Rod Photoreceptor Temporal Properties in Retinitis Pigmentosa

摘要

One of the characteristic signs of retinitis pigmentosa (RP) is the progressive loss of night vision. We have previously shown that the gain of rod photoreceptor activation is moderately reduced in some patients with RP, but this decrease in activation kinetics is not sufficient to account for the night blindness. Recently, single rod recording from animal models of RP showed rods under degeneration remain saturated for shorter periods than normal rods; i.e. are less able to sustain the rod photoresponse. Using paired-flash ERG, here we determine whether rod phototransduction inactivation parameters might also be abnormal in patients with RP. Inactivation parameters were derived from 13 subjects with normal vision, 16 patients with adRP, and 16 patients with autosomal recessive/isolate (rec/iso) RP. The adRP cases included 9 patients with rhodopsin mutations and 7 patients with peripherin/RDS mutations. The inactivation phase was derived using a double-flash paradigm, with a test flash of 2.7 log scot td-sec followed at varying intervals by a 4.2 log scot td-sec probe flash. Derived rod photoresponses to this just-saturating test flash in normal subjects exhibit a critical time to the initiation of recovery (Tsat) of 525±90 (SD) msec. The values of Tsat were 336±104 (SD) msec in patients with adRP (P<0.001) and 271±45 (SD) msec (P<0.001) in patients with rec/iso RP. When Tsat values were categorized by mutations, the values were 294±91 (SD) msec (P<0.001) for rhodopsin mutations, and 389±100 (SD) msec (p=0.01) for peripherin/RDS mutations. Overall, Tsat in patients with RP was significantly correlated with the amplitude of ISCEV standard rod response (r = 0.56; P < 0.001) and the gain of the activation phase of phototransduction (r=0.6, P<0.001). Tsat may be a useful marker for therapeutic efficacy in future clinical trials in RP.