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ObjectiveTo compare subjective and objective clinical tests used in the screening for hydroxychloroquine retinal toxicity to multifocal electroretinography (mfERG) reference testing.DesignProspective, single-center, case control study.ParticipantsFifty-seven patients with a previous or current history of hydroxychloroquine treatment of more than 5 years' duration.MethodsParticipants were evaluated with a detailed medical history, dilated ophthalmologic examination, color fundus photography, fundus autofluorescence (FAF) imaging, spectral-domain (SD) optical coherence tomography (OCT), automated visual field testing (10-2 visual field mean deviation [VFMD]), and mfERG testing. We used mfERG test parameters as a gold standard to divide participants into 2 groups: those affected by hydroxychloroquine-induced retinal toxicity and those unaffected.Main outcome measuresWe assessed the association of various imaging and psychophysical variables in the affected versus the unaffected group.ResultsFifty-seven study participants (91.2% female; mean age, 55.7±10.4 years; mean duration of hydroxychloroquine treatment, 15.0±7.5 years) were divided into affected (n = 19) and unaffected (n = 38) groups based on mfERG criteria. Mean age and duration of hydroxychloroquine treatment did not differ statistically between groups. Mean OCT retinal thickness measurements in all 9 macular subfields were significantly lower (

BackgroundInherited retinal diseases are uncommon, and the likelihood of having more than one hereditary disorder is rare. Here, we report a case of Stargardt disease and congenital stationary night blindness (CSNB) in the same patient, and the identification of two novel in-frame deletions in the GRM6 gene.Materials and methodsThe patient underwent an ophthalmic exam and visual function testing including: visual acuity, color vision, Goldmann visual field, and electroretinography (ERG). Imaging of the retina included fundus photography, spectral-domain optical coherence tomography (OCT), and fundus autofluorescence. Genomic DNA was PCR-amplified for analysis of all coding exons and flanking splice sites of both the ABCA4 and GRM6 genes.ResultsA 46-year-old woman presented with recently reduced central vision and clinical findings of characteristic yellow flecks consistent with Stargardt disease. However, ERG testing revealed an ERG phenotype unusual for Stargardt disease but consistent with CSNB1. Genetic testing revealed two previously reported mutations in the ABCA4 gene and two novel deletions in the GRM6 gene.ConclusionsDiagnosis of concurrent Stargardt disease and CSNB was made on the ophthalmic history, clinical examination, ERG, and genetic testing. This case highlights that clinical tests need to be taken in context, and that co-existing retinal dystrophies and degenerations should be considered when clinical impressions and objective data do not correlate.

Purpose: To study the prevalence, microbial profile, and risk factors for ocular involvement in patients with candidemia. Methods: The records of all inpatient consultations requested at the Brigham and Women's Hospital from October 2009 to June 2011 to evaluate for ocular involvement in patients with candidemia were retrospectively reviewed. Results: Forty-nine consults were requested to rule out ocular involvement in patients with candidemia. The mean patient age was 55 years. In decreasing frequency, the organisms isolated were Candida albicans (42%), Candida parapsilosis (23%), Candida glabrata (17%), Candida tropicalis (8%), Candida dublinensis (5%), Candida krusei (3%), and Candida lusitaniae (3%). All patients were on antifungal treatment at the time of their initial ophthalmologic examination. One patient had evidence of chorioretinitis, and 3 patients had nonspecific fundus findings. Common risk factors included presence of an intravenous catheter, malignancy, and abdominal surgery. Conclusions: Ocular involvement is rare among patients with systemic Candida infection. [ABSTRACT FROM AUTHOR]