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Can I read Transient pupillary light reflex in CEP290- or NPHP5-associated Leber congenital amaurosis: Latency as a potential outcome measure of cone function on EtoBox?

Transient pupillary light reflex in CEP290- or NPHP5-associated Leber congenital amaurosis: Latency as a potential outcome measure of cone function by Krishnan, Arun K.; Jacobson, Samuel G.; Roman, Alejandro J.; Iyer, Bhavya S.; Garafalo, Alexandra V.; Héon, Elise; Cideciyan, Artur V. is a Neuroscience article available to read on EtoBox.

What is Transient pupillary light reflex in CEP290- or NPHP5-associated Leber congenital amaurosis: Latency as a potential outcome measure of cone function about?

Mutations in photoreceptor cilium genes CEP290 and NPHP5 cause a form of Leber congenital amaurosis (LCA) which typically lacks rods but retains central cones. The current study evaluated the transient pupillary light reflex (TPLR) as an objective outcome measure to assess efficacy of ongoing and future therapies. Eleven eyes of six patients selected for retained cone function were tested with TPLR using full-field stimuli in the dark-adapted state. Stimuli were red or blue with 1 s duration and spanned a 6-log unit dynamic range. TPLR response amplitude was quantified at fixed times of 0.9 and 2 s after stimulus onset and TPLR latency was defined as the time to reach 0.3 mm constriction. Full-field stimulus testing (FST) and static perimetry were used to correlate subjective perception with objective TPLR parameters. TPLR and FST thresholds with both red and blue stimuli were abnormally elevated in patients to near -1.25 log phot-cd·m consistent with the lack of rods. TPLR latencies were delayed on average but showed some differences among patients. Remnant extrafoveal vision was correlated with faster TPLR latencies. Our results support the use of a short TPLR protocol with full-

Who reads Transient pupillary light reflex in CEP290- or NPHP5-associated Leber congenital amaurosis: Latency as a potential outcome measure of cone function?

It is typically read by researchers, students, and practitioners in Neuroscience.

Author
Krishnan, Arun K.; Jacobson, Samuel G.; Roman, Alejandro J.; Iyer, Bhavya S.; Garafalo, Alexandra V.; Héon, Elise; Cideciyan, Artur V.
Publisher
Elsevier Science; Elsevier ; Elsevier Ltd.; Elsevier BV (ISSN 0042-6989)
Published
2020
Language
EN
Field
Neuroscience (Life Sciences)

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