Detection of fenestrated central scotoma by scanning laser ophthalmoscope microperimetry in a patient with Leber’s hereditary optic neuropathy after visual recovery

Abstract

Landmark-driven fundus microperimetry using a scanning laser ophthalmoscope (SLO) was performed to assess the local sensitivities of the central retina and to determine the fixation point in a patient with Leber’s hereditary optic neuropathy (LHON) after visual recovery. A 16-year-old Japanese boy was diagnosed as having LHON with an 11778 mutation of mitochondrial DNA. Fourteen months after starting idebenone and vitamin therapy, his vision began to recover and reached 1.0 in both eyes. His corrected visual acuity, Humphrey 10-2 threshold test, SLO microperimetry, critical flicker frequency (CFF), and visual evoked potentials (VEPs) were tested after visual recovery. At an early stage of visual recovery, the Humphrey 10-2 threshold test revealed a fenestrated scotoma in the right eye and a dense central scotoma in the left eye. SLO microperimetry of the left eye, however, detected a few spots that were sensitive to a stimulus intensity of 0 dB on the temporal side of the fovea. A fixation point was detected on the nasal side of the fovea and it was relatively stable in both eyes. CCF and VEPs deteriorated despite the recovery of vision in both eyes. The ability to detect small islands of vision and to identify fixation points are two advantages of SLO microperimetry over Humphrey perimetry.