Abstract
PURPOSE. Recent studies show that patients with Usher syndrome type 2 (USH2) have abnormal cone structure and density in the central retina. This occurs in the presence of normal acuity, opening the quest for additional sensitive functional measures of central cone function in USH. We tested here whether focal macular cone electroretinogram (fERG) could be such a tool. METHODS. This retrospective study of central cone function loss was based on data from 47 patients with USH2 from the Ophthalmology Department of the Policlinico Gemelli/Catholic University in Rome. The analysis focused on the decrease of the fERG, obtained in response to a 41-Hz sinusoidal modulation of a uniform field presented to the central 18°, generated by red light-emitting diodes (LEDs) and superimposed on an equiluminant steady adapting background. fERG decrease was compared with the decrease of best-corrected visual acuity and Goldmann kinetic perimetry V4E field. RESULTS. fERG follow-up data document a severe and precocious loss of central cone function in USH2 patients, preceding losses in other measures of cone function. fERG is already reduced to 40% of control at the beginning of the second decade of life, and by 25 years of age, all USH2 patients have fERGs less than 30% of control values. CONCLUSIONS. fERG represents a sensitive tool to evaluate central cone function in USH2, anticipating the decline of other central cone function measures, such as visual acuity and Goldmann perimetry.
| Original language | English |
|---|---|
| Pages (from-to) | 3827-3835 |
| Number of pages | 9 |
| Journal | Investigative Ophthalmology and Visual Science |
| Volume | 59 |
| Issue number | 10 |
| DOIs | |
| Publication status | Published - Aug 1 2018 |
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Keywords
- Cone photoreceptors
- Goldmann perimetry
- Retina
- Retinitis pigmentosa
ASJC Scopus subject areas
- Ophthalmology
- Sensory Systems
- Cellular and Molecular Neuroscience
Cite this
Central retina functional damage in usher syndrome type 2 : 22 years of focal macular ERG analysis in a patient population from central and southern Italy. / Galli-Resta, Lucia; Placidi, Giorgio; Campagna, Francesca; Ziccardi, Lucia; Piccardi, Marco; Minnella, Angelo; Abed, Edoardo; Iovine, Silvia; Maltese, Paolo; Bertelli, Matteo; Falsini, Benedetto.
In: Investigative Ophthalmology and Visual Science, Vol. 59, No. 10, 01.08.2018, p. 3827-3835.Research output: Contribution to journal › Article
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TY - JOUR
T1 - Central retina functional damage in usher syndrome type 2
T2 - 22 years of focal macular ERG analysis in a patient population from central and southern Italy
AU - Galli-Resta, Lucia
AU - Placidi, Giorgio
AU - Campagna, Francesca
AU - Ziccardi, Lucia
AU - Piccardi, Marco
AU - Minnella, Angelo
AU - Abed, Edoardo
AU - Iovine, Silvia
AU - Maltese, Paolo
AU - Bertelli, Matteo
AU - Falsini, Benedetto
PY - 2018/8/1
Y1 - 2018/8/1
N2 - PURPOSE. Recent studies show that patients with Usher syndrome type 2 (USH2) have abnormal cone structure and density in the central retina. This occurs in the presence of normal acuity, opening the quest for additional sensitive functional measures of central cone function in USH. We tested here whether focal macular cone electroretinogram (fERG) could be such a tool. METHODS. This retrospective study of central cone function loss was based on data from 47 patients with USH2 from the Ophthalmology Department of the Policlinico Gemelli/Catholic University in Rome. The analysis focused on the decrease of the fERG, obtained in response to a 41-Hz sinusoidal modulation of a uniform field presented to the central 18°, generated by red light-emitting diodes (LEDs) and superimposed on an equiluminant steady adapting background. fERG decrease was compared with the decrease of best-corrected visual acuity and Goldmann kinetic perimetry V4E field. RESULTS. fERG follow-up data document a severe and precocious loss of central cone function in USH2 patients, preceding losses in other measures of cone function. fERG is already reduced to 40% of control at the beginning of the second decade of life, and by 25 years of age, all USH2 patients have fERGs less than 30% of control values. CONCLUSIONS. fERG represents a sensitive tool to evaluate central cone function in USH2, anticipating the decline of other central cone function measures, such as visual acuity and Goldmann perimetry.
AB - PURPOSE. Recent studies show that patients with Usher syndrome type 2 (USH2) have abnormal cone structure and density in the central retina. This occurs in the presence of normal acuity, opening the quest for additional sensitive functional measures of central cone function in USH. We tested here whether focal macular cone electroretinogram (fERG) could be such a tool. METHODS. This retrospective study of central cone function loss was based on data from 47 patients with USH2 from the Ophthalmology Department of the Policlinico Gemelli/Catholic University in Rome. The analysis focused on the decrease of the fERG, obtained in response to a 41-Hz sinusoidal modulation of a uniform field presented to the central 18°, generated by red light-emitting diodes (LEDs) and superimposed on an equiluminant steady adapting background. fERG decrease was compared with the decrease of best-corrected visual acuity and Goldmann kinetic perimetry V4E field. RESULTS. fERG follow-up data document a severe and precocious loss of central cone function in USH2 patients, preceding losses in other measures of cone function. fERG is already reduced to 40% of control at the beginning of the second decade of life, and by 25 years of age, all USH2 patients have fERGs less than 30% of control values. CONCLUSIONS. fERG represents a sensitive tool to evaluate central cone function in USH2, anticipating the decline of other central cone function measures, such as visual acuity and Goldmann perimetry.
KW - Cone photoreceptors
KW - Goldmann perimetry
KW - Retina
KW - Retinitis pigmentosa
UR - http://www.scopus.com/inward/record.url?scp=85051234911&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85051234911&partnerID=8YFLogxK
U2 - 10.1167/iovs.17-23703
DO - 10.1167/iovs.17-23703
M3 - Article
AN - SCOPUS:85051234911
VL - 59
SP - 3827
EP - 3835
JO - Investigative Ophthalmology and Visual Science
JF - Investigative Ophthalmology and Visual Science
SN - 0146-0404
IS - 10
ER -