Childhood versus early-teenage onset Leber’s hereditary optic neuropathy: visual prognosis and capacity for recovery

Abstract

ObjectiveIn Leber’s hereditary optic neuropathy (LHON) in children and teenagers, the influence of age on visual prognosis has not yet been investigated.MethodsPatients from the mitoNET registry with LHON onset at age 4–16 years with at least 4 years of follow-up without treatment were included. Visual acuity (VA) at baseline, lowest VA ever recorded (nadir) and VA at end of follow-up were compared between childhood onset (ChO, ≤12 years of age) and early-teenage onset (eTO; 13–16 years).ResultsOut of 231 patients with LHON, 19 met the inclusion criteria (8.2%). There were 11 patients in the ChO and 8 patients in the eTO group. Mean age at onset was 8.6 (SD 2.1) years (ChO) and 15.4 (SD 0.7) years (eTO) (p<0.00001). Follow-up was mean 184 (SD 129) months (ChO) and 119 (SD 78) months (eTO) (p=0.22). Baseline VA was similar between both groups in better (p=0.96) and worse eyes (p=0.54). In worse eyes, both groups deteriorated similarly (p=0.79) until nadir and showed similar recovery until end of follow-up (p=0.38). In better eyes, both groups deteriorated similarly (p=0.16) until nadir. From nadir until end of follow-up, better eyes in the ChO group showed a significantly better recovery (−0.35 (SD 0.36) vs −0.01 (SD 0.06) logMAR; p=0.02) than eTO eyes.ConclusionVisual prognosis of LHON in children is much more favourable in cases of childhood onset (≤12 years of age) as compared with teenage onset (13–16 years), mostly due to better recovery from nadir in childhood onset.