american journal of ophthalmology volume 25 issue 4 1942 [doi 10.1016%2fs0002-9394%2842%2990900-0]...

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T E M P O R A R Y A M A U R O S I S A N D H E M I A N O P S I A D U E T O E P I L E P S Y *

A R T H U R K I N G , M . D., A N D F R A N K B . W A L S H , M . D .

Baltimore

Temporary amaurosis due to epilepsy

was first described in the latter half of

the nineteenth century. Since then occa

sional reports of this condition have ap

peared in the English literature, but, so

far as we know, the American literature

is quite barren on the subject. Although

we have nothing new to offer concerning

this condition, reports are so infrequent

that a short note may be of some value.

Transient stupor, drowsiness, hemi-

plegias, cranial-nerve palsies, and hemi-

anesthesias are frequently observed after

epileptic attacks. These conditions can be

found by objective examination of the

patient, and very little cooperation on his

part is necessary. Aphasia is less com

monly demo nstrated because most per

sons in a postepileptic state are too

drowsy to perform the required tests. To

procure visual fields the patient has to be

fully awake, conscious, and cooperative.

Blindness arid visual-field defects may be

overlooked because of the postconvulsive

stupor.

As transient loss of vision and visual-

field defects often occur in cases of in

creased intracranial pressure, in tumors

near the visual tracts, and from cerebral

edema caused by trauma or vascular ac

cidents, we were careful to eliminate all

these factors in the cases we wish to dis

cuss.

W e have observed only two patients

in whom blind ness persisted after full

return of the other functions. Although

the condition was temporary, it persisted

for a surprisingly long period in both

cases.

* From the Department of Neurology, Bal

timore City Hospitals, and the Department of

Ophthalmology, Johns Hopkins Hospital.

C A S E

REPORTS

Case 1

B. R., a white female, 22

months old, was seen in the Harriet

Lane Home.

The family and past histories were

without significance. There was nothing

remarkable about her birth or develop

ment until the time of onset of the pres

ent illness.

Seven months before the onset of her

difficulty the patient had fallen out of a

crib. She remained quite normal there

after for several weeks and then sud

denly had a generalized convulsion fol

lowed by several more within a few

days.

Observations at a local hospital,

where she remained for a month, dis

closed that she had had 84 generalized

convulsions. It was also determined that

the child was totally blind. After the

convulsions were controlled vision gradu

ally returned, but it was eight weeks

before it was thought that vision was

normal.

Following this the child was well until

at the age of 21 months three generalized

convulsions occurred within a week.

These were followed by a free interval

of a week, when the fits recurred. Before

her admission to the Johns Hopkins Hos

pital she had had 18 convulsions within

24 hours. The convulsions were usually

ushered in with a cry, the face became

cyanotic, she foamed at the mouth, and

finally all the extremities jerked and be

came rigid.

Several days after cessation of the epi

leptic attacks the general physical exami

nation was negative except for neurologic

signs: ataxia of the extremities and in

creased activity of the tendon reflexes.

398



T E M P O R A R Y A M A U R O S I S D U E T O E P I L E P S Y

399

The eyes on external examination seemed

quite normal, the pupils reacted normally

to light, and the fundi showed no abnor

malities, but there was complete blind

ness.

There was no blink response to

objects held close to the patient's face,

nor was there nystagmus when the gaze

was directed toward a revolving drum.

Laboratory examinations, including

spinal-fluid studies and Wassermann re

actions, were negative. A ventriculogram

was done, showing the ventricular sys

tem to be normal.

About 10 days after the last convulsion

there was return of the blink reflex, and

appropriate movements of the eyes were

observed when tested with the revolving

drum. The child was discharged at the

end of six weeks with normal vision and

almost complete disappearance of the

other neurologic signs. Both the neuro

logic and ophthalmologic findings were

thought to be postepileptic residua. After

the child had been home several weeks

the convulsions returned and all the pre

vious signs and symptoms, including

blindness, recurred.

C O M M E N T : This case is in the same

general category as those, occurring in

infants, described by Ashby and Stephen-

son in 1903. Th ese auth ors postulated

that this form of amaurosis occurring in

young children and infants is due to

anesthesia of the vital centers. The con

vulsions precipitating the blindness were,

in their experience, unusually violent.

Besides these visual disturbances, aphasia

and hemiplegia were frequently associ

ated findings. The phenomena were usu

ally transient, but one case of persistent

blindness was recorded, while in other

infants the hemiplegia was permanent.

In 1918, Pr itch ard reporte d am auro sis

following convulsions in a child two

years of age. Blindness was still present

10 days after the fits had stopped, and it

was not until six weeks had elapsed that

recovery was complete. Our case was

similar to his, but differed in that the

pupils in our patient reacted to light. This

led to the conclusion that the interruption

of the visual pathways was above the

geniculate bodies in our case.

The aforementioned authors speak

only of postepileptic amaurosis in chil

dren. It is a more unusual finding in

adults. W e have recently observed an

adult patient who had a transient

homonymous hemianopsia following

grand- and petit-mal epilepsy.

Case 2

H. M., a male, Negro, aged

45 years, was seen in the Baltimore City

Hospital.

The patient was first admitted to the

hospital in M arch , 1940, with frost-bite

of the feet. The left leg was amputated

below the knee and the stump healed

promptly.

About December, 1940, he began to

have intermittent headaches, localized at

first in the right frontal region, later be

coming bi frontal, an d his eyes felt sore.

Nausea and dizziness frequently accom

panied the headaches, but there was no

vomiting. Transient diplopia was experi

enced several times. For one month the

patient experienced intermittent attacks,

during which he would see little people,

men, animals, and irregular objects walk

ing before him. He realized that they

were not real, but they persisted when

he closed his eyes. The march of the

hallucinatory figures was always from

left to right in the field of vision. These

attacks would last for several hours and

they occurred at any time of the day. On

Jan ua ry 29, 1941, he had several gener

alized convulsions without warning or

aura. Consciousness was lost for about

half an hour and this was followed by a

severe episode of vomiting.

Physical examination after the con

vulsions revealed nothing remarkable ex-



4

ARTHUR KING

AND

FRANK

B.

WALSH

cept that the patient was very drowsy.

Two days later a complete neurologic ex-

amination

was

made,

and a

left homony-

mous hemianopsia was found. This was

confirmed

by

perimetric studies. Vision

was 20/40

in

each

eye. The

optic fundi

were entirely normal. The cranial nerves

were intact except for a mild left lower

facial paralysis

of

central type. Strength

in the left hand was not quite equal to

that

of the

right,

and

fine movements

were

a

little clumsy. Muscle tone

was

everywhere normal. Therewas no lossof

sensation cerebellar tests were well per

formed; speech was normal, and there

was no difficulty in reading or writing.

Neither apraxia

nor

aphasia could

be

demonstrated. The tendon reflexes were

normal. Hoffmann's sign or ankle clonus

on

the

right could

not be

elicited.

The

plantar response on the right was dorsi-

flexion (left leg ampu ta ted ) .

Laboratory studies were normal. Blood

and cerebrospinal-fluid Wassermann

re-

actions were negative. The spinal-fluid

pressure three days after the convulsions

w as

200 mm.

water .

The

Pandy reaction

was negative, and the fluid contained no

cells.

The patient was rexamined by Dr.

F rank

R.

Ford

one

week after

the

grand-

mal episode, and the neurologic findings

were unchanged.

The

patient

had

several

petit-mal attacks during

the

next

two

weeks. At times he saw people, both

large and small, who were always Ne-

groes dancing from left

to

right

in the

blind fields. They were always dressed

in bright-red

or

green clothes. Move

ments were rapid, often dancing and jig

gling, and at times some of the figures

would throw silvery material about, while

others would seem

to

sweep

it up. One

day he described animals vividly, most

of these being dogs. That these hallucina

tions weredue to an irritative rather than

to a psychic stimulus was at least par

tially substantiated

by the

fact that

the

patient realized that the moving objects

were not real, and that the associated

sensations

of

touch, smell,

and

weight

were not present.

On February 19th, he had a different

sort

of

attack, which lasted

for two

days.

H e was found wiping and brushing his

left hand

and the

left side

of the bed

clothes. When asked why he was doing

this he stated that fluffy white, blue, and

yellow sticky

stuff was

falling

on him'

and on the bed. Several times he en-

quired of the examiner whether he did

not also

see it. All

that

day he

wiped

the

left side of his face and his left hand

with acloth and made picking motions at

the left side

of the bed. The

next

day he

was still both ered with this sticky stuff,

but in addition tothis healsosaw insects

and fishes

in his

blind fields.

A

psychiatric

consultant thought that all these com

plaints were

on an

irritative basis.

During these attacks the patient was

not

at all

drowsy,

and the

hemianopsia

was easily demonstrated

at all

times

and

by many observers.The petit-mal attacks

stopped without sedation at the end of

48 hours after

the

episode

of the

sticky

stuff. After that he had no further at-

tacks of any kind.

On February 28, 1941, it was discov

ered that

the

hemianopsia

had

disap

peared. Vision

was

20/203

in

each

eye

at this time.No abnormalities of thecen

tral nervous system could

be

demon

strated.

The patient was studied from the psy

chiatric standpoint,

but no

deviations

from the normal could be brought out.

H e was quite intelligent, and reading,

writing, addition, multiplication,

and

speech were normal. Extensive tests for

aphasia

and

apraxia revealed nothing.

At

no time

did

anyone have

the

impression

thathemightbe in some sortof psychotic

state.

X-ray s tudies of the skull and chest

were normal.Athorotrast injectionof the



T E M P O R A R Y A M A U R O S I S D U E T O E P I L E P S Y

401

right internal carotid artery was done,

and no abnormality of the right middle

cerebral arterial system was seen. An

encephalogram showed a small dilated

area in the temporal horn of the right

lateral ventricle. Cortical atrophy was

noted in the region where the temporal

and parietal lobes met.

After a free period of about a month

without medication we tried to induce

some further epileptic attacks by hyper-

ventilation and by intravenous injection

of 4 c.c. of metrazol. These efforts were

unsuccessful. D uring July, 1941, the pa

tient again had a generalized convulsion,

and the hemianopsia recurred. It lasted

for two days. No petit-mal attacks were

noted at that time.

C O M M E N T : The diagnosis of brain tu

mor seemed obvious when the patient was

first examined. The history of a left-

sided convulsion in a 40-year-old man,

who later showed a left homonymous

hemianopsia and visual hallucinations

seemed to make the diagnosis of a neo

plasm of the right temporal lobe inescap

able.

It must be confessed that we were

chagrined when the hemianopsia disap

peared and failed to recur. Occasionally

visual-field defects may disappear when a

neoplasm is actually present, but these

reappear within a few days. It was only

when an encephalogram definitely ruled

out a tumor that we realized that we were

dealing with postepileptic blindness.

D I S C U S S I O N

Several explanations have been offered

for the occurrence of temporary amauro-

sis and hemianopsia following epileptic

seizures. In 1897, Harris discussed tem

porary hemianopsias at great length he

described them as a part of migraine,

hysteria, and cerebral accidents. In addi

tion he recorded four cases associated

with epilepsy. In one of these the patient

had transient unilateral deafness. Harris

studied two patients who saw faces,

people, animals, and oth ers, in the blind

parts of the visual fields and he concluded

that this phenomenon was due to irritation

of the higher reflex centers, probably in

the region of the angular gyrus. Tran

sient hemianopsias were found to last 24

hours or more after cessation of con

vulsions. All his patients had major epilep

sy and the visual disturbances invariably

followed the attacks. He stated that blind

ness was due to exhaustion of the cortex

following the violent epileptic discharge.

His communication should be read in the

original.

Gowers was also acquainted with post-

epileptic blindness, as were several other

observers quoted by Harris. None of

these offered furth er exp lanatio n than

that of Harris. As has been mentioned

un der case 1, Ashby a nd Stevenson con

sidered the blindness as seen in children

to be due to cortical anesthesia.

In 1938, Mecca raised the question of

angiospasm of the retinal arteries as a

cause of amaurosis following epileptic

attacks, and reviewed the literature con

cerning the ophthalmologic findings

during and immediately following con

vulsions. From his survey it is quite evi

dent that there is no characteristic pic

ture .

Dilatation or spasm of the arteries,

ischemia or hyperemia of the fundus,

turgidity of the veins and papillary atro

phy have all been described. He reported

a case of a nine-year-old girl who had

suffered grand-mal attacks since her first

year of life and who gave a history of

recurrent attacks of dimness of vision

lasting for several hours following the

convulsions. After a particularly severe

seizure she became completely blind.

Nothing could be found on examination

except changes in the optic fundi. The

retinae were pale, the arteries very small,

and the maculae cherry-red. Obviously

the picture was that of occlusion of cen

tral retinal arteries. At this time vision

was limited to distinguishing bright lights,



4 2

A R T H U R K I N G A N D F R A N K B . W A L S H

an d th e re was b a re ly p e rcep t ib l e p u p i l l a ry

reac t io n to l i g h t . Ace ty l ch o l in e was g iv en .

By the end of a m on th v is ion wa s 1 /10

in the r igh t eye and 1 /4 in the lef t . The

fu n d i n o w sh o wed ty p i ca l ch an g es asso

cia ted wi th an o ld obst ruct ion of the

cen t r a l r e t i n a l a r t e ry . As emb o l i an d

ar t e r io sc l e ro s i s were e l imin a t ed , t h e o n ly

o ther poss ib i l i ty seemed to be a v io len t

an d p ro lo n g ed co n s t r i c t i o n o f t h e cen t r a l

r e t i n a l a r t e r i e s , wh ich p e r s i s t ed u n t i l t h e

n e r v o u s e l e m e n t s o f t h e r e t i n a s u c

c u m b e d .

M e c c a q u o t e d s e v e r a l a u t h o r s w h o w e r e

rep u ted to h av e seen ce reb ra l v esse l s

a t o p era t i o n b e fo re an d d u r in g ep i l ep t i c

se i zu res . As t h e o cu la r a r t e r i e s a re c lo se ly

re l a t ed emb ry o lo g ica l ly t o t h o se o f t h e

b ra in , i t was Mecca ' s o p in io n th a t s imi l a r

ev en t s may t ak e p l ace i n t h e r e t i n a . On

o n e o ccas io n h e o b se rv ed th e r e t i n a l a r

t e r i e s t o r emain co n t r ac t ed fo r 3 5 min

u t es a f t e r a co n v u l s io n . T o s u b s t an t i a t e

th i s p o in t sev era l o th e r au th o r s , i n c lu d in g

H u g h l i n g s J a c k s o n , a r e r e f e r r e d t o a s

h a v i n g r e p o r t e d s i m i l a r o b s e r v a t i o n s .

M e c c a c o n s i d e r s t h a t t h e c o n s t r i c t i o n o f

the visual f ields, select ive loss of color

v i s io n , an d amau ro s i s wi th f i x ed p u p i l s

can b e ex p la in ed in n o o th e r way th an b y

a s s u m i n g a t e m p o r a r y n o n f u n c t i o n i n g

re t i n a d u e to i sch emia cau sed b y sp asm

o f t h e r e t i n a l a r t e r i e s .

Th i s l a s t -men t io n ed th eo ry o f r e t i n a l

i sch emia may h o ld g o o d fo r so me cases ,

b u t i t i s imp o ss ib l e t o ex p la in t h e h emi -

an o p s i a o b se rv ed in o u r case 2 o n th i s

b as i s .

Al so p a t i en t s may b e q u i t e b l i n d ,

yet thei r pupi l s react p rompt ly to l igh t ,

a s was a l so o b se rv ed in o u r case 1 . Th i s

l a t t e r o b se rv a t io n l ead s t o t h e su sp i c io n

of a les ion poster io r to the f iber t racts

s u b s e r v i n g t h e p u p i l l a r y r e a c t i o n s . F i

nal ly , the phenomena of v isual - f ie ld con

s t r i c t i o n an d ap p aren t l o ss o f co lo r v i s io n

mig h t we l l b e ex p la in ed b y p o s t ep i l ep t i c

s tu p o r o r l ack o f a t t en t io n . Al th o u g h

sp asmo d ic co n s t r i c t i o n o f ce reb ra l an d

re t i n a l a r t e r i e s as a p a r t o f ep i l ep sy can

n o t b e ca t eg o r i ca l l y d en ied , t h e re i s co n

s iderab le d i f ference of op in ion as to i t s

e x i s t e n c e . I n o u r o w n e x p e r i e n c e w e h a v e

not observed i t . The re t inal vessels in

b o th o u r p a t i en t s were n o rmal a t t h e t ime

o f o b se rv a t io n .

C O N C L U S I O N S

Th e case o f an i n fan t h as b een d e

sc r ib ed in wh o m th ere was t o t a l b l i n d n ess

l as t i n g fo r sev era l d ay s fo l l o win g sev ere

g en era l i zed co n v u l s io n s . A seco n d case

w i t h a h o m o n y m o u s h e m i a n o p s i a w a s o b

se rv e d in an ad u l t fo l l o win g g r an d - a n d

p e t i t - m a l s e iz u r e s . W e l l - f o r m e d a n d c l e a r

ly d esc r ib ed v i su a l h a l lu c in a t io n s were

a s s o c i a t e d w i t h t h e h e m i a n o p s i a .

W e b e l iev e t h a t a m ajo r i t y o f cases o f

b l in d n ess fo l l o win g ep i l ep sy , an d ce r

t a in ly t h o se i n wh ich th e p u p i l l a ry r e

f lexes r em ain i n t ac t o r in wh ich h e m i

an o p s i a i s p resen t , a r e b es t ex p la in ed o n

t h e b a s i s o f t e m p o r a r y e x h a u s t i o n o f t h e

v i su a l co r t ex d u e to t h e ep i l ep t i c d i s

c h a r g e . A t t e n t i o n h a s b e e n d r a w n t o a

case d esc r ib ed b y Mecca in wh ich sp asm

o f t h e r e t i n a l v esse l s seemed to b e t h e

c a u s e o f a m a u r o s i s .

R E F E R E N C E S

Ashby, H., and Stephe nson, S. Ac ute am auro sis following infantile convulsions. Lancet, 1903,

v. 1, pp. 1294-1296 and 1616-1617.

Gow ers, Sir W . R. A manual of diseases of the nervous system. Philadelphia, P. Blakiston's

Son & Co., 1892/1895.

H arr is, W. Hem ianopia with especial reference to its transient varieties. Brain, 1897, v. 20,

pp .308-364.

Mecca, M. Rep erti oculari nell'epilessia essentielle con particulare reguardo allo spasmo vasale.

Ann. di Ottal. e Clin. Ocul., 1938, v. 66, pp. 457-469.

Pr itch ard , E. A case of am aurosis following violent convulsions. Pro c. Roy. Soc. Med. (Sec

tion for Study Dis. Child ren ), 1918, v.

11,

p p. 14-16.

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