- Email: [email protected]
Homonymous Hemianopia*
616
Ε.
Η.
B O T T E R E L L ,
L.
Α.
L L O Y D
A N D
Η.
J.
H O F F M A N
ping of the aneurysm is undertaken soon after the onset of oculmotor palsy. Other forms of treatment are much less effective in restoring third-nerve function. Toronto General Hospital.
neck and none of these recovered full function of their third nerve. Four patients were treated conservatively because of age or general state and none of these fully recovered function of their third nerve. CONCLUSION
ACKNOWLEDGMENTS
W e w i s h to e x p r e s s our gratitude to D r s . W . M. Lougheed and T. P . M o r l e y for permission t o include the records of their patients, and to Prof. T. O l s z e w s k i of the Department of Neuropathology, U n i v e r s i t y of T o r o n t o , for making available his autopsy studies.
Full recovery of third-nerve function following oculomotor palsy due to supraclinoid internal carotid berry aneurysms is likely to occur only if treatment by intracranial clip-
REFERENCES 1. Jefferson, G.: Isolated oculomotor palsy caused by intracranial aneurysm. Proc. Roy. Soc. M e d , 4 0 : 419, 1946. 2. H y l a n d , Η . H , and H . J. M . Barnett: T h e pathogenesis of cranial nerve palsies associated with intracranial aneurysms. P r o c . Roy. Soc. M e d , 4 7 : 1 4 1 , 1954. 3. W a l s h , F . B . : Clinical N e u r o - O p h t h a l m o l o g y . Baltimore, W i l l i a m s & W i l k i n s , 1957, ed. 2, p. 833.
HOMONYMOUS A
HEMIANOPIA*
REVIEW OF ONE HUNDRED J.
LAWTON Durham,
SMITH,
North
Quantitative perimetry is a clinical examination essential to proper evaluation of the neurologic patient. To emphasize the fundamental role of quantitative study of the visual fields in topical neurologic diagnosis, the following review of 100 cases of homonymous hemianopia is presented. MATERIAL
One hundred cases with homonymous visual field defects were selected for review from records of patients seen in neuroophthalmologic consultation over two and one-half years. Criteria for selection were: ( 1 ) visual field examination must have been done by me in each instance, ( 2 ) the defect must have been present at the time of examination, and ( 3 ) the patient had to be ex* F r o m the D i v i s i o n of Ophthalmology, D u k e U n i v e r s i t y School of Medicine. T h i s study w a s supported in part by U . S . Public H e a l t h Service Grant 81-1656.
CASES
M.D.
Carolina
amined at both the perimeter and tangent screen (confrontation fields alone were insufficient). Conventional tangent screen techniques were used and an Aimark projection perimeter was employed for peripheral field examinations. It should be noted that a common cause of homonyous field defects, ophthalmic migraine, was thus eliminated from consideration for, although many migraine patients give vivid histories of hemianopias, it is infrequent for these defects to be present at the time of the perimetric examination. The cases here considered are primarily those which would be seen in consultation by an ophthalmologist. The records were analyzed for the following data: age, sex, race, chief complaint, duration of complaint, visual acuity, visual field findings, measurements of palpebral fissures and pupils, optokinetic responses, ophthalmoscopic findings, ophthalmodynamometry, neurologic findings, results of arteriog-
HOMONYMOUS
raphy and encephalography, surgical findings, histologic corroboration, follow-up and final diagnosis. The visual field findings evaluated included: congruity, incongruity, splitting of fixation, sparing of fixation, defect denser above, defect denser below, importance of color techniques, perimeter of greater value, tangent screen of greater value, and perimeter and screen of equal value in detecting the hemianopia. FINDINGS
The location and incidence of the lesions producing hemianopia are seen in Table 1. I. OCCIPITAL LOBE
There were 39 cases in which the causative lesion was considered to be in the occipital lobe. The lesions were proved in 12 cases and, in 27, the diagnosis was based on clinical findings only. A. Proved occipital lobe cases. Of the 12 proved occipital lobe cases, six were in males (five white and one Negro), with an age range of 30 to 70 years, average 52 years. Six were in females (five white and one Negro), with an age range of 12 to 46 years, average 28 years. Eight cases were proved by surgery and biopsy, two by arteriography, one by autopsy and one by biopsy of primary malignancy elsewhere. The etiology of the 12 proved occipital lobe lesions was: Tumors Arteriovenous malformations Infarct ( A n t o n e ' s s y n d r o m e ) Trauma Carcinoid syndrome Vascular
6 2 1 1 1 1
Histologic examination of the six proved occipital lobe tumors revealed : Glioblastoma multiforme Ependymoma Metastatic bronchogenic carcinoma Metastatic breast carcinoma Metastatic undifferentiated carcinoma
2 1 1 1 1
Summarizing the etiology of the proved occipital lesions:
HEMIANOPIA
617
T A B L E
1
LESIONS IN HOMONYMOUS
HEMIANOPIA
O p t i c tract Lateral geniculate T e m p o r a l lobe Parietal lobe Occipital lobe
3 % 1% 24% 3 3 % 39%
T u m o r s ( 3 primary, 3 metastatic) V a s c u l a r accidents Arteriovenous malformations Trauma
6 3 2 1
Etiology in the six males was tumor in three, and vascular in three. Etiology in the six females was tumor in three, arteriovenous malformation in two, and trauma in one. Vision in these proved occipital lobe cases was 2 0 / 3 0 or better in both eyes in 10 patients, and less than 2 0 / 7 0 in both eyes of two others ; however, both of the cases with poorer vision had demonstrated bilateral occipital lobe lesions. The optokinetic nystagmus signs were negative in six of the proved cases (normal, symmetrical response), positive (abnormal, asymmetric response) in five and absent in one. It is of note that of the five cases of proved occipital lobe lesions with positive optokinetic nystagmus signs three had tumors and two had arteriovenous malformations, all five representing mass lesions. Visual fields in the proved occipital lobe cases revealed the following data: D e n s e defects ( o u t to confrontation) Splitting o f fixation S p a r i n g of fixation D e n s e r below D e n s e r above Perimeter better Perimeter-screen Screen better
9 6 Nil 3 Nil 1 1 2
B. Not proved occipital lobe cases. There were 27 cases in which the diagnosis of an occipital lobe lesion appeared warranted on clinical grounds but in which unequivocal anatomic proof was lacking. These cases are considered separately. Of the 27 cases with such clinical diagnoses, 22 were in males (21 white and one N e g r o ) , with an age range
618
J. L A W T O N
of 22 to 74 years, average 54 years. There were five females (five white), with an age range of 37 to 78 years, average 54 years. Etiology in the five females was vascular in four and tumor in one. All five of these patients had negative optokinetic nystagmus signs. A bilateral homonymous hemianopia was present in one of these patients. Field data on these five cases were: Splitting of fixation Sparing o f D e n s e r above D e n s e r below Screen better P e r i m e t e r better
fixation
2 1 2 Nil 1 1
Vision was 20/40 or better in both eyes of all but one of the females ; bilateral hemianopia in the one case explained vision of 20/70, R.E. ; 20/40, L.E. Of the 22 cases of not-proved occipital lobe lesions in males, there were 20 with negative optokinetic responses and two with positive. The two patients with positive findings were clinically considered to have vascular lesions, but tumors could not be excluded. Vision was normal ( 2 0 / 4 0 or better in both eyes) in 20 of the 22 males. The other two patients had cataracts and macular lesions to account for reduced acuity. Field data on the male cases revealed: Splitting of fixation Sparing of fixation D e n s e r above D e n s e r below Congruous Incongruous Color perimetry of value "Cresent" phenomenon Perimeter better Perimeter-screen Screen better
8 5 7 Nil 12 Nil 1 2 2 4 6
Summary of occipital lobe cases. If the data for the proved and not-proved occipital lobe cases are combined, the following points are evident: there were 28 males and 11 females, with an average age of 50 years. The optokinetic nystagmus sign was negative in 31 cases, positive in seven (all of which demonstrated mass lesions) and absent in one. Vision was normal in 34 patients. Bilateral
SMITH
occipital lobe lesions accounted for reduced acuity in three cases, and ocular disease was present in two others. The etiology was due to a vascular lesion in 24 cases, tumors in seven and other causes in eight. The visual field defects split fixation in 14 cases and spared fixation in five. The defects were congruous in 12 instances, and slightly incongruous in one. The defects were denser above in seven patients and denser below in three. The perimeter was considered of greater value in three patients and of equal value with the screen in five. The tangent screen appeared of greater value in the detection of eight cases of hemianopia due to the presence of paracentral defects in most instances. In patients with a clinical diagnosis of posterior cerebral artery occlusion, it was of interest that splitting of fixation was encountered 3:1 more frequently than was sparing of fixation. I I . PARIETAL LOBE
There were 17 proved and 16 unproved parietal lobe lesions. A. Proved parietal lobe lesions. Of the 17 proved cases, 10 were in males ( nine white and one Negro), with an age range of 34 to 64 years, average 51 years. There were seven females (all white), ranging in age from 38 to 71 years, average 53 years. The diagnoses and clinical evidence used for substantiation in the seven proved cases in females were: T u m o r (proved by arteriography) Spindle cell sarcoma (proved by autopsy) A n e u r y s m (proved by arteriography) Internal carotid occlusion (proved by arteriography) Intracerebral hematoma (proved by surgery) Glioblastoma multiforme (proved by sur-
gery)
Subdural hematoma (proved by s u r g e r y )
Thus, among the females there were three cases of tumors, three of vascular lesions and one of trauma. In these seven cases, the optokinetic nystagmus signs were positive in six cases and "equivocal" in one.
HOMONYMOUS
Of the 10 proved parietal lobe lesions in males, six were tumors (two primary and four metastatic), two vascular lesions, one porencephalic cyst and one traumatic. These cases were confirmed by surgery in five cases, by arteriography in four and by biopsy of a primary malignancy elsewhere in one. Of the 10 males, nine had positive optokinetic nystagmus signs and one was "equivocal on the tape and positive on the drum." Vision was normal ( 2 0 / 4 0 or better in both eyes) in 11 patients, could not be accurately determined in four (dysphasia and obtundation) and was reduced due to ocular lesions in two others. Visual fields in the proved parietal lobe cases showed: D e n s e defects ( o u t to confrontation, and hence congruity could not be determined) Splitting of fixation Sparing of fixation Denser below D e n s e r above P e r i m e t e r better Perimeter-screen Screen better Congruous Incongruous, slightly Color perimetry helpful
11
5
Nil
5 2 1 1
2
Nil
2
1
B. Not proved parietal lobe lesions. Of the 16 unproved parietal lobe cases, seven were females (all white) ranging in age from 59 to 82 years, average 73 years. Nine were in males (seven white and two N e g r o ) , ranging from 43 to 96 years, average 58 years. Of the seven females, five had vascular lesions and two had tumors. Six of the seven females showed a positive optokinetic nystagmus sign ; it was not recorded in one case. Vision was normal in three of the females. Dysphasia, parkinsonism with debilitation, metaherpetic keratitis and central retinal artery occlusion accounted for the lowered acuity in the other four patients. Of the nine male not-proved parietal lobe cases, the etiology was vascular in six, tumor in two and congenital in one. The optokinetic response was positive in seven of the males, and difficult to assess in two
HEMIANOPIA
619
others (due to sedation and obtundation). Vision was normal ( 2 0 / 4 0 or better in both eyes) in five of the males, not listed in one, reduced due to ocular trauma in two and could not be assessed because of global aphasia in one. Visual field data on the not-proved parietal lobe cases revealed: D e n s e defects ( c o n g r u i t y could not be assessed) 13 Perimeter-screen 3 P e r i m e t e r better 1 Screen better Nil Splits 4 Spares Nil D e n s e r below 1 D e n s e r above Nil
Summary of parietal lobe cases. Combining the data for all parietal lobe cases, the following points emerge: There were 33 cases, with 19 males ( 1 6 white and three Negro) and 14 females (all white). The average age was 58 years. Etiology was vascular in origin in 16, due to a tumor in 13 and due to other causes in four. The optokinetic nystagmus signs were positive in 28, equivocal in two, difficult to assess or not recorded in three. The visual field defects were quite dense in 24 patients and therefore congruity could not be determined. Since the defects were denser below in six and denser above in two cases of partial hemianopia, it would appear that parietal lobe field defects are denser below 3:1 more often than above. The perimeter and tangent screen appeared to be of equal value in detecting parietal lobe field defects. Splitting of fixation was noted in nine patients, and sparing in none. N o congruous defects were found. It is evident that parietal lobe lesions cause splitting of fixation. I I I . TEMPORAL LOBE
There were 14 proved and 10 not proved temporal lobe lesions in this series. A. Proved temporal lobe lesions. There were 14 cases of proved temporal lobe lesions. Of these 10 cases were in females, with an aee range of 25 to 57 years, average 40 years.
620
J. L A W T O N
Four cases were in males, with an age range of 30 to 53 years, average 36 years. It should be stressed that all but one of the 14 proved temporal lobe lesions was a tumor. Surgery and histologic study proved the etiology of the temporal lobe lesions in the 10 females, showing teratoma, astrocytoma, oligodendroglioma, astrocytoma, glioblastoma multiforme, glioma, cystic glioma, glioma, temporal lobectomy for seizures and metastatic breast carcinoma (biopsy of primary plus roentgen evidence). In the four males, the etiology was found by surgery and biopsy to be due to an astrocytoma in three patients ; one patient was found by arteriography to have a deep temporal lobe tumor stain. Vision was normal ( 2 0 / 3 0 or better in both eyes) in 13 of the proved cases ; it was not listed in one male. The optokinetic responses were of interest. All 10 of the female cases had negative signs. This was considered significant since the postoperative examination in four of these patients was at six months, five years, eight years, and 17 years, respectively. In the four males, two had negative optokinetic signs and one equivocal. One patient with a tumor, when first examined, had a negative optokinetic response which became positive six months later. Visual fields in the proved cases revealed: D e f e c t denser above ( M e y e r ' s loop field 11 defect) Full hemianopia 3 7 Incongruous 1 N e a r l y congruous 2 Perimeter better 2 Perimeter-screen 1 Screen better 3 Color perimetry helpful Splits fixation 3 Nil Spares fixation
B. Not proved temporal lobe cases. Ternporal lobe lesions were diagnosed on clinical grounds in 10 patients. Of these seven were in males (six white and one Negro) and three were in females (all white). The average age of the males was 37 years (range
SMITH
24 to 51 years) and of the females 36 years ( range 11 to 66 years). Etiology in the males was tumor in three, vascular two, trauma one, and tuberculoma one. The causative lesions in the females were tumor in two and birth injury in one. Vision was better than 2 0 / 4 0 in both eyes in all of the women, and in five of the seven men. Vision was not recorded in one man in whom it had fallen from 2 0 / 3 0 to hand movements during a two-year observation period due to a tuberculoma of the optic nerve. The optokinetic nystagmus signs were negative in two of the three women (not recorded in the other), and in five of the seven men. They were positive in two of the men. Visual field data in the not-proved temporal lobe cases revealed: D e f e c t denser above ( M e y e r ' s loop defects) 7 Full hemianopia 3 Perimeter better S Perimeter-screen 4 Screen examination only 1 Incongruous 7 Congruous Nil Color perimetry helpful 1
Summary of temporal lobe cases. There were 24 temporal lobe lesions, 14 of which were proved. Thirteen were in females and 11 in males. The average age of all of the temporal lobe cases was 38 years. The etiologic findings were striking in that a neoplasm was found in 18 patients, vascular lesions in only two, and other causes in four patients. Of the 14 patients with proved lesions, a tumor was found in all but one case, making the ratio of tumors to vascular lesions 9:1 in the patients presenting temporal lobe lesions with homonymous field defects. The optokinetic nystagmus sign was negative in 19, positive in three and not recorded in two. Vision was normal in 21 patients, not recorded in two and reduced because of a local eye lesion in one. Visual fields in the temporal lobe cases revealed that the typical Meyer's loop upper quadrantanopia was present in 18 cases, with a full hemianopia in only six. Thus, a
HOMONYMOUS
Meyer's loop field defect is seen 3:1 more frequently than a full hemianopia in temporal lobe disease. The defects were incongruous in 14 cases, and nearly congruous in only one, showing that temporal lobe lesions have a marked tendency toward incongruity. Of note is that the perimeter seemed to be more helpful in detecting the defect in seven patients, the screen in only two. This finding seemed to be corroborated by the observation that color peripheral fields with the Aimark perimeter were helpful in four cases, or one of six temporal lobe field defects. IV.
HEMIANOPIA
621
V i s u a l field e x a m i n a t i o n revealed that o n l y a temporal island remained in the left eye. T h e field w a s within normal limits in the right e y e t o 1/330 and 6 / 1 0 0 0 w h i t e but there w a s a complete temporal hemianopia in the right eye t o 3 / 3 3 0 red. T h e left optic disc w a s pale. T h e optokinetic responses w e r e symmetrical o n both sides. R o e n t g e n o g r a m s revealed a g r o s s l y enlarged sella turcica. T h e patient declined further studies and the clinical impression w a s either a pituitary tumor o r aneurysm. T h e h o m o n y m o u s aspect to his visual field defect corroborated involvement at the junction of optic tract and chiasm on the left. DISCUSSION
The material presented herein is summarized in Table 2.
TRACT A N D LATERAL GENICULATE
The diagnosis of lesions of the optic tract and lateral geniculate body by perimetric techniques is quite difficult and this difficulty seems to be reflected in the infrequency with which such lesions were found in this series. The one patient considered to have a lateral geniculate body lesion was a 59-year-old white woman (reported elsewhere*) who developed a field defect during a stereotactic procedure for unilateral parkinsonism. Visual field examination in this patient revealed a left lower quadrantanopia which was extremely incongruous. The optokinetic nystagmus sign was negative. Tract hemianopias were considered to be present when extreme incongruity, a negative optokinetic response, evidence of Behr's pupil and evidence of neurologic disease in neighboring structures were demonstrated. The number of tract lesions is considered too small for statistical interpretation. One patient is cited as an example of a tract defect: A 67-year-old N e g r o w a s seen in the Massachusetts E y e and E a r Infirmary in 1953, complaining of decreasing visual acuity in the left eye for o n e year. A t that time, a nasal field defect had been found in the left eye. F i v e years later, the patient returned and w a s found to h a v e vision of 2 0 / 2 0 in the right e y e and 1/200 in the left eye. * Smith, J. L., N a s h o l d , B. S., and Kreshon, M . J . : Ocular signs a f t e r stereotactic lesions in the pallidum and thalamus. Α Μ Α A r c h . Ophth., 65: 532 ( A p r . ) 1961.
SUMMARY
This review of 100 cases of homonymous visual field defects examined by me permits the following conclusions: Temporal lobe lesions produce one fourth of the cases of hemianopias. They usually occur in a younger age group and are about equally distributed between males and females. Such field defects have ominous significance since the most frequent etiologic lesion is a neoplasm; the tumor/vascular ratio was 9:1 in this series. These field defects are typically incongruous and are usually found more readily at the perimeter than the tangent screen. The 3/330 red isopter on the Aimark projection perimeter is at times quite helpful in their detection. These lesions are associated with a normal, symmetrical optokinetic response, unless parietal lobe extension has occurred. Parietal lobe lesions produce one third of the cases of hemianopias, and occur in an older age group. The incidence appears to be slightly greater in males than females, probably reflecting the greater number of vascular lesions in the male group. The ratio of tumors and vascular lesions is about equal. The optokinetic nystagmus sign is of great help in these cases ; there were 28 instances of a positive optokinetic nystagmus sign. Not a single case of parietal lobe hemianopia showed a normal optokinetic response. There is no question but that parietal lobe lesions
622
J. L A W T O N T A B L E
SMITH 2
HOMONYMOUS HEMIANOPIA
Temporal N o . Cases Males Females A g e range Average age Etiology Tumor Vascular Other Optokinetic R e s p o n s e Positive Negative Absent N o t recorded Equivocal Fields Denser a b o v e Denser below Incongruous Congruous Perimeter better Perimeter = screen Screen better Split fixation Spare fixation Color helpful Vision 2 0 / 4 0 O . U . or better Less E y e disease N o t recorded Dysphasia
SUMMARY
Parietal
Occipital
24
33
39
11
19
28
13
14
11
11-57
34-96
12-78
38
58
50
18
13
7
2
16
24
4
4
8
3
28
19
nil
7 31 1
2
7
1
6
3
14
2
1
1
nil
12
7
2
3
7
4
5
2
2
8
9
14
20
nil 1
21
5
nil
19
34
1
6
2
2
1
3 (bilateral occipital lesions)
can exist in the presence of a negative optokinetic response, but, if the lesion is deep enough to produce hemianopia, the optokinetic response is invariably asymmetric. This series confirms Kestenbaum's data regarding this finding. Parietal lobe field defects are detected with equal facility on the perimeter and tangent screen. Such defects are denser below 3:1 as often as they are denser above. They split fixation in each instance in this study, with no sparing noted with parietal lobe disease. Occipital lobe lesions are the most common cause of hemianopia, producing two fifths of the cases. They are more frequent in males than females (28:11 in this series). The average age was SO years, definitely older than in the temporal lobe group. The etiology
7
of an occipital lobe field defect is just the opposite of that of temporal lobe lesions for vascular lesions are more frequent than tumors by a striking degree, 3:1 in this study. The optokinetic sign is usually negative in the presence of an occipital lobe field defect. Cogan's rule was evaluated in this study. This states that, in the presence of an occipital lobe field defect, a negative optokinetic nystagmus sign points to a vascular lesion but a positive sign points to a tumor or mass lesion. There were seven positive optokinetic responses in this series of occipital lobe lesion. There were seven positive optokinetic and two arteriovenous malformations—in each instance, a mass lesion was present. Thus, Cogan's rule was found to be of great clinical value.
HOMONYMOUS
Occipital lobe field defects are found more readily on the tangent screen than the perimeter, due to the frequency of paracentral defects. They are congruous and, interestingly, are denser above 2:1 as often as below. They usually split fixation (3:1 over sparing). Posterior cerebral artery occlusions usually split fixation rather than spare it. A very important point concerning vision in the presence of hemianopia should be stressed. Homonymous hemianopia does not explain a reduction in visual acuity! Thus, a patient can see 2 0 / 2 0 with one half of
HEMIANOPIA
623
the macula. In every instance, without exception, in the entire series the visual acuity was within normal limits in each patient with hemianopia, unless cause for the reduction in acuity was readily evident on routine ophthalmologic examination. Division of Ophthalmology. T h e patients seen in this study w e r e e x a m i n e d at the Massachusetts E y e and E a r Infirmary, D u k e U n i v e r s i t y Medical Center and D u r h a m V e t e r a n s Administration Hospital. Grateful acknowledgment is g i v e n to Dr. D a v i d G. Cogan for his help and encouragement and permission to see some of these patients.
REFERENCE Kestenbaum, A . : Clinical M e t h o d s of N e u r o - O p h t h a l m o l o g i c E x a m i n a t i o n . N e w Y o r k , Grune & Stratton, 1961, ed. 2.
H O M O N Y M O U S
H E M I A N O P I A
A F T E R
O C C I P I T A L
L O B E C T O M Y *
ALFRED HUBER, M . D . Zurich,
The phenomenon of macular sparing in homonymous hemianopia has been the object of world-wide discussion for many years (Wilbrand, Heine, Lenz, Pfeifer, Foerster, Penfield, Putnam, Poliak, Rönne, v. Monakow, Adler Fliegelmann, Verhoeff, Halstead, Walker, Bucy, David, Hacean, Ajuriaguerra, Austin, Lewey, Grant, Dubois-Poulsen). It has assumed even more interest since neurosurgery has provided the opportunity to study visual field defects after removal of well-determined areas of the visual pathway in man. Professor Walsh, to whom this article is dedicated, devotes several pages of his wonderful book to this problem. Therefore, it was thought that he might be interested in hearing about some new findings on this subject. In co-operation with the Department of Neurosurgery of the University of Zurich * F r o m the U n i v e r s i t y E y e Clinic and the N e u rosurgery Clinic o f the U n i v e r s i t y of Zurich.
Switzerland
(Prof. Krayenbühl) I have had occasion to examine 11 cases of occipital lobectomies performed because of tumors of this area of the brain. In occipital lobectomy, very frequently combined with opening of the posterior horn of the lateral ventricle, the whole calcarine cortex and a considerable part of the optic radiation is removed—in other words, total removal of that part of the optic pathway which is considered to produce homonymous hemianopia with macular sparing. Our method of examination was to determine all visual functions, including visual acuity, color sense, motility and so forth. Perimetry was done by means of different apparatuses: the Bjerrum-screen, Maggiore perimeter, Amsler chart and especially the Goldmann perimeter. Different test objects of varying size, luminosity and color were used. Special care was given to the problem of the maintenance of good central fixation during perimetry. The fixation can be steadily controlled at the Goldmann perimeter
Ε.
Η.
B O T T E R E L L ,
L.
Α.
L L O Y D
A N D
Η.
J.
H O F F M A N
ping of the aneurysm is undertaken soon after the onset of oculmotor palsy. Other forms of treatment are much less effective in restoring third-nerve function. Toronto General Hospital.
neck and none of these recovered full function of their third nerve. Four patients were treated conservatively because of age or general state and none of these fully recovered function of their third nerve. CONCLUSION
ACKNOWLEDGMENTS
W e w i s h to e x p r e s s our gratitude to D r s . W . M. Lougheed and T. P . M o r l e y for permission t o include the records of their patients, and to Prof. T. O l s z e w s k i of the Department of Neuropathology, U n i v e r s i t y of T o r o n t o , for making available his autopsy studies.
Full recovery of third-nerve function following oculomotor palsy due to supraclinoid internal carotid berry aneurysms is likely to occur only if treatment by intracranial clip-
REFERENCES 1. Jefferson, G.: Isolated oculomotor palsy caused by intracranial aneurysm. Proc. Roy. Soc. M e d , 4 0 : 419, 1946. 2. H y l a n d , Η . H , and H . J. M . Barnett: T h e pathogenesis of cranial nerve palsies associated with intracranial aneurysms. P r o c . Roy. Soc. M e d , 4 7 : 1 4 1 , 1954. 3. W a l s h , F . B . : Clinical N e u r o - O p h t h a l m o l o g y . Baltimore, W i l l i a m s & W i l k i n s , 1957, ed. 2, p. 833.
HOMONYMOUS A
HEMIANOPIA*
REVIEW OF ONE HUNDRED J.
LAWTON Durham,
SMITH,
North
Quantitative perimetry is a clinical examination essential to proper evaluation of the neurologic patient. To emphasize the fundamental role of quantitative study of the visual fields in topical neurologic diagnosis, the following review of 100 cases of homonymous hemianopia is presented. MATERIAL
One hundred cases with homonymous visual field defects were selected for review from records of patients seen in neuroophthalmologic consultation over two and one-half years. Criteria for selection were: ( 1 ) visual field examination must have been done by me in each instance, ( 2 ) the defect must have been present at the time of examination, and ( 3 ) the patient had to be ex* F r o m the D i v i s i o n of Ophthalmology, D u k e U n i v e r s i t y School of Medicine. T h i s study w a s supported in part by U . S . Public H e a l t h Service Grant 81-1656.
CASES
M.D.
Carolina
amined at both the perimeter and tangent screen (confrontation fields alone were insufficient). Conventional tangent screen techniques were used and an Aimark projection perimeter was employed for peripheral field examinations. It should be noted that a common cause of homonyous field defects, ophthalmic migraine, was thus eliminated from consideration for, although many migraine patients give vivid histories of hemianopias, it is infrequent for these defects to be present at the time of the perimetric examination. The cases here considered are primarily those which would be seen in consultation by an ophthalmologist. The records were analyzed for the following data: age, sex, race, chief complaint, duration of complaint, visual acuity, visual field findings, measurements of palpebral fissures and pupils, optokinetic responses, ophthalmoscopic findings, ophthalmodynamometry, neurologic findings, results of arteriog-
HOMONYMOUS
raphy and encephalography, surgical findings, histologic corroboration, follow-up and final diagnosis. The visual field findings evaluated included: congruity, incongruity, splitting of fixation, sparing of fixation, defect denser above, defect denser below, importance of color techniques, perimeter of greater value, tangent screen of greater value, and perimeter and screen of equal value in detecting the hemianopia. FINDINGS
The location and incidence of the lesions producing hemianopia are seen in Table 1. I. OCCIPITAL LOBE
There were 39 cases in which the causative lesion was considered to be in the occipital lobe. The lesions were proved in 12 cases and, in 27, the diagnosis was based on clinical findings only. A. Proved occipital lobe cases. Of the 12 proved occipital lobe cases, six were in males (five white and one Negro), with an age range of 30 to 70 years, average 52 years. Six were in females (five white and one Negro), with an age range of 12 to 46 years, average 28 years. Eight cases were proved by surgery and biopsy, two by arteriography, one by autopsy and one by biopsy of primary malignancy elsewhere. The etiology of the 12 proved occipital lobe lesions was: Tumors Arteriovenous malformations Infarct ( A n t o n e ' s s y n d r o m e ) Trauma Carcinoid syndrome Vascular
6 2 1 1 1 1
Histologic examination of the six proved occipital lobe tumors revealed : Glioblastoma multiforme Ependymoma Metastatic bronchogenic carcinoma Metastatic breast carcinoma Metastatic undifferentiated carcinoma
2 1 1 1 1
Summarizing the etiology of the proved occipital lesions:
HEMIANOPIA
617
T A B L E
1
LESIONS IN HOMONYMOUS
HEMIANOPIA
O p t i c tract Lateral geniculate T e m p o r a l lobe Parietal lobe Occipital lobe
3 % 1% 24% 3 3 % 39%
T u m o r s ( 3 primary, 3 metastatic) V a s c u l a r accidents Arteriovenous malformations Trauma
6 3 2 1
Etiology in the six males was tumor in three, and vascular in three. Etiology in the six females was tumor in three, arteriovenous malformation in two, and trauma in one. Vision in these proved occipital lobe cases was 2 0 / 3 0 or better in both eyes in 10 patients, and less than 2 0 / 7 0 in both eyes of two others ; however, both of the cases with poorer vision had demonstrated bilateral occipital lobe lesions. The optokinetic nystagmus signs were negative in six of the proved cases (normal, symmetrical response), positive (abnormal, asymmetric response) in five and absent in one. It is of note that of the five cases of proved occipital lobe lesions with positive optokinetic nystagmus signs three had tumors and two had arteriovenous malformations, all five representing mass lesions. Visual fields in the proved occipital lobe cases revealed the following data: D e n s e defects ( o u t to confrontation) Splitting o f fixation S p a r i n g of fixation D e n s e r below D e n s e r above Perimeter better Perimeter-screen Screen better
9 6 Nil 3 Nil 1 1 2
B. Not proved occipital lobe cases. There were 27 cases in which the diagnosis of an occipital lobe lesion appeared warranted on clinical grounds but in which unequivocal anatomic proof was lacking. These cases are considered separately. Of the 27 cases with such clinical diagnoses, 22 were in males (21 white and one N e g r o ) , with an age range
618
J. L A W T O N
of 22 to 74 years, average 54 years. There were five females (five white), with an age range of 37 to 78 years, average 54 years. Etiology in the five females was vascular in four and tumor in one. All five of these patients had negative optokinetic nystagmus signs. A bilateral homonymous hemianopia was present in one of these patients. Field data on these five cases were: Splitting of fixation Sparing o f D e n s e r above D e n s e r below Screen better P e r i m e t e r better
fixation
2 1 2 Nil 1 1
Vision was 20/40 or better in both eyes of all but one of the females ; bilateral hemianopia in the one case explained vision of 20/70, R.E. ; 20/40, L.E. Of the 22 cases of not-proved occipital lobe lesions in males, there were 20 with negative optokinetic responses and two with positive. The two patients with positive findings were clinically considered to have vascular lesions, but tumors could not be excluded. Vision was normal ( 2 0 / 4 0 or better in both eyes) in 20 of the 22 males. The other two patients had cataracts and macular lesions to account for reduced acuity. Field data on the male cases revealed: Splitting of fixation Sparing of fixation D e n s e r above D e n s e r below Congruous Incongruous Color perimetry of value "Cresent" phenomenon Perimeter better Perimeter-screen Screen better
8 5 7 Nil 12 Nil 1 2 2 4 6
Summary of occipital lobe cases. If the data for the proved and not-proved occipital lobe cases are combined, the following points are evident: there were 28 males and 11 females, with an average age of 50 years. The optokinetic nystagmus sign was negative in 31 cases, positive in seven (all of which demonstrated mass lesions) and absent in one. Vision was normal in 34 patients. Bilateral
SMITH
occipital lobe lesions accounted for reduced acuity in three cases, and ocular disease was present in two others. The etiology was due to a vascular lesion in 24 cases, tumors in seven and other causes in eight. The visual field defects split fixation in 14 cases and spared fixation in five. The defects were congruous in 12 instances, and slightly incongruous in one. The defects were denser above in seven patients and denser below in three. The perimeter was considered of greater value in three patients and of equal value with the screen in five. The tangent screen appeared of greater value in the detection of eight cases of hemianopia due to the presence of paracentral defects in most instances. In patients with a clinical diagnosis of posterior cerebral artery occlusion, it was of interest that splitting of fixation was encountered 3:1 more frequently than was sparing of fixation. I I . PARIETAL LOBE
There were 17 proved and 16 unproved parietal lobe lesions. A. Proved parietal lobe lesions. Of the 17 proved cases, 10 were in males ( nine white and one Negro), with an age range of 34 to 64 years, average 51 years. There were seven females (all white), ranging in age from 38 to 71 years, average 53 years. The diagnoses and clinical evidence used for substantiation in the seven proved cases in females were: T u m o r (proved by arteriography) Spindle cell sarcoma (proved by autopsy) A n e u r y s m (proved by arteriography) Internal carotid occlusion (proved by arteriography) Intracerebral hematoma (proved by surgery) Glioblastoma multiforme (proved by sur-
gery)
Subdural hematoma (proved by s u r g e r y )
Thus, among the females there were three cases of tumors, three of vascular lesions and one of trauma. In these seven cases, the optokinetic nystagmus signs were positive in six cases and "equivocal" in one.
HOMONYMOUS
Of the 10 proved parietal lobe lesions in males, six were tumors (two primary and four metastatic), two vascular lesions, one porencephalic cyst and one traumatic. These cases were confirmed by surgery in five cases, by arteriography in four and by biopsy of a primary malignancy elsewhere in one. Of the 10 males, nine had positive optokinetic nystagmus signs and one was "equivocal on the tape and positive on the drum." Vision was normal ( 2 0 / 4 0 or better in both eyes) in 11 patients, could not be accurately determined in four (dysphasia and obtundation) and was reduced due to ocular lesions in two others. Visual fields in the proved parietal lobe cases showed: D e n s e defects ( o u t to confrontation, and hence congruity could not be determined) Splitting of fixation Sparing of fixation Denser below D e n s e r above P e r i m e t e r better Perimeter-screen Screen better Congruous Incongruous, slightly Color perimetry helpful
11
5
Nil
5 2 1 1
2
Nil
2
1
B. Not proved parietal lobe lesions. Of the 16 unproved parietal lobe cases, seven were females (all white) ranging in age from 59 to 82 years, average 73 years. Nine were in males (seven white and two N e g r o ) , ranging from 43 to 96 years, average 58 years. Of the seven females, five had vascular lesions and two had tumors. Six of the seven females showed a positive optokinetic nystagmus sign ; it was not recorded in one case. Vision was normal in three of the females. Dysphasia, parkinsonism with debilitation, metaherpetic keratitis and central retinal artery occlusion accounted for the lowered acuity in the other four patients. Of the nine male not-proved parietal lobe cases, the etiology was vascular in six, tumor in two and congenital in one. The optokinetic response was positive in seven of the males, and difficult to assess in two
HEMIANOPIA
619
others (due to sedation and obtundation). Vision was normal ( 2 0 / 4 0 or better in both eyes) in five of the males, not listed in one, reduced due to ocular trauma in two and could not be assessed because of global aphasia in one. Visual field data on the not-proved parietal lobe cases revealed: D e n s e defects ( c o n g r u i t y could not be assessed) 13 Perimeter-screen 3 P e r i m e t e r better 1 Screen better Nil Splits 4 Spares Nil D e n s e r below 1 D e n s e r above Nil
Summary of parietal lobe cases. Combining the data for all parietal lobe cases, the following points emerge: There were 33 cases, with 19 males ( 1 6 white and three Negro) and 14 females (all white). The average age was 58 years. Etiology was vascular in origin in 16, due to a tumor in 13 and due to other causes in four. The optokinetic nystagmus signs were positive in 28, equivocal in two, difficult to assess or not recorded in three. The visual field defects were quite dense in 24 patients and therefore congruity could not be determined. Since the defects were denser below in six and denser above in two cases of partial hemianopia, it would appear that parietal lobe field defects are denser below 3:1 more often than above. The perimeter and tangent screen appeared to be of equal value in detecting parietal lobe field defects. Splitting of fixation was noted in nine patients, and sparing in none. N o congruous defects were found. It is evident that parietal lobe lesions cause splitting of fixation. I I I . TEMPORAL LOBE
There were 14 proved and 10 not proved temporal lobe lesions in this series. A. Proved temporal lobe lesions. There were 14 cases of proved temporal lobe lesions. Of these 10 cases were in females, with an aee range of 25 to 57 years, average 40 years.
620
J. L A W T O N
Four cases were in males, with an age range of 30 to 53 years, average 36 years. It should be stressed that all but one of the 14 proved temporal lobe lesions was a tumor. Surgery and histologic study proved the etiology of the temporal lobe lesions in the 10 females, showing teratoma, astrocytoma, oligodendroglioma, astrocytoma, glioblastoma multiforme, glioma, cystic glioma, glioma, temporal lobectomy for seizures and metastatic breast carcinoma (biopsy of primary plus roentgen evidence). In the four males, the etiology was found by surgery and biopsy to be due to an astrocytoma in three patients ; one patient was found by arteriography to have a deep temporal lobe tumor stain. Vision was normal ( 2 0 / 3 0 or better in both eyes) in 13 of the proved cases ; it was not listed in one male. The optokinetic responses were of interest. All 10 of the female cases had negative signs. This was considered significant since the postoperative examination in four of these patients was at six months, five years, eight years, and 17 years, respectively. In the four males, two had negative optokinetic signs and one equivocal. One patient with a tumor, when first examined, had a negative optokinetic response which became positive six months later. Visual fields in the proved cases revealed: D e f e c t denser above ( M e y e r ' s loop field 11 defect) Full hemianopia 3 7 Incongruous 1 N e a r l y congruous 2 Perimeter better 2 Perimeter-screen 1 Screen better 3 Color perimetry helpful Splits fixation 3 Nil Spares fixation
B. Not proved temporal lobe cases. Ternporal lobe lesions were diagnosed on clinical grounds in 10 patients. Of these seven were in males (six white and one Negro) and three were in females (all white). The average age of the males was 37 years (range
SMITH
24 to 51 years) and of the females 36 years ( range 11 to 66 years). Etiology in the males was tumor in three, vascular two, trauma one, and tuberculoma one. The causative lesions in the females were tumor in two and birth injury in one. Vision was better than 2 0 / 4 0 in both eyes in all of the women, and in five of the seven men. Vision was not recorded in one man in whom it had fallen from 2 0 / 3 0 to hand movements during a two-year observation period due to a tuberculoma of the optic nerve. The optokinetic nystagmus signs were negative in two of the three women (not recorded in the other), and in five of the seven men. They were positive in two of the men. Visual field data in the not-proved temporal lobe cases revealed: D e f e c t denser above ( M e y e r ' s loop defects) 7 Full hemianopia 3 Perimeter better S Perimeter-screen 4 Screen examination only 1 Incongruous 7 Congruous Nil Color perimetry helpful 1
Summary of temporal lobe cases. There were 24 temporal lobe lesions, 14 of which were proved. Thirteen were in females and 11 in males. The average age of all of the temporal lobe cases was 38 years. The etiologic findings were striking in that a neoplasm was found in 18 patients, vascular lesions in only two, and other causes in four patients. Of the 14 patients with proved lesions, a tumor was found in all but one case, making the ratio of tumors to vascular lesions 9:1 in the patients presenting temporal lobe lesions with homonymous field defects. The optokinetic nystagmus sign was negative in 19, positive in three and not recorded in two. Vision was normal in 21 patients, not recorded in two and reduced because of a local eye lesion in one. Visual fields in the temporal lobe cases revealed that the typical Meyer's loop upper quadrantanopia was present in 18 cases, with a full hemianopia in only six. Thus, a
HOMONYMOUS
Meyer's loop field defect is seen 3:1 more frequently than a full hemianopia in temporal lobe disease. The defects were incongruous in 14 cases, and nearly congruous in only one, showing that temporal lobe lesions have a marked tendency toward incongruity. Of note is that the perimeter seemed to be more helpful in detecting the defect in seven patients, the screen in only two. This finding seemed to be corroborated by the observation that color peripheral fields with the Aimark perimeter were helpful in four cases, or one of six temporal lobe field defects. IV.
HEMIANOPIA
621
V i s u a l field e x a m i n a t i o n revealed that o n l y a temporal island remained in the left eye. T h e field w a s within normal limits in the right e y e t o 1/330 and 6 / 1 0 0 0 w h i t e but there w a s a complete temporal hemianopia in the right eye t o 3 / 3 3 0 red. T h e left optic disc w a s pale. T h e optokinetic responses w e r e symmetrical o n both sides. R o e n t g e n o g r a m s revealed a g r o s s l y enlarged sella turcica. T h e patient declined further studies and the clinical impression w a s either a pituitary tumor o r aneurysm. T h e h o m o n y m o u s aspect to his visual field defect corroborated involvement at the junction of optic tract and chiasm on the left. DISCUSSION
The material presented herein is summarized in Table 2.
TRACT A N D LATERAL GENICULATE
The diagnosis of lesions of the optic tract and lateral geniculate body by perimetric techniques is quite difficult and this difficulty seems to be reflected in the infrequency with which such lesions were found in this series. The one patient considered to have a lateral geniculate body lesion was a 59-year-old white woman (reported elsewhere*) who developed a field defect during a stereotactic procedure for unilateral parkinsonism. Visual field examination in this patient revealed a left lower quadrantanopia which was extremely incongruous. The optokinetic nystagmus sign was negative. Tract hemianopias were considered to be present when extreme incongruity, a negative optokinetic response, evidence of Behr's pupil and evidence of neurologic disease in neighboring structures were demonstrated. The number of tract lesions is considered too small for statistical interpretation. One patient is cited as an example of a tract defect: A 67-year-old N e g r o w a s seen in the Massachusetts E y e and E a r Infirmary in 1953, complaining of decreasing visual acuity in the left eye for o n e year. A t that time, a nasal field defect had been found in the left eye. F i v e years later, the patient returned and w a s found to h a v e vision of 2 0 / 2 0 in the right e y e and 1/200 in the left eye. * Smith, J. L., N a s h o l d , B. S., and Kreshon, M . J . : Ocular signs a f t e r stereotactic lesions in the pallidum and thalamus. Α Μ Α A r c h . Ophth., 65: 532 ( A p r . ) 1961.
SUMMARY
This review of 100 cases of homonymous visual field defects examined by me permits the following conclusions: Temporal lobe lesions produce one fourth of the cases of hemianopias. They usually occur in a younger age group and are about equally distributed between males and females. Such field defects have ominous significance since the most frequent etiologic lesion is a neoplasm; the tumor/vascular ratio was 9:1 in this series. These field defects are typically incongruous and are usually found more readily at the perimeter than the tangent screen. The 3/330 red isopter on the Aimark projection perimeter is at times quite helpful in their detection. These lesions are associated with a normal, symmetrical optokinetic response, unless parietal lobe extension has occurred. Parietal lobe lesions produce one third of the cases of hemianopias, and occur in an older age group. The incidence appears to be slightly greater in males than females, probably reflecting the greater number of vascular lesions in the male group. The ratio of tumors and vascular lesions is about equal. The optokinetic nystagmus sign is of great help in these cases ; there were 28 instances of a positive optokinetic nystagmus sign. Not a single case of parietal lobe hemianopia showed a normal optokinetic response. There is no question but that parietal lobe lesions
622
J. L A W T O N T A B L E
SMITH 2
HOMONYMOUS HEMIANOPIA
Temporal N o . Cases Males Females A g e range Average age Etiology Tumor Vascular Other Optokinetic R e s p o n s e Positive Negative Absent N o t recorded Equivocal Fields Denser a b o v e Denser below Incongruous Congruous Perimeter better Perimeter = screen Screen better Split fixation Spare fixation Color helpful Vision 2 0 / 4 0 O . U . or better Less E y e disease N o t recorded Dysphasia
SUMMARY
Parietal
Occipital
24
33
39
11
19
28
13
14
11
11-57
34-96
12-78
38
58
50
18
13
7
2
16
24
4
4
8
3
28
19
nil
7 31 1
2
7
1
6
3
14
2
1
1
nil
12
7
2
3
7
4
5
2
2
8
9
14
20
nil 1
21
5
nil
19
34
1
6
2
2
1
3 (bilateral occipital lesions)
can exist in the presence of a negative optokinetic response, but, if the lesion is deep enough to produce hemianopia, the optokinetic response is invariably asymmetric. This series confirms Kestenbaum's data regarding this finding. Parietal lobe field defects are detected with equal facility on the perimeter and tangent screen. Such defects are denser below 3:1 as often as they are denser above. They split fixation in each instance in this study, with no sparing noted with parietal lobe disease. Occipital lobe lesions are the most common cause of hemianopia, producing two fifths of the cases. They are more frequent in males than females (28:11 in this series). The average age was SO years, definitely older than in the temporal lobe group. The etiology
7
of an occipital lobe field defect is just the opposite of that of temporal lobe lesions for vascular lesions are more frequent than tumors by a striking degree, 3:1 in this study. The optokinetic sign is usually negative in the presence of an occipital lobe field defect. Cogan's rule was evaluated in this study. This states that, in the presence of an occipital lobe field defect, a negative optokinetic nystagmus sign points to a vascular lesion but a positive sign points to a tumor or mass lesion. There were seven positive optokinetic responses in this series of occipital lobe lesion. There were seven positive optokinetic and two arteriovenous malformations—in each instance, a mass lesion was present. Thus, Cogan's rule was found to be of great clinical value.
HOMONYMOUS
Occipital lobe field defects are found more readily on the tangent screen than the perimeter, due to the frequency of paracentral defects. They are congruous and, interestingly, are denser above 2:1 as often as below. They usually split fixation (3:1 over sparing). Posterior cerebral artery occlusions usually split fixation rather than spare it. A very important point concerning vision in the presence of hemianopia should be stressed. Homonymous hemianopia does not explain a reduction in visual acuity! Thus, a patient can see 2 0 / 2 0 with one half of
HEMIANOPIA
623
the macula. In every instance, without exception, in the entire series the visual acuity was within normal limits in each patient with hemianopia, unless cause for the reduction in acuity was readily evident on routine ophthalmologic examination. Division of Ophthalmology. T h e patients seen in this study w e r e e x a m i n e d at the Massachusetts E y e and E a r Infirmary, D u k e U n i v e r s i t y Medical Center and D u r h a m V e t e r a n s Administration Hospital. Grateful acknowledgment is g i v e n to Dr. D a v i d G. Cogan for his help and encouragement and permission to see some of these patients.
REFERENCE Kestenbaum, A . : Clinical M e t h o d s of N e u r o - O p h t h a l m o l o g i c E x a m i n a t i o n . N e w Y o r k , Grune & Stratton, 1961, ed. 2.
H O M O N Y M O U S
H E M I A N O P I A
A F T E R
O C C I P I T A L
L O B E C T O M Y *
ALFRED HUBER, M . D . Zurich,
The phenomenon of macular sparing in homonymous hemianopia has been the object of world-wide discussion for many years (Wilbrand, Heine, Lenz, Pfeifer, Foerster, Penfield, Putnam, Poliak, Rönne, v. Monakow, Adler Fliegelmann, Verhoeff, Halstead, Walker, Bucy, David, Hacean, Ajuriaguerra, Austin, Lewey, Grant, Dubois-Poulsen). It has assumed even more interest since neurosurgery has provided the opportunity to study visual field defects after removal of well-determined areas of the visual pathway in man. Professor Walsh, to whom this article is dedicated, devotes several pages of his wonderful book to this problem. Therefore, it was thought that he might be interested in hearing about some new findings on this subject. In co-operation with the Department of Neurosurgery of the University of Zurich * F r o m the U n i v e r s i t y E y e Clinic and the N e u rosurgery Clinic o f the U n i v e r s i t y of Zurich.
Switzerland
(Prof. Krayenbühl) I have had occasion to examine 11 cases of occipital lobectomies performed because of tumors of this area of the brain. In occipital lobectomy, very frequently combined with opening of the posterior horn of the lateral ventricle, the whole calcarine cortex and a considerable part of the optic radiation is removed—in other words, total removal of that part of the optic pathway which is considered to produce homonymous hemianopia with macular sparing. Our method of examination was to determine all visual functions, including visual acuity, color sense, motility and so forth. Perimetry was done by means of different apparatuses: the Bjerrum-screen, Maggiore perimeter, Amsler chart and especially the Goldmann perimeter. Different test objects of varying size, luminosity and color were used. Special care was given to the problem of the maintenance of good central fixation during perimetry. The fixation can be steadily controlled at the Goldmann perimeter