Peripheral Cone Dystrophy

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Abstract

A 19-year-old man reported bilateral peripheral visual field loss, hemeralopia, and photophobia. Examination and testing was in keeping with peripheral cone dystrophy. This rare entity is discussed.

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Peripheral cone dystrophy: a variant of cone dystrophy with predominant dysfunction in the peripheral cone system.

N Kondo, Hiroko Terasaki, Shinji Ueno … (2004)

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. Three observational case reports with electrophysiologic and psychophysical studies. 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. 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. 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.

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Case of Unilateral Peripheral Cone Dysfunction

Yujin Mochizuki, Kei Shinoda, Celso Matsumoto … (2012)

Purpose Peripheral cone dystrophy is a subgroup of cone dystrophy, and only 4 cases have been reported. We present a patient with unilateral peripheral cone dysfunction and report the functional changes determined by electrophysiological tests and ultrastructural changes determined by spectral domain optical coherence tomography (SD-OCT). Case A 34-year-old woman complained of blurred vision in both eyes. Our examination showed that her visual acuity was 0.05 OD and 0.2 OS. A relative afferent pupillary defect was present in her right eye. The results of slit-lamp examination, ophthalmoscopy, and fluorescein angiography were normal except for pallor of the right optic disc. SD-OCT showed a diffuse thinning of the retina in the posterior pole of the right eye. A severe constriction of the visual fields was found in both eyes but more in the right eye. The photopic full-field electroretinograms (ERGs) were reduced in the right eye but normal in the left eye. The multifocal ERGs were severely reduced throughout the visual field except in the central area of the right eye. The multifocal ERGs from the left eye were normal. The pattern visual evoked responses were within the normal range in both eyes. She had a 5-year history of sniffing paint thinner. Results Although the visual dysfunction was initially suspected to be due to psychological problems from the results of subjective tests, objective tests indicated a peripheral cone dysfunction in the right eye. The pathophysiological mechanism and the relationship with thinner sniffing were not determined. Conclusions Our findings indicate that peripheral cone dysfunction can occur unilaterally. Electrophysiology and SD-OCT are valuable tests to perform to determine the pathogenesis of unusual ocular findings objectively.

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Author and article information

Journal

Title: Journal of Neuro-Ophthalmology

Abbreviated Title: Journal of Neuro-Ophthalmology

Publisher: Ovid Technologies (Wolters Kluwer Health)

ISSN (Print): 1070-8022

Publication date Created: 2014

Publication date (Print): December 2014

Volume: 34

Issue: 4

Pages: 366-368

Article

DOI: 10.1097/WNO.0000000000000119

PubMed ID: 24705257

SO-VID: af2efec2-63cf-4007-bfa9-7c94e463b51f

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Peripheral Cone Dystrophy : A Diagnostic Improbability?

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