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Peripheral cone dystrophy: a variant of cone dystrophy with predominant dysfunction in the peripheral cone system.
Ophthalmology. 2004 Apr; 111(4):732-9.O

Abstract

PURPOSE

To determine the phenotype of 3 patients from 2 pedigrees with an unusual form of cone dystrophy in which the peripheral cone system is more affected than the central cone system, and whose rod system is relatively normal.

DESIGN

Three observational case reports with electrophysiologic and psychophysical studies.

METHODS

Three patients underwent fundus photography, fluorescein angiography, color vision testing, Goldmann visual field testing, full-field electroretinograms (ERGs), focal macular cone ERGs, and rod-cone perimetry, in addition to routine ophthalmologic examinations. Multifocal ERGs also were recorded from 2 patients.

RESULTS

The fundus examination and fluorescein angiogram results essentially were normal except for a mild temporal pallor of the optic disc in 2 patients. The corrected visual acuity ranged from 20/16 to 20/100. Color vision was normal in 1 patient, but was abnormal in 2 patients. A relative paracentral scotoma was detected in 2 patients. Full-field ERG cone responses were reduced significantly, but rod responses were normal in all patients, as in patients with typical cone dystrophy. However, the focal macular cone ERGs were well preserved in all patients. Psychophysical rod-cone perimetry demonstrated that the peripheral cone system was impaired, whereas the rod sensitivity was completely normal. The results of the multifocal ERG in 2 patients supported the findings made by the full-field and focal macular ERGs.

CONCLUSIONS

These findings demonstrate that there is a subgroup of patients with cone dystrophy where the peripheral cone system is more affected than the central cone system.

Authors+Show Affiliations

Department of Ophthalmology, Nagoya University School of Medicine, 65 Tsuruma-cho, Showa-ku, Nagoya 466-8550, Japan. kondomi@med.nagoya-u.ac.jpNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

15051206

Citation

Kondo, Mineo, et al. "Peripheral Cone Dystrophy: a Variant of Cone Dystrophy With Predominant Dysfunction in the Peripheral Cone System." Ophthalmology, vol. 111, no. 4, 2004, pp. 732-9.
Kondo M, Miyake Y, Kondo N, et al. Peripheral cone dystrophy: a variant of cone dystrophy with predominant dysfunction in the peripheral cone system. Ophthalmology. 2004;111(4):732-9.
Kondo, M., Miyake, Y., Kondo, N., Ueno, S., Takakuwa, H., & Terasaki, H. (2004). Peripheral cone dystrophy: a variant of cone dystrophy with predominant dysfunction in the peripheral cone system. Ophthalmology, 111(4), 732-9.
Kondo M, et al. Peripheral Cone Dystrophy: a Variant of Cone Dystrophy With Predominant Dysfunction in the Peripheral Cone System. Ophthalmology. 2004;111(4):732-9. PubMed PMID: 15051206.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Peripheral cone dystrophy: a variant of cone dystrophy with predominant dysfunction in the peripheral cone system. AU - Kondo,Mineo, AU - Miyake,Yozo, AU - Kondo,Nagako, AU - Ueno,Shinji, AU - Takakuwa,Hideo, AU - Terasaki,Hiroko, PY - 2002/07/18/received PY - 2003/07/30/accepted PY - 2004/3/31/pubmed PY - 2004/4/16/medline PY - 2004/3/31/entrez SP - 732 EP - 9 JF - Ophthalmology JO - Ophthalmology VL - 111 IS - 4 N2 - PURPOSE: To determine the phenotype of 3 patients from 2 pedigrees with an unusual form of cone dystrophy in which the peripheral cone system is more affected than the central cone system, and whose rod system is relatively normal. DESIGN: Three observational case reports with electrophysiologic and psychophysical studies. METHODS: Three patients underwent fundus photography, fluorescein angiography, color vision testing, Goldmann visual field testing, full-field electroretinograms (ERGs), focal macular cone ERGs, and rod-cone perimetry, in addition to routine ophthalmologic examinations. Multifocal ERGs also were recorded from 2 patients. RESULTS: The fundus examination and fluorescein angiogram results essentially were normal except for a mild temporal pallor of the optic disc in 2 patients. The corrected visual acuity ranged from 20/16 to 20/100. Color vision was normal in 1 patient, but was abnormal in 2 patients. A relative paracentral scotoma was detected in 2 patients. Full-field ERG cone responses were reduced significantly, but rod responses were normal in all patients, as in patients with typical cone dystrophy. However, the focal macular cone ERGs were well preserved in all patients. Psychophysical rod-cone perimetry demonstrated that the peripheral cone system was impaired, whereas the rod sensitivity was completely normal. The results of the multifocal ERG in 2 patients supported the findings made by the full-field and focal macular ERGs. CONCLUSIONS: These findings demonstrate that there is a subgroup of patients with cone dystrophy where the peripheral cone system is more affected than the central cone system. SN - 0161-6420 UR - https://www.unboundmedicine.com/medline/citation/15051206/Peripheral_cone_dystrophy:_a_variant_of_cone_dystrophy_with_predominant_dysfunction_in_the_peripheral_cone_system_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0161-6420(03)01520-3 DB - PRIME DP - Unbound Medicine ER -