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Erica Z. Oltra, Clement C. Chow, Felix Y. Chau, Jennifer I. Lim, Heather E. Moss; Neurocognitive Function and Retinal Thinning by Spectral-Domain Optical Coherence Tomography in Sickle Cell Patients. Invest. Ophthalmol. Vis. Sci. 2012;53(14):1160.
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Our group has shown that many sickle cell patients have macular thinning on spectral domain optical coherence tomography (SD-OCT). Cognitive deficits in sickle cell patients have been described and some pediatric studies have shown an association with radiographic markers of cerebrovascular disease. We hypothesized that retinal thinning in patients with sickle cell is a marker for cerebral vasculopathy that would be associated with reduced cognitive function. The purpose of this investigation was to compare cognitive function in sickle cell patients with and without retinal thinning and with and without history of stroke.
Sickle cell patients with and without history of stroke were recruited from an academic retina clinic. All patients had at least a high school education. Retinal thickness was measured using the SD-OCT and stratified into normal and thin based on temporal macula thickness. Cognitive testing was performed by administering the Philadelphia Brief Assessment of Cognition. This is a validated cognition screen that generates subscores for executive function, language, visuospatial operations and memory. The relationship between these subscores, degree of retinal thinning and history of stroke was investigated using linear and logistic regression accounting for age and education.
24 patients were enrolled (age 38.1±13.2 yrs, 66% female). Binocular average temporal macular thickness was 244.3 μm±7.5 and 202.3±13.8 in the normal and thin groups respectively (p<0.00001). 4 subjects had a history of stroke. Age and education were similar in all groups. Sickle cell subjects performed below average on tests of executive function (6.4±2.5 for those with history of stroke and 10.9±2.4 for those without history of stroke compared to published norm of 14.1±2.15). Multiple regression accounting for education identified correlations between history of stroke and all cognitive testing subscores (p=0.008-0.026), and between age and memory (p<0.00001) and visual spatial (p=0.022) subscores, but not between retinal thinning and cognitive impairment (p=0.12-0.45). Logistic regression did not identify correlations between retinal thinning, history of stroke (p=0.113), age (p=0.686) or education (p=0.074).
We confirm that executive function is compromised in patients with sickle cell and that history of stroke is associated with impaired cognitive testing. We did not find a significant correlation between retinal thinning and cognitive function or history of stroke. Further investigation is necessary to define the relationship between retinal thinning, retinal microvascular disease and cerebral vascular disease in sickle cell.
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