Aims-To report the clinical features of five patients with non-progressive
central ring scotomas of acute onset associated with excellent retained vis
ual acuity.
Methods-Complete neuro-ophthalmological examinations were performed. Visual
fields were performed by tangent screen, Goldmann, or Humphrey perimetry.
In some cases further testing was carried out including fundus photography,
fluorescein angiography, ERG, VEP, and neuroimaging.
Results-The patients were three women and two men whose ages ranged from 25
to 57 years. Four patients were heavy caffeine consumers while the fifth p
atient experienced an episode of hypotension. Vision loss was acute in all
cases. The onset of vision loss was bilateral/simultaneous in three cases,
bilateral/sequential in one case, and unilateral In one case. All affected
eyes retained visual acuities of 20/25 or better. Colour vision was subnorm
al in three of four cases. Visual field defects were characterised by a cen
tral ring scotoma having an outer diameter less than 10 degrees. Fundus exa
mination demonstrated temporal optic nerve pallor in three patients (five o
f 10 affected eyes) and reddish, petaloid macular lesions in one patient. G
ood visual acuity was maintained for the duration of follow up in all five
patients.
Conclusion-Central ring scotomas excellent retained visual acuity may prese
nt as an acute, bilateral syndrome in patients who are heavy caffeine consu
mers. The configuration of visual field loss and its location, combined wit
h the presence of temporal pallor in five eyes, suggest that the defect loc
alises to the inner layers of the macula. While these cases could be consid
ered an expansion of the clinical spectrum of acute macular neuroretinopath
y, some may represent a distinct entity.