Long-term decline of central cone function in retinitis pigmentosa evaluated by focal electroretinogram

Benedetto Falsini, Lucia Galli-Resta, Antonello Fadda, Lucia Ziccardi, Marco Piccardi, Giancarlo Iarossi, Giovanni Resta

Research output: Contribution to journalArticlepeer-review

9 Citations (Scopus)


PURPOSE: We evaluated long-term changes of central cone-mediated function in retinitis pigmentosa (RP) patients by recording focal electroretinograms (fERG). METHODS: A cohort of 43 RP patients was followed from 4 to 16 years (average follow-up 9.3 years, average 10 examinations/patient) by recording the fERG response to a flickering uniform red field overlaying the central 18° of visual field (VF). Statistical censoring led to a reduced dataset of 32 patients (autosomal dominant 9, recessive 5, sporadic 5, x-linked 1, Usher II 12), from which long-term decay rates were estimated by global fitting of individual fERG amplitude time-curves. RESULTS: Long-term follow-up of central cone FERG amplitude showed two main features: short-term variability and long-term decline. fERG short-term variability range was 0.14 to 0.2 log units. Mean yearly decay rate of central fERG was 5.6% (95% confidence interval [CI] 4%-7%). Yearly decline depended on inheritance pattern, being significantly greater in autosomal recessive and sporadic compared to autosomal dominant RP. The degree of central cone fERG decline was unrelated to the size of the residual VF. CONCLUSIONS: The decline of central cone function is significantly slower than global cone function decline in RP. Central cone fERG loss is independent of residual VF.
Original languageEnglish
Pages (from-to)7701-7709
Number of pages9
Publication statusPublished - 2012


  • Adolescent
  • Child
  • Child, Preschool
  • Disease Progression
  • Electroretinography
  • Female
  • Follow-Up Studies
  • Humans
  • Male
  • Retinal Cone Photoreceptor Cells
  • Retinitis Pigmentosa
  • Time Factors
  • Visual Acuity
  • Visual Fields


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