Abstract
Quantitative layer-by-layer perimetry provides a non-invasive, psychophysical means of evaluating the functional characteristics of human retina. By comparing the results obtained from normal, healthy subjects to those of patients exhibiting retinal abnormalities, insight may be gained into both the underlying pathophysiology of those abnormalities as well as their effects upon visual function.
Here, we report data obtained from a patient with gyrate atrophy of the retina and choroid. Evaluation of the spatial interaction properties of the fovea and two parafoveal areas of this patient’s retina revealed a profound loss of spatial sensitization found in normal subjects. Testing performed at the same retinal locations, but using a stimulus which probed transient-like properties of the inenr retina, revealed significant, though somewhat less dramatic deviations from normal. The foveal results showed no significatt changes when retested 19 months after the initial evaluation. Thus, we conclude that this patient’s visual loss was likely due to physiological effects whose influence was not limited to the outer retina, but included the inner retinal layer, as well.
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
References
Aishinoff, S., McCulloch, J.C., Parker, J.A., Phillips, M.J. and Marliss, E.B. Ornithine (ORN) metabolism and liver pathology in hyperornithinaemia and gyrate atrophy of the choroid and retina (HOGA) (absract). Clin. Res. 25: 321A (1977).
Enoch, J.M. Quantitative layer-by-layer perimetry; Proctor Lecture. Invest. Ophthalmol. Vis. Sci. 17: 208–257 (1978).
Enoch, J.M., Fitzgerald, C.R., and Campos, E.C. Quantitative Layer-by-Layer Perimetry: An Extended Analysis. Grune and Stratton, Inc., New York, (1981).
Enoch, J.M., O’Donnell, J.J., Williams, R.A., and Essock, E.A. The relationship between observed retinal boundaries and the functional limit of vision in gyrate atrophy. Arch. Ophthalmol, (submitted).
Jacobson, E. Ein Fall von Retinitis pigentosa atypica. Klin. Monatsbl. Augenheilkd. 26:202–206 (1888).
Kaiser-Kupfer, M.I., Kuwabara, T., Askanas, V., Brody, L., Takki, K., Dworestzky, I., and Engel, W.K. Systemic manifestations of gyrate atrophy of the choroid and retina. Ophthalmol. 88: 302–306 (1981).
Kaiser-Kupfer, M.I., Valle, D. and Del Valle, L.A. A specific enzyme defect in gyrate atrophy. Amer. J. Ophthalmol. 85: 200–204 (1978).
Kennaway, N.G., Weleber, R.G. and Buist, N.R.M. Gyrate atrophy of choroid and retina: Deficient activity of ornithine ketoacid aminotransferase in cultured skin fibroblasts, (correspondence) N. Engl. J. Med. 297: 1180 (1977).
Kennaway, N.G., Weleber, R.G. and Buist, N.R.M. Gyrate atrophy of the choroid and retina with hyper ornithinemia: Biochemical and histologic studies and response to vitamin B6. Amer. J. Hum. Genet. 32: 529–541 (1980).
McCulloch, C. and Marliss, E.B. Gyrate atrophy of the choroid and retina with hyperornithinemia. Am. J. Ophthalmol. 80:1047–1057 (1975).
McCulloch, J.C., Arshinoff, S.A., Marliss, E.B. and Parker, J.A. Hyperornithinemia and gyrate atrophy of the choroid and retina. Ophthalmol. 85: 918–928 (1978).
O’Donnell, J.J., Sandman, R.P. and Martin, S.R. Deficient L-ornithine; 2-oxoacid aminotransferase activity in cultured fibroblasts from a patient with gyrate atrophy of the retina. Biochem. Biophys. Res. Commun. 79: 396–399 (1977).
Sengers, R.C.A., Trijbels, J.M.F., Brussaart, J.H. and Deutman, A.F. Gyrate atrophy of the choroid and retina and ornithine-detoacid aminotransferase deficiency (abstract) Pediat. Res. 10: 894 (1976).
Shih, V., Berson, E.L., Mandell, R. and Schmidt, S.Y. Ornithine ketoacid transaminase deficiency in gyrate atrophy of the choroid and retina. Am. J. Hum. Genet. 30: 174–179 (1978).
Sim ell, O. and Takki, K. Raised plasma ornithine and gyrate atrophy of the choroid and retina. Lancet 1: 1031 (1973).
Sipila, I., Simell, O., Rapola, J., Sainio, K. and Tuuteri, L. Gyrate atrophy of the choroid and retina with hyperornithinemia: tubular aggregates and type 2 fiber atrophy in muscle. Neurology 29: 996–1005 (1979).
Takki, K. Gyrate atrophy of the choroid and retina associated with hyperornithinaemia. Brit. J. OphthalmoL 58: 3–23 (1974).
Takki, K.K. and Milton, R.C. The natural history of gyrate atrophy of the choroid and retina. Ophthalmol. 88: 292–301 (1981).
Trijbels, J.M.F., Sengers, R.C.A., Bakkeren, J.A.J.M., De Kort, A.F.M. and Deutman, A.F. L-ornithine-ketoacid-transaminase deficiency in cultured fibroblasts of a patient with hyperornithinaemia and gyrate atrophy of the choroid and retina. Clin. Chim. Acta 79: 371–377 (1977).
Valle, D., Kaiser-Kupfer, M.I. and Del Valle, L.A. Gyrate atrophy of the choroid and retina: Deficiency of ornithine aminotransferase in transformed lymphocytes. Proc. Natl. Acad. Sci. USA 74: 5159–5161 (1977).
Yasuma, Y., Hamer, R.D., Lakshminarayanan, V., Enoch, J.M. and O’Donnell, J.J. Directional sensitivity measured in a patient with gyrate atrophy: 1. Measurements in different parts of the remaining retina. Arch. Ophthalmol, (submitted).
Author information
Authors and Affiliations
Editor information
Rights and permissions
Copyright information
© 1985 Dr. W. Junk Publishers, Dordrecht, The Netherlands
About this chapter
Cite this chapter
O’Donnell, J., Fendick, M., Enoch, J.M. (1985). Abnormal Inner Retinal Function in Gyrate Atrophy. In: Heijl, A., Greve, E.L. (eds) Sixth International Visual Field Symposium. Documenta Ophthalmologica Proceedings Series, vol 42. Springer, Dordrecht. https://doi.org/10.1007/978-94-009-5512-7_69
Download citation
DOI: https://doi.org/10.1007/978-94-009-5512-7_69
Publisher Name: Springer, Dordrecht
Print ISBN: 978-94-010-8932-6
Online ISBN: 978-94-009-5512-7
eBook Packages: Springer Book Archive