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 L1, Placidi G2, Campagna F2, Ziccardi L3, Piccardi M2, Angelo Maria Minnella, Abed E2, Iovine S2, Maltese P4, Bertelli M4, B. Falsini*

*Autore corrispondente per questo lavoro

Risultato della ricerca: Contributo in rivistaArticolopeer review

2 Citazioni (Scopus)

Abstract

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.
Lingua originaleInglese
pagine (da-a)3827-3835
Numero di pagine8
RivistaInvestigative Ophthalmology and Visual Science
Volume59
Numero di pubblicazione10
DOI
Stato di pubblicazionePubblicato - 2018

All Science Journal Classification (ASJC) codes

  • Oftalmologia
  • Sistemi Sensoriali
  • Neuroscienze Cellulari e Molecolari

Keywords

  • Usher syndrome
  • cones
  • electroretinogram

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