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Postgraduate Medicine

ISSN: 0032-5481 (Print) 1941-9260 (Online) Journal homepage: http://www.tandfonline.com/loi/ipgm20

Cortical blindness

Walter G. Drymalski

To cite this article: Walter G. Drymalski (1980) Cortical blindness, Postgraduate Medicine, 67:4,
149-156, DOI: 10.1080/00325481.1980.11715430

To link to this article: http://dx.doi.org/10.1080/00325481.1980.11715430

Published online: 07 Jul 2016.

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Download by: [Cornell University Library] Date: 08 July 2017, At: 17:25
Cortical blindness
The changing incidence and shifting etiology

Walter G. Drymalski, MD

Con si der The incidence and etiology of cortical blindness-a disorder that usually
occurs after cerebrovascular accidents, cardiorespiratory arrest, severe
What are the most common head trauma, or bacterial meningitis-may be changing with the develop-
characteristics of cortical ment of effective means of cardiopulmonary resuscitation, as three cases
blindness? discussed here show.

Under what circumstances is the For a long time cortical blindness thrombi lodged in the distal poste-
disorder difficult to diagnose? bas been considered a rare event rior circulation. In a smaller series
resulting from bilateral damage to reported in 1957 by Bergman,5
Which diagnostic test is the most the occipital lobes or the optic radi- cerebrovascular occlusion caused
useful for fo/low-up evaluation? ations of the brain beyond the lat- cortical blindness in 7 of 12 pa-
eral geniculate body. The causes tients; carbon monoxide poisoning,
are few. trauma, Hodgkin 's disease, and
In the pediatrie age-group, progressive subcortical leukoen-
causes include head trauma, men- cephalopathy were also reported as
ingitis, and cardiorespiratory ar- causes.
rest.1-3 The head trauma is
usually sufficiently severe to result Recent case studies
in generalized CNS depression, It is important to note that the
but prognosis for recovery of sight foregoing studies were completed
is favorable. 2 Transient cortical prior to the 1960s-before effec-
blindness in children after seem- tive methods of cardiopulmonary
ingly minor head trauma also has resuscitation were developed. More
been reported. 1 Cortical blindness recent reports2-6·s of cortical blind-
as a sequela of bacterial meningitis ness in adults and the three follow-
is rare, and the incidence attribut- ing cases-which occurred in a
able to each of the various pa tho- two-year period at two community
gens is not known. hospitals-may indicate an inci-
In adults, the most common dence higher than originally sus-
causes are cerebrovascular acci- pected and a shift in the primary
dents and episodes of cardiorespi- etiology from cerebrovascular acci-
ratory arrest. ln the largest series dents to severe hypoxic or hypoten-
to date (58 cases), Symonds and sive episodes occurring during car-
Mackenzie• reported in 1957 that diorespiratory arrest.
cortical blindness was caused al- CASE t-A 71-year-old man was
most exclusively by thrombosis or brought to the emergency depart-
embolization of cerebral vessels. ment of Borgess Hospital, Kalama-
They believed that most of the epi- zoo, Michigan, in October 1975 by
sodes of cortical blindness were ambulance for evaluation of in-
caused by microemboli from verte- creasing dyspnea over the previous
bral or basilar artery plaques or 24 hours. The patient had been
continued

'VOL 67/NO 4/ APRIL 1980/POSTGRADUATE UEOICINI' • CORTICAL IILINDNE88 149


Cortical blindness should be suspected when a
patient with normal pupillary reflexes complains of
blindness even though the results of an ophthal·
moscopic examination are normal.

Walter G. Drymalski sion. He bad adult-onset type 1


Dr Drymalski is a fellow in (insulin-dependent) diabetes meiJi-
rheumatology, University of tus and was taking 40 units of
Michigan Medical sèhool, Ann
Arbor, and Wayne County isophane (NPH) insulin daily. He
General Hospital, Eloise, bad bad a myocardial infarction
Michigan. He was a resident in two years before this admission
internai medicine, Bronson
Methodist and Borgess hospi- and a right hemispheric cerebro-
tals, Southwestern Michigan vascular accident one year before.
Area Health Education Center, Blood pressure with the patient
Kalamazoo. His current re-
search interests are in hyper- seated was 180/120 mm Hg, ca-
trophie pulmonary osteoarthrop- rotid pulses were full and without
athy. bruits, and heart rate was regular
with an atrial gallop. Neurologie
examination showed increased
deep tendon reflexes on the left
receiving treatment for angina on! y gross perception of light and side and slight ataxia. Methyldopa,
pectoris and severe congestive complete inability to identify ob- propranolol, and furosemide were
heart failure. His wife said that his jects or colors. A neurologie con- administered.
mental clarity and ability bad been sultant found normal optic fundi On the third day, the patient be-
declining within the past few years. and cranial nerves, bilateral ca- came agitated and complained of
On physical examination, the pa- rotid bruits on auscultation, and no being unable to see. Examination
tient did not respond to commands nystagmus in response to optoki- at that time showed reactive pupils,
and bad peripheral cyanosis. Pupils netic stimulation with a rotating normal fundi, blood pressure of
were dilated but reactive; systolic drum. He also found the patient to 140/100 mm Hg, and blood glu-
blood pressure was 50 mm Hg with be disoriented to place and time cose leve! of 105 mg/dl. The corn-
supraventricular tachycardia in a and noted occasional visual halluci- plaint of blindness was not believed
rate of 130 to 150/min. Cardiopul- nations. initially, and chlorpromazine was
monary resuscitation was begun. In other respects, the patient im- administered to control agitation.
The patient improved steadily, proved, and he was discharged on a By the fourth day, the patient
and in 72 hours the resuscitation regimen for treatment of conges- was disoriented and more agitated.
was stopped. At that time he was tive heart disease. Within a month Left-sided hemiparesis developed;
awake although disoriented to he was readmitted for exacerbation blood pressure was llO/ 60 mm
place and time. He also com- of congestive heart failure. No Hg. The patient stated that he
plained of blindness. lnitially, the change in blindness was noted at could see, although when examined
complaint was attributed to the pa- that time, and although he was ori- he was unable to identify objects or
tient's dementia; therefore, com- ented and cooperative, visual field visually follow their movement.
plete assessment was delayed for examination was not requested. The neurologie consultant
severa! days. CASE 2-A 41-year-old man was agreed that cortical blindness, as
Over the next few days, the pa- admitted to Bronson Methodist manifested by intact pupillary
tient began to deny his blindness, Hospital, Kalamazoo, Michigan, in reflexes and absence of response to
although examination revealed July 1976 for essential hyperten- optokinetic stimulation, may have

150 CORTICAL IILINDNE88 • VOL 67/NO 4/ APRIL 1980/POSTGRADUATE UEOICINI'


Other than complaints of
blindness, the most
common symptom of cor-
tical blindness is deniai
of blindness, known as
Anton's syndrome.

occurred. A radioisotope brain not follow commands. Within three right temporal field but believed
scan showed decreased uptake in hours irregular, unstereotyped, that the fundi were otherwise nor-
the left parietal lobe and several jerking movements of the extremi- mal. Visual field examination was
small foci of decreased uptake in ties developed. The neurologie con- not performed because the patient
the right posterior parietal lobe. sultant, who felt these signs were was unable to cooperate.
After prolonged hospitalization consistent with myoclonic-type sei- Eight days after the patient's ad-
for intensive physical therapy, the zures, advised against immediate mission, gastrointestinal bleeding
patient recovered only minimal use treatment because the movements developed, followed by renal fait-
of his left leg and no apparent use were infrequent. Cranial nerves ure. Peritoneal dialysis was unsuc-
of his left arm, although evaluation were thought to be intact, and no cessful, and the patient died six
was complicated by his deniai of focal abnormalities were identified. days later.
that arm. The patient continued to The next day, the patient ap- Autopsy showed aortic stenosis
deny his blindness but was unable peared more alert. He followed with marked left ventricular hyper-
to consistently identify colors or simple commands but with a ten- trophy, acute bronchopneumonia,
figures. Visual field examination dency toward perseveration. He also and acute ischemie colitis. Grossly,
was not done during his hospitali- began talking at intervals and, dur- the brain was normal, but micro-
zation. ing one of these periods, com- scopie study revealed focal laminar
During more than one year of plained of blindness. vacuolization and neuronal degen-
follow-up, no significant change in On examination, the pupils re- eration throughout the cortex,
the patient's blindness was noted. acted to light, but there was no including sections taken from cal-
CASE ~A 65-year-old man was blinking response to threatening carine sulci.
brought to the emergency depart- gestures. Optokinetic stimulation
ment at Borgess Hospital in Sep- also produced no response. Clinical presentation
tember 1977 with a complaint of The following day, he no longer Cortical blindness should be sus-
rapid onset of dyspnea white rest- complained of blindness and, at pected when a patient with normal
ing. Two days previously, digoxin one time, seemed successful in pupillary reflexes complains of
therapy had been started for prob- identifying objects. However, later blindness even though the results
able aortic stenosis with dyspnea the same day, an EEG showed of an ophthalmoscopic examination
on exertion. At that time, the pa- diffuse slowing, particularly in the are normal. Too often the corn-
tient also had had symptoms con- occipital regions. Photic stimula- plaint of blindness is attributed to
sistent with angina pectoris, and an tion produced no response on the dementia or hysteria, which can
ECG had shown left bundle- EEG. delay diagnosis, as in cases 1 and 2.
branch block. Five days after admission, the Cortical blindness is far more
Shortly after arrivai, the patient patient still did not respond to difficult to diagnose in patients
had respiratory arrest, and intuba- optokinetic stimulation, and the who cannot easity communicate
tion and resuscitation were begun. EEG, which showed sorne increase their loss of sight, such as the very
When his condition stabilized, he in background slow-wave activity, young, the very debilitated, and
was transferred to the intensive still indicated no significant change those on respirators. In addition,
care unit. White being examined with photic stimulation. diagnosis is difficult in patients
there, he opened his eyes in re- The ophthalmologic consultant with Anton's syndrome-a form of
sponse to noises or voices but would found a cholesterol embolus in the anosognosia. In these types of pa-
continued

VOL 67/NO 4/ APRIL 1980/POSTGRADUATE UEOICINI' o CORTICAL IILINDNE88 151


Besides the ophthalmoscopic examination, other
essential diagnostic tests include bedside
examinations, such as optokinetic stimulation,
EEG with photic stimulation, and visual field
assessment.

optic nerve.9 As indicated in cases ophthalmoscopic findings (ie, no


Table 1. Characterietiçs of cortical sign of optic atrophy, generalized
blindneee l and 2, in which deniai of blind-
ness occurred only after an initial retinal degeneration, or papille-
Claseictriad complaint of blindness, Anton's dema). Table l lists classic charac-
Blindness. evidenced by
Failure to react to threatening syndrome may, at times, arise from teristics of the disorder. One of the
gestures most common is the absence of
No response to optokinetic the patient's confusion at the hospi-
stimulation tal staffs failure to confirm his or , blinking as a reflex to threatening
Consistent EEG findings gestures made by the examiner.
Intact pupillary reflexes ber initial complaint.
Normal fundi Spatial disorientation, Joss of vi- Also common is the absence of
Additional findinge suai imagery, Joss of color vision, blinking when a bright light is
Unformed visual hallucinations and visual agnosia also have been shone on the face of a patient who
Deniai of blindness (Anton's
syndrome) associated with cortical blindness, bas been in semidarkness. The lat-
although they were not common ter response may occasionally be
occurrences in the Symonds and normal in the absence of signifi-
tients, close and consistent clinicat Mackenzie study, 4and their inci- cant retained cortical vision, but
observation will detect decreased dence in cases occurring after car- also may be the first response to
visual attentiveness and sightless diorespiratory arrest bas not been return during recovery.
behavior. reported. In addition, these phe- Optokinetic stimulation tests for
In about 50% of patients whose nomena were absent in two of the nystagmus should be given to con-
blindness is caused by emboli or three cases cited here. scious patients. These tests involve
other occlusive vascular events, his- Finally, visual hallucinations use of a revolving drum with
tory of significant prodromes is evi- may accompany cortical blindness. alternating dark and light vertical
dent. The most frequent complaint As cases l and 2 demonstrate, it stripes or pulling a long piece of
is transient visual impairment; Jess may be difficult to distinguish material with alternating designs
often the complaint is vertigo, and these hallucinations from those of across the patient's visual field. In
!east often brief Joss of conscious- preexistent or concurrent demen- patients with normal pupillary
ness suggesting vertebrobasilar tia. In case l the patient bad prob- reflexes, cortical blindness is con-
insufficiency. 4 In addition, white able preexistent dementia, which firmed if one of these maneuvers
Joss of vision is usually acute, it could have been exacerbated by his does not produce nystagmus. The
may also occur progressively over hospitalization and Joss of sight, hysterical patient cannot voluntar-
severa! da ys in a minority of pa- causing more obvious confusion ily suppress nystagmus, white the
tients.4 and hallucination. In case 2 onset patient whose blindness is caused
Of the clinicat symptoms associ- of visual hallucinations and cortical by other factors will not have nor-
ated with cortical blindness, none blindness occurred simultaneously mal pupillary reflexes.
is constant or pathognomonic. One with onset of abnormal behavior. EEG with photic stimulation bas
of the most common is Anton's been proposed as a diagnostic and
syndrome. This syndrome, which in Physical examination prognostic test for cortical blind-
the past was considered to occur Specifie bedside examinations are ness.2·10 Responses consistent with
only with cortical blindness, bas necessary to confirm cortical blind- cortical blindness are failure to
also occurred with blindness ness in patients with normal pupil- suppress background activity with
caused by damage to the retina or lary responses to light and normal photic stimulation and the absence
continued

152 CORTICAL IILINDNE88 o VOL 67/NO 4/ APRIL 1980/POSTGRADUATE UEOICINI'


After cardiorespiratory
arrest, the most common
visual field defect is
loss of central vision
with varying degree of
peripheral sparing.

of short-latency visually evoked re-


sponse waves.z.a.:; However, studies
have not shown any consistent abil-
ity for this procedure to predict re-
covery of sight. 3
ln the cooperative patient, as-
sessment of visual fields may be
one of the most useful methods for
follow-up of cortical blindness. Pat-
terns of visual field Joss may also
delineate the pathogenesis.
ln their study, Symonds and
Mackenzie• found that the most
common visual field pattern (in 27
of 58 cases) was recovery of cen-
tral vision with continued absence
of any peripheral vision. Only four
patients demonstrated Joss of cen-
tral vision with partial or complete
spa ring of peripheral vision. The
others either remained totally blind
( 14) or regained varying amounts
of central and peripheral vision
(13). The authors noted that the
area of central vision in the striate
cortex is closest to the occipital
poles. They proposed that since the
occipital poles receive a rich collat- Figure 1. "Watershed" area of middle and posterior cerebral arteries shown in view of optic
eral supply from the "watershed" pathways exposed from below.
of the middle and posterior cere-
bral arteries, they most likely
would be spared from specifie oc- are usually the first to be affected ing, rather than the preservation of
clusive events occurring within the by generalized hypoxia because central vision seen with focal occlu-
occipital regions. they are the farthest from the ori- sive events in the posterior or mid-
It is important to note that none gins of cerebral circulation 11 dle cerebral arteries.
of the cases discussed by Symonds (figure 1). The conclusion seems Unfortunately, there are not an
and Mackenzie included histories obvious: ln cortical blindness oc- adequate number of case reviews
of cardiac or respira tory arrest curring after cardiorespiratory ar- in recent literature to document
with subsequent resuscitation. rest, the most common visual field this hypothesis. Hoyt and Walsh6
Other studies have shown that the defect is Joss of central vision with reported a case of cardiac arrest in
cerebrovascular "watershed" areas varying degrees of peripheral spar- which visual field testing showed
continued

VOL 67/NO 4/ APRIL 1980/POSTGRADUATE MEDICINE • CORTICAL IILINDNE88 155


The majority of patients with cortical blindness have
at least soma recovery of sight, but the extent
of recovery appears to be determined by the cause
of the disorder. The amount of ti me for recovery
has not been determined.

loss of central vision with return of and Mackenzie's4 58 cases result- cerebrovascular accidents. Progno-
sorne peripheral vision. Sabah8 re- ed in permanent blindness. Of sis for recovery of at )east partial
ported complete recovery of visual Duchowny and associates'3 six pe- sight is good, although proper visual
fields in two patients; however, the diatrie patients, only one remained testing is required to determine the
visual fields were not tested imme- completely blind, while three re- extent of improvement.~
diately after cardiac arrest. Other covered completely. Abraham and
reports of occipital blindness after associates 10 reported that of three Address reprint requests to Walter G.
cardiac arrest do not mention vi- adult patients with cortical blind- Drymalski, MD, Department of Internai
Medicine, Wayne County General Hospital,
suai field findings. 7 ln the cases 1 ness, one remained completely Eloise, Ml 48185.
have reported here, visual field blind, one recovered completely,
testing was not performed in cases and one recovered partially. Of
Referenc:es
1 and 2 because its potential use- Bergman's5 seven patients who sur-
1. Griffith JH, Dodge PR. Transient blind-
fulness was not appreciated at the vived acute episodes, two had com- ness following head injury in children.
time; in case 3 the patient was not plete return of vision and three had N Engl J Med 1961;278:648-51
cooperative enough to be tested. partial recovery. 2. Ramet AB, MIUBOII JI, Wllmer E. Acute
cerebral blindness in children: six cases
Furthermore, when possible, vi- To what extent the recovery of studied clinically and electrophysiologically.
suai field testing should be an inte- sight is determined by the cause of Neurology 1970;20:1147-56
gral part of evaluation because it the disorder and the amount of 3. Ducbowny MS, Weiss IP, Majlessi H, et
aL Visual evoked responses in childhood
assesses the patient's functional time required for recovery, wheth- cortical blindness after head trauma and
visual status and allows rehabilita- er complete or partial, have not meningitis: a longitudinal study of six cases.
tion to be tailored to individual been delineated. Until more spe- Neurology 1974;24:933-40
4. Symonds C, Mackenzie 1. Bilateral Joss of
abilities. lt is important to remem- cifie information becomes avail- vision from cerebral infarction. Brain 1957;
ber that the patient who retains able, it seems best to give each pa- 80:415-54
central vision may have nearly nor- tient an optimistic forecast. 5. Bergman PS. Cerebral blindness. Arch
Neurol 1957;78:568-84
mal visual acuity, but will behave 6. Hoyt WF, Walsh FB. Cortical blindness
as though blind because of the se- Summary with partial recovery following acute cere-
vere restriction of peripheral visual bral anoxia from cardiac arrest. Arch
Ophthalmol 1958;60: 1061-9
fields. Such a patient may act to- Once thought to be rare, cortical 7. Weinberger HA, fanderWoude R, Maier
tally blind and be treated as such, blindness today appears to occur HC. Prognosis of cortical blindness follow-
yet potential for rehabilitation is more frequently. (fhree cases de- ing cardiac arrest in children. JAMA
1962; 179: 126-9
far grea ter than that of the patient scribed here occurred within a two- 8. Sabah AH. Blindness after cardiac ar-
with no sight preserved. year period.) ln addition, the disor- rest. Postgrad Med J 1968;44:513-6
der may have undergone a shift in 9. Redlich FC, Dorsey JF. Deniai of blind-
ness by patients with cerebral disease. Arch
Prognosis etiology since the development of Neurol 1945;53:407-17
Prognosis for patients with cortical effective means of cardiopulmonary 10. Abraham FA, Melamed E, Lery S. Prog-
blindness is hopeful. Two thirds or resuscitation within the past two nostic value of visual evoked potentials in
occipital blindness following basilar artery
more of those affected will have at decades. Severe hypoxic or hypo- occlusion. Appl Neurophysiol 1975;38:
least sorne recovery of sight, al- tensive episodes occurring during 126-35
though this may not be appreciated cardiorespiratory arrest may have Il. Brierly JB. Cerebral hypoxia. ln: Black-
wood W, Corsellis JA, cds. Greenfield's
without follow-up visual field ex- become a more common cause of neuropathology. 3rd cd. London: E Arnold,
amination. Only 14 of Symonds cortical blindness in adults than 1976:56-79

156 CORTICAL 8l.INIINE88•VOL 67/NO 4/APRIL 1980/POSTGRADUATE UF!ltC:INF