June 2013
Volume 54, Issue 15
Free
ARVO Annual Meeting Abstract  |   June 2013
Rule of multifocal electroretinogram in follow-up of ABCA4-related Stardgardt’s disease
Author Affiliations & Notes
  • Ada Orrico
    Second University of Naples, Naples, Italy
  • Francesco Testa
    Second University of Naples, Naples, Italy
  • Settimio Rossi
    Second University of Naples, Naples, Italy
  • Paolo Melillo
    Second University of Naples, Naples, Italy
  • Antonella de Benedictis
    Second University of Naples, Naples, Italy
  • Michele Della Corte
    Second University of Naples, Naples, Italy
  • Francesca Simonelli
    Second University of Naples, Naples, Italy
  • Footnotes
    Commercial Relationships Ada Orrico, None; Francesco Testa, None; Settimio Rossi, None; Paolo Melillo, None; Antonella de Benedictis, None; Michele Della Corte, None; Francesca Simonelli, None
  • Footnotes
    Support None
Investigative Ophthalmology & Visual Science June 2013, Vol.54, 1371. doi:
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      Ada Orrico, Francesco Testa, Settimio Rossi, Paolo Melillo, Antonella de Benedictis, Michele Della Corte, Francesca Simonelli; Rule of multifocal electroretinogram in follow-up of ABCA4-related Stardgardt’s disease. Invest. Ophthalmol. Vis. Sci. 2013;54(15):1371.

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

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Abstract
 
Purpose
 

The aim of this study is to investigate the rule of multifocal electroretinogram (mfERG) to follow-up patients with Stardgardt’s disease in order to provide prognostic information.

 
Methods
 

We followed up 15 patients (9 female and 6 male) with ABCA4-related Stardgardt disease for three years by performing the following examinations: ophthalmoscopic exam, best corrected visual acuity (BCVA), full field electroretinogram (ERG) and mfERG. BCVA was measured with Early Treatment Diabetic Retinopathy Study (ETDRS) charts. The fundus lesion were classified according to Fishman stages. ERGs and mfERGs were recorded following International Society for Clinical Electrophysiology of Vision standard guidelines. The ERG responses were categorized according to Lois’s classification. In mfERGs a stimulus array of 103 hexagons was adopted, the responses were spatially averaged in six rings and the amplitude and latency parameters of the three waves (N1, P1 and N2) were computed.

 
Results
 

The patients had the following demographic characteristics: age 15-50 yrs, disease length 1-23 yrs, age of onset 12 - 38 yrs. The BCVA ranged from 0 to 1.82 logMar (0.84 ± 0.33 logMar). Fundus lesions were classified as Fishman stage I, II and III in 12, 2 and 1 patients, respectively. ERG responses belonged to Lois class I, II and III in 12, 2 and 1 patients, respectively. None of the patients showed a normal mfERGs in any rings. In the fifty per cent of patients a subnormal but recordable response was assessed in the last three rings. We observed significant differences (p-value < 0.05) in some parameters of mfERG responses between baseline and last follow-up measurements (peak amplitude of N1 wave, latency and peak amplitude of N2 wave in Ring 6, and peak amplitude of N2 wave in Ring 5). Moreover, we observed a significant correlation between BCVA and amplitude of P1 wave in Ring 6 (rho=-0.30; p=0.03). Finally, we observed significant correlation between BCVA lost in the three year follow-up and the amplitude of N1 wave in Ring 6 (rho=0.397;p=0.046).

 
Conclusions
 

This study revealed significant differences in mfERG responses after three year follow-up. Selected mfERG parameters, recorded at the baseline, seemed to have a prognostic value to predict the visual acuity lost in the following three years. Further studies on a larger sample of patients would be needed to confirm the prognostic value of mfERG in Stardgardt disease.

 
Keywords: 494 degenerations/dystrophies • 696 retinal degenerations: hereditary • 509 electroretinography: clinical  
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