Clinical Features Of Leber’s Hereditary Optic Neuropathy And The Features Analyzed By OCT

[Objects]To analyse clinical features of Leber’s hereditary optic neuropathy (LHON),and to study the retinal never fiber layer thickness（RNFLT） and macularthickness（MT）by optical coherence tomography (OCT).[Methods]Ninety LHON patients in our hospital from December2009to March2013were collected. Analyse clinical features. Thirty-five LHON patients fromSeptember2011to July2012, and30healthy controls were collected, according todisease duration, affected eyes were divided into five groups which separated by3,6,9,12months of time nodes. Five female patients,18female carriers, and25healthy controls were collected. RNFLT and MT were compared.[Results]1. Clinical Features Age of disease onset between3-44a (mean17.88±7.55a),male: female was9:1. Visual acuity ranging from light perception to0.8,66.7%ofthem <0.1. That of77.3%pupillary light reflex were sensitive,22.7%wereinsensitve,14.8%of them with relative afferent pupillary defect (RAPD). Therewas no obvious optic disc edema in the early phase,54.3%of them have clearborder and reddish color, and in the atrophic phase,67.9%of them have optic discwere pallor. About visual field,71.9%showed central or paracentral scotoma.74.1%F-VEP and all P-VEP were abnormal.2. RNFLT changes of LHON patients Compared to the control group, thefirst group showed a thicker RNFL in the average, superior, nasal and inferiorquadrants (P=0.013,0.007,0.008,0.010); no significant change was detected inthe temporal quadrant. The second group showed a thinner RNFL in the temporalquadrant; no significant changes were detected in the other quadrants. The third and fourth groups showed a thinner RNFL in the average, temporal, superior andinferior quadrants (P=0.001,0.001,0.025,0.001), except for the nasal quadrant.The fifth group showed a thinner RNFL in all quadrants (P=0.000). BCVA didn’tcorrelate with RNFLT (P＞0.073).3. MT changes of LHON patients Compared to the control group, the firstgroup had a thinner MT in4medial and nasal side of lateral sub-area (P<0.05forall) but had no significant changes in other areas. The second group had a thinnerMT in cube average thickness,4medial, and temporal and nasal side of lateralsub-areas (P<0.05for all) except for the superior and inferior side of lateralsub-areas. All the measurements in the last three groups showed a significantreduction (p<0.05). BCVAdidn’t correlate with MT (p=0.218).4. Changes of RNFLT and MT in LHON female carrier Compared to thecontrol group, female carriers had a thinner MT in4medial sub-areas (P<0.05forall) but had no significant changes in the other areas and RNFLT. Compared to thefemale carrier, except for the central sub-area MT and nasal quadrant RNFLT,female patients showed significant reduction in all the other areas (P<0.05for all).[Conclusions]1. LHON mostly occurred in young adult male, and manifested as painless centralvision defect, visual impairment was severe. There was no obvious optic discedema and exudation in early phase. Most patients have abnormal VEP.2. The RNFLT undergone a first thickening then thinning process. The temporalquadrant affected firstly, and nasal lastly. BCVAdidn’t correlate with RNFLT.3. MT reduction prior to RNFLT,4medial and nasal side of lateral sub-areasthinned firstly, temporal side of lateral sub-area thinned secondly, superior andinferior side of lateral sub-areas thinned lastly. BCVAdidn’t correlate with MT.4. Female carriers have normal RNFLT, but have thinner MT at4medial sub-area.