May 2006
Volume 47, Issue 13
Free
ARVO Annual Meeting Abstract  |   May 2006
Impairment of L– and M–Cone–Driven Electroretinographic Responses in Patients With Spinocerebellar Ataxias and No or Minimal Visual Symptoms
Author Affiliations & Notes
  • G. Stifano
    Catholic University, Rome, Italy
    Ophthalmology,
  • L. Montrone
    Catholic University, Rome, Italy
    Ophthalmology,
  • L. Ziccardi
    Catholic University, Rome, Italy
    Ophthalmology,
  • G. Iarossi
    Catholic University, Rome, Italy
    Ophthalmology,
  • P. Piscopo
    Catholic University, Rome, Italy
    Ophthalmology,
  • M. Masciullo
    Catholic University, Rome, Italy
    Neurology,
  • G. Silvestri
    Catholic University, Rome, Italy
    Neurology,
  • B. Falsini
    Catholic University, Rome, Italy
    Ophthalmology,
  • E. Balestrazzi
    Catholic University, Rome, Italy
    Ophthalmology,
  • Footnotes
    Commercial Relationships  G. Stifano, None; L. Montrone, None; L. Ziccardi, None; G. Iarossi, None; P. Piscopo, None; M. Masciullo, None; G. Silvestri, None; B. Falsini, None; E. Balestrazzi, None.
  • Footnotes
    Support  Ministero Universita' e Ricerca Scientifica, Fondi di Ateneo
Investigative Ophthalmology & Visual Science May 2006, Vol.47, 787. doi:
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      G. Stifano, L. Montrone, L. Ziccardi, G. Iarossi, P. Piscopo, M. Masciullo, G. Silvestri, B. Falsini, E. Balestrazzi; Impairment of L– and M–Cone–Driven Electroretinographic Responses in Patients With Spinocerebellar Ataxias and No or Minimal Visual Symptoms . Invest. Ophthalmol. Vis. Sci. 2006;47(13):787.

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

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Abstract

Purpose: : To evaluate Long (L)– and Middle (M)–wavelength cone–driven electroretinographic responses in patients with spino–cerebellar ataxias (SCAs) and no or minimal visual symptoms

Methods: : Focal (central 18°) electroretinograms (F–ERGs) in response to red (R)–green (G) flickering (41 Hz) uniform stimuli, modulated in counterphase with a luminance color ratio (R/R+G) corresponding to the points of L–cone and M–cone silent substitution (L–cone: 0.78; M–cone: 0.46) were recorded in 11 autosomal dominant SCA 2 genotype patients, in nine autosomal recessive AR–SCA of unknown genotype, and in 15 age–matched normal controls. All patients had normal visual acuity and moderate, non–specific color vision defects (error score 4–9) at desaturated Farnsworth D–15 test. The ERG fundamental response component amplitude and phase, isolated by Fourier analysis, were measured

Results: : When compared to controls, either SCA 2 or AR–SCA patients showed a loss in L– but not M–cone driven ERGs mean amplitude (by 35%, p < 0.05). ERG M–cone, but not L–cone–driven responses had a phase delay (by 90°, about 6.5 milliseconds, p < 0.01). L–cone driven response losses of individual patients were negatively correlated (p < 0.05) with Farnsworth D–15 error score

Conclusions: : The results indicate an abnormality of both L– and M–cone driven retinal responses SCA clinical phenotypes usually not associated with retinal degeneration, suggesting a subclinical impairment of the cone system in the visually asymptomatic disease sub–types

Keywords: neuro-ophthalmology: diagnosis • color vision • electroretinography: clinical 
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