TY - JOUR
T1 - 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.
AU - L1, Galli-Resta
AU - G2, Placidi
AU - F2, Campagna
AU - L3, Ziccardi
AU - M2, Piccardi
AU - Minnella, Angelo Maria
AU - E2, Abed
AU - S2, Iovine
AU - P4, Maltese
AU - M4, Bertelli
AU - Falsini, B.
PY - 2018
Y1 - 2018
N2 - PURPOSE:\r\nRecent 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.\r\n\r\nMETHODS:\r\nThis 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.\r\n\r\nRESULTS:\r\nfERG 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.\r\n\r\nCONCLUSIONS:\r\nfERG 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:\r\nRecent 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.\r\n\r\nMETHODS:\r\nThis 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.\r\n\r\nRESULTS:\r\nfERG 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.\r\n\r\nCONCLUSIONS:\r\nfERG 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 - Usher syndrome
KW - cones
KW - electroretinogram
KW - Usher syndrome
KW - cones
KW - electroretinogram
UR - https://publicatt.unicatt.it/handle/10807/125267
UR - https://www.scopus.com/inward/citedby.uri?partnerID=HzOxMe3b&scp=85051234911&origin=inward
UR - https://www.scopus.com/inward/record.uri?partnerID=HzOxMe3b&scp=85051234911&origin=inward
U2 - 10.1167/iovs.17-23703
DO - 10.1167/iovs.17-23703
M3 - Article
SN - 0146-0404
VL - 59
SP - 3827
EP - 3835
JO - Investigative Ophthalmology and Visual Science
JF - Investigative Ophthalmology and Visual Science
IS - 10
ER -