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8/10/2019 American Journal of Ophthalmology Volume 25 issue 4 1942 [doi 10.1016%2Fs0002-9394%2842%2990900-0] King
http:///reader/full/american-journal-of-ophthalmology-volume-25-issue-4-1942-doi-1010162fs0002-9394284229909 1/5
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
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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-
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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
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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,
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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.