EurekaMag.com logo
+ Site Statistics
References:
52,725,316
Abstracts:
28,411,598
+ Search Articles
+ Subscribe to Site Feeds
EurekaMag Most Shared ContentMost Shared
EurekaMag PDF Full Text ContentPDF Full Text
+ PDF Full Text
Request PDF Full TextRequest PDF Full Text
+ Follow Us
Follow on FacebookFollow on Facebook
Follow on TwitterFollow on Twitter
Follow on Google+Follow on Google+
Follow on LinkedInFollow on LinkedIn

+ Translate

Electrophysiological study on cone rod dystrophy of bull's eye macular lesion type






Nihon University Journal of Medicine 28(3): 175-188

Electrophysiological study on cone rod dystrophy of bull's eye macular lesion type

Nine cases of cone-rod dystrophy of bull's eye macular lesion type were examined electrophysiologically. 7 eyes of 7 normal cases were studied as a control by the same electrophysiological techniques. 1) Widespread cone-dysfunction could be detected from abnormalities of the photopic b wave, rapid off decay and 30 Hz flicker ERG. 2) The degree of cone-dysfunction which was evaluated from the abnormalities of the photopic b wave and 30 Hz flicker ERG was found not to correlate with the extent of the pathologic lesion as demonstrated by fluorescein angiography and ophthalmoscopy. 3) The results of color vision tests in 5 cases were compared with their spectral response curves, which were obtained from the amplitude of the rapid off decay of ERG stimulated by monochromatic light of various wavelengths. The total error scores of the color vision tests were found to correlate with the amplitude of the monochromatic ERG. 4) Even in cases which showed ophthalmoscopic abnormalities only in the macular area, an abnormal scotopic b wave was observed. These results indicate that rod dysfunction precedes discernible ophthalmoscopic abnormalities.


Accession: 005349865



Related references

The PROM1 mutation p.R373C causes an autosomal dominant bull's eye maculopathy associated with rod, rod-cone, and macular dystrophy. Investigative Ophthalmology & Visual Science 51(9): 4771-4780, 2010

Macular dystrophy with protan genotype and phenotype studied with cone type specific ERGs. Current Eye Research 22(3): 221-228, 2001

Cone dysfunction in patients with late-onset cone dystrophy and age-related macular degeneration. Archives of Ophthalmology 121(11): 1557-1561, 2003

Novel GUCA1A mutations suggesting possible mechanisms of pathogenesis in cone, cone-rod, and macular dystrophy patients. Biomed Research International 2013: 517570-517570, 2014

Electrophysiological testing as a method of cone-rod and cone dystrophy diagnoses and prediction of disease progression. Documenta Ophthalmologica. Advances in Ophthalmology 130(2): 103-109, 2015

Analysis of macular cone photoreceptors in a case of occult macular dystrophy. Clinical Ophthalmology 7(): 859-864, 2013

Early detection of central visual function decline in cone-rod dystrophy by the use of macular focal cone electroretinogram. Investigative Ophthalmology & Visual Science 54(10): 6560-6569, 2013

Mutation in the gene GUCA1A, encoding guanylate cyclase-activating protein 1, causes cone, cone-rod, and macular dystrophy. Ophthalmology 112(8): 1442-1447, 2005

Depolarizing pattern of macular cone ERG in human macular dystrophy. IOVS 39(4): S723, March 15, 1998

Histo pathogenic study of the macular dystrophy of the cornea fehrs dystrophy or groenouws type ii. Ophthalmic Research 7(4): 261-269, 1975