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Images in Neurology
November 2002

Progressive Visual Loss in Optic Nerve Hypoplasia and Bilateral Microdiscs

Arch Neurol. 2002;59(11):1829-1830. doi:10.1001/archneur.59.11.1829

A 55-YEAR-OLD man in good general health without a family history of visual loss was referred for progressive loss of visual acuity. His visual acuity had deteriorated from 20/32 OU in 1992 to 20/60 OU in 2000. The refractive error was +2.25/−0.75/170° OD and +4.00/−1.75/23° OS. Visual fields showed bilateral concentric constriction of 40°(Goldmann III-4) in 1997. On follow-up 3 years later, his visual fields showed variable constriction, ranging from 10° to 20° OU. Slitlamp biomicroscopy revealed normal anterior ocular segments. Ophthalmoscopy showed a normal fundus but bilaterally extremely small optic discs with an area of 0.36 mm2 OD and an area of 0.11 mm2 OS as measured by confocal scanning laser tomography (Figure 1). The retinal and choroidal vascular anatomy were normal on fluorescein angiography. Electroretinography showed normal photopic and scotopic responses to whole-field stimulation. For both eyes, amplitudes of the visual evoked potentials were abnormally low, with a more pronounced reduction for the left eye than for the right eye. High-resolution magnetic resonance imaging (turbo spin-echo repetition time, 4230 ms/echo time, effective echo time, 119 ms; echo train length, 15; slice thickness, 3 mm; matrix, 270 × 512; field of view, 18 × 24 cm; number of excitations, 6; in-plane resolution, 0.67 mm × 0.47 mm; frequency selective fat suppression) showed extremely thin optic nerves and optic chiasm, but no other abnormality that indicated more widespread developmental abnormalities (eg, septo-optic dysplasia1) was identified on cranial magnetic resonance imaging (Figure 2). The neurologic examination disclosed no nystagmus or other abnormal signs, and serum levels of the hypothalamic hormones were unremarkable. Autoantibody screening was negative as were routine biochemical, blood cell count, and clotting studies. None of the known mutations for Leber hereditary optic atrophy were detected. Optic nerve hypoplasia, also known as microdiscs or micropapillae, was diagnosed in the absence of any specific cause (eg, septo-optic dysplasia, Leber hereditary optic atrophy).2,3

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