ajopht 1977 rush

7/23/2019 Ajopht 1977 Rush

http://slidepdf.com/reader/full/ajopht-1977-rush 1/5

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

7/23/2019 Ajopht 1977 Rush


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.

7/23/2019 Ajopht 1977 Rush


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-

7/23/2019 Ajopht 1977 Rush


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,

7/23/2019 Ajopht 1977 Rush


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

Books

ajopht 1977 rush

Documents