BILATERAL HOMONYMOUS HEMIANOPIA EARL W. NEPPLE, M.D., RICHARD E. APPEN, M.D., AND JOSEPH F. SACKETT, M.D. Madison, Wisconsin
Bilateral homonymous hemianopia is considered to be rare. 1-4 However, we have seen 15 patients with this visual impairment during the past four years. The visual defect results from separate lesions of the central nervous system that affect the postchiasmal visual pathways on both sides of the brain. We describe herein patients with unequivocal clinical or radiologic documentation of this con dition. SUBJECTS AND METHODS
All patients were referred to us for neuro-ophthalmic consultation. We paid particular attention to refraction and ex amination of pupils, ocular motility, fundi, and visual fields by kinetic Goldmann perimetry. Clinical examination was supplemented by computed axial tomog raphy in five patients, by cerebral angiography in five patients, by technetium brain scan in seven patients, and by au topsy in two patients. CASE REPORTS Case 1 (GROUP l)—h 73-year-old man received a cardiac pacemaker in 1968 following Stokes-Adams attacks. In 1970, he observed the abrupt onset of flashing lights in the vision of both eyes. Visual acuity then was 6/6 (20/20) in both eyes, and a left homonymous superior quadrantanopia was present. In 1972, the patient began complaining of vague difficulties in reading. In 1974, visual acuity re mained 6/6 (20/20) in both eyes. Examination was otherwise normal except for visual fields that showed a dense right homonymous upper quadrant defect and a less dense left homonymous superior quadrant defect (Fig. 1). The patient died six months From the Departments of Ophthalmology (Drs. Nepple and Appen) and Radiology (Dr. Sackett), University of Wisconsin Medical Center, Madison, Wisconsin. Reprint requests to Richard E. Appen, M.D., De partment of Ophthalmology, University of Wiscon sin Hospitals, 1300 University Ave., Madison, WI 53706. 536
later from histiocytic malignant lymphoma. At au topsy, the brain showed infarcts of the right temporo-occipital area and of the left occipital lobe (Fig. 2). There was marked arteriosclerosis of the aorta, the coronary arteries, and the cerebral arteries.
Comment—Arteriosclerosis with sec ondary cerebral infarcts was the cause of the bilateral homonymous hemianopia in this man and in five other patients (Cases 1-6) and was the most common diagnostic category. Although a sequential occur rence of the homonymous field defects was documented in this patient, the histo ry and setting indicated a simultaneous onset in most of our patients. Bilateral lesions were discovered simultaneously for the first time in 11 of our 15 patients. Basilar artery insufficiency was consid ered to be the cause of the bilateral ho monymous hemianopia in the two pa tients with ocular motility findings indicating brain stem dysfunction.5 Kearns8 described three patients with bi lateral homonymous hemianopia caused by basilar artery thrombosis and empha sized the association of brain stem signs with the bilateral homonymous hemiano pia. Case 7 (GROUP 2)—Three weeks after closed head trauma, a 48-year-old man became increasingly leth argic and developed dilated, unreactive pupils; blepharoptosis; and impaired adduction of the left eye. Arteriography showed a large left subdural hematoma, which was operated on shortly there after. Neuro-ophthalmic examination 18 days after the operation showed visual acuity of 6/6 (20/20) in both eyes with normal pupils and fundi. A small left hypertropia was a residual of the left third nerve paresis. Visual fields showed a right homonymous hemianopia combined with a left homonymous su perior quadrantanopia (Fig. 3). Computed tomogra phy documented bilateral occipital lobe infarctions (Fig. 4, left). The computed tomography one section more cephalad (Fig. 4, right) showed a normal superior portion of the right occipital lobe corre sponding to the left homonymous inferior quadrant of remaining vision.
Comment—This patient and two others (Cases 7-9) sustained bilateral homony mous hemianopia secondary to uncal her-
AMERICAN JOURNAL OF OPHTHALMOLOGY 86:536-543, 1978
Vol. 86, NO. 4
BILATERAL HOMONYMOUS HEMIANOPIA
537
1
Fig. 1 (Nepple, Appen, and Sackett). Case 1. Visual field. Dense right homonymous superior quadrant and less dense left homonymous superior quadrant defect.
niation following subdural hematomas, as described by Hoyt 7 and Klintworth. 8 Incapacitating field contraction sparing only four degrees occurred in one patient who maintained visual acuity of 6/6 (20/ 20) (Case 9), whereas a 12-year-old boy functioned relatively well with a residual homonymous quadrant of vision (Case 8). These infarcts result from increased intracranial pressure shifting the uncal portions of the temporal lobes downward at the tentorium, with subsequent com pression of the posterior cerebral arteries against the tentorial edge. Such herniation can be caused by any expanding supratentorial lesion regardless of loca tion. Occasionally, homonymous hemianopia may be a sign of tentorial herniation even without pupillary dilation from oculomotor nerve dysfunction. 7 Case 10 (Group 3)—A 27-year-old woman had had severe intermittent headaches associated with nausea, vomiting, and transient dimming of vision for ten years. Five years before our examination of the patient, a left homonymous superior quadrantanopia was documented after one of these episodes. At that time, bilateral carotid arteriography was nor mal. Nine months before the present hospitalization, she awoke with severe headache, slurred speech,
Fig. 2 (Nepple, Appen, and Sackett). Case 1. Infe rior surface of brain demonstrates the old infarct in the right occipitotemporal lobes and smaller infarct in the left occipital lobe.
538
AMERICAN JOURNAL OF OPHTHALMOLOGY
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•i
I 3;
t 1 1
\
v*-i7*^i
OCTOBER, 1978
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E
/ ^>*.
/
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Fig. 3 (Nepple, Appen, and Sackett). Case 7. Visual field. Right homonymous hemianopia combined with left homonymous superior quadrantanopia. and additional blurring of vision. On admission, visual acuity was 6/4.5 (20/15), and ocular motility, pupils, and fundi were normal. Visual fields showed a left superior homonymous quadrantanopia with a right homonymous superior quadrantanopia, simu lating an altitudinal visual field defect (Fig. 5). Cerebral angiography showed occlusion of both posterior cerebral arteries (Figs. 6, right, and 7, right). Three years later her visual acuity was un changed and she was otherwise well.
Comment—This woman, whose tenyear history of intermittent headache is typical of migraine, sustained permanent cerebral damage secondary to ischemia of migraine, as has been reported. 9 - 1 2 The other patient of our series with migraine (Case 11) also had bilateral cerebral infarcts, indicated by computed tomogra-
Fig.4 (Nepple, Appen, and Sackett). Case 7. Left, Computed tomography demonstrates bilateral occipital lobe radiodensity secondary to infarction. Right, computed tomography section 1 cm more cephalad than section in Figure 4, left, shows relatively normal right occipital lobe corresponding to the normal left homonymous inferior quadrant of vision.
Vol. 8 6 , N O . 4
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il;
HEMIANOPIA
539
1*4* 1 1 T
3
Fig. 5 (Nepple, Appen, and Sackett). Case 10. Visual field. Left homonymous superior quadrantanopia combined with a right homonymous superior quadrantanopia simulates an altitudinal visual field defect.
phy. H e also retained good visual acuity despite extensive loss of visual field (Fig. 8). Case 12 (Group 4)—A 54-year-old woman com plained of poor vision bilaterally since an episode of anoxia from cardiac arrest during elective herniorrhaphy. Best corrected visual acuity was R.E.: 6/400 (3/200); L.E.: 6/600 (2/200). Intraocular pressures, pupils, fundi, optokinetic nystagmus, and electroretinogram were all normal. This patient was one of only three in the series who sustained loss of central
vision (Fig. 9). The small vertical steps at the midline indicate the bilateral homonymous nature of the field defects.
Comment—The middle and posterior cerebral arteries perfuse adjacent regions of the brain. The boundary between the two areas is served by the distal branches of both vessels, which are under lowest pressure. This area is termed a watershed zone and may be infarcted when hypoten-
Fig. 6 (Nepple, Appen, and Sackett). Left, Normal lateral view of vertebral arteriography. Superior cerebellar artery (crossed arrow) serves as landmark indicating location of the tentorium. Arteries cephalad to that artery are supratentorial and are the parieto-occipital branches of the posterior cerebral arteries (arrow). Right, Case 10. Lateral view of vertebral anteriogram demonstrates nonfilling of the parietooccipital branches of the posterior cerebral arteries above the tentorium. Proximal segments of posterior cerebral arteries are indicated by arrows, and the superior cerebellar artery by crossed arrow.
540
AMERICAN JOURNAL OF OPHTHALMOLOGY
OCTOBER, 1978
Fig. 7 (Nepple, Appen, and Sackett). Left, Frontal view of normal vertebral arteriography. Posterior cere bral arteries with parieto-occipital branches (arrows) are lateral to the superior cerebellar arteries (crossed (arrow). Right, Case 10. Frontal projection of vertebral arteriography shows nonfilling of parieto-occipital branches of the posterior cerebral arteries (arrows) above the level of the superior cerebellar arteries (crossed arrow).
sion affects both arteries, but is spared when there is occlusion of only one of them. 3 ' 4,13 When cardiac arrest occurs, the p u m p failure causes greatest ischemia at the watershed zone and thereby accounts for the predominant loss of central visual field, as occurred in this patient. In con trast, embolic occlusion of a posterior cerebral artery spares the watershed zone because of its perfusion by the corre sponding middle cerebral artery.
This patient was one of the remaining four whose diseases were caused by diverse factors. The patients with bilateral homonymous hemianopia secondary to pro gressive multifocal leukoencephalopathy (Case 14) and cardiac surgery (Case 13) have been previously described. 14 ' 15 The other patient (Case 15) was a 45year-old woman with a history suggestive of migraine. Following discovery of her bilateral homonymous hemianopia,
Fig. 8 (Nepple, Appen, and Sackett). Case 11. Visual field. Almost total left homonymous hemianopia combined with right homonymous inferior field loss.
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it
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I
Fig. 9 (Nepple, Appen, and Sackett). Case 12. Visual field. Vertical steps indicate the bilateral homony mous character of the central visualfieldloss, slightly more extensive in the left homonymousfieldthan in the right.
diagnostic studies revealed a type 4 hyperlipidemia, which resolved after dis continuation of oral contraceptives. Her angiogram documented severe bilateral posterior cerebral artery disease. RESULTS
The group studied consisted of ten men and five women ranging in age from 12 to 73 years (Table). Results of external, pu pil, motility, and fundus examinations were normal in 11 patients. Visual acuity was generally equal in both eyes and was 6/7.5 (20/25) or better bilaterally in 11 of the 15 patients. One patient (Case 5) had visual acuity of R.E.: 6/6 (20/20), L.E.: 6/12 (20/40). The reason for the reduced visual acuity of the left eye remained uncertain. Three patients had visual acu ity of 6/60 (20/200) or less. Optokinetic nystagmus was normal in all ten patients tested. Four patients had abnormal neuro-ophthalmic findings other than the visual fields. Of two patients with ocular motility abnormalities, one man (Case 6) had internuclear ophthalmoplegia, skew
deviation, and nystagmus. The other (Case 11) had a right sixth nerve paresis, skew deviation, and a right Homer's syn drome. In one patient (Case 4) ophthalmoscopic examination revealed two cho lesterol crystals. A positive Marcus Gunn pupil sign that was difficult to explain was present in one patient (Case 5). In all patients the medical history was important in clarifying the cause of the bilateral homonymous hemianopia. For nine patients, a definitive neurologic di agnosis was established by techniques other than neuro-ophthalmic examina tion, including cerebral angiography (three patients); computed tomography (three patients); technetium Tc 99m brain scan (one patient); and autopsy (two pa tients). Another patient (Case 6) had a computed tomography scan that con firmed infarction of the right occipital lobe, but visual fields showed a definite right homonymous defect in addition to the total left homonymous hemianopia. Bilateral cortical occipital lesions were diagnosed clinically in the six remaining patients.
AMERICAN JOURNAL OF OPHTHALMOLOGY
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OCTOBER, 1978
TABLE B I L A T E R A L HOMONYMOUS HEMIANOPIA
Age (yrs)
Sex
1
73
M
2
64
M
Case No.
3
49
F
4
64
M
5
60
F
6
50
M
7
48
M
8
12
M
9
44
M
10
27
F
11
37
M
12
54
F
13
48
M
14
63
M
15
45
F
Visual Acuity*
Bilateral Homonymous Hemianopia Other At First Neuro-Ophthalmic Examination f Abnormalities
6/6 (20/20) 6/6 (20/20) 6/4.5 (20/15) 6/6 (20/20) R.E.:6/6 (20/20) L.E.:6/12 (20/40) 6/7.5 (20/25) 6/6 (20/20) 6/6 (20/20) 6/6 (20/20) 6/4.5 (20/15) 6/6 (20/20) 6/400 (3/200) 6/60 (20/200) 6/60 (20/200) 6/6 (20/20)
Positive Diagnostic Studies
Etiology
Autopsy
ASVDJ
CT$ Scan
ASVD
None
ASVD
None
ASVD
Angiography
ASVD
CT Scan
ASVD
+
CT Scan
Tentorial herniation
+
None
Tentorial herniation
None
Tentorial herniation
Angiography
Migraine
+
CT Scan
Migraine
None
Cardiac arrest
+
Brain scan
Cardiac surgery
+
Autopsy
PML||
+
Angiography
Idiopathicl
+
* Corrected and equal in both eyes except in Case 5. t + designates present; - , absent. X ASVD designates arteriosclerotic vascular disease. § CT designates computed tomography. PML designates progressive multifocal leukoencephalopathy. The patient was taking an oral anovulatory agent at the time of onset of bilateral homonymous hemianopia.
DISCUSSION
Forster 16 first described a patient with a well-documented partial bilateral homon ymous hemianopia in 1890. The 49-yearold patient sustained a right, followed by a left homonymous hemianopia with sparing of central vision. Recent ophthal mic papers in English have not empha sized this condition. Previous ophthalmic reports 6,17 and neurologic studies 3 , 4 have not presented documentation of the visu al field abnormalities by means of modern
techniques. T h e characteristics of the ophthalmologic examination that should suggest this diagnosis include (1) similar ly shaped visual field defects on corre sponding sides of the vertical midline for each eye; (2) equal visual acuity for each eye, which generally is normal (as in 75% [12 of 15] of our patients); (3) normal pupils and fundi; (4) ocular motility that is generally normal unless there are coex isting brain stem lesions. The cerebral lesions almost invariably result from cerebrovascular insufficiency,
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BILATERAL HOMONYMOUS HEMIANOPIA
most commonly caused by arteriosclero sis, tentorial hemiation from increased intracranial pressure, and migraine. The cerebral lesions are generally in the oc cipital lobes, and the posterior cerebral arteries are the vessels most commonly affected. Brain tumor did not occur in our series, and no bilateral homonymous hemianopia occurred in 849 patients with visual field defects and brain tumors studied by TOnnis as reported by Schaublin.4 Contrary to prior reports, the occur rence of this visual abnormality is not rare. The ophthalmologist must consider this diagnosis in the examination of any patient with bilateral visual deficits that are not accounted for by visible ocular abnormalities. SUMMARY
Bilateral homonymous hemianopia oc curred in 15 patients seen during a fouryear period. Persons with this visual defi cit had similarly shaped visual field defects on corresponding sides of the vertical midline for each eye, equal visual acuity which is generally normal, and normal pupil and fundus examina tions. The most common causes of the bi lateral posterior cerebral artery insuffi ciency were arteriosclerosis (40%), uncal hemiation (20%), and migraine (13%). REFERENCES 1. Frontera, A. T.: Bilateral homonymous hemi
543
anopia with preservation of central vision. Mt. Sinai J. Med. N. Y. 41:480, 1974. 2. Godtfredsen, E.: Bilateral homonymous hemi anopia. Acta Ophthalmol. (Suppl.) 125:20, 1975. 3. Symonds, C , and MacKenzie, I.: Bilateral loss of vision from cerebral infarction. Brain 80:415, 1957. 4. Schaublin, A.: Horizontal hemianopias. Confin. Neurol. 29:327, 1967. 5. Hollenhorst, R. W.: Carotid and vertebralbasilar arterial stenosis and occlusion. Neuroophthalmologic considerations. Trans. Am. Acad. Ophthalmol. Otolaryngol. 66:166, 1962. 6. Reams, T. P., Wagner, H. P., and Millikan, C. H.: Bilateral homonymous hemianopsia. Arch. Ophthalmol. 53:560, 1955. 7. Hoyt, W. F.: Vascular lesions of the visual cortex with brain hemiation through the tentorial incisura. Arch. Ophthalmol. 64:44, 1960. 8. Klintworth, G. K.: The neuro-ophthalmic man ifestations of transtentorial hemiation. In Smith, J. L. (ed.): Neuroophthalmology Symposium of the University of Miami and the Bascom Palmer Eye Institute. St. Louis, C. V. Mosby, 1972, pp. 113-131. 9. Connor, R.: Complicated migraine. Lancet 2: 1072, 1962. 10. Harrington, D. O.: The Visual Fields. St. Louis, C. V. Mosby, 1976, pp. 375-379. 11. Kaul, S. N., Duboulay, G. H., Kendall, E., and Russell, R. W.: Relationship between visual field defect and arterial occlusion in the posterior cerebral circulation. J. Neurol. Neurosurg. Psychiatr. 37:1022, 1974.. 12. Pierce, J.: The ophthalmological complica tions of migraine. J. Neurol. Sci. 6:73, 1968. 13. Hoyt, W. F., and Walsh, F. B.: Cortical blind ness with partial recovery following acute cerebral anoxia from cardiac arrest. Arch. Ophthalmol. 60: 1061, 1958. 14. Appen, R. E., Roth, H., ZuRhein, G. M., and Varakis, J. N.: Progressive multifocal leukoencephalopathy. Arch. Ophthalmol. 95:656, 1977. 15. Taugher, P. J.: Visual loss after cardiopulmonary bypass. Am. J. Ophthalmol. 81:280, 1976. 16. Forster, O.: Cber rindenblindheit. Arch. Oph thalmol. 36:94, 1890. 17. Reese, F. M.: Bilateral homonymous hemian opsia. Am. J. Ophthalmol. 38:44, 1954.
Bilateral homonymous hemianopia is considered to be rare. 1-4 However, we have seen 15 patients with this visual impairment during the past four years. The visual defect results from separate lesions of the central nervous system that affect the postchiasmal visual pathways on both sides of the brain. We describe herein patients with unequivocal clinical or radiologic documentation of this con dition. SUBJECTS AND METHODS
All patients were referred to us for neuro-ophthalmic consultation. We paid particular attention to refraction and ex amination of pupils, ocular motility, fundi, and visual fields by kinetic Goldmann perimetry. Clinical examination was supplemented by computed axial tomog raphy in five patients, by cerebral angiography in five patients, by technetium brain scan in seven patients, and by au topsy in two patients. CASE REPORTS Case 1 (GROUP l)—h 73-year-old man received a cardiac pacemaker in 1968 following Stokes-Adams attacks. In 1970, he observed the abrupt onset of flashing lights in the vision of both eyes. Visual acuity then was 6/6 (20/20) in both eyes, and a left homonymous superior quadrantanopia was present. In 1972, the patient began complaining of vague difficulties in reading. In 1974, visual acuity re mained 6/6 (20/20) in both eyes. Examination was otherwise normal except for visual fields that showed a dense right homonymous upper quadrant defect and a less dense left homonymous superior quadrant defect (Fig. 1). The patient died six months From the Departments of Ophthalmology (Drs. Nepple and Appen) and Radiology (Dr. Sackett), University of Wisconsin Medical Center, Madison, Wisconsin. Reprint requests to Richard E. Appen, M.D., De partment of Ophthalmology, University of Wiscon sin Hospitals, 1300 University Ave., Madison, WI 53706. 536
later from histiocytic malignant lymphoma. At au topsy, the brain showed infarcts of the right temporo-occipital area and of the left occipital lobe (Fig. 2). There was marked arteriosclerosis of the aorta, the coronary arteries, and the cerebral arteries.
Comment—Arteriosclerosis with sec ondary cerebral infarcts was the cause of the bilateral homonymous hemianopia in this man and in five other patients (Cases 1-6) and was the most common diagnostic category. Although a sequential occur rence of the homonymous field defects was documented in this patient, the histo ry and setting indicated a simultaneous onset in most of our patients. Bilateral lesions were discovered simultaneously for the first time in 11 of our 15 patients. Basilar artery insufficiency was consid ered to be the cause of the bilateral ho monymous hemianopia in the two pa tients with ocular motility findings indicating brain stem dysfunction.5 Kearns8 described three patients with bi lateral homonymous hemianopia caused by basilar artery thrombosis and empha sized the association of brain stem signs with the bilateral homonymous hemiano pia. Case 7 (GROUP 2)—Three weeks after closed head trauma, a 48-year-old man became increasingly leth argic and developed dilated, unreactive pupils; blepharoptosis; and impaired adduction of the left eye. Arteriography showed a large left subdural hematoma, which was operated on shortly there after. Neuro-ophthalmic examination 18 days after the operation showed visual acuity of 6/6 (20/20) in both eyes with normal pupils and fundi. A small left hypertropia was a residual of the left third nerve paresis. Visual fields showed a right homonymous hemianopia combined with a left homonymous su perior quadrantanopia (Fig. 3). Computed tomogra phy documented bilateral occipital lobe infarctions (Fig. 4, left). The computed tomography one section more cephalad (Fig. 4, right) showed a normal superior portion of the right occipital lobe corre sponding to the left homonymous inferior quadrant of remaining vision.
Comment—This patient and two others (Cases 7-9) sustained bilateral homony mous hemianopia secondary to uncal her-
AMERICAN JOURNAL OF OPHTHALMOLOGY 86:536-543, 1978
Vol. 86, NO. 4
BILATERAL HOMONYMOUS HEMIANOPIA
537
1
Fig. 1 (Nepple, Appen, and Sackett). Case 1. Visual field. Dense right homonymous superior quadrant and less dense left homonymous superior quadrant defect.
niation following subdural hematomas, as described by Hoyt 7 and Klintworth. 8 Incapacitating field contraction sparing only four degrees occurred in one patient who maintained visual acuity of 6/6 (20/ 20) (Case 9), whereas a 12-year-old boy functioned relatively well with a residual homonymous quadrant of vision (Case 8). These infarcts result from increased intracranial pressure shifting the uncal portions of the temporal lobes downward at the tentorium, with subsequent com pression of the posterior cerebral arteries against the tentorial edge. Such herniation can be caused by any expanding supratentorial lesion regardless of loca tion. Occasionally, homonymous hemianopia may be a sign of tentorial herniation even without pupillary dilation from oculomotor nerve dysfunction. 7 Case 10 (Group 3)—A 27-year-old woman had had severe intermittent headaches associated with nausea, vomiting, and transient dimming of vision for ten years. Five years before our examination of the patient, a left homonymous superior quadrantanopia was documented after one of these episodes. At that time, bilateral carotid arteriography was nor mal. Nine months before the present hospitalization, she awoke with severe headache, slurred speech,
Fig. 2 (Nepple, Appen, and Sackett). Case 1. Infe rior surface of brain demonstrates the old infarct in the right occipitotemporal lobes and smaller infarct in the left occipital lobe.
538
AMERICAN JOURNAL OF OPHTHALMOLOGY
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•i
I 3;
t 1 1
\
v*-i7*^i
OCTOBER, 1978
i^^^^^^^^^m
E
/ ^>*.
/
\ '1° /
\
^^ \
Fig. 3 (Nepple, Appen, and Sackett). Case 7. Visual field. Right homonymous hemianopia combined with left homonymous superior quadrantanopia. and additional blurring of vision. On admission, visual acuity was 6/4.5 (20/15), and ocular motility, pupils, and fundi were normal. Visual fields showed a left superior homonymous quadrantanopia with a right homonymous superior quadrantanopia, simu lating an altitudinal visual field defect (Fig. 5). Cerebral angiography showed occlusion of both posterior cerebral arteries (Figs. 6, right, and 7, right). Three years later her visual acuity was un changed and she was otherwise well.
Comment—This woman, whose tenyear history of intermittent headache is typical of migraine, sustained permanent cerebral damage secondary to ischemia of migraine, as has been reported. 9 - 1 2 The other patient of our series with migraine (Case 11) also had bilateral cerebral infarcts, indicated by computed tomogra-
Fig.4 (Nepple, Appen, and Sackett). Case 7. Left, Computed tomography demonstrates bilateral occipital lobe radiodensity secondary to infarction. Right, computed tomography section 1 cm more cephalad than section in Figure 4, left, shows relatively normal right occipital lobe corresponding to the normal left homonymous inferior quadrant of vision.
Vol. 8 6 , N O . 4
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il;
HEMIANOPIA
539
1*4* 1 1 T
3
Fig. 5 (Nepple, Appen, and Sackett). Case 10. Visual field. Left homonymous superior quadrantanopia combined with a right homonymous superior quadrantanopia simulates an altitudinal visual field defect.
phy. H e also retained good visual acuity despite extensive loss of visual field (Fig. 8). Case 12 (Group 4)—A 54-year-old woman com plained of poor vision bilaterally since an episode of anoxia from cardiac arrest during elective herniorrhaphy. Best corrected visual acuity was R.E.: 6/400 (3/200); L.E.: 6/600 (2/200). Intraocular pressures, pupils, fundi, optokinetic nystagmus, and electroretinogram were all normal. This patient was one of only three in the series who sustained loss of central
vision (Fig. 9). The small vertical steps at the midline indicate the bilateral homonymous nature of the field defects.
Comment—The middle and posterior cerebral arteries perfuse adjacent regions of the brain. The boundary between the two areas is served by the distal branches of both vessels, which are under lowest pressure. This area is termed a watershed zone and may be infarcted when hypoten-
Fig. 6 (Nepple, Appen, and Sackett). Left, Normal lateral view of vertebral arteriography. Superior cerebellar artery (crossed arrow) serves as landmark indicating location of the tentorium. Arteries cephalad to that artery are supratentorial and are the parieto-occipital branches of the posterior cerebral arteries (arrow). Right, Case 10. Lateral view of vertebral anteriogram demonstrates nonfilling of the parietooccipital branches of the posterior cerebral arteries above the tentorium. Proximal segments of posterior cerebral arteries are indicated by arrows, and the superior cerebellar artery by crossed arrow.
540
AMERICAN JOURNAL OF OPHTHALMOLOGY
OCTOBER, 1978
Fig. 7 (Nepple, Appen, and Sackett). Left, Frontal view of normal vertebral arteriography. Posterior cere bral arteries with parieto-occipital branches (arrows) are lateral to the superior cerebellar arteries (crossed (arrow). Right, Case 10. Frontal projection of vertebral arteriography shows nonfilling of parieto-occipital branches of the posterior cerebral arteries (arrows) above the level of the superior cerebellar arteries (crossed arrow).
sion affects both arteries, but is spared when there is occlusion of only one of them. 3 ' 4,13 When cardiac arrest occurs, the p u m p failure causes greatest ischemia at the watershed zone and thereby accounts for the predominant loss of central visual field, as occurred in this patient. In con trast, embolic occlusion of a posterior cerebral artery spares the watershed zone because of its perfusion by the corre sponding middle cerebral artery.
This patient was one of the remaining four whose diseases were caused by diverse factors. The patients with bilateral homonymous hemianopia secondary to pro gressive multifocal leukoencephalopathy (Case 14) and cardiac surgery (Case 13) have been previously described. 14 ' 15 The other patient (Case 15) was a 45year-old woman with a history suggestive of migraine. Following discovery of her bilateral homonymous hemianopia,
Fig. 8 (Nepple, Appen, and Sackett). Case 11. Visual field. Almost total left homonymous hemianopia combined with right homonymous inferior field loss.
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BILATERAL HOMONYMOUS HEMIANOPIA
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I
Fig. 9 (Nepple, Appen, and Sackett). Case 12. Visual field. Vertical steps indicate the bilateral homony mous character of the central visualfieldloss, slightly more extensive in the left homonymousfieldthan in the right.
diagnostic studies revealed a type 4 hyperlipidemia, which resolved after dis continuation of oral contraceptives. Her angiogram documented severe bilateral posterior cerebral artery disease. RESULTS
The group studied consisted of ten men and five women ranging in age from 12 to 73 years (Table). Results of external, pu pil, motility, and fundus examinations were normal in 11 patients. Visual acuity was generally equal in both eyes and was 6/7.5 (20/25) or better bilaterally in 11 of the 15 patients. One patient (Case 5) had visual acuity of R.E.: 6/6 (20/20), L.E.: 6/12 (20/40). The reason for the reduced visual acuity of the left eye remained uncertain. Three patients had visual acu ity of 6/60 (20/200) or less. Optokinetic nystagmus was normal in all ten patients tested. Four patients had abnormal neuro-ophthalmic findings other than the visual fields. Of two patients with ocular motility abnormalities, one man (Case 6) had internuclear ophthalmoplegia, skew
deviation, and nystagmus. The other (Case 11) had a right sixth nerve paresis, skew deviation, and a right Homer's syn drome. In one patient (Case 4) ophthalmoscopic examination revealed two cho lesterol crystals. A positive Marcus Gunn pupil sign that was difficult to explain was present in one patient (Case 5). In all patients the medical history was important in clarifying the cause of the bilateral homonymous hemianopia. For nine patients, a definitive neurologic di agnosis was established by techniques other than neuro-ophthalmic examina tion, including cerebral angiography (three patients); computed tomography (three patients); technetium Tc 99m brain scan (one patient); and autopsy (two pa tients). Another patient (Case 6) had a computed tomography scan that con firmed infarction of the right occipital lobe, but visual fields showed a definite right homonymous defect in addition to the total left homonymous hemianopia. Bilateral cortical occipital lesions were diagnosed clinically in the six remaining patients.
AMERICAN JOURNAL OF OPHTHALMOLOGY
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OCTOBER, 1978
TABLE B I L A T E R A L HOMONYMOUS HEMIANOPIA
Age (yrs)
Sex
1
73
M
2
64
M
Case No.
3
49
F
4
64
M
5
60
F
6
50
M
7
48
M
8
12
M
9
44
M
10
27
F
11
37
M
12
54
F
13
48
M
14
63
M
15
45
F
Visual Acuity*
Bilateral Homonymous Hemianopia Other At First Neuro-Ophthalmic Examination f Abnormalities
6/6 (20/20) 6/6 (20/20) 6/4.5 (20/15) 6/6 (20/20) R.E.:6/6 (20/20) L.E.:6/12 (20/40) 6/7.5 (20/25) 6/6 (20/20) 6/6 (20/20) 6/6 (20/20) 6/4.5 (20/15) 6/6 (20/20) 6/400 (3/200) 6/60 (20/200) 6/60 (20/200) 6/6 (20/20)
Positive Diagnostic Studies
Etiology
Autopsy
ASVDJ
CT$ Scan
ASVD
None
ASVD
None
ASVD
Angiography
ASVD
CT Scan
ASVD
+
CT Scan
Tentorial herniation
+
None
Tentorial herniation
None
Tentorial herniation
Angiography
Migraine
+
CT Scan
Migraine
None
Cardiac arrest
+
Brain scan
Cardiac surgery
+
Autopsy
PML||
+
Angiography
Idiopathicl
+
* Corrected and equal in both eyes except in Case 5. t + designates present; - , absent. X ASVD designates arteriosclerotic vascular disease. § CT designates computed tomography. PML designates progressive multifocal leukoencephalopathy. The patient was taking an oral anovulatory agent at the time of onset of bilateral homonymous hemianopia.
DISCUSSION
Forster 16 first described a patient with a well-documented partial bilateral homon ymous hemianopia in 1890. The 49-yearold patient sustained a right, followed by a left homonymous hemianopia with sparing of central vision. Recent ophthal mic papers in English have not empha sized this condition. Previous ophthalmic reports 6,17 and neurologic studies 3 , 4 have not presented documentation of the visu al field abnormalities by means of modern
techniques. T h e characteristics of the ophthalmologic examination that should suggest this diagnosis include (1) similar ly shaped visual field defects on corre sponding sides of the vertical midline for each eye; (2) equal visual acuity for each eye, which generally is normal (as in 75% [12 of 15] of our patients); (3) normal pupils and fundi; (4) ocular motility that is generally normal unless there are coex isting brain stem lesions. The cerebral lesions almost invariably result from cerebrovascular insufficiency,
Vol. 86, NO. 4
BILATERAL HOMONYMOUS HEMIANOPIA
most commonly caused by arteriosclero sis, tentorial hemiation from increased intracranial pressure, and migraine. The cerebral lesions are generally in the oc cipital lobes, and the posterior cerebral arteries are the vessels most commonly affected. Brain tumor did not occur in our series, and no bilateral homonymous hemianopia occurred in 849 patients with visual field defects and brain tumors studied by TOnnis as reported by Schaublin.4 Contrary to prior reports, the occur rence of this visual abnormality is not rare. The ophthalmologist must consider this diagnosis in the examination of any patient with bilateral visual deficits that are not accounted for by visible ocular abnormalities. SUMMARY
Bilateral homonymous hemianopia oc curred in 15 patients seen during a fouryear period. Persons with this visual defi cit had similarly shaped visual field defects on corresponding sides of the vertical midline for each eye, equal visual acuity which is generally normal, and normal pupil and fundus examina tions. The most common causes of the bi lateral posterior cerebral artery insuffi ciency were arteriosclerosis (40%), uncal hemiation (20%), and migraine (13%). REFERENCES 1. Frontera, A. T.: Bilateral homonymous hemi
543
anopia with preservation of central vision. Mt. Sinai J. Med. N. Y. 41:480, 1974. 2. Godtfredsen, E.: Bilateral homonymous hemi anopia. Acta Ophthalmol. (Suppl.) 125:20, 1975. 3. Symonds, C , and MacKenzie, I.: Bilateral loss of vision from cerebral infarction. Brain 80:415, 1957. 4. Schaublin, A.: Horizontal hemianopias. Confin. Neurol. 29:327, 1967. 5. Hollenhorst, R. W.: Carotid and vertebralbasilar arterial stenosis and occlusion. Neuroophthalmologic considerations. Trans. Am. Acad. Ophthalmol. Otolaryngol. 66:166, 1962. 6. Reams, T. P., Wagner, H. P., and Millikan, C. H.: Bilateral homonymous hemianopsia. Arch. Ophthalmol. 53:560, 1955. 7. Hoyt, W. F.: Vascular lesions of the visual cortex with brain hemiation through the tentorial incisura. Arch. Ophthalmol. 64:44, 1960. 8. Klintworth, G. K.: The neuro-ophthalmic man ifestations of transtentorial hemiation. In Smith, J. L. (ed.): Neuroophthalmology Symposium of the University of Miami and the Bascom Palmer Eye Institute. St. Louis, C. V. Mosby, 1972, pp. 113-131. 9. Connor, R.: Complicated migraine. Lancet 2: 1072, 1962. 10. Harrington, D. O.: The Visual Fields. St. Louis, C. V. Mosby, 1976, pp. 375-379. 11. Kaul, S. N., Duboulay, G. H., Kendall, E., and Russell, R. W.: Relationship between visual field defect and arterial occlusion in the posterior cerebral circulation. J. Neurol. Neurosurg. Psychiatr. 37:1022, 1974.. 12. Pierce, J.: The ophthalmological complica tions of migraine. J. Neurol. Sci. 6:73, 1968. 13. Hoyt, W. F., and Walsh, F. B.: Cortical blind ness with partial recovery following acute cerebral anoxia from cardiac arrest. Arch. Ophthalmol. 60: 1061, 1958. 14. Appen, R. E., Roth, H., ZuRhein, G. M., and Varakis, J. N.: Progressive multifocal leukoencephalopathy. Arch. Ophthalmol. 95:656, 1977. 15. Taugher, P. J.: Visual loss after cardiopulmonary bypass. Am. J. Ophthalmol. 81:280, 1976. 16. Forster, O.: Cber rindenblindheit. Arch. Oph thalmol. 36:94, 1890. 17. Reese, F. M.: Bilateral homonymous hemian opsia. Am. J. Ophthalmol. 38:44, 1954.
