Comparative Analysis of Functional and Structural Decline in Retinitis Pigmentosas

• Published in: International journal of molecular sciences 2020-04, Vol.21 (8), p.2730
• Main Authors: Cabral, Thiago, Lima de Carvalho, Jr, Jose Ronaldo, Kim, Joonpyo, Oh, Jin Kyun, Levi, Sarah R, Park, Karen Sophia, Duong, Jimmy K, Park, Junhyung, Boudreault, Katherine, Belfort, Jr, Rubens, Tsang, Stephen H
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Aged; Aged, 80 and over; Apoptosis; Child; Cohort Studies; Comparative analysis; Correlation analysis; Disease Progression; Electrodes; Electroretinograms; Electroretinography; Electroretinography - methods; Female; Flicker; Follow-Up Studies; Horizontal cells; Humans; Imaging techniques; Male; Middle Aged; Optical Coherence Tomography; Patients; Prognosis; Retina; Retina - diagnostic imaging; Retina - pathology; Retina - physiopathology; Retinal Dystrophies - congenital; Retinal Dystrophies - diagnostic imaging; Retinal Dystrophies - pathology; Retinitis; Retinitis pigmentosa; Retinitis Pigmentosa - diagnostic imaging; Retinitis Pigmentosa - genetics; Retinitis Pigmentosa - pathology; Retinitis Pigmentosa - physiopathology; Retrospective Studies; Structure-function relationships; Tomography, Optical Coherence
• ISSN: 1422-0067; 1661-6596; 1422-0067
• DOI: 10.3390/ijms21082730

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 ( = 0.011), while implicit times remained unchanged. For SD-OCT, the EZ line decreased by 204.1 ± 34.7 µm/year ( < 0.001). Horizontal and vertical hyperautofluorescent ring diameters decreased by 161.9 ± 25.6 µm/year and 146.9 ± 34.6 µm/year, respectively ( = 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 ( = 0.020) and EZ line ( = 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.