Abstract
We examined cone and rod electroretinograms to ganzfeld stimuli in a patient with crystalline retinopathy. The 54-year-old man complained of night blindness, blurred vision, and metamorphopsia in both eyes. His visual acuity was 10/200 in the right eye and 10/20 in the left eye; his subjective dark-adaptation threshold was elevated 1 log unit, and he made one tritan error on the Farnsworth Panel D-15. Specular microscopic examinations revealed tiny crystalline deposits in the limbal cornea bilaterally. Ophthalmoscopically, crystalline deposits were found in the posterior fundi. His light-adapted cone electroretinograms to white stimuli were diminished (about 30% of those of normal controls), with normal implicit times. His darkadapted rod electroretinogram amplitudes were 10% of those of normal controls. The S-cone electroretinogram was not detectable to different spectral stimuli with strong white background, while the L-M-cone responses appeared normal in waveforms with reduced amplitude. These ERG results indicated that the patient's S-cone system is more highly impaired than the L-M-cone system, supporting the psychophysical evidence that the S-cone system is more vulnerable than other cone systems in retinal diseases.
Similar content being viewed by others
References
Bietti GB. Ueber familiares Vorkommen von ‘Retinitis punctata albescens’ (verbunden mit ‘Dystrophia marginalis cristallinea corneae’): glitzern des Glaskörpers und anderen degenerativen Augenveränderungen. Klin Monatsbl Augenheilkd 1937; 99: 737–56.
Grizzard WS, Deutman AF, Nijhuis F, de Kerk AA. Crystalline retinopathy. Am J Ophthalmol 1978; 86: 81–8.
Hayasaka S, Okuyama S. Crystalline retinopathy. Retina 1984; 4: 177–81.
Wilson DJ, Weleber RG, Klein ML, Welch RB, Green WR. Bietti's crystalline dystrophy: a clinicopathologic correlative study. Arch Ophthalmol 1989; 107: 213–21.
Kaiser-Kupfer MI, Chan CC, Markello TC, Crawford MA, Caruso RC, Csaky KG, Guo J, Gahl WA. Clinical biochemical and pathologic correlations in Bietti's crystalline retinopathy. Am J Ophthalmol 1994; 118: 569–82.
Gouras P, MacKay CJ. Electroretinographic responses of the short-wavelength sensitive cones. Invest Ophthalmol Vis Sci 1990; 31: 1203–9.
Gouras P, MacKay CJ, Yamamoto S. The human S-cone electroretinogram and its variation among subjects with and without L and M-cone function. Invest Ophthalmol Vis Sci 1993; 34: 2437–42.
Hood DC, Benimoff NI, Greenstein VC. The response range of the blue-cone pathways: a source of vulnerability to disease. Invest Ophthalmol Vis Sci 1984; 25: 864–7.
Greenstein VC, Hood DC, Ritch R, Steinberger D, Carr RE. S (blue) cone pathway vulnerability in retinitis pigmentosa, diabetes and glaucoma. Invest Ophthalmol Vis Sci 1989; 30: 1732–7.
Swanson WH, Birch DG, Anderson JL. S-cone function in patients with retinitis pigmentosa. Invest Ophthalmol Vis Sci 1993; 34: 3045–55.
Miyake Y, Yagasaki K, Ichikawa H. Differential diagnosis of congenital tritanopia and dominantly inherited juvenile optic atrophy. Arch Ophthalmol 1985; 103: 1496–501.
Bailey JE, Montag E. Short wavelength sensitive cone system function in hereditary tritanopia. Invest Ophthalmol Vis Sci 1992; 33(suppl): 701.
Kamiyama M, Yamamoto S, Hirata H. S-cone ERG in diabetes with and without retinopathy. Invest Ophthalmol Vis Sci 1995; 36 (Suppl): 5480.
de Monasterio FM, McCrane EP, Newlander JK, Schein SJ. Density profile of blue-sensitive cones along the horizontal meridian of macaque retina. Invest Ophthalmol Vis Sci 1985; 26: 289–302.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Yamamoto, S., Kataoka, Y., Kamiyama, M. et al. Nondetectable S-cone electroretinogram in a patient with crystalline retinopathy. Doc Ophthalmol 90, 221–227 (1995). https://doi.org/10.1007/BF01203858
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF01203858