Natural course of ocular function in pigmented paravenous retinochoroidal atrophy.
OBJECTIVE
To estimate mean rates of change of ocular function in patients with pigmented paravenous retinochoroidal atrophy (PPRCA).


METHODS
Retrospective observational case series.


METHODS
Fifteen patients aged 8 to 67 years with PPRCA were followed for 3 to 35 years (average follow-up time, 13 years) with measures of visual acuity, visual field area, and full-field electroretinogram amplitude. Mean annual exponential rates of change were quantified by repeated measures longitudinal regression.


RESULTS
Estimated mean annual rates of change were -2.0% for visual acuity, +0.3% for visual field area, and -3.4% and -6.7% for 0.5 Hz and 30 Hz electroretinogram amplitudes, respectively.


CONCLUSIONS
Patients with PPRCA have a slowly progressive disease with respect to the loss of peripheral vision.
Berman-Gund Laboratory for the Study of Retinal Degenerations, Harvard Medical School, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, USA.
John Y
JY
Choi
Michael A
MA
Sandberg
Eliot L
EL
Berson
D000293:Adolescent; D000328:Adult; D000368:Aged; D001284:Atrophy; D002648:Child; D002829:Choroid / Q000473:pathology; D018450:Disease Progression; D004596:Electroretinography; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D010857:Pigment Epithelium of Eye / Q000473:pathology; D012160:Retina / Q000502:physiology*; D012174:Retinitis Pigmentosa / Q000503:physiopathology*; D012189:Retrospective Studies; D014786:Vision Disorders / Q000503:physiopathology*; D014792:Visual Acuity / Q000502:physiology*; D014794:Visual Fields / Q000502:physiology*