Acta Ophthalmol Scand 2000 Feigl-1

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  ACTA OPHTHALMOLOGICA SCANDINAVICA  2000

Optical coherence tomography

 OCT)  n 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) concern-

in;̂ 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 OC T there was a hand of higher reflectivity

(115 ^m) overlying an intact hand corresponding to the retinal pigment epithel-

ium (RPE)/ choriocapillaris complex. Retinal thickness was within the normal

range.

Conclusion: OCT can he an additional valuahle tool in acute macular neuroreti-

nopathy  us  it is a disease with discrete pathology and often normal results in

other diagnostic tests.

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

Acta Ophthalmol. Scand. 2000: 78: 714-716

Copyright SI

  cta

 Ophlhalmot

 Scard

 2000

tSSN

 1395 3907

A

cule macular neuroretinopathy  was

.first discribed

  by Bos and

  Deut-

matin  in 1975 and is a  rare uni-or bilat-

eral 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 acu-

ity, often with  a  preceding flu-like disease

(Bos  Deutmann  1975;  Miller  et al.

1989).

  Opiilhalmoscopy shows wedge-

shaped, red-brown lesions arranged radi-

ally  in the  macula.  The  lesions  are sug-

gested

  to be

  located

  in the

  outer retina,

FA  and  standard electroretinography

(Sieving et al.  1984) typically are nor tnal .

whereas Amsler grid

  and

  visual fields

 re-

veal parafoveal scotomas. Visual deficit

does

 not

  improve

 as

 persistance

 of

 macul-

opathy  and  scotomas have been  de-

scribed  up to 9  years (Desai  et  al . l993)

(e.g steroids)  has not  been proven  to

date.

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 subma ndi bula r lytnph nodes,

and pain  in  her joints. Othe r than taking

oral antibiotics after

  the

  onset

  of

  v'isual

problems,  she did not  receive  any  treat-

ment.

Visual acuity  was 20/20  on her  right

eye;

 on her

  left

  eye she had a

  decreased

visual acuity (20/100) because

  of

  squint-

ing  as a  child  and  amblyopia. Amsler

right  eye.  Direct ophthalmoscopy 

vealed red-brown lesions (Fig. 1) in

macula area

  and

  visual field test

(Octopus

  M2.

  Interzeag. Switzerlan

showed paracentral scotomas  (Fig

while  the  results  on her  left  eye  w

within the norm al range. First order K

nels

 of

 mf-ERG (Reti scan. Roland

 C

suU. Wiesbaden. Germany) were norm

Fig,  1.  Fundus examiniition showed  a

reddish-brown lesion on the right eye.

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ACTA OPHTHALMOLOGICA SCANDINAVICA

  2000

Fig. 3. a. OCT resu lts (longitu dinal scan and a rea showed on Fig. 1) on her right eye revealed a hyperrefleciivity (arrow ) above an und isturbe d Rl 'E/

chorio capillaris b and wh ich spares the foveal depression, b. Longitud inal O CT scan thro ugh the fovea on her left eye was within the norm al ran ge.

on her right eye and could not be per-

formed on her left eye because of ambly-

opia 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 hyp er reflectivity of 115

j^m  overlying an intact RPE/choriocapil-

laris complex sparing the foveal de-

pression, 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 scot-

omas 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 nor-

mal angiographic findings in most ofthe

patients, except a very slight hypolluo-

rescencc and not a dramatically blocked

flourescencc that would be expected from

blood (Kalina 1999). Nonetheless, a vas-

cular etiology has been discussed as pa-

tients 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 ec-

lampsia (Kalina 1999) and oral contra-

ceptive use (Desai et al. 1993) have been

described to resetiible AMN. Recently.

heavy calTeitie con sum ptio n as well as hy-

potension have been published as ad-

ditional causes in AMN (Kerrison et al.

2000).

sensory retina with intact RPE and reti-

nal 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 nor-

mal, but early receptor potentials are re-

duced, 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 path-

ology 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 n orma l thick-

ness of the retinal layers including the

nerve fibre layer (NFL) and a stnall area

of hyper reflectivity overlying an undi s-

turbed RPE/choriocapillaris complex in

OCT Hyperreflectivity in OCT is found

in several disorders including inflamma-

tory processes, chorioretinal neovascu-

larisations. hard exudates. fibrosis. and

hemorrhages,

Considering hyperreflectivity due to an

inflammatory process maybe our findings

could reflect a swelling ofthe photorecep-

tor ceils or an increase ofth e intercellular

matrix because of inflammatory cells in

the outer layer of the retina.

As in many patients with acute macu-

lar 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

agent for an autoimmune response in-

ducing blast transformation of lympho-

cytes,

  causing an immune memory and

therefore ati inllamniatory reaction (Nus-

senblatt 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 incit-

ing cause in AMN.

Nevertheless, whether hyperrefiectivity

in our pa tient is due to an inflamm atory

or a preceding vascular event which leads

to a small hemorrhagic lesion that would

explain the reddish-brown colour i.s still

unclear and canno t be differentiated with

OCT. But our observations may under-

line the the location of AMN in the outer

retinal layer.

References

Bos PJ & Deutmann AF (1975): Acute macu-

lar neuroretinopathy. Am J Ophthalmol 80;

573-584 .

Desai UR. Sudhamathi K & Natarajan S

(1993); Intravenous epinephrine and acute

macular neuroretinopathy (Letter). Arch

Ophthalmol III; 1026-1027.

Gu zak SV, Kalinii RE & Chenoweth RG

(1983); Acute macular neuroretinopathy fol-

lowing adverse reaction lo intravenous con-

trast media. Reiina 3; 312-317.

Kalina RE (1999); Acute macular neiirorclino-

pathy. Retina-Vitreoiis-Macula (Guyer DR.

Yunniizzi LA. Chang S, Shields JA. Green

WR) Chapter 49: 593-596.

Kerrison JB. Pollock SC. Biousse V  New-

man NJ (2000): Coffee iind doughnut macu-

lopathy : a cause ol" acute eeniral ring scot-

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—   ACTA OPHTHALMOLOGICA SCANDINAVICA  2000

(1989): Acute macular neurorclinopalhy.

Ophthalmology 96: 265-269.

Nussenblall RB, Gery I  Ballintine EJ (1980):

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  S-antigen.

  Am J Oph thal -

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O'Brien DM. Farmer SG, Kaiina RE  Leon

JA (1989): Aculc macular ncuroreiinopathy

following intravenous sympathomimetics.

Retina 9: 281 -28 6. . .. .

Sieving PA. Fishman GA, Salazano T & Rabb

MF (1984): Acute maeular neuroretinopa-

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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

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