ajopht 1977 rush
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A C U T E M A C U L A R N E U R O R E T I N O P A T H Y
J A M E S
A. R U S H , M . D .
Charlottesville Virginia
Recent ly ,
Bo s and Deu tman
1
examined
four patients with an unusual macular
affliction
character ized by min ima l de-
press ion o f visio n and s coto mas that cor-
responded to peculiar foveal lesions situ-
ated in the superficial retina. They termed
this i l lness acute macular neuroret ino-
pathy.
The i r
four patients were women, aged
24 to 3 3 years , an d all we re takin g oral
contraceptiv es. Tw o of the four pat ients
had exper ienced a mild, apparently infec-
t ious i l lness: one had an acute ente r i t is ,
and the other, a bou t of influenza .
T h r e e of the four patients had bilateral
involvem ent, a l though one of these did
not man ifest simulta neo us bilate ral in-
volvement. Fundi and visual f ields were
abno rmal, but f luorescein angiogram s in
all
four, and electroretinograms with
electro-oculogram s (E O G ) in both pa-
tients tested, were normal.
Recent ly ,
I observ ed a wo ma n with a
remarkably similar malady. To my
knowledge, this is the first case reported
in the United
States.
C A S E
R E P O R T
A 24-year-old white woman complained of see-
ing spots in her left eye. She had been healthy until
late in
December 1975, when
she
contracted
a
cold. Six
weeks
later, she developed an acute
pharyngitis, that, although culture-negative,
was
treated with erythromycin. When her symptoms
resolved
two weeks
later,
she
noted three
tear-
shaped spots in front of her left eye. They were
constant, and always appeared where she focused.
At an ocular examination two days later, without
cessation of symptoms, she admitted to having had
migraine in high school. She had been taking oral
contraceptives for many years. Her general history
was otherwise unremarkable.
At that visit on March 10, 1976, her best corrected
acuity was 6/6
(20/20)
in each eye. Both anterior
segments were normal. Direct and indirect ophthal-
moscopy demonstrated pertinent
findings
restricted
to the macula of the left eye. In the fovea, there were
three
discrete, although faint, reddish-brown
lesions
that
had an arrowhead or a wedge shape, pointing
toward a normal foveolar light reflex (Fig.
1).
Biomi-
croscopy
through a Goldmann contact
lens
showed
three superficial, vague areas of dusty, dusky depos-
its that merged imperceptibly with normal retina.
There
were no deep areas of altered pigmentation or
exudative deposits in the retina. No commonly iden-
tifiable changes
in
retinal structure were noted.
Fluorescein angiograms were normal (Fig. 2),
confirming the integrity of the retinal pigment epithe-
lium and retinal vasculature, and substantiating the
gossamer-like quality of the observed lesions. The
patient was able to reproduce scotomas on an
Amsler grid (Fig. 3). Goldmann perimetry revealed
no abnormality other than that on the Amsler grid,
although the varying illuminance and size of the test
objects corresponded well with both the densities of
the ophthalmoscopically observed lesions and the
subjective appreciation described
by
the patient.
No change
in
the visual acuity, and
no
change
in
the subjective scotomas, occurred in six months of
follow-up observation. The lesions neither coa-
lesced nor regressed. The patient
still finds
the
scotomas most bothersome, but has refused to con-
sider cessation
of
the oral contraceptive hormone.
From the University of Virginia Medical Center,
E ye
Clinic, Charlottesville, Virginia.
Reprint
requests
to
James
A.
Rush,
M.D.,
1404
Hazel St., Charlottesville, VA 22901 .
Fig.
1 (Rush). Fovea of the left eye. Normal light
reflex is surrounded by one temporal and two nasal
wedges or
teardrops, pointing
to the
foveola (ar-
rows).
490
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VOL.
83, NO. 4
A C U T E
MACULAR NEURORETINOPATHY
491
Fig.
2
(Rush). Left
eye.
Late
phase fluorescein
angiogram showing absence of window defects,
blocked
transmission, or capillary leakage.
D I S C U S S I O N
Several idiopathic acute posterior pole
lesions have been described recently. In
1 9 6 8 , G as s
2
first described three young
wome n with modera tely severe loss of
central vision bilate rally, in whose pos-
terior pole areas were seen multiple
cream-colored
lesions, apparently located
at the pigm ent e pithe lial level. He termed
this entity acute posterior multifocal plac-
oid pigment epitheliopathy, and noted
that these lesions did not extend beyond
the equator and generally were less
than
1
disk diameter in size. Despite a superfi-
cial
resemblance to choroiditis, no incit-
ing agent was ascertained, and his pa-
tients recovere d normal, or nearly n ormal,
acuity in several months.
Later reports documented that this con-
dition was not benign, being associated
with papillitis and retinovasculitis.
3
It
has been ass ociat ed with bilateral serous
detachments in two patients, 4
and erythe-
ma nodosum in two others.
5 , 6
Several
patients developed this after an acute
viral i l ln ess .
7 , 8
Twenty-seven of the 51 patients
2 - 1 3
with acute posterior multifocal placoid
pigment epitheliopathy were female, but
only three of them were known to be
taking oral cont race ptive s. All patients
recovered and only one patient had a
recurrence.
9
Despite speculation concern-
ing the cause—a viral infection
1 0
or its
sequelae
1 1
—etiology and pathogenesis
are unknow n. Whe ther it is primarily a
disease of choriocapillaris or retinal pig-
ment epit heliu m is still bein g debat-
e d .
1 2 , 1 3
Clinically distinct, acute retinal pig-
ment epitheliitis was described in
1 9 7 2 .
1 4
Only four cases have been reported
s i n c e . 1 5 , 1 6
Th e lesions were ophthalmo-
scopically
distinct, and restricted to the
macula. They were multiple, and resem-
ble d gray to bla ck pigm ent spots, sur-
rounded
by halo zones. They were usual-
ly monocular, asymptomatic, and caused
a mo derate visual loss—mo st cases better
than
6/22
2 0 / 7 0 )
and only one had 6/30
(20/100)—although recovery was the rule.
Fluorescein angiography showed hypo-
fluorescence in the cen tra l dark areas and
+
->
r
Fig. 3 (Rush). Amsler grid demonstrates the
tear-
drop
or wedge shapes of
parafoveal
scotomas point-
ing toward fixation. Large horizontal scotoma was
most
prominent, followed by inferonasal scotoma
whose corresponding lesion was closest to the fove-
ola.
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492
AMERICAN JOURNAL OF
OPHTHALMOLOGY APRIL,
1977
hyperfluorescence in the halo zones.
Leakage
was not demonstrate d. T he se le-
sions did not look like those of acute
posterior multifocal placoid pigment epi-
theliopathy, being considerably smaller,
and healing without scar formation.
Howev er, the retinal pigm ent epithe lium
is implicated because the
EOGs ,
when
they have been recorded, have been base-
line
or subnormal.
Like
acute posterior
multifocal
placo id pigment epitheliopa-
thy, the illness is idiopathic, and recovery
is
com mon. Only one of the ten reported
cases occurred after an acute viral illness.
Acute macular neuroretinopathy
1
was
first de scr ibe d in 1 97 5, whe n four de-
tailed and two supplemental cases from
Eur op e were reported. Typ ica lly, acute
macular neuroretinopathy manifests three
to five reddish-b rown foveal lesio ns, oc-
curs pred omi nan tly in wom en and often
after
mild viral illnesse s. Th es e lesions
assume a pecu liar wedge-shaped or tear-
drop configuration, pointing to the umbo,
and characteristically do not cause a de-
cline in visual acuity (Snellen). Yet, they
are bothersome, as they infringe
upon
the foveola and produce small central
scotomas. The lesions are subtle, and
are best seen with the direct ophthalmo-
scope
by using red-free light and motion
parallax.
Our case is typical of acute macular
neuroretinopathy: a young woman recov-
ering from a viral
upper
respiratory
infec-
tion noted the onset of three central
scoto-
mas corresponding to parafoveal deposits
o f the
unusual
shape and coloration pre-
viously described. Visual acuity,
periph-
eral fields, and fluorescein angiograms
were normal. Central fields, as depicted
on Amsler grid, showed scotomas corre-
sponding to the
fundus
lesions.
O f seven patients, six women inclu ding
my patient with acute macular neurore-
tinopathy were taking oral contraceptives.
None of the other women had a remis-
sion, but no knowledge of discontin-
uation of the hormone is available. My
patient was bothered by disruptive
sco-
tomas, but declined to consider long-
term cessation of the pill. Therefore,
wheth er th e association is causal or casual
is
speculative.
There is some overlapping in these
three different conditions
(Table).
They
are more common in women, but are
increasingly being diagnosed in men.
1
They may o ccu r after viral illnesses. Th ey
may be related to oral hormones. They
occur
prim arily, yet not ex clusiv ely, in
younger people. They
affect
the posterior
pole.
These
lesio ns, although superficially
similar, differ in appearance, and differ on
fluorescein angi ogra phy. Fur the rmo re,
acute ma cular neuroretinopath y is a dis-
ease of the more superficial sensory layers
o f the retina, whereas the other two enti-
ties have involved the retinal pigment
epithelium.
Although acute mac ular neuroretinopa-
thy is associated with the least decline in
visual acuity, it is the one disease that
undergoes the least amoun t of ameliora-
tion of symptoms. Whereas acute posteri-
or
multifocal placoid pigment epithelio-
pathy is associated with an initial
dramat-
ic decline in acuity, and the acute retinal
epitheliitis is manifested by a moderate to
severe loss of vision, recovery, although
slow,
ultimately occ urs. On the other
hand,
in acute macu lar neuroretinop athy,
the paracentral scotomas have never been
documented to regress.
Final ly, all three illnesse s are idiopath -
ic .
As the number of cases of acute poster-
io r
multifocal placoid pigment epithelio-
pathy mounts, the association with viral
i l lness is bec om in g a potential source of
research. Although most cases of acute
macu lar neuroretino pathy occurre d after
a
viral illness, a relationship to viral in-
fection has not yet been proven. Further-
mor e, altho ugh all six wom en with acu te
macular neuroretinopathy had been re-
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V O L . 83, NO. 4
ACUTE MACULAR NEURORETINOPATHY
493
T A B L E
D I F F E R E N T I A L D I A G N O S I S O F
M A C U L A R
L E S I O N S *
A P M P P E
2
1 3
Pigment
Epitheliitis
14
16
Acute Macular
Neuroretinopathy
1
Sex 2 4
M,27 F
4
M, 6 F
1 M, 5 F
Appearance Cream-colored
plaques Gray-black spots
Red-brown
wedge
with halo
Location
Posterior
pole
Macula
Fovea
to equator
Systemic
infection
Common Rare 4
of 6
Associated ocular Common None None
inflammation
Laterality Usually bilateral Usually monocular Either
Diminished acuity
Marked Moderate
Mild
Fluorescein
study
Abnormal Abnormal Normal
Recovery
Months to years Weeks to months None
Cause
Possibly Unknown Possibly infection
infection-related
or
hormone
Pathology
Choriocapillaris RPE
Sensory retina
and RPE
*M indicates male; F , female;
R P E ,
retinal pigment epithelium; and A P M P P E , acute posterior multifocal
placoid
pigment epitheliopathy.
ceiving oral contraceptives, this avenue of
research may be restricted because one
other patient was a man.
1
Fur the r aware ness of the exis tenc e of,
and the differentiation among, these three
illnesses , and the accu mula tion of more
clinical exper ienc e, will necessarily lea d
to a further delinea tion o f pathophy si-
ology.
S U M M A R Y
A 24-year-old white woman convalesc-
ing from a viral upper respiratory in fec-
tion suddenly developed three positive
scotomas around the fixation point in her
left eye. Th e fundus had three corre-
sponding lesions that appeared to be
charac teristic of acute mac ular neurore-
tinopathy. Situated in the fovea, subt le,
reddish-br own, wedge- or tear-shaped le-
sions were best seen with the direct oph-
thalmoscope using red-free light. Visual
acuity was 6/6
2 0 / 2 0 ) ,
and peripher al
fields wer e norm al. T h e pat ient 's repro-
duct ion o f the scoto ma s on an Amsle r grid
sheet mirror ed the obser ved ophth alm o-
scopic findings. Fluorescein angiograms
were norm al. Sh e had been taking oral
contra ceptiv es for many years. Although
viral illness and oral contrac eptives have
bee n associated with acute mac ular neur-
oretinopa thy, no etiology was proven, and
no treatment is know n. Th e patien t re-
mains symptomatic after a six-month
follow-up.
R E F E R E N C E S
1.
Bos, P. J . M., and Deutman, A. F. ; Acute
macular neuroretinopathy. Am. J . Ophthalmol. 80:
573, 1975.
2.
Gass,
J .
D. M.: Acute
posterior
multifocal plac-
oid pigment epitheliopathy.
Arch.
Ophthalmol. 80:
177, 1968.
3.
Kirkham, T. H., Ffytche, T.
J .
and Sanders,
M. D.: Placoid pigment epitheliopathy with retinal
vasculitis and papillitis. Br .
J .
Ophthalmol. 56:875,
1972.
4 .
Bird,
A. C , and Hamilton, A. M.: Placoid
pigment epitheliopathy. Br .
J .
Ophthalmol. 56:881,
1972.
5.
Deutman, A.
F.,
Oosterhuis,
J .
A., Boen-tan, T.
N., and Aan de Kerk, A.
L .:
Acute posterior multifo-
cal placoid pigment epitheliopathy. Br .
J .
Ophthal-
mol. 56:863 ,
1972.
6.
Van Buskirk, E . M., Lessei, S., and
Friedman,
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494
AMERICAN
JOURNAL
O F OPHTHALMOLOGY
APRIL, 1977
E. :
Pigmentary epitheliopathy and erythema nodo-
sum.
Arch.
Ophthalmol. 85:369 , 1971.
7.
Ryan,
S. J .
and Maumenee,
A.
E. : Acute pos-
terior multifocal placoid pigment epitheliopathy.
Am. J. Ophthalmol.
74:1066 ,
1972.
8. Annesley, W. H., Tomer, T. L., and Shields,
J .
A.: Multifocal placoid pigment epitheliopathy. Am.
J .
Ophthalmol. 76:511 , 1973.
9. Lewis, R. A.: Acute posterior multifocal plac-
oid pigment epitheliopathy. Arch. Ophthalmol. 93:
235 ,
1975.
10 . Azar, P. Jr., Gohd, R. S., Waltman, D., and
Gitter,
K. A.: Acute multifocal placoid pigment
epitheliopathy associated with an adenovirus type 5
infection. Am.
J .
Ophthalmol. 80:1003 , 1975.
11 . Holt, W. S., Regan, C. D. S., and Trempe, C :
Acute posterior multifocal placoid pigment epitheli-
opathy. Am.
J .
Ophthalmol. 81:403 , 1976.
12 .
Savino,
P. J .
Weinberg,
R. J .
Yassin, J.
G.,
and Pilkerton, A. R.: Diverse manifestations of acute
posterior multifocal placoid pigment epithelio-
pathy. Am. J. Ophthalmol. 77:659 , 1974.
13.
Fishman,
G.
A., Rabb, M. F. , and Kaplan,
J . :
Acute posterior multifocal placoid pigment epitheli-
opathy. Arch. Ophthalmol. 92:173 , 1974.
14 . Krill, A. E . , and Deutman, A. F.: Acute retinal
epitheliitis. Am. J . Ophthalmol. 74:193 , 1972.
15 .
Deutman, A.
F .:
Acute retinal epitheliitis. Am.
J .
Ophthalmol. 78:571 , 1974.
16. Friedman, M . W.: Bilateral recurrent acute
retinal
pigment epitheliitis. Am. J . Ophthalmol. 79:
567 , 1975.
O P H T H A L M I C M I N I A T U R E
But
sudd enly the Mir ror we nt altog eth er dark, as dark as if a hole
had op ene d in the wor ld of s ight , and Fr od o look ed into emp tines s .
In the blac k aby ss there appea red a s ing le E y e that s low ly grew,
until i t fil led ne arly all the Mir ror. So ter ribl e was i t that F ro do stood
rooted , una ble to cry out or to wi thd raw his gaze. T he Ey e was
r immed wi th fire, but was
i tself
glazed, yel lo w as a cat ' s , wa tchful
an d intent, and the bla ck sli t of i ts pu pil op en ed on a pit , a w in do w
into nothing.
J .
R. R . To l k i e n , The Lord of the
Rings
Part 1. The Fellowship of the Ring
N ew
York,
Ba l l an t i ne
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