Comparative Analysis of Functional and Structural Decline in Retinitis Pigmentosas

International Journal of Molecular Sciences

JimmyK. Duong¹  Rubens Belfort²  KarenSophia Park³  Katherine Boudreault³  Thiago Cabral³  JoseRonaldo Lima de Carvalho³  JinKyun Oh³  SarahR. Levi³  StephenH. Tsang³  Joonpyo Kim⁴  Junhyung Park⁵ 

[1] Department of Biostatistics, Columbia University, New York, NY 10032, USA;Department of Ophthalmology, Federal University of São Paulo (UNIFESP), São Paulo, SP 04039-032, Brazil;Department of Ophthalmology, Jonas Children’s Vision Care and Bernard & Shirlee Brown Glaucoma Laboratory, Columbia University, New York, NY 10032, USA;Department of Statistics, Seoul National University, Seoul 08826, Korea;Department of Statistics, University of California, Los Angeles (UCLA), Los Angeles, CA 90095, USA;

关键词: retinitis pigmentosa;    ERG;    inherited retinal dystrophy;   

DOI  :  10.3390/ijms21082730

来源: DOAJ

【 摘 要 】

Retinitis pigmentosa (RP) is a category of inherited retinal dystrophies that is best prognosticated using electroretinography (ERG). In this retrospective cohort study of 25 patients with RP, we evaluated the correlation between 30 Hz flicker ERG and structural parameters in the retina. Internationally standardized 30 Hz flicker ERG recordings, short-wavelength autofluorescence (SW-AF), and spectral domain–optical coherence tomography (SD-OCT) were acquired at two visits at least one year apart. Vertical and horizontal hyperautofluorescent ring diameter measurements with SW-AF, as well as ellipsoid zone (EZ) line width measurements with SD-OCT, were used as structural parameters of disease progression. The 30 Hz flicker ERG amplitude decreased by 2.2 ± 0.8 µV/year (p = 0.011), while implicit times remained unchanged. For SD-OCT, the EZ line decreased by 204.1 ± 34.7 µm/year (p < 0.001). Horizontal and vertical hyperautofluorescent ring diameters decreased by 161.9 ± 25.6 µm/year and 146.9 ± 34.6 µm/year, respectively (p = 0.001), with SW-AF. A correlation was found between the progression rates of the 30 Hz flicker amplitude recorded with Burian–Allen electrodes and both the horizontal ring diameter (p = 0.020) and EZ line (p = 0.044). SW-AF and SD-OCT, two readily available imaging techniques, may be used to prognosticate disease progression because of the reliability of their measurements and correlation with functional outcome.

【 授权许可】

Unknown