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ACTA OPHTHALMOLOGICA SCANDINAVICA 2000

Optical coherence tomography


(OCT) in acute macular
neuroretinopathy
Beatrix Feigl and Anton Haas
Department of Ophthaltnology, University of Graz, Austria
ABSTRACT.
Purpose: To evaluate the value of ocular coherence tomography (OCT) concernin;^ diagnosis and pathogenesis of acute macular neuroretinopathy.
Methods: A 33-year old woman complained of sudden onset of central scotomas
in her right eye hecause of acute niacutar neuroretinopathy. We performed a
direct ophthalmoscopy, a visual Held testing, a fluorescein angiography (FA) a
multifocal ERG (mf-ERG) and an OCT.
Re.sults: We found typical paracentral scotoma in visual field testing, a normal
FA and nif-KRG in her right eye. In OCT there was a hand of higher reflectivity
(115 ^m) overlying an intact hand corresponding to the retinal pigment epithelium (RPE)/ choriocapillaris complex. Retinal thickness was within the normal
range.
Conclusion: OCT can he an additional valuahle tool in acute macular neuroretinopathy us it is a disease with discrete pathology and often normal results in
other diagnostic tests.

right eye. Direct ophthalmoscopy revealed red-brown lesions (Fig. 1) in the


macula area and visual field testing
(Octopus M2. Interzeag. Switzerland)
showed paracentral scotomas (Fig. 2)
while the results on her left eye were
within the normal range. First order Kernels of mf-ERG (Reti scan. Roland ConsuU. Wiesbaden. Germany) were normal

Key words: optical coherence tomography - OCT - acute macular neuroretinopathy


Acta Ophthalmol. Scand. 2000: 78: 714-716
Copyright SI Acta Ophlhalmot Scard 2000. tSSN 1395-3907

cule macular neuroretinopathy was


.first discribed by Bos and Deutmatin in 1975 and is a rare uni-or bilateral tTiaculopathy of unknown etiology
itivolving mainly young women between
the ages of 20 and 30 years. Most patients
experience sudden onset of paracentral
scototnas with preserved good visual acuity, often with a preceding flu-like disease
(Bos & Deutmann 1975; Miller et al.
1989). Opiilhalmoscopy shows wedgeshaped, red-brown lesions arranged radially in the macula. The lesions are suggested to be located in the outer retina,
FA and standard electroretinography
(Sieving et al. 1984) typically are nortnal.
whereas Amsler grid and visual fields reveal parafoveal scotomas. Visual deficit
does not improve as persistance of maculopathy and scotomas have been described up to 9 years (Desai et al.l993)
and a benefit of any medical treatment

714

(e.g steroids) has not been proven to


date.

Fig, 1. Fundus examiniition showed a


reddish-brown lesion on the right eye.

Case Report
A 33-year-old female patient complained
about sudden visual disturbances with
central, greyish, swirling scotomas on her
right eye. A week before she had suffered
from a flu-Uke disease with fever, swelling
of the left submandibular lytnph nodes,
and pain in her joints. Other than taking
oral antibiotics after the onset of v'isual
problems, she did not receive any treatment.
Visual acuity was 20/20 on her right
eye; on her left eye she had a decreased
visual acuity (20/100) because of squinting as a child and amblyopia. Amsler
grid examination showed metamorphopsia in the temporal superior area on her

Fig. 2. The visual field on the rigiit eye revealed


typical paracentral scotoma.

ACTA OPHTHALMOLOGICA SCANDINAVICA

2000

Fig. 3. a. OCT results (longitudinal scan and area showed on Fig. 1) on her right eye revealed a hyperrefleciivity (arrow) above an undisturbed Rl'E/
choriocapillaris band which spares the foveal depression, b. Longitudinal OCT scan through the fovea on her left eye was within the normal range.

on her right eye and could not be performed on her left eye because of amblyopia and difficulties in central fixation.
The horizontal OCT scan through the
tnacula (Humphrey Instruments. Zeiss.
Obcrkochen. Germany) on her right eye
showed a batid of hyper reflectivity of 115
j^m overlying an intact RPE/choriocapillaris complex sparing the foveal depression, and normal results on her left
eye (Fig. 3a. b).

Discussion
The lypica! hallmarks of acute macular
neuroretinopathy are a younger age of
onset, female gender, paracentral scotomas and reddish-brown macular lesions,
but the pathogenesis is still unknown. It
is not clear cither why the lesions appear
red. A thin retinal blood layer has been
discussed but seems unlikely due to normal angiographic findings in most ofthe
patients, except a very slight hypolluorescencc and not a dramatically blocked
flourescencc that would be expected from
blood (Kalina 1999). Nonetheless, a vascular etiology has been discussed as patients with acute hypertension caused by
intravenous sytnpathomimetics (O'Brien
ct ai. 1989). patients receiving a contrast
agent for computed tomography (Guzak
ct al, 1983). as well as patients with eclampsia (Kalina 1999) and oral contraceptive use (Desai et al. 1993) have been
described to resetiible AMN. Recently.
heavy calTeitie consumption as well as hypotension have been published as additional causes in AMN (Kerrison et al.
2000).
Stereo viewing suggests location of
retinal disturbatice in the external neuro-

sensory retina with intact RPE and retinal blood vessels, although an affection
of the inner layers of the retina causing
temporal disc pallor has been postulated
(Kerrison etal. 2000).
Standard electroretinography is normal, but early receptor potentials are reduced, confirming assignment of the
pathology to the photoreceptors (Sieving
et al. 1984). Therefore, one would expect
also reduction in mf-ERG as it refiects
photoreceptor function in well defined
small areas. We could not tind any pathology in mf-ERG. but maybe the area
was too small to be detected. As we used
a 61 hexagonal stimulus perhaps the use
of more stimulus segments, for example
of 103 or 206 hexagons, would have been
of greater diagnostic value.
In our patient we found normal thickness of the retinal layers including the
nerve fibre layer (NFL) and a stnall area
of hyper reflectivity overlying an undisturbed RPE/choriocapillaris complex in
OCT Hyperreflectivity in OCT is found
in several disorders including inflammatory processes, chorioretinal neovascularisations. hard exudates. fibrosis. and
hemorrhages,
Considering hyperreflectivity due to an
inflammatory process maybe our findings
could reflect a swelling ofthe photoreceptor ceils or an increase ofthe intercellular
matrix because of inflammatory cells in
the outer layer of the retina.
As in many patients with acute macular neuroretinopathy a preceding flu-like
disease can be found and an autoitnmune
response to retinal antigens may also play
a causative role in this entity.
There are several antigens from the
photoreceptor region, such as for exatnple the S-antigen, that may be an

agent for an autoimmune response inducing blast transformation of lymphocytes, causing an immune memory and
therefore ati inllamniatory reaction (Nussenblatt et al,1980).
So possibly an initial process like a
viral infection with subsequent exposure
of normally sequestered antigens from
the retinal photoreceptors to the immune
system and sensitization may be the inciting cause in AMN.
Nevertheless, whether hyperrefiectivity
in our patient is due to an inflammatory
or a preceding vascular event which leads
to a small hemorrhagic lesion that would
explain the reddish-brown colour i.s still
unclear and cannot be differentiated with
OCT. But our observations may underline the the location of AMN in the outer
retinal layer.

References
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573-584.
Desai UR. Sudhamathi K & Natarajan S
(1993); Intravenous epinephrine and acute
macular neuroretinopathy (Letter). Arch
Ophthalmol III; 1026-1027.
Guzak SV, Kalinii RE & Chenoweth RG
(1983); Acute macular neuroretinopathy following adverse reaction lo intravenous contrast media. Reiina 3; 312-317.
Kalina RE (1999); Acute macular neiirorclinopathy. Retina-Vitreoiis-Macula (Guyer DR.
Yunniizzi LA. Chang S, Shields JA. Green
WR) Chapter 49: 593-596.
Kerrison JB. Pollock SC. Biousse V & Newman NJ (2000): Coffee iind doughnut maculopathy: a cause ol" acute eeniral ring scotomas. Br .1 Ophthiilniol 84 (2): 158 164.
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ACTA OPHTHALMOLOGICA SCANDINAVICA

(1989): Acute macular neurorclinopalhy.


Ophthalmology 96: 265-269.
Nussenblall RB, Gery I & Ballintine EJ (1980):
Cellular immune rcspotisivcness of uveitis
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O'Brien DM. Farmer SG, Kaiina RE & Leon
JA (1989): Aculc macular ncuroreiinopathy
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. .. .

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2000

Sieving PA. Fishman GA, Salazano T & Rabb


MF (1984): Acute maeular neuroretinopathy: early receptor potential change suggests
photoreceptor pathology. Br J Ophlhalmoi
68: 229.
Received on May 29th, 2000.
Accepted on July 1 hh. 2000.

Corresponding author:
Beatrix Feigl. M.D.
Deparlment ot Ophthalmology
Univ. Augenklinik
Auenbruggerplaiz 4
8036 Graz
Austria
Tel: 0316 385 2394
Fax: 0316 385 3261
e-mail; beatrix.feigl@kftiiiigraz.ac.at