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Marie-Helene Errera, Andrew R. Webster, Anthony . G. Robson, Tracey Wong, Philip G. Hykin, Bishwanath Pal, Anthony T. Moore, Graham E. Holder; Unilateral Retinitis Pigmentosa? A Retrospective Case Series Of Unilateral Pigmentary Retinopathy. Invest. Ophthalmol. Vis. Sci. 2011;52(14):4995.
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To survey the clinical characteristics of patients with unilateral pigmented retinopathy.
Thirty seven patients were ascertained retrospectively following pattern and full field electroretinography (PERG;ERG) that incorporated the International standards. Clinical findings, fundus photographs and fundus autofuorescence were reviewed.
Of 37 patients, 29 women and 8 men between 13 and 67 years of age were ascertained. Associated conditions, that may explain aetiology were noted in 13 cases (35%) and included direct ocular trauma, prior ocular inflammation, systemic carcinoma, pregnancy induced hypotension and meningitis. An inherited disorder was definitely identified in 2 patients; one had a RP1 germline mutation and dominant family history and one was a female carrier of X-linked RP. Peripheral field loss and photopsias were common symptoms. Some were asymptomatic and were found to have UPR as part of a routine spectacle check-up. Reduced visual acuity was a later feature of the disorder. Fundoscopy revealed a range of abnormalities and varying degrees of intraretinal pigmentary deposition with 12.5% of patient not having frank bone-spicule pigmentation. Two patients had cystoid macular oedema (5%). On electrophysiologic findings, all patients presented a generalised loss of retinal function affecting both rod and cone systems and a dysfunction at the level of the retinal pigment epithelium/ photoreceptor complex. Pattern ERG was abnormal in 29 patients (78.4%) indicating macular involvement. There was subclinical involvement in the better seeing eye in 6 cases (16%) as assessed by electrophysiology with borderline or marginally subnormal full field ERGs, multifocal ERG and fundus autofluorescence imaging.
True unilateral RP is an uncommon cause of unilateral pigmentary retinopathy. Post traumatic and post-inflammatory disorders are more common, but the aetiology of most cases is unknown. Nevertheless, mosaisism for a mutant gene during development cannot be excluded.
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