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TOPICAL DIAGNOSIS OF CHIASMAL AND RETROCHIASMAL DISORDERS and acalculia) in association with a right homonymous hemianopia (289,290). Lesions in the nondominant parietal lobe may cause impaired constructional ability, dyscalculia, and, most commonly, inattention or neglect (291,292). Indeed, left spatial neglect after right hemisphere lesions may accentuate the left hemianopia, hemianesthesia, and hemiplegia and contribute to poor recovery (293). LESIONS OF THE OCCIPITAL LOBE AND VISUAL CORTEX In many cases the most posterior visual radiation and visual cortex are affected together. Most lesions in the occipital lobe are vascular or traumatic in origin, with tumor, abscess, demyelination, and toxic disorders of white matter occurring less frequently. Cole, a neurologist, has written a first-person account of his experience as a patient with an occipital stroke, which includes some of the symptomatology described below (294). Because of the close anatomic relationship of fibers from corresponding portions of the two retinas, lesions of the occipital lobe cause defects that are not only overwhelmingly homonymous but also increasingly congruous the more posteriorly situated the lesion (295–299). Automated static perimetry may inaccurately portray homonymous visual fields as incongruous, compared to Goldmann or tangent screen perimetry (300). Unilateral Lesions of the Posterior Occipital Lobe Defects seen with these lesions are exclusively homonymous. With lesions of the tip of the occipital lobe (occipital pole), the field defects are always central homonymous scotomas that are exquisitely congruous (257,296,298,299) (Fig. 12.33). After a detailed examination of three patients with homonymous visual field defects produced by well- 539 circumscribed lesions in the occipital lobe, Horton and Hoyt (298) estimated that the central 10⬚ of the visual field are represented by at least 50–60% of the posterior striate cortex and that the central 30⬚ is represented by about 80% of the cortex (Figs. 12.34 and 12.35). These findings were corroborated by McFadzean et al. (299). Instead of using lesions for mapping, it is possible to use functional MR imaging to detect those areas of the cortex activated by visual stimuli, and thus to produce a retinotopic map noninvasively (301). Wong and Sharpe used functional MR imaging to map the retinal representation on the occipital lobe and found that the central 15% of vision occupied only 37% of the cortex (302). Probably the most accurate method for determining cortical magnification (i.e., the degree by which areas of central retina are processed by magnified areas of visual cortex) is the use of angioscotoma mapping (303,304). Adams and Horton (305) used this mapping technique in squirrel monkeys and found that the central 16⬚ of visual field is represented by approximately 70% of cortex. Similar cortical magnification of the central visual field is seen in the extrastriate areas V2 and V3 (306). The detailed anatomy of the visual cortex is discussed in Chapter 1. Lesions located more anteriorly may produce primarily central field defects that break out into the periphery (Fig. 12.36). Impressive congruity is a feature of such field defects (257,307,308). The phenomenon of sparing of the macula is often seen in such cases (see below). An unusual visual field defect is homonymous hemianopia associated with sparing of the horizontal or vertical meridian. This results from sparing from injury of the corresponding cortical representation of the meridian, with the vertical meridian represented at the lips of the calcarine fissure and the horizontal meridian represented at the base of the fissure (309,310). Figure 12.33. Exquisitely congruous homonymous hemianopic scotomas in a patient with an infarct of the inferior lip of the left calcarine cortex. Note the few degrees of macular sparing. 540 CLINICAL NEURO-OPHTHALMOLOGY Figure 12.34. A 30-year-old woman reported several episodes of flashing colored lights in her right upper quadrant of vision. A, Merged 30-2 and 60-2 full-threshold visual field tests using a Humphrey Field Analyzer. A homonymous, congruous scotoma was present in the right upper quadrant. In the inset, the visual fields are mapped at the tangent screen. The scotoma extended from 6⬚ to 18⬚. B, Parasagittal magnetic resonance image of the left occipital lobe. The lesion (cross-hatched area on the right) is within the visual cortex (stippled area on the right). The calcarine sulcus (solid arrow) and the parieto-occipital sulcus (open arrow) are marked by dots. On biopsy, the lesion was a presumed tuberculoma. (From Horton JC, Hoyt WF. The representation of the visual field in human striate cortex. Arch Ophthalmol 1991;109⬊816–824.) Both Allen and Carman (311) and Barkan and Boyle (312) reported a syndrome in which an otherwise healthy patient develops the sudden onset of an isolated homonymous paracentral scotoma associated with normal visual acuity and no ophthalmoscopic abnormalities. Clinically, such a lesion must occur as the result of a vascular event in the posterior occipital cortex; however, in both cases, the homonymous scotomas were incongruous. The explanation for this apparent clinical–anatomic discrepancy is unclear. Unilateral Lesions of the Anterior Occipital Lobe Because the temporal field in each eye is larger than the nasal field, the fibers subserving that portion of the peripheral temporal field that has no nasal correlate must be un- paired throughout the postchiasmal portion of the visual sensory pathway. Damage to these unpaired peripheral fibers produces a monocular defect in the extreme temporal visual field. This field defect is crescentic and its widest extent is in the horizontal meridian, where it extends from 60⬚ out to approximately 90⬚. Because of its peculiar shape, a defect in this region has been termed a temporal crescent or halfmoon syndrome (Figs. 12.35C, 12.37, and 12.38). Brouwer and Zeeman (313) demonstrated that after destruction of the peripheral nasal retina there was degeneration of a bundle of fibers localized in the median portion of the optic nerve, which continued into the optic chiasm to the median portion of the optic tract and spread ventrally into the lateral geniculate body. Wilbrand (6) confirmed that this bundle contained TOPICAL DIAGNOSIS OF CHIASMAL AND RETROCHIASMAL DISORDERS 541 Figure 12.35. Humphrey visual fields (30-2 program) (A) in a 40-year-old man who suffered a right occipital infarction (B) involving the superior bank of the calcarine cortex (arrowhead). The infarction spared the posterior striate cortex as well as the anterior striate cortex at the junction of the parieto-occipital and calcarine fissures (arrow). C, The visual fields showed sparing of central fixation within 6⬚ as well as sparing of the left monocular temporal crescent. (From McFadzean R, Brosnahan D, Hadley D, et al. Representation of the visual field in the occipital striate cortex. Br J Ophthalmol 1994;78⬊185–190.) only crossed fibers. In 1929, Förster (quoted by Walsh [314]) produced the sensation of light in the extreme temporal periphery of one eye by electrically stimulating the most anterior portion of the striate cortex. Polyak (315) confirmed these findings and postulated that the most anterior portion of the striate cortex harbored the projected monocular temporal crescent of the contralateral eye. Ask-Upmark (316) and Kronfeld (317) performed further studies in patients in whom the temporal crescent was selectively spared or damaged. They agreed that lesions of the posterior striate cortex tended to spare the temporal crescent, whereas lesions of the anterior striate cortex could selectively produce a defect in it or eliminate it entirely. Spence and Fulton (318) extirpated the entire left occipital lobe in a chimpanzee and later resected part of the right occipital lobe. They subsequently observed that the animal had vision only in the temporal periphery of the left eye. Postmortem examination of the brain disclosed that only the anterior portion of the right striate cortex was intact, confirming the conclusions reached by Polyak (315), Ask-Upmark (316), and Kronfeld (317). According to Bender and Strauss (208), the unpaired nasal fibers corresponding to the extreme temporal peripheral visual field come together in the most anterior portion of the striate cortex, with the fibers for the inferior portion of the 542 CLINICAL NEURO-OPHTHALMOLOGY Figure 12.36. Inferior homonymous hemianopic scotomas that break out to the periphery in a patient with an infarct of the left superior occipital cortex. Figure 12.37. Visual field defects in a 22-year-old woman who suffered bilateral occipital lobe infarctions. There is a complete left homonymous hemianopia and an incomplete right inferior homonymous quadrantanopia. The temporal crescent is absent from the right visual field. TOPICAL DIAGNOSIS OF CHIASMAL AND RETROCHIASMAL DISORDERS 543 Figure 12.38. Visual field defects in a 56-year-old woman with a right occipital lobe infarction. There is a left homonymous hemianopia with sparing of the inferior portion of the left temporal crescent. crescent terminating above the calcarine fissure and the fibers for the superior portion of the crescent terminating below the fissure. Bender and Strauss (208) found that in 100 patients with verified cerebral tumor affecting the posterior optic radiation, there was a unilateral temporal crescentic defect in the visual field of 10 patients. The recognition of such a defect has value in the early diagnosis of a lesion of the posterior optic radiation or anterior visual cortex. Theoretically, a lesion anywhere in the postchiasmal visual pathway, from the optic tract to the occipital lobe (and particularly the dorsal LGB), could produce a monocular field defect in the temporal periphery (244); however, in practice, only lesions in the posterior optic radiation or anterior visual cortex produce such selective damage. Both upper and lower temporal crescents may be scotomatous in the field of one eye, or only the upper or lower temporal crescent may be affected. Frequently, such a field defect is followed later by the development of an homonymous hemianopia (316). Bender and Strauss (208) fairly often found an unpaired crescentic relative scotoma medial to the peripheral absolute scotoma, indicating to them that the optic radiation has a lamellar arrangement. Just as the temporal crescent may be affected by lesions destroying the most anterior occipital lobe, it may likewise be spared when the remainder of the temporal visual field is affected in an homonymous hemianopia (284,314,319,320) (Figs. 12.35 and 12.38). Such sparing seems to occur exclusively in patients with occipital lobe lesions that spare the anterior aspect of the visual cortex (208). Two important facts should be kept in mind when considering the temporal crescent syndrome. First, monocular peripheral temporal visual field defects are probably caused most often by retinal lesions, not by intracranial ones. Thus, the nasal retinal periphery should be carefully examined ophthalmoscopically in such cases. Second, because these defects begin approximately 60⬚ from fixation, central field testing (i.e., that performed using a tangent screen or most automated static perimetry programs) gives normal results and will not detect such defects (321,322) (Fig. 12.39). Bilateral Effects of Unilateral Occipital Lobe Lesions Although patients with unilateral lesions of the occipital lobe typically have visual deficits totally restricted to the contralateral field, area V1 and adjacent extrastriate areas often function or fail in concert. Pathologic lesions of the occipital lobe thus may disrupt not only area V1 but also adjacent underlying white matter and extrastriate cortex. Such damage may alter interhemispheric connections along their presplenial course and even disturb visual cortical–subcortical connections. Nonetheless, there is excellent evidence that there are regions of cortex ipsilateral to the field defect that process visual information (323,324). Rarely, cortical plasticity in the setting of congenital cerebral lesions can lead to developmental reorganization of the visual processing areas so that the visual field is represented ipsilaterally (325). There is also indirect evidence for reorganization of projections to extrastriate visual processing areas after injury to V1 (326). For example, in a study by Bender and Teuber (327,328), dark adaptation was delayed in the apparently normal field. Rizzo and Robin (329) studied the vision of 12 patients with unilateral lesions of the visual cortex. All patients had homonymous defects in the contralateral visual fields, as expected, and all had visual acuity of 20/30 or better in both eyes. The first experiment performed by Rizzo and Robin (329) tested the subjects’ ability to respond to transient sig- 544 CLINICAL NEURO-OPHTHALMOLOGY Figure 12.39. Migrainous loss of the temporal crescent. A, A 31-year-old woman with a history of migraine without aura had the sudden onset of a bright light in the temporal field of vision of the right eye, followed by loss of vision in this same region, and subsequent headache. The field deficit persisted for a few days and then resolved. B, Visual fields performed 5 days later were full in both eyes. Magnetic resonance imaging performed at the time of the initial deficit was completely normal. nals presented at unpredictable temporal intervals and spatial locations among many spatially random and identical distracter elements. The results showed that compared with controls, the lesion group had a significantly reduced sensitivity to signal and increased response times in both hemifields. In a second experiment, Rizzo and Robin (329) tested the useful field of view in two of the patients under conditions of differing attention demand. Both patients showed bilateral constriction, consistent with the results of the first experiment. Rizzo and Robin suggested that one possible explanation for the bilateral effects of unilateral occipital lobe lesions is damage to interhemispheric connections along their presplenial course, affecting the synthesis of visual information from both hemifields. The disturbances are task-dependent and seem to result from a global reduction in visual attention capacity. Subsequent experiments by Battelli et al. (329a) showed ipsilateral effects of right parietal lobe lesions on motion detection and suggested that some of these effects could be mediated by specific alterations in attention circuits. Although these disturbances are subtle compared with the homonymous visual field defects that occur in patients with unilateral occipital lobe lesions, they may be responsible for various unexplained complaints of reduced performance in tasks with high visual information-processing demands such as reading and driving. Macular Sparing When a portion of the central field of each eye in homonymous hemianopia is preserved as a result of deviation of the vertical meridian between the functioning and nonfunction- TOPICAL DIAGNOSIS OF CHIASMAL AND RETROCHIASMAL DISORDERS ing halves of the visual fields, there is said to be ‘‘sparing of the macula’’ or ‘‘macular sparing.’’ In a majority of such cases, visual acuity is normal, with the zone of preserved visual field ranging from 1⬚ or 2⬚ in width to almost 10⬚ (Fig. 12.40). Another type of field in which there is sparing is the ‘‘overshot’’ field (284). In such a visual field, the sparing is not central alone but extends along the entire vertical meridian. A complete discussion of this phenomenon appears in the paper by Safran et al. (330). Deviation of the vertical meridian accounting for macular sparing is a much-debated phenomenon. Although it is usually seen in patients with retrogeniculate (usually occipital) lesions, the absence of sparing does not imply that the lesion is pregeniculate or noncortical. The etiology of macular spar- 545 ing is controversial. Some authors consider it an artifact of testing. Others believe it is a real phenomenon related to the representation of a small portion of each macula in each occipital lobe. Finally, some authors believe that macular sparing results from incomplete damage of the visual pathways or occipital lobe. This section addresses each of these theories in turn. What has been represented as wide sparing may often be shown in reality to be much narrower when ocular fixation is carefully controlled. Hence, imprecisely charted fields tend to show a wider sparing than actually exists. In many cases of homonymous hemianopia with sparing, it is possible to prove a shift in fixation by charting the blind spot in the single field in which it can be performed. Another method Figure 12.40. A 28-year-old woman suffered a persistent visual field defect after an unusually severe migraine attack. A, Full-threshold 60⬚ visual fields using a Humphrey Field Analyzer show the central 15⬚ of the left hemifield is intact. In the inset, the visual fields mapped at the tangent screen show that the field defect bisects the blind spot representation of the left eye. B, T1-weighted parasagittal magnetic resonance image through the right occipital lobe shows an arteriovenous malformation involving the anterior portion of the right calcarine cortex. The posterior margin of the lesion is situated 31 mm from the occipital tip, marking the approximate location of the representation of the left eye’s blind spot. The calcarine (curved arrow) and parieto-occipital (straight arrow) sulci are indicated. (From Horton JC, Hoyt WF. The representation of the visual field in human striate cortex. Arch Ophthalmol 1991;109⬊816–824.) 546 CLINICAL NEURO-OPHTHALMOLOGY is performing a tangent screen examination using three or more identical stimuli slowly brought horizontally from the defective hemifield toward the vertical meridian. One stimulus moves toward fixation, as the others simultaneously move inward to points 10–15⬚ above and below fixation. With genuine macular sparing, the central stimulus is perceived before those above and below it. If there is defective fixation and shifting of the entire hemifield, however, all targets are simultaneously perceived. It is impossible to obtain absolutely stable fixation, and physiologic movements of the fixing eye so slight that they cannot be detected by ordinary means probably account for 1–2⬚ of deviation of the vertical meridian about the central area. Verhoeff (331) emphasized the importance of the optic fixation reflexes in cases of macular sparing. He suggested that loss of integration between the seeing field and the blind field in the conscious visual cortical area in use might result in eccentric fixation. As a result of fixation slippage, the original macular field would be split but would appear to be spared during field testing. Sugishita et al. (332) used fundus perimetry combined with fundus image analysis in two subjects with left occipital lesions and ‘‘macular sparing.’’ Two of the four eyes tested showed eccentric fixation, and miniature eye movements to the blind hemifield were observed in 4–10% of the trials. Using microperimetry and a scanning laser ophthalmoscope, Bischoff et al. (333) also demonstrated rapid fixation shifts in their patients with ‘‘macular sparing’’ of 1–5⬚. These authors concluded that this typical macular sparing may be a functional adaptation of the ocular motor system to enhance the central visual field toward the blind hemifield. In 1928 Lenz (334) proposed that a small fiber tract subserving the macular region branched from the optic tract through the corpus callosum to the opposite visual cortex, providing representation of the macula in both occipital lobes. The presence of bilateral representation of the macula was further suggested by Penfield et al. (277), who studied five patients after radical extirpation of the occipital lobe. In two instances, the posterior portion of the lobe was excised. In both, there was complete homonymous hemianopia with splitting of the macula. In two other cases, a large portion of the lobe was removed, and it was thought that the entire calcarine cortex was included. Nevertheless, following surgery, the patient had an homonymous hemianopia with macular sparing and retention of normal visual acuity. The fifth and most interesting case studied by Penfield et al. (277) was one in which there was complete destruction of the calcarine cortex of one side. This pathologic process resulted in homonymous hemianopia with sparing. When the diseased occipital lobe was extirpated, the line of resection was so far forward that it was adjacent to the splenium of the corpus callosum. Subsequent to the resection there were further field changes in that the vertical meridian failed to deviate about the point of fixation. Thus, ‘‘sparing of the macula’’ was converted into ‘‘splitting of the macula.’’ Ohki et al. (335) described a case that was the reverse of Penfield’s fifth case. The patient was a 62-year-old man who experienced a left parieto-occipital hemorrhage. When first examined, the patient had a right homonymous hemianopia with macular splitting. As the hemorrhage resolved, the patient developed macular sparing, initially of 5⬚ and then of 10⬚. From a study of reported cases of occipital lobectomy and five cases studied personally, Dubois-Poulsen et al. (336) also supported the concept of macular representation in both occipital lobes as an explanation for macular sparing. These researchers endorsed a theory that had first been proposed by Morax (337), who suggested that in the foveal region of the retina there is a small vertical band of ganglion cells, some of which have crossed and others uncrossed fibers. Gramberg-Danielsen (338) supported this thesis in a thorough review of the problem of macular sparing. He emphasized the inexact nature of the vertical border between the two hemifields and called attention to a functional vertical overlap of the two sides of the visual field in the form of a narrow band that is widest near the fovea. At the retinal level, ganglion cells and their dendritic processes overlap and intertwine without any morphologic suggestion of the functional vertical separation of the two halves of the retina, corresponding to the temporal and nasal halves of the visual field. Halstead et al. (339) carefully studied the visual fields of two patients who underwent complete occipital lobectomy and found ‘‘splitting’’ in one case and ‘‘sparing’’ in the other. From a study of occipital lobectomy cases, Huber (340) (who subsequently declared macular sparing to be an artifact [333,341]) also confirmed the phenomenon of macular sparing, favoring the theory of bilateral foveal representation probably explained at the level of the retina. Cowey (342) found occasional anomalous receptive fields for cells in the squirrel monkey’s cortical fovea. All of his animals had previously had an occipital lobectomy. Recording from a microelectrode in a single cortical unit in the foveal area of the monkey’ intact occipital lobe, he was able to evoke responses from the parafoveal area of the ‘‘blind’’ visual field. He recognized the similarity of his experimental finding to the clinical observation of macular sparing following occipital lobectomy in man. It is clear from histochemical studies that, to some extent, a small portion of each macula probably has representation in each occipital lobe. As noted in Chapter 1, there is a small area of nasotemporal overlap on either side of the vertical meridian in which some axons from ganglion cells temporal to the fovea cross within the chiasm, whereas some axons from ganglion cells nasal to the fovea remain uncrossed (210,211,213,214,343). In the monkey, the nasotemporal overlap is smallest (about 1⬚ of visual angle) near the fovea and increases at least twofold in more peripheral retina (213,214). Scanning laser ophthalmoscopy has demonstrated this zone of nasotemporal overlap in humans that extends into the blind hemifield, with a slightly concave shape (344). Despite the presence of a nasotemporal overlap that exists in nonhuman primates and presumably in humans as well, the mere presence of an overlap does not necessarily have major visual consequences. Sugishita et al. (332) concluded that dual macular representation, if it exists in humans, must be less than 0.4⬚ wide, and Brysbaert (345) also questioned the clinical significance of bilateral macular representation. Of more importance, however, is that a ganglion cell located TOPICAL DIAGNOSIS OF CHIASMAL AND RETROCHIASMAL DISORDERS on one side of the fovea does not necessarily receive input from a photoreceptor located on the same side. The much larger width of the cone pedicle compared with the foveal cone soma creates a packing problem that is only partly resolved by lateral displacement of cone pedicles and via the nerve fiber layer of Henle. This lateral shift could theoretically connect photoreceptors on one side of the vertical meridian to seemingly distant ganglion cells on the opposite side (266). Such an anatomic relationship would have no visual consequences, because it is the position of the photoreceptor and not the ganglion cell body that determines the location of visual input to the brain. The anatomic inexactness of the vertical demarcation can be observed clinically by visual field testing (289). Nonetheless, the most likely cause of true macular sparing in clinical practice is incomplete damage to the visual pathways, particularly the occipital lobe. Wilbrand (347) suggested that macular sparing was the result of residual intact areas of the visual pathways. McAuley and Russell (308) noted an association between macular sparing and retained portions of the most posterior portion of the occipital cortex (Fig. 12.41). These observations are supported by the work of Horton and Hoyt (298), who demonstrated the extremely high cortical magnification of the macular representation (Fig. 12.40). The unique dual blood supply of the occipital cortex from the posterior cerebral and middle cerebral arteries (see Chapters 1 and 39) provides a mechanism for this partial damage. In patients with homonymous hemianopia and substantial macular sparing, not only is the location of the lesion almost always the occipital lobe, but also the pathogenesis is likely posterior cerebral artery infarction. In these cases, the most common cause for macular sparing is reten- 547 tion of some functioning posterior occipital cortex. In other cases, macular sparing may reflect fixation artifact or bilateral representation. Together, the consequences of these theories can explain the great variability in macular sparing seen in retrochiasmal disease. Bilateral Occipital Lobe Lesions Bilateral lesions of the occipital lobes may occur simultaneously or consecutively. Because such lesions are neurologically asymptomatic except with respect to the visual system, patients with unilateral lesions that have caused a homonymous field defect may not be aware of the defect until their attention is called to it (e.g., during a routine ocular examination or after a motor vehicle accident) or until they experience a similar event on the opposite side. Förster first described a patient with a well-documented, partial, bilateral homonymous hemianopia in 1890 (348). The patient initially sustained a right homonymous hemianopia followed by a left homonymous hemianopia with sparing of central vision. Numerous case reports subsequently documented this condition and its many causes (349–358). Bilateral homonymous hemianopia may appear simultaneously. In a majority of these cases, there is initially complete visual loss; however, this total blindness is usually transient, lasting from minutes to days (359,360). Nepple et al. (356) reviewed the findings in 15 patients with bilateral homonymous hemianopia. All patients had similarly shaped visual field defects on corresponding sides of the vertical midline for each eye, symmetric (and usually normal) visual acuity, normal pupils and fundi, and normal ocular motility unless there was a coexisting brain stem lesion. The majority Figure 12.41. A 52-year-old man with coronary artery disease and thrombocytopenia purpura noticed trouble seeing to the left side with his left eye. A, Examination was normal except for visual fields, which showed an incongruous left homonymous quadrantic defect, denser in the left eye. (Figure continues.) 548 CLINICAL NEURO-OPHTHALMOLOGY Figure 12.41. Continued. B and C, Magnetic resonance images (B, T2-weighted; C, T1-weighted after gadolinium) showed a curvilinear enhancing lesion in the medial, anterior, right occipital lobe consistent with infarction (arrows). of patients had bilateral posterior cerebral artery insufficiency caused by arteriosclerosis (40%), uncal herniation from subdural hematoma (20%), or migraine (13%). Most of these patients had a history suggestive of simultaneous bilateral occurrence. Brain tumor did not occur in this series; conversely, in 849 patients with visual field defects and brain tumors reviewed by Schaublin (361), there were no patients with bilateral homonymous hemianopias. Bilateral homonymous hemianopia may also occur from consecutive events, invariably vascular (Fig. 12.42). This is a much more common phenomenon than is bilateral simultaneous homonymous hemianopia. In such cases, the patient Figure 12.42. A, Bilateral homonymous hemianopic defects secondary to bilateral posterior cerebral artery infarction. Note the dense right homonymous hemianopia combined with a left inferior homonymous quadrantanopia with sparing of the left temporal crescent. (Figure continues.) TOPICAL DIAGNOSIS OF CHIASMAL AND RETROCHIASMAL DISORDERS 549 Figure 12.42. Continued. B–D, T2-weighted magnetic resonance images demonstrated bilateral posterior cerebral artery infarctions, worse on the left than the right, and extending less inferiorly and less anteriorly on the right. experiences an acute homonymous hemianopia with retention of normal vision with or without sparing of the macula. At a later time, varying from weeks to years, the patient develops a sudden homonymous hemianopia on the opposite side, again with or without macular sparing. After this second event, the patient is either blind or retains only a small central field around the point of fixation, similar to that seen in simultaneous cases. The syndrome of bilateral nonsimultaneous homonymous hemianopia from infarction of both occipital lobes is most frequently observed in elderly atherosclerotic individuals and may be associated with hypertensive crisis. In some instances, there are premonitory visual disturbances such as flashing lights or visual hallucinations. Because the vascular supply to association areas of the visual cortex may be interrupted, there may also be visual agnosia and other disturbances of higher cortical function. Bilateral vascular lesions of the occipital lobes produce 550 CLINICAL NEURO-OPHTHALMOLOGY bilateral homonymous lesions that are characteristic and vary only in their extent. They may be complete or scotomatous, and they may or may not be accompanied by macular sparing. Bender and Furlow (362,363), for instance, described ‘‘central scotomas’’ produced by bilateral lesions of the occipital lobes. Such defects are, in reality, bilateral homonymous scotomas that may be detected by careful field testing along the vertical midline (364) (Fig. 12.43A and B). Occasionally, such scotomas have enough central sparing to produce ‘‘ring’’ scotomas. One such patient had complete blindness transiently following a tractor accident. When vi- sion returned, the patient noted that although he could see clearly in the exact center of his field, the paracentral area was blurred. Visual field testing demonstrated bilateral, congruous, homonymous scotomas with macular sparing (Fig. 12.43C and D). Bilateral occipital lobe disease, whether from infarction, tumor, or trauma, may result in various degrees of bilateral homonymous hemianopia (Fig. 12.44). There may be complete (cortical or cerebral) blindness. The hemianopia may be complete on one side and incomplete (and congruous) on the other, or there may be a hemianopia on one side and a Figure 12.43. A and B, Bilateral homonymous hemianopic scotomas in a patient with bilateral nonsimultaneous occipital lobe strokes. C and D, Note vertical step that differentiates these defects from a true central scotoma with macular sparing in a patient who suffered trauma to the occipital region. The tractor on which he was riding overturned, pinning him underneath for several minutes. Initially, he was completely blind, but vision returned within several minutes. He subsequently realized that he had a ‘‘ring’’ of blurred vision around fixation. Note that the homonymous scotomas respect the vertical midline. TOPICAL DIAGNOSIS OF CHIASMAL AND RETROCHIASMAL DISORDERS Figure 12.44. A 40-year-old man suffered occipital head trauma with a depressed skull fracture and visual complaints. Visual acuity was 20/20 in both eyes. A, Visual fields revealed exquisitely congruous bilateral homonymous scotomas, larger on the left than the right. A computed tomography scan (B) revealed a right occipital pole lesion, but magnetic resonance images (C and D) confirmed bilateral occipital pole involvement. 551 552 CLINICAL NEURO-OPHTHALMOLOGY Figure 12.45. After strenuous exercise, a 33-year-old man suffered intermittent confusion, gait disturbances, and right facial weakness, followed by permanent left hemiparesis and difficulties with vision. A, Visual fields revealed a right homonymous hemianopia with macular sparing and a left superior homonymous quadrantanopia. B, Magnetic resonance imaging revealed the corresponding infarctions in the occipital lobes bilaterally. Note the involvement of the inferior occipital lobe on the right and the entire calcarine cortex on the left, with sparing of the most posterior pole. The patient also had cerebellar and brain stem infarctions, all secondary to artery-to-artery emboli from a vertebral artery dissection. quadrantanopia on the other (307,365) (Figs. 12.42 and 12.45). In some instances, there may be a bilateral homonymous hemianopia with bilateral macular sparing of a different degree on each side. The remaining visual field thus appears to be severely constricted, and such patients may be thought to have bilateral optic nerve or retinal disease or may even be thought to have nonorganic visual field loss. As with bilateral homonymous hemianopic scotomas, however, careful testing along the vertical midline will establish the bilateral nature of the field defect and its correct origin (Fig. 12.46). Crossed quadrant hemianopia results when patients develop bilateral quadrantic defects, either simultaneously or more commonly consecutively, that affect the superior oc- cipital lobe above the calcarine fissure on one side and the inferior occipital lobe below the fissure on the other side. Such defects are sometimes called checkerboard fields and are infrequent (Fig. 12.47). Felix (366) described a case and reviewed the literature up to 1926, which included only two other cases. In the case reported by Felix and in one other case, this field defect followed a simultaneous bilateral homonymous hemianopia, although a case described by Groenouw (quoted by Felix [366]) occurred after two separate vascular events occurring 10 months apart. Although bilateral altitudinal field defects are usually caused by bilateral anterior optic nerve disease (e.g., glaucoma or nonarteritic anterior ischemic optic neuropathy), they can also be caused by bilateral occipital lobe disease TOPICAL DIAGNOSIS OF CHIASMAL AND RETROCHIASMAL DISORDERS 553 Figure 12.46. Bilateral homonymous hemianopias with macular sparing in a patient with severe cerebrovascular occlusive disease. The patient initially presented with 20/20 vision in both eyes but with ‘‘tubular’’ fields. The macular sparing respects the vertical midline. (e.g., from trauma, infarction, hemorrhage, and rarely tumors). Such lesions were studied extensively by Holmes and Lister (375) and were subsequently described in more detail by Holmes (367), who observed that bullet wounds interrupting both occipital lobes were usually fatal if the lower portions were damaged because death occurred from intracranial bleeding as a result of laceration of the dural sinuses in the region of the torcular herophili. When the upper portions of the visual cortex or posterior radiations were damaged, however, the patient often survived, and the resultant field defects in such cases were altitudinal, with loss of the entire inferior field of both eyes. Money and Nelson (1943) described field defects similar to those reported by Holmes (367), and Wortham et al. (368) reported bilateral inferior hemianopia occurring during or immediately following thoracoplasty. The defect was Figure 12.47. Crossed quadrant (checkerboard) hemianopia. These field defects occurred suddenly in a 70-year-old woman with basilar artery disease. Note the quadrantic defects in the right upper field and the left lower field with the narrow congruous isthmus near fixation. Also note the sparing of the left temporal crescent and the incongruity of the field defects along the upper vertical meridian and the right horizontal meridian. (Courtesy of University of California Hospital, San Francisco.) 554 CLINICAL NEURO-OPHTHALMOLOGY thought to have resulted from anoxia, and slight improvement occurred over the next several months’ observation. Newman et al. (369) described two cases of bilateral altitudinal hemianopia, one inferior and one superior, secondary to occipital infarctions that were demonstrated by CT scanning, and a similar case was confirmed both on neuroimaging and pathology by Vanroose et al. (370). Other cases secondary to occipital infarction or hemorrhage occur, occasionally in the setting of cardiac surgery or hypoxia (308,353,368, 371–376). Based on the preceding, it can be understood why superior altitudinal defects from occipital lobe trauma are less common than inferior altitudinal defects. An example of the former, occurring in a patient where a bullet passed from right to left through the inferior portions of both occipital lobes but missing the dural sinuses, is depicted in Figure 12.48. On the other hand, both superior (Fig. 12.49) and inferior bilateral altitudinal defects can occur with vascular disease affecting the occipital lobes. When the lesion is far posterior, the field defect remains altitudinal but is central and scotomatous (Fig. 12.50). Spalding (296) confirmed the impressive congruity of these small field defects. Cortical (Cerebral) Blindness Cortical blindness and cerebral blindness are considered in the same section because in many instances it is impossible to differentiate between them on clinical grounds. The term ‘‘cortical blindness’’ indicates loss of vision in both eyes from damage to the striate cortex. ‘‘Cerebral blindness’’ is a more general term indicating blindness from damage to any portion of both visual pathways posterior to the lateral geniculate bodies. Thus, cortical blindness is a subset of cerebral blindness. Patients with cerebral blindness may have other neurologic deficits, including hemiplegia, sensory disorders, aphasia, and disorientation. Because of the similarity in the visual system symptomatology between these two conditions, for the sake of simplicity the term ‘‘cortical blindness’’ will be used in this section. The essential features of cortical blindness as outlined by Marquis (377) are (a) complete loss of all visual sensation, including all appreciation of light and dark; (b) loss of reflex lid closure to bright illumination and to threatening gestures; (c) retention of the reflex constriction of the pupils to illumination and to convergence movements (the near-response); (d) integrity of the normal structure of the retinas as verified with the ophthalmoscope; (e) retention of full extraocular movements of the eyes, unless there is also damage to ocular motor structures. Many neuro-ophthalmologists use the term ‘‘cortical blindness’’ not only where the visual acuity is light perception or no light perception but also with any level of visual acuity, as long as the visual acuity is equal in the two eyes (assuming the anterior visual pathways are normal). Hypoxia or ischemia of the occipital lobes is the most common etiologic factor producing cortical blindness. Such damage is necessarily bilateral. Most commonly, an infarction in the posterior cerebral artery territory is initially unrecognized, but this previously silent hemianopia contributes to complete cortical blindness when a contralateral lesion occurs (378,379). The most common mechanism for the infarction is cerebral embolism from either the heart or the more proximal vessels of the vertebrobasilar system (380,381) (Fig. 12.51). Prolonged hypotension can cause cortical blindness from bilateral watershed infarctions at the parieto-occipital junction. Courville (382), among others, recognized that hypoxia causes laminar cortical necrosis and that the visual, premotor, and parietal areas are particularly apt to be damaged by this process. Courville (382) expressed the opinion that these areas seemingly had a higher oxygen requirement. Lindenberg (383) emphasized that the affected areas represent the border zones of cerebral circulation. Cortical blindness is observed under many circumstances other than infarction, including trauma, neoplasm, malignant hypertension, toxemia of pregnancy, diseases of white matter (e.g., Schilder’s disease, adrenoleukodystrophy, Pelizaeus-Merzbacher disease, metachromatic leukodystrophy, progressive multifocal leukoencephalopathy), CreutzfeldtJakob disease, mitochondrial encephalopathy lactic acidosis and stroke-like episodes (MELAS), cerebral angiography, ventriculography, blood transfusions, uremia, acute intermittent porphyria, syphilis, infectious and neoplastic meningitis, bacterial endocarditis, subacute sclerosing panencephalitis, hepatic encephalopathy, sudden elevation or reduction in intracranial pressure, cardiac arrest, hypoglycemia, correction of hyponatremia, epilepsy, electroshock, and after exposure to carbon monoxide, nitrous oxide, ethanol, licorice, methamphetamine, mercury, lead, cis-platinum, cyclosporin A, tacrolimus (FK506), methotrexate, vincristine, vindesine, and interferon (379,384–418). The mechanism of injury underlying the cortical blindness caused by these events or substances is not always known, but vascular insufficiency plays a role in many of them. In certain situations, cortical blindness is transient. This is particularly true in patients who experience transient vascular insufficiency in the vertebrobasilar system, in hypertensive syndromes following restoration of normal blood pressure, after removal of many of the toxic agents listed above, and after trauma (417,419–421). Children are more likely to experience recovery from cortical blindness than adults, regardless of the underlying cause. Barnet et al. (419) studied six children who developed transient cortical blindness after respiratory or cardiac arrest, head trauma, or meningitis. In all cases, visual acuity returned to some extent in 1–10 weeks. Greenblatt (422) emphasized three clinical patterns of transient cortical blindness following head trauma: 1. Juvenile (up to age 8 years). These patients experience blindness of short duration (hours), accompanied by somnolence, irritability, and vomiting, with an excellent prognosis for full recovery. 2. Adolescent (late childhood through teenage years). These patients have blindness that does not develop immediately after the trauma but occurs several minutes later. The blindness lasts minutes to hours, is usually unaccompanied by other neurologic or systemic deficits, and also tends to resolve completely. 3. Adult. These patients have immediate blindness, a TOPICAL DIAGNOSIS OF CHIASMAL AND RETROCHIASMAL DISORDERS Figure 12.48. A 24-year-old man suffered a gunshot wound to the left temporal region with exit of the bullet via the right occipital pole. Visual acuity was 20/20 in both eyes, but there was cerebral dyschromatopsia and prosopagnosia. A, Visual fields showed a right superior homonymous quadrantanopia (probably resulting from damage to the left inferior radiations at the temporal–occipital lobe junction) and a left superior homonymous quadrantic scotoma (probably from damage to the right inferior occipital pole). B–E, Magnetic resonance images showed the hematoma created by the bullet track. 555 556 CLINICAL NEURO-OPHTHALMOLOGY Figure 12.49. A 71-year-old man had acute onset of complete visual loss, followed by clearing inferiorly. A, Examination was normal except for visual fields that showed bilateral superior altitudinal visual field defects (bilateral superior homonymous quadrantanopias). B–F, T2-weighted magnetic resonance images (B–D, axial views; E and F, sagittal views to the right and left of midline, respectively) demonstrated bilateral posterior cerebral artery infarctions involving primarily the inferior occipital lobes. (Figure continues.) TOPICAL DIAGNOSIS OF CHIASMAL AND RETROCHIASMAL DISORDERS 557 Figure 12.49. Continued. protracted course with other neurologic defects, and a variable visual outcome. Postictal cortical blindness is not uncommon (395, 423,424), and ictal cortical blindness as a manifestation of occipital seizures may occur (409,425–429). Ashby and Stephenson (430) analyzed 11 cases of blindness after seizures and concluded that there is a form of amaurosis that occurs in young children and infants that is postictal and is caused by depression of the visual centers. The seizures that accompany such blindness are usually violent and may be associated with aphasia and paresis of hemiplegic distribution. The hemiplegia may be permanent, although the visual loss is generally transient. Joseph and Louis (429) reviewed the literature concerning seizure-induced bilateral blindness and divided affected patients into three groups. Fifteen patients, all under 12 years of age, had a long succession of seizures or status epilepticus. They developed blindness that was permanent or that lasted many months. Ten patients, seven of whom were between the ages of 10 and 20 years, had bilateral occipital seizures with blindness corresponding to atypical spike and wave activity. The blindness lasted less than 30 minutes in all cases. Finally, 18 patients had a focal seizure originating in or adjacent to an occipital lobe, with ictal spread to involve both occipital lobes. In these patients, blindness occurred either ictally or postictally. Because focal lesions may be responsible for ictal amaurosis, MR imaging 558 CLINICAL NEURO-OPHTHALMOLOGY Figure 12.50. Occipital injury (shell fragment). Bilateral inferior hemianopic quadrantic scotomas, according to Holmes. (From Traquair HM. An Introduction to Clinical Perimetry, 3rd edition. St Louis, CV Mosby, 1940.) should be performed in all cases (409). Complete recovery from ictal or postictal blindness can occur even though the blindness lasts for several days. Visual-Evoked Responses in Cortical Blindness Figure 12.51. Bilateral simultaneous posterior cerebral artery occlusion secondary to embolic infarction from a cardiac arrhythmia. The patient was initially cortically blind with no light perception vision. Over the course of a few weeks he had 20/30 vision in each eye because of 5⬚ of macular sparing. The relationship between cortical blindness and the visual-evoked response (VER) is unclear. One of the earliest human studies on this subject was performed by Kooi and Sharbrough (431). Their patient had suffered head trauma and was initially blind with no evoked responses to light stimuli. Subsequently, both visual acuity and VERs underwent gradual and parallel improvement. This promising correlation was corroborated by other investigators (432–434). However, Spehlmann et al. (435) reported persistence of the VER to light in a blind patient who had extensive bilateral destruction of cortical visual areas and their association areas. Similarly, Celesia et al. (436) and Hess et al. (437) demonstrated normal VERs in both complete and incomplete cortical blindness. Aldrich et al. (379) found abnormal pattern and flash VERs in 15 of 19 patients with cortical blindness, but there was no correlation with the severity of visual loss or with visual outcome. Indeed, all four patients with normal VERs during blindness had poor visual outcomes, and three patients with good outcomes had no initial responses to pattern-reversal VERs. These investigators found that electroencephalography was a more helpful diagnostic indicator: the presence of a posterior dominant alpha rhythm responsive to eye opening appears to be incompatible with complete or incomplete cortical blindness. Hence, it would appear that at least in adults, VERs are not useful in establishing either the diagnosis or prognosis in patients with cortical blindness (379,438). TOPICAL DIAGNOSIS OF CHIASMAL AND RETROCHIASMAL DISORDERS The usefulness of VERs in children with cortical visual dysfunction is controversial. Barnet et al. (419) reported two children who showed well-formed VERs shortly after the onset of their acute disease, even though they were completely blind. Bodis-Wollner et al. (439) described normal VERs to both flash and alternating grating patterns in a blind child in whom CT scanning showed destruction of the cerebral cortex corresponding to areas 18 and 19 bilaterally with preservation of primary visual cortex (area 17). Frank and Torres (440) recorded VERs to brief light flashes from occipital regions in a group of 30 cortically blind children aged 4 months to 15 years. These investigators compared their results with those obtained from 31 children of similar age range who had similar central nervous system diseases but no evidence of blindness. Only one patient with cortical blindness showed no response, and the presence of abnormal responses was not incompatible with normal vision. Mellor and Fielder (441) reported that poor or even absent VERs in infancy did not always indicate a poor prognosis, and Hoyt (442) showed a similar lack of correlation. Other authors also noted poor correlation of VERs and visual function in children with bilateral cerebral abnormalities, especially those with associated neurologic deficits (443–445), and it is not uncommon to see patients free of clinical neurologic or visual deficits who have incidentally discovered abnormal VERs. Nevertheless, some investigators argue that the VER is indeed helpful in establishing both diagnosis and prognosis in children with cortical visual dysfunction. In a study of six children with cortical blindness after either bacterial meningitis or head trauma, Duchowny et al. (446) found that changes in short-latency VER components correlate with visual ability, whereas changes in longer-latency VER components correlate with the level of psychomotor function. Kupersmith and Nelson (447) reported that the presence of a VER wave, whether normal or abnormal, indicated the future development of vision. Lambert et al. (448) and Bencivenga et al. (449) also reported some correlation of the VER response to cerebral visual function. McCulloch and Taylor (450) argued that flash VERs are an excellent indicator of visual recovery for those children who have acuteonset cortical blindness and no preexisting neurologic disorder. In this subset of patients, many with presumed hypoxia, those with abnormal VERs had permanent visual impairment or blindness, and all but one patient with absent VERs remained blind (451,452). The variable results from previous studies may reflect the many different underlying etiologies of cortical blindness in children and the many other associated neurologic factors that might contribute to poor recovery. Optokinetic Nystagmus in Cortical Blindness Optokinetic nystagmus (OKN), the slow component of which is generally considered a conscious pursuit movement, is often used to distinguish patients with true blindness from those with nonorganic blindness. Most investigators agree that OKN is absent in patients with total cortical blindness (385,453–455). In fact, Brindley et al. (455) repeatedly 559 examined a patient with total cortical blindness over a 3.5year period and could never elicit OKN. Ter Braak (456) separated OKN into two types: passive and active. According to this investigator, passive OKN is produced by the movement of the majority of contrasts in the visual field and may still be present after ablation of the visual cortex, whereas active OKN depends on the movement of specific objects in the visual field and thus requires ‘‘vision’’ for its production. Using these definitions, Ter Braak et al. (457) examined a 71-year-old man with longstanding cortical blindness and found passive OKN in one direction. Subsequent necropsy revealed almost total destruction of both striate cortices and degeneration of both LGBs. Despite these observations, the presence of OKN in an individual complaining of complete blindness or perception of light only is almost always evidence of nonorganic visual loss (see also Chapter 21). Cortical Blindness with Denial of Blindness (Anton’s Syndrome) Patients with cortical blindness are often unaware of their deficit. This denial of blindness, a type of anosognosia, is called Anton’s syndrome (458). The syndrome also occurs in patients with blindness from causes other than occipital lobe disease, including cataracts, retinopathies, or optic atrophy (459). The explanation for Anton’s syndrome is unclear. Lessell (459) reviewed the various theories and concluded that each explains some of the cases. Geschwind (quoted by Lessell [459]) suggested that patients with denial commonly have an alteration in emotional reactivity. Such patients have emotions that are described as coarse and shallow and may predispose them to deny their blindness. Lessell also stated that ‘‘psychiatric’’ denial may occur as an accentuation of a common response to illness. In patients with Korsakoff’s syndrome, denial of blindness may represent a memory disorder. Such patients cannot remember from minute to minute that they truly are blind. Finally, it is probable that in many patients with cortical blindness, there are lesions in various areas of the brain responsible for recognition and interpretation of visual images. In such patients, denial of blindness is caused not by the lesion in the primary visual pathway but by a lesion in another region of the brain. Other Visual Features of Occipital Lobe Damage Bender and Teuber (327) made noteworthy observations based on their study of 140 individuals who suffered penetrating head wounds in World War II. It was their belief that when macular function is spared, there is either an incomplete homonymous hemianopia or development of a pseudofovea. They also noted that when an incomplete homonymous field defect is produced by an occipital lobe lesion, there are often disturbances in the area of the homonymous visual fields that seem spared when ‘‘ordinary’’ methods of perimetry are performed. For instance, critical flicker fusion frequency falls in field areas adjacent to the scotoma and to a lesser degree in field areas far distant from the scotoma. 560 CLINICAL NEURO-OPHTHALMOLOGY Dark adaptation is delayed in the apparently normal field. Luminous objects can be discerned within the scotomatous field when patients are placed in total darkness. Finally, when simple figures are presented to the patient so that part of the figure is in the area of scotoma and part in the seeing field, the entire figure is seen (completion phenomenon) (460). There are several different explanations for these observations. First, the methods of perimetry used in the 1940s were clearly less sensitive than automated static perimetry. It thus is likely that many of the patients studied by Bender and Teuber (327) actually had larger field defects than were detected by what was then ‘‘routine’’ perimetry. Second, it is likely that the observation of luminous objects in blind areas of the field represented artifacts of testing, since it is clear that some ‘‘luminous’’ objects have a larger area of stimulation of the retina than might normally be assumed. In addition, minor movements of the eyes during testing that might not be detected in total darkness with the equipment available to Bender and Teuber (327) may have moved the luminous target into the seeing field adjacent to the blind field. Some patients with homonymous hemianopia, especially those with a vascular occlusion in the occipital lobe, report phosphenes in the blind visual field, particularly early in the course of their disorder (461–464) (see also the section on visual hallucinations in Chapter 13). Among 96 patients with homonymous hemianopia or quadrantanopia, most of vascular origin, Kolmel (463) noted that 14 had experienced colored patterns in their blind hemifield, typically red, green, blue, and yellow. Many of the visual field defects in these patients resolved substantially, suggesting that the phosphenes may be viewed as a prognostically favorable symptom. Of 32 patients with ischemic infarction of the retrochiasmal pathways studied prospectively by Vaphiades et al. (464), positive spontaneous visual phenomena in the blind hemifield were present in 13 patients (41%). Classifying these positive visual phenomena as photopsias, phosphenes, palinopsia, or visual hallucinations had no value in localizing the site of the lesion. However, there was a significant difference in the severity of associated neurologic deficits between hemianopic patients with and without these positive visual phenomena; specifically, larger lesions destroying anteriorly located visual association areas precluded the development of these positive phenomena. The authors proposed that visual association areas bordering damaged primary cortex were the source of these visual symptoms when they were released from normal inhibitory inputs from primary visual cortex. An unusual visual phenomenon associated with unilateral or bilateral occipital lesions is cerebral diplopia or polyopia, in which two or more images are perceived monocularly (465,466). Cerebral polyopia may be due to reorganization of one or more of the multiple representations of the visual field in the visual cortex (466). Unlike monocular polyopia from irregularities of the ocular refractive media, cerebral polyopia does not improve with pinhole. This entity is discussed further in Chapter 13. Lesions of the Striate Cortex Without Defects in the Visual Field It is generally assumed that any lesion of the striate cortex produces a defect in the field of vision, but there are cases on record that suggest that this assumption may be incorrect. Teuber (467) stated that he had records on an extraordinary case of a 1-year-old child who sustained an accidental gunshot wound through the right parietal lobe. The bullet remained in the right calcarine area, where it could still be shown radiologically 30 years later. Teuber et al. could not demonstrate any evidence of a field defect, even though they used refined tests of flicker fusion and light thresholds. Weiskrantz (468) stressed that it is conceivable that an injury of the striate cortex must produce a critical minimum amount of damage before it produces any demonstrable scotoma. Evidence supporting this has been contributed by N. R. Miller, who searched in vain for tiny scotomas produced by stereotactically implanted depth electrodes (0.5 mm in diameter) that were inserted directly into and through the macular area of the occipital pole and the occipital portion of the optic radiations. It is unlikely that standard white-onwhite automated perimetry can identify visual field defects associated with these small injuries, although it is possible that static color field testing could identify such defects. Similarly, stereotactic pallidotomy, a procedure used to treat patients with Parkinson’s disease, requires precise positioning before the electrode is activated. The positioning includes placement of the electrode in the ipsilateral optic tract, the cells of which are then identified by recording from the electrode (468a). The electrode is then pulled back out of the optic tract into the globus pallidus, where a lesion is then made. It is difficult to identify visual field defects in the majority of patients in whom stereotactic pallidotomy is performed, regardless of whether the testing is performed using kinetic or static automated perimetry. Weiskrantz and Cowey (469) contributed neurophysiologic evidence to support the aforementioned observations and conclusions. They showed that quantitative lesions in the macular cortex of the monkey produce a smaller drop in visual acuity than would be predicted from equivalent lesions of the macula. They suggested that the discrepancy between retinal and cortical effects is probably best explained in terms of lateral interaction of neuronal elements at the retinal level, the geniculate level, or both. An alternative explanation is that the visual cortex has a higher relative magnification factor for the foveal representation than is accounted for by the concentration of retinal ganglion cells in the macula (305). Lesions of the Extrastrate Cortex with Defects in the Visual Field As noted in Chapter 1, the striate cortex (V1, or Brodmann area 17) is the primary visual cortex and the principal recipient of output from the LGB. Surrounding the striate cortex within the occipital lobe are two visual association areas, Brodmann areas 18 and 19, also called the extrastriate visual cortex. Studies from primate electrophysiologic and pathway tracing methods indicate that areas 18 and 19 together repre- TOPICAL DIAGNOSIS OF CHIASMAL AND RETROCHIASMAL DISORDERS sent at least five distinct cortical areas devoted to visual processing (278,470–472). These visual areas are designated V2, V3, V3a, V4, and V5 in the monkey, and corresponding regions are present in the human occipital cortex (472). Functional MR imaging has recently been able to demonstrate multiple visual areas in the human, each with their own retinotopic mapping (473,474). Regions V2 and V3 are the major recipient areas for projections from both the magnocellular and parvocellular systems in V1, the primary striate cortex. However, V1 also directly projects to areas V4 and V5, bypassing V2 (475). The sizes of V1 and V2 are highly correlated, but not V1 and V3 (306). Feinsod et al. (476) suggested that a lesion in the extrastriate cortex alone might cause defects in the visual field. Others reported superior visual field defects from damage to the 561 ‘‘nonvisual’’ lingual and fusiform gyri (373,477). Polyak (478) reported a case in which paracentral quadrantic scotomas were caused by an infarction primarily located in a region corresponding to the ventral portion of areas V2 and V3. Horton and Hoyt (278) reported two patients with homonymous quadrantic defects caused by tumors, one in the cuneus of the occipital lobe and the other in the upper peristriate cortex (Figs. 12.52 and 12.53). These investigators proposed not only that a lesion of V2/V3 may be sufficient to create a visual field defect, but also that such lesions are the principal cause of quadrantic defects that strictly respect the horizontal meridian. This hypothesis has been supported by functional MR imaging studies demonstrating selective loss of activation of extrastriate cortex areas in a patient with an upper right homonymous quadrantic defect (479). Figure 12.52. A 39-year-old woman had experienced multicolored visual hallucinations in her left lower quadrant since childhood. A, Magnetic resonance imaging revealed a lesion within the cuneus of the right occipital lobe that on en bloc resection was found to be a grade I astrocytoma. B, Postoperative magnetic resonance image revealed the area of resection. C, Postoperative visual fields demonstrated a left inferior homonymous quadrantanopia with precise respect of the horizontal and vertical meridians. The patient could detect gross hand motion within the quadrantic defect. (From Horton JC, Hoyt WF. Quadrantic visual field defects. A hallmark of lesions in extrastriate [V2/V3] cortex. Brain 1991;114⬊1703–1718.) 562 CLINICAL NEURO-OPHTHALMOLOGY Figure 12.53. A 40-year-old man experienced increasing episodes of flashing lights in the left lower quadrant of vision and was found to have a large tumor of the right parieto-occipital cortex. A, On magnetic resonance imaging the calcarine sulcus (between arrows) and the occipital pole are preserved. B, After resection of the mass, he had a left lower quadrantanopia precisely bordering the horizontal meridian with sparing of the central 10⬚. (From Horton JC, Hoyt WF. Quadrantic visual field defects. A hallmark of lesions in extrastriate [V2/ V3] cortex. Brain 1991;114⬊1703–1718.) Cortical Visual Loss with Normal Neuroimaging William F. Hoyt has suggested that the essence of neuroophthalmology is diagnosis in the presence of normal neuroimaging. A not uncommon example occurs when there is unilateral or bilateral homonymous hemianopia in the presence of normal MR imaging. Brazis et al. (480) reviewed several causes of this phenomenon, including the Heidenhain variant of Creutzfeldt-Jakob disease (481,482), variant Alzheimer dementia (483,484), nonketotic hypoglycemia, and ischemia without infarction. Many of these disorders can be detected with positron emission tomography scanning or functional MR imaging, which is sensitive to the effects of neural activity on blood oxygenation levels (485). Another etiology for visual loss without abnormalities on conventional MR imaging is occipital lobe seizures, which can disrupt visual perception not only during the ictus but also postictally (486) and even permanently (487). Dissociation of Visual Perception The chapter that follows (Chapter 13) discusses the topographic diagnosis of disorders of visual processing more dis- tal to the primary visual cortex. Because many of these disorders result from lesions in the extrastriate cortex of the occipital lobes, it is worth mentioning a few of the more prominent syndromes in this chapter. As suggested by studies in monkeys, the human visual cortex is highly specialized with respect to specific functions (472). For instance, there are distinct areas of the cortex that subserve color vision. These can be identified either by studying patients with known lesions or by functional MR imaging mapping of activation of cortex after appropriate stimulation. One area in the lingual and fusiform gyri of the prestriate cortex corresponds to area V4 in the monkey and is responsible for color (472,488,489). Other areas adjacent to V3 can be identified by functional MR imaging as containing retinotopic maps for color (473). Lesions in any of these regions may spare the visual field but produce acquired dyschromatopsia in the contralateral hemifield (477,490,491). The disturbance of color vision in such cases may be permanent, especially if there are bilateral lesions. Conversely, transient achromatopsia may reflect temporary ischemia in this region, a phenomenon that may occur in migrainous aura (492). The area of functional specialization for visual motion is TOPICAL DIAGNOSIS OF CHIASMAL AND RETROCHIASMAL DISORDERS localized to the temporoparieto-occipital junction, a region corresponding to area V5 in the monkey (472,493,494). Lesions in this region, especially when bilateral, may cause a selective deficit in the perception of visual movement without a visual field defect and without impairment of nonvisual movement perception (i.e., movement perceived by acoustic or tactile stimulation) (495,496). A similar loss of motion detection, but in a single direction at a time, can be reproduced by electrical stimulation of the posterior temporal lobe (497). In some patients, damage to an occipital lobe can cause a complete homonymous hemianopia to nonmoving objects, with retention of the ability to detect moving objects within the blind hemifield: static–kinetic dissociation, also called the Riddoch phenomenon. In 1917 Riddoch (498) noted that some wounded soldiers with complete homonymous hemianopia developed or retained the ability to perceive small moving objects in the affected hemifield. Riddoch divided his cases into three groups: those who perceived motion only on the affected side; those who perceived both moving and stationary objects but to a different degree; and those with no dissociation between moving and stationary objects. Static–kinetic dissociation may have prognostic significance since it usually means that some degree of recovery can be expected. Such a phenomenon has been observed not only after occipital trauma but also after removal of tumor, cortical blindness from cardiac arrest, and occipital apoplexy in patients with occipital arteriovenous malformations (see also (499)). Preservation of the human correlate of area V5 may be required for intact visual motion processing, perhaps via extrageniculostriate pathways (500) (see below). Static–kinetic dissociation is not a purely pathologic phenomenon, nor is it limited to the striate cortex. In fact, all normal individuals perceive moving objects better than static objects of the same size, shape, and luminance. If objects are sufficiently small or dull, they will be perceived only during kinetic perimetry and not during static perimetry. Safran and Glaser (501) demonstrated variable degrees of physiologic dissociation of kinetic and static stimuli in 7 of 15 normal subjects. Interestingly, the dissociation occurred for achromatic target perception but not for chromatic perception of red. Safran and Glaser attributed this physiologic dissociation to a successive lateral summation of images, as previously noted by Greve (502). Spatial summation is more pronounced for rods than for cones, and this may explain the lack of dissociation of static and kinetic targets when hue recognition is required. Selective excitation of movementdependent channels appears to be required for perception of movement (503). In addition, pathologic static–kinetic dissociation occurs in patients with nonoccipital lobe lesions. Zappia et al. (504) reported two patients, one with a lesion in the optic tract and one with a lesion of the optic chiasm. Both patients showed a dissociation between perception of static and kinetic objects in their affected hemifields. In addition, Safran and Glaser (501) studied 11 patients with compression of the anterior visual pathways and found some degree of static–kinetic dissociation in the defective field of all of them. The Riddoch phenomenon of static–kinetic dissociation 563 is one form of a general category of visual phenomena designated as ‘‘blindsight’’ (468,505–510). In a classic experiment, Mohler and Wurtz (511) removed the occipital lobe in trained monkeys. The monkeys appeared to be blind in the appropriate hemifield for several weeks but then began to make appropriate saccades into the previously blind hemifield. Mohler and Wurtz then extirpated the superior colliculi in the monkeys, who then became permanently blind in the hemifield. In another set of monkeys, Mohler and Wurtz first removed the superior colliculi. The monkeys showed some mild and transient difficulty with saccadic eye movements but no evidence of visual loss. The monkeys were then subjected to unilateral occipital lobe excision, following which they became permanently blind in the contralateral hemifield of both eyes. These experiments indicated that nonhuman primates have a subcortical vision sensory system that likely involves connections between the retina and the superior colliculus in addition to the retinogeniculo-occipital pathway for normal visual function. A similar subcortical pathway may exist in humans. Indeed, some patients with extensive damage to the occipital lobes appear to retain a rudimentary form of vision involving the perception of visual stimuli other than just moving objects. Most patients are not consciously aware of this ability to look, point, detect, and discriminate without truly ‘‘seeing.’’ In many cases blindsight is likely a result of islands of preserved area 17, as demonstrated by positron emission tomography, single photon-emission computed tomography, and image stabilization perimetry (512,513). Celesia et al. (512) argued that the sparing of portions of primary visual cortex, combined with probable scatter of light into the intact visual field during testing (514), accounts for blindsight in humans. However, other investigators contend that blindsight is a genuine phenomenon that reflects nonstriate visual pathways (see Cowey and Stoerig [508] for review), which can occur in the absence of functioning area 17 (515). In humans, examples of pathways invoked as an explanation for blindsight are a retinocollicular projection that reaches extrastriate cortical visual areas via the pulvinar nucleus (516) and a direct geniculo-extrastriate cortical projection (508). Interestingly, there appears to be an increase in the sensitivity of blindsight in a given individual over time, suggesting that training of visual function may have rehabilitative implications. Another way in which visual perceptions may be dissociated is in the syndrome of unilateral inattention or neglect. Patients with this defect may appear to have normal visual function if tested in routine fashion, since they can correctly perceive objects in each hemifield with either eye. However, when two test objects are presented in the right and left hemifields of each eye simultaneously, the patient perceives only the test object in the hemifield ipsilateral to the lesion. This phenomenon, called visual extinction, can occur following damage to the parietal or occipitoparietal cortex as well as to the frontal lobe and thalamus. It can be demonstrated not only in patients with partial hemianopic defects on the same side but also in patients with previously recovered hemianopias or no documented visual field defects. Unilateral neglect can result from lesions in several different regions of the brain. This finding reflects the complex inte- 564 CLINICAL NEURO-OPHTHALMOLOGY grated network that exists for the modulation of directed attention within extrapersonal space (291). Functional MR imaging can demonstrate activation of visual cortex without activation of more distal cortical processing areas, demonstrating an anatomic basis for extinction (517). lated to direct downward pressure on the cerebellum by the tumor. Nonvisual Symptoms and Signs of Occipital Lobe Disease Spontaneous recovery of homonymous visual field defects occurs in no more than 20% of patients within the first several months after brain injury (523–525). Thus, despite a certain amount of plasticity even in the adult cerebral cortex (526,527), patients with visual field defects have a consistently poor rehabilitation outcome (528–530). The exact anatomic location of the lesion causing the homonymous hemianopia does not appear to affect the functional outcome (531); however, the greater the number of associated neurologic deficits, the more difficult the rehabilitation and the poorer the functional performance (532). Contributing further to the poor functional improvement is the advanced age of most of these patients, a factor associated even in normal individuals with progressive cognitive, sensory, and motor deficits (533). Finally, the presence of neglect, especially in patients with nondominant hemisphere lesions, also interferes with the rehabilitation process. Several techniques may assist in the treatment and rehabilitation of patients with homonymous hemianopia (534). A mirror attachment can be used to project the mirror image of the blind field into the seeing half-field (535). The mirror is attached to the nasal side of the spectacle frame behind the lens. Burns et al. (536) found that three of six patients learned to make some use of the device. For many patients, however, such mirrors do not prove satisfactory because the patient has to turn the head toward the mirror, because the mirror produces a scotoma that blocks a portion of the seeing field, and because spatial disorientation is common. Images seen in the mirror are reversed and projected to the opposite side of the midline (537), causing confusion. Mirrors on prongs fixed to a frame are available, but they are usually discarded by the patient because of the cosmetic appearance of such devices. Mintz (538) devised a small adjustable mirror that is mounted on a clip placed over the spectacle frames on the nasal side. Waiss and Cohen (539) suggested the use of a temporal mirror coating on the back surface of spectacles. Smith (540) advocated the use of Fresnel press-on prisms in patients with a homonymous hemianopia. The prism is placed on the outside half of the lens ipsilateral to the hemianopia with the base oriented toward that side (i.e., for a patient with a left homonymous hemianopia, a 30-diopter prism was placed base-out on the left half of the left lens). Perlin and Dziadul (537) advocated placing 20 prism-diopter Fresnel prisms in front of both eyes, allowing for a 5⬚ movement of the eyes before they encounter the prism edge (see also [541]). Prism power and placement are then modified, depending on patient adaptation. The goal is to increase the patient’s scanning skills over time. Rossi et al. (542) randomly assigned 39 patients with stroke and homonymous hemianopia or unilateral visual neglect to treatment with 15 prism-diopter plastic press-on Fresnel prisms or to no treatment. Although the prism-treated group later performed As might be expected, vascular lesions of the occipital lobe are usually asymptomatic, except with regard to the visual system. Many patients with occipital infarctions experience acute pain of the head, brow, or eye ipsilateral to the lesion. This presumably reflects the dual trigeminal innervation of the posterior dural structures and the periorbital region (518). Patients with homonymous hemianopia may also complain of disturbances of equilibrium, particularly a feeling that their body is swaying toward the side of the hemianopia. Rondot et al. (519) called this phenomenon ‘‘visual ataxia’’ and proposed that the postural defect reflects unopposed tonic input of vision from the intact hemifield rather than any true vestibular impairment or neglect. If a homonymous hemianopia is unrecognized by the patient, the first signs of its presence may be the functional consequence of hemifield blindness. Most clinicians have never seen the ‘‘door sign’’ (520), in which a patient with homonymous hemianopia acquires a linear wound in the forehead ipsilateral to the field defect from inadvertently striking the head on a door frame. However, it is not unusual to see patients with homonymous hemianopia that was discovered after a motor vehicle accident in which little or no injury was sustained to account for the field defect or the infarct in the occipital lobe (subsequently detected by neuroimaging) that clearly predated the accident. If the posterior cerebral artery is occluded proximally, patients with an infarct in the occipital lobe may also have hemiplegia from damage to the posterior internal capsule or cerebral peduncle, language dysfunction from damage to posterior parietal structures, or symptoms reflecting damage to the ipsilateral thalamus (380,521). In addition, because the condition is ordinarily seen in patients with severe atherosclerosis, there may be other evidence of vertebrobasilar insufficiency, including ocular motor abnormalities referable to the rostral brain stem. Patients who develop a bilateral homonymous hemianopia in this setting may be more severely impaired than patients with blindness from bilateral retinal or optic nerve disease and may be difficult to rehabilitate (see below). Tumors of the occipital lobe may cause nonvisual manifestations by virtue of their mass effect. Parkinson and Craig (522) examined the signs and symptoms of 50 patients with verified tumors limited to the occipital lobe. Headache was the most common symptom, occurring in 45 patients (90%). Other symptoms and signs included nausea and vomiting (46%), ataxia (30%), hallucinations that were usually unformed (24%), seizures (18%), and mental changes (16%). Most of the signs and symptoms noted by Parkinson and Craig are nonlocalizing and related to increased intracranial pressure. Ataxia and other cerebellar signs are usually re- TREATMENT AND REHABILITATION FOR HOMONYMOUS HEMIANOPIA
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