S. Cuore, Rome, Italy
  • Lucia Ziccardi
    Ophthalmology, Bietti Foundation IRCCS, Rome, Italy
  • Antonello Fadda
    Health and Technology, Istituto Superiore di Sanita, Rome, Italy
  • Angelo Minnella
    Ophthalmology, Universita' Cattolica del S. Cuore, Rome, Italy
  • Dario Marangoni
    Ophthalmology, Universita' Cattolica del S. Cuore, Rome, Italy
  • Silvia Bisti
    Scienze Cliniche ed Applicate Biotecnologiche, Universita dell'Aquila, L'Aquila, Italy
  • Giovanni Resta
    Informatics and Telematics, CNR, Pisa, Italy
  • Lucia Galli Resta
    Neuroscience Institute, CNR, Pisa, Italy
  • Footnotes
    Commercial Relationships Benedetto Falsini, None; Marco Piccardi, None; Lucia Ziccardi, None; Antonello Fadda, None; Angelo Minnella, None; Dario Marangoni, None; Silvia Bisti, None; Giovanni Resta, None; Lucia Galli Resta, None
  • Footnotes
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Investigative Ophthalmology & Visual Science June 2013, Vol.54, 5110. doi:
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      Benedetto Falsini, Marco Piccardi, Lucia Ziccardi, Antonello Fadda, Angelo Minnella, Dario Marangoni, Silvia Bisti, Giovanni Resta, Lucia Galli Resta; Long-Term Decay of Central Cone Function in Cone-Rod Dystrophy Evaluated by Focal Electroretinogram. Invest. Ophthalmol. Vis. Sci. 2013;54(15):5110.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose: To describe the natural history of cone-rod dystrophy (CRD) as determined by longitudinal focal ERG (fERG) recording, and determine the usefulness of fERG in monitoring and anticipating CRD progression.

Methods: A cohort of 35 CRD patients (17 males, 18 females, age range at baseline: 9-59 years, 22 autosomal recessive, eight sporadic, two X-linked, and three dominant) was followed from 2.5 to 12.6 years (average follow-up 6.4 years) by clinical examination and by recording the fERG response to a (41 Hz) flickering uniform red field overlying the central 18 degrees of visual field (average of 1 clinical-fERG examination/patient/year, all patients had at least 3 visits). Short-term repeatability and long-term changes in fERG amplitudes were determined for each patient.

Results: The repeatability range for fERG amplitude was -54% to 67% (95th percentile). Based on these cut-off criteria, patients in our cohort resulted either stationary (STAT, n=18) or declining (DEC, n=17). Of the STAT patients, eight had high (≥ 1 µV) and 10 low amplitude (0.5-1 µV) fERG. Of the DEC patients, 11 had a slow (9%/year) and six a fast (33%/year) fERG amplitude decay (between-group difference, p < 0.01). All STAT patients also had stable visual acuity. Fifteen out of the 17 DEC patients (88%) also had a visual acuity loss (≥ 2 lines) during follow-up. All acuity losses were preceded and/or accompanied by fERG losses.

Conclusions: fERG represents a direct and sensitive assay to follow the progression of central retinal dysfunction in CRD. This approach might help to anticipate for an individual patient the clinical rate and timing of disease progression

Keywords: 461 clinical (human) or epidemiologic studies: natural history • 509 electroretinography: clinical • 696 retinal degenerations: hereditary  
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