Chapter 17: Afferent Lesions

CHAPTER 17 Afferent Lesions CONTENTS A. Summary ......• . . . . . . . . . . . . . . . . . . . B. Historical Setting . . . . . . . . . . . . . . . . . . . . C. Appearance and Clinical Significance . . . . . . 1. Lesions in the Retina or Optic Nerve .... (a) Assumption of Separate Afferent Fibers for the Pupil and for Vision . . . . . . . . (b) Pupillary Equality . . . . . . . . . . . . . . . 2. Lesions in the Optic Chiasm or Tract .... 915 917 917 917 920 924 927 (a) Behr's Pupillary Phenomena . . . . . . . . (b) ''Wernicke's Sign" or Pupil Hemiakinesia . . . . . . . . . . . . . . . . . . 3. Lesions in the Afferent Pupillary Fibers beyond the Optic Tract . . . . . . . . . . . . . . 4. Lesions in the Supra-geniculate Visual Pathways (Optic Radiations and Cortex) .. 927 931 931 937 A. Summary The following paragraphs pertain to clinical conditions in man. Animals below primates have a different fiber distribution in their afferent pupillary neurons (see Chapter 3). Since both the retinal rods and cones are receptors for the pupil, light reflexes can be elicited from all parts of the retina; and retinal damage of any kind will reduce them. 1 In patients with lesions in the retina or optic nerve of one eye the light reflexes remain normal when the good eye is stimulated, but they show "low-intensity" form when the bad eye is stimulated. These reflexes look exactly like normal reflexes to dim light: the latent period is prolonged, and the contractions are inextensive, relatively slow, and short-lasting. For patients with unilateral or with asymmetric retinal or optic nerve damage, a good clinical test consists in moving a flashlight from one eye of the patient to the other and-after a few seconds-back again to the first eye: both pupils will contract when the light is moved to the good eye, and they will dilate when the light is shifted to the bad eye (Figure 17-1,A). With bilateral impairment of the retina or optic nerve, stimulation of either eye ( or of 1. For pupillary findi ngs in squint amblyopia, and due to opacities of the ocular media or to refractive errors, see Chapter 3. Figure 17-1. Appearance of patients with uncomplicated lesions in the retina or optic nerve. In darkness or in dim light the pupils are normal in size (Al). When the lesion is unilateral both pupils contract well when the good eye is exposed to light (A2), but they become noticeably larger when the light is shifted to the bad eye (A3). Reactions to bilateral light and to near vision remain normal (A4 and AS) , and the right and left pupils remain equal at all times. If they become unequal to a clinically noticeable degree, there is additional damage in other neurons of the pupillary reflex arc. Bilateral damage to the retina or optic neive, and lesions in the optic chiasm or tract, cause subnormal contractions when either eye alone or when both eyes together are stimulated. Nearvision contractions may be surprisingly extensive, but in patients blind for a long time it may not be possible to elicit the near response. both of them together) will elicit subnormal contractions of "low-intensity" form (Figure 17-1,B). More recently, pupillary "edge light cycles" also have been used to reveal such impairment. While useful empirically, this technique has some problems (see Chapter 3). Lesions in the optic chiasm or tract cause "lowintensity" reflexes when either eye is stimulated: · in patients with such damage the pupillary field defects always match the visual field defects. Complete interruption of an optic tract destroys the uncrossed optic fibers from the eye on the side of the lesion (temporal retina) and the crossed fibers from the opposite eye (nasal retina). Since the nasal retina contains more ganglion R L AlA2 ~ -~---------.- ~ A3 ~ A4l~ :.;:::::::::::: A5 ~ ~ J 915 916 / IV. Pupillary Pathology: Symptomatology cells than the temporal retina, the chiasmal fiber distribution is not symmetric. Instead, there are more crossed than uncrossed fibers. Stimulation of an eye on the side of an optic tract lesion (crossed fibers spared) therefore produces distinctly more extensive pupillary contractions than stimulation of the opposite eye (uncrossed fibers spared). But in the absence of additional damage to other pupillary neurons, the right and left pupils remain equal, at least within clinically discernible limits. It has often been said that there are separate retinal ganglion cells and afferent optic fibers to serve vision and to serve the pupil. But clinical evidence for this assumption remained unconvincing, while physiologic evidence partly supported and partly contradicted it. Recent findings in retinal physiology appear to explain these discrepancies. There are at least three types of retinal ganglion cells which project afferent fibers to the forebrain and to the midbrain in different proprotions (see Table 13 in Chapter 3). One type (The W or gamma-cells) project exclusively to the midbrain, and they form the chief afferent connections for the pupil. The other two (Y and X cells, or alpha and beta cells, respectively) send projections mainly to the lateral geniculate nucleus, but also send collateral branches to the midbrain (the Y cells more so than X cells); and some of these also appear to serve the pupil. Afferent pupilJary fibers leave the optic tract before it reaches the lateral geniculate ganglion and travel byway of the brachia of the superior colliculi to the pretectaJ area. Damage in that area beyond the optic tract affects the pupils while vision remains normal. Conversely, destruction of the suprageniculate visual path does not abolish pupillary reflexes, although long-term lesions cause elevation of the pupillary threshold in the corresponding portion of the retina. The mechanisms responsible for this effect have been debated for many years but can be explained by the new retinal findings just mentioned, as described in detail in Chapter 3. 7~-c---l.r. 1~- - - - -2n. 2.5"'- - - 3,,, 1 6 1 \ '--"~ 5 , - rA'\-v--,'1\,-.. - - - - - - - - - - - - - - - - - - - t 4,,___ _ _ _ _ _~ --~~~~M;,;:x~:;:;:;;;:;;;:::;::;:.~-.....,,;-1 3 ,_•-•-•--+-•- •-•--+- +-•---.--.--.--r-+-+-+-•-•-•-+-•~~ .-m--H·~•:__ 5ln-1\--;A.-~-A- - t \ ~ - f . ~'.-p' •~-A:-1\-f 4 /. ~.--¾--V,l,-__v,.- '/4.~l- ~• ,,. - ,,. ~.-.__,;µ':',:+-~-~-V...----'v--.--V;;V.- ~ ~f\1 '- 4 5 6-+-- - ---------1 rE-~~~-~A'---~---~~-~-A~~-r-~/r~ ~---~ -....,:w- 3- I , ; 1 - - - - - -- --l-1 ,; ,-..., I .r. r- I .,.--1-----------t 0. !sec . -+ Figure 17-2. Psychogenic loss of vision (or malingering) without related pupilary deficit. Three weeks before examination the patient, a 17-year-old boy, had been in a scuffle with a schoolmate. He was struck on the left temple and knocked down, but did not lose consciousness. He went home, had a good supper, and went to sleep. The following day he felt fine but failed an examination. Several hours later he complained of headaches and blurred vision, and about 6 hours after the examination he said he could not see at all out of his left eye, save for a small area in the extreme temporal field. He never was drowsy or confused. Opthalmologic and neurologic examinations were entirely negative. The patient continued to complain about pain in his left eye and about throbbing pain behind his eye. His vision was 20/25+ on the right side and finger counting in the temporal periphery on the left side. A " malingering test" was said to have been negative. The pupils reacted normally to light and to darkness, except for slight consensual deficit: On stimulation of the left eye the right pupil (solid lines) lagged a bit behind the left pupil (broke n lines). Otherwise all reactions were symm etric, both when bright light was used (A, B, C, D, E) and when the light was attenuated by neutral grey filters (density 2 logs in F and 6 logs in G). Even near the pupillary threshold right-eye and left-eye stimulation caused responses of equal amplitude (Gt and G2). Comment: A normal pupillary threshold is not compatible with extensive unilateral visual loss. When the patient was told firmly that his visual problem would rule out participation in sports, his vision rapidly returned to normal. 17. Afferent Lesions / 917 B. Historical Setting . It has been known for a very long time that pupillary size is related to vision. Galen, in the second century AD, knew that the pupils usually were large when vision was poor. He and his contemporaries used this fact as a prognostic sign when considering couching a cataract: the pupils were small when the good eye was open and the bad eye was covered up, and they enlarged when the good eye was covered and the bad eye opened instead. Galen did not think of this pupillary movement as a response elicited by light, because he believed that the " essential principle" for vision arose from the brain: in healthy individuals abundant "animal spirits" flowed from the brain through the hollow optic nerves to the eyes, to shine out of the eyes and to illuminate the world. The expression that a person was "robbed of the light of his eyes" derived from this theory. Centuries later, Rhazes (850-932) realized that normal pupils contracted to light shining upon the eye, and dilated when the light was reduced (see Chapter 3). And much later again it was found that only light which passed through the pupil and reached the retina caused the contraction, not light falling upon the iris itself (Scheiner, 1616). Further, the pupil of the other eye, which was not stimulated, constricted also, " by consensus" (Plempius, 1648). From this expression came the term "consensual light reflex. " Clearly, the brain transmitted the optic stimulus to the oculomotor nerves of both eyes (Whytt, 1717). Investigators have differed about the identity of the retinal receptors (from 1880 to 1960); about the retinal ganglion cells serving the pupil (from 1870 to the present); about the afferent fibers in the optic nerve, chiasm, and tracts (from 1870 to the present); and about a possible influence of the visual cortex upon light reflexes (from 1883 to the present). These discussions arc considered in Chapter 3 and below. They played a role in clinical thinking about the influence of afferent lesions upon pupillary movements. In 1855 von Graefe mentioned that pupillary contraction to light " always" had been accepted as a sign for the presence of vision. And pupil reflexes have been used widely to distinguish organic blindness from hysteria or malingering (Figure 17-2). In the opposite situation, poor light reflexes alterted the physician to retinal or optic nerve damage even though visual acuity appeared normal (see Figure 17-7). In 1884 Hirschberg described a young girl in whom asymmetric afferent conduction of the pupillary light reflex revealed retrobulbar neuritis. The consensual reaction of her affected eye was more extensive than its direct reaction: when the good eye was shaded both pupils dilated, and they contracted when the bad eye was shaded and the good one was opened. Later, other patients have been described in whom, after a bout of retrobulbar neuritis, the pupils continued to show an afferent deficit while visual acuity had returned to normal. Such reliance on pupillary reactions in the diagnosis of afferent damage was widespread during the last decades of the nineteenth century, but the finding is usually ascribed to Marcus Gunn (1904), and the eponym " Marcus Gunn pupil" has been coined. We would , however, discourage its use: the term is historically unjust; and further, Marcus Gunn's name is already associated with the jaw-winking phenomenon (' Marcus Gunn syndrome" ), with AV nicking of retinal vessels ("Gunn's sign" ), and with glistening pecks near the optic disk ("Gunn's dots" ). The pupil sign was popularized by Kestenbaum (1946) under the name of Marcus Gunn and of "pseudoanisocoria," because the pupils were larger when one eye than when the other was covered. But both pupils become large when the good eye is shaded, and both constrict when the bad eye is covered instead, so that the two pupils remain equal at all times. We therefore find the term "pseudo-anisocoria" unfortunate: it is easily misunderstood as meaning that anisocoria develops, which is not the case (see below). For such unilateral or asymmetric afferent defects the name "relative afferent pupillary deficit,' advocated by H.S. Thompson, appears the most descriptive. Under this name the phenomenon has become quite popular in recent years (Table 17-1 ). C. Appearance and Clinical Significance 1. Lesions in the Retina or Optic Nerve Since the retinal rods and cones are the receptors for the pupillary light reflex, any kind of retinal or optic nerve lesion can reduce afferent pupillary impulses and cause " low-intensity' reflexes. These look exactly like responses elicited by dim light from a normal eye. In fact, by reducing the intensity of the light to the patient's good eye with grey filters it is possible to match these reactions to those produced when the bad eye is stimulated with the full brightness of the light (Figure 17-3). A rough numerical value can thus be placed on the relative afferent deficit of the bad eye (in log units of stimulus attenuation to the good eye needed to produce the match). " Low-intensity" light reflexes have prolonged latent periods, reduced extent and speed, and relatively short duration. In answer to a bright, continuous light, such pupils contract at first but soon redilate even though the stimulus is still present (Figure 17-4). This feature has been called "pupi.llary escape"; and it distinguishes "low-intensity" reactions from those caused by lesions in the motor path of the light reflex. These also are slow Table 17-1. - YEAR Recent clinical publications on afferent pupillary deficit AUTHOR 1961 1966 Krilger Thompson ~ Stanle.r & Baise 1971 Alexandridis & Weddigen 1973 Smith 1975 Alexandridis & Bischoff 1975 Mc Crar.r 1975 Nikoskelainen 1976 Kaback, Burde & Becker 1976 Prrwes 1976 Tbom2son 1977 Merritt 1978 Meienberg & Kommerell 1978 Tbom2son 1979 Brill 1979 Ellis 1979 Fison, Garlick &Smith 1979 Kahn, Moss & Podos 1979 Osher, Schields &La~an 1979 ThomEson 1980 Argyropoulos & al CONDITION !YEAR ambl.ro2ia afferent EuEil deficit minimal OEtic neuroEath:t: hereditary pigmentary retinal dageneration afferent defects (method) post-surgery retinal detachment retinal & 02tic nerve disease optic neuritis 1980 1980 AUTHOR Bovino & Burton Thompson, Watzke & Weinstein 1981 Alexandridis & al. 1981 Cox, Thompson & Corbett 1981 Newsome & al. 1982} Alexandr id is 1982 Alexandridis & al. 1981 Thompson, Corbett & Cox 1982 Gladstone 1982 Thompson glaucoma as~metric glaucoma optic nerve disease optic nerve conaitions --- 1983 1983 subtle optic nerve lesions 1983 1984 cataract unilateral retinitis Eigmentosa acute optic neuritis 1984 unilateral afferent defects 1985 1985 1985 1986 1986 glaucoma melanoma of the optic disc technique of measuring optic nerve disease Greenwald& Folk Portnoy, Thompson Lennarson &Corbett Thompson & Cox Folk, Thompson, Han &Brown Han, Thompson & Folk Jiang & Thom:eson ThomQSOn Thom2son & Corbett Cox Servais, Thompson & Hayreh CONDITION retinal detachment macular disease optic nerve disease optic neuritis macular degeneration retrobulbar neuritis technique of measuring occult temporal arteritis pupil vs visual acuity and visual field amblyopia amblyopia optic neuritis central serous retinopatby optic neuritis retinitis pigmentosa r etinal & 02tic nerve disease aHerent pupil deficit measurement technique central retinal vein occlusion --Left eye Light, Left 100 % ----·Right eye 7 6 5 4 ..... 3 ~ 2 '-7 ~6 Light, Right 100 % B ~5 ~ C:::i4 ~3 ~ 7 6 Light. Left 10- 3 ·6 C 5 SECONDS F igure 17-3. Method of quantifying afferent deficit in patients with unilateral visual impairment. The patient had right-sided afferent pupillary deficit because of retrobulbar neuritis. A: During the time periods shaded dark grey the normal left eye was exposed to standard intensity light flashes, and extensive light reflexes resulted. B: When the same stimuli were placed on the left eye (lightly shaded areas) the reactions we re only shallow, but no anisocoria resulted. C: With 3.6 log units of neutral grey filters interposed between th e light and the good left eye, the reflexes 918 were decreased almost as much as in B. Comment: By attenuati ng the light intensity used to stimulate the good eye so as to match the reactions produced when the bad eye was stimulated with bright light, a rough numerical value can be placed on the pupillometer deficit of the bad eye (in this case a little more than 3.6 log units). (From H.S. Thomp on , Amer. J. Optha/., 62 [1966):860); published with permission from the American Journal of Opthalmology, 0 The Ophthalmic Publishing Company) 17. Afferent Lesions / 919 and shallow, but they last at least as long as do normal reflexes. Because of this "escape" mechanism, the pupils of patients with retinal or optic nerve lesions are rather large in the light-adapted state. This fact is probably the source of the term "amaurotic mydriasis" which often has been used to describe them. It is, however, important to remember that the pupils of such patients are not "dilated" : their size is normal in darkness; and they appear larger than normal only because of the defective light reflex. For the darkness reflex the " low-intensity" pattern is especially characteristic: the pupils, fairly large after light-adaptation, as just mentioned, dilate little or not at all when this light is interrupted for a short time; and they constrict relatively well when the light is readmitted (Figure 17-4). The mechanism of this behavior is discussed in Chapter 4. Clinically, it is a useful feature: "low-intensity" darkness reflexes confirm the existence Light, Left 100% --Left eye ----·Right eye of a suspected afferent defect and make it easy to distinguish the condition from inextensive light reflexes due to parasympathetic deficit or to unusually strong central inhibition. A simple clinical test for unilateral or assymmetric afferent impairment is the "swinging flashlight test" advocated by Levatin and later many others. With the patient in a dimly illuminated room and looking far away, one eye-say, the good (or better) one-is exposed to a flashlight beam, and both pupils become small. After some seconds, the light is shifted to the bad ( or worse) eye, and the pupils enlarge. As the light beam is thus directed alternately into one eye and then the other, consistent pupillary contractions and dilations will reveal the relative afferent defect. Such test , as well as clinical pupillograms, are useful because they demonstrate the presence of afferent impairment in an objective and reasonably quantitative way. M.E. Age 15 A Figure 17-4. Pupilary "escape" in a patient with bilateral afferent impairment (A), compared with a normal subject (B and C) . The patient was an obese 15-year-old girl who complained of severe headaches. She was found to have bilateral papilledema and increased intracranial pressure. Extensive diagnostic investigation failed to reveal the cause of her trouble. During the next months the working diagnosis was pseudotumor cerebri. She was treated with steroids and the papilledema receded. About 2 months later her headache recurred and her vision was reduced to light perception. One month later a craniotomy was done for decompression, and the cortex and meninges were found to be studded with nodules of metastatic melanoma. The patient died about 2 months later; and since only the brain was examined, the location of the primary tumor remained unknown. The neuropathologist's report stated that " microscopically, both optic nerves were diffusely infiltrated by the tumor. The infiltration was peripheral, entering the nerves from the leptomeninges; however, much viable nerve B remained. " Line I shows the patient 's reactions to bright light and to darkness; line 2 shows a normal person' reactions under the same conditions. In line 3 light of reduced intensity was used in the normal person (attenuated by neutral grey filter of 102 density). Note the " escape" of the patient's light reflex, compared to the firm, extensive contraction of the normal subject's pupils. With 2 log units of neutral grey filter interposed between the normal eye and the light the same pupillary "escape" was produced as in the patient with bright light. In the darkness reflexes the normal pupil dilated well to interruptions of the light (white bars); and they recontracted quickly when the light was readmitted. In contrast, both the patient's eye and the normal eye with attenutated light bad "low intensity" darkness reflexes: the dark-dilations were missing and the secondary contractions were hallow and short-lasting. (From H.S. Thompson.Amer. J. Ophthal. , 62 [ I 966]:860; published with permission from The American Journal of Ophthalmology, 0 The Ophthalmic Publishing Company) 920 I IV. PupilJary Pathology: Symptomatology As de cribed in more detail in Chapter 3, pupillary light reflexe arc related to the threshold of light perception but not nece arily to vi ual acuity. The pupils urn up the total afferent output of the retinal ganglion cell , and fairly good reflexes may be obtained in patient with only peripheral or scattered areas of the vi ual field pared, even when the e do not allow good vi ion (Figure 17-5); and small foveal le ions may interfere with vi ual acuity more everely than with pupillary con trictions elicited by diffuse, strong light which reache the entire retina and stimulates the large number of peripheral rods. 2 On the other hand, pupillary deficit may continue to be pre ent during remission after an attack of retrobulbar neuriti with residual loss of periphral retinal function even though foeveal acuity ha returned to normal. For demon tration of pupillary field lo in uch case mall dim test lights must be u ed to prevent pupillornotor effects of stray light. Pupillary te t for afferent deficit allow assessment of retinal or ptic nerve function at different stages of a di ea e proce , or under different physiologic condition . For example, Czarnecki (1979) found improvement of pupillary afferent deficit in a patient with multiple clero i after drinking cold water. In patient with cataract but with a healthy retina the reactions elicited from the affected eye may be no wor e than or even better than normal. Apparently diffu ion of the light by the cataract to all areas of the retina, and reduced retinal bleach in the affected eye, can make up for the loss of light intensity due to absorption by the cataract. This was not true at a time when surgery was done only for very dense cataracts. (a) Assumption of Separate Afferent Fibers for the Pupil and for Vision Ever since von Gudden (1885) had observed nerve fibers of different caliber in the optic nerves and tracts of mammals, he and others believed that the thicker fibers served the pupil while the thinner fibers served visual perception (Table 17-2). And since the thicker fibers looked more robust under the microscope, they were often said to be more resistant than the thinner ones to mechanical or toxic injury. This was thought to explain occasional findings of apparently blind patients with active pupil reflexes. The opposite opinion was held by others who said the pupillary fibers were thinner and more fragile than the visual ones, and that they had a specific affinity to a syphilitic toxin; and that this caused the loss of the pupillary light reflexes without loss of vision in patients with the Argyll Robertson syndrome. No one ever tried to verify either of these theories by anatomic studies. The clinical evidence brought forth to support the idea of separate pupillary and visual afferent fibers was weak. It remained far from proven that the pupils 2. ln amblyopia, also, there is no relation between pupillary afferent deficit and the depth of visual impairment (see Chapter 3). continued to react to light in eyes truly blind due to retinal or afferent path disease, since the published reports of "blind" patients with active pupil reflexes had many flaw . -:-The 'blindness" often was not total, and vanous degrees of vi ion remained intact (Table 17-3,A); and since the pupillary threshold was so low, patients without much useful vision may have fairly good light reflexes. -In many cases, the pupillary contractions were almost certainly due to causes other than light, such as lid-close reactions or inadvertent convergence-accommodation efforts (Table 173,B). --Sometimes vision was lost very shortly before ( or regained shortly after) the pupil reflexes, leaving doubt as to whether a dissociation between the two functions really existed (Table 17-3,C). -In other cases, malingering or hysteria were not ruled out with certainty (Table 17-3,D). -In yet others, the blindness was very probably cortical, at least in part (Table 17-3,F); or the patients were in poor general or mental condition, so that it was difficult to assess the severity of their visual loss. -Very often, stray light was inadequately controlled , especially in patients with unilateral afferent lesions. Meticulous care must be taken to prevent the stimulating light from reaching the healthy eye. In this large literature we were unable to find a single case (and have seen none ourselves) which would prove the existence of separate afferent pupillary fibers beyond doubt. But it should be noted that this lack of clinical evidence did not disprove the existence of separate pupillary retinal ganglion cells or afferent fibers; and some physiologic evidence was brought forth that appeared to support the assumption (see Chapter 3). All that could be said until recently was that the question had not been decided conclusively either way. One point, however, appeared obvious: if separate retinal neurons and afferent fibers existed for the pupil and for vision, selective damage to one or the other kind of fibers in the optic nerve or tract could not explain blindness with active pupils or good vision with fixed pupils, since the afferent optic fibers arc closely packed in the optic nerve and tract. It is difficult to imagine a disease process such as tumor, trauma, or infection that would selectively destroy all fibers serving one function while those for the other would remain untouched. To overcome this objection a number of authors said that the afferent pupil fibers were not intermingled with the visual fibers but travelled as a separate fascicle from the eye to the midbrain. Therefore they could be destroyed without injury to the visual fibers, and vice versa (Table 17-4). There were, however, good reasons to discard this notion. (1) As seen from Table 17-4, the "pupillary fascicles " assumed by different authors varied in position in the optic nerve and tract, and the ~~y1 ~ 5 •......... \ \ li9ht .J,..<j,ht ... _ - _,/'/_-- saa.:,: a•-~--• A2 -A3 . ~--- _; - -dark- i\~ {le,$ 1'!/'/ 7':J,ht -----r-:-J --- -- A'+ -~ ciarl<.- n~ left "" CS L Tl • Otsu.al ftelol.s dark- dark1'1ess l~ft Figure 17-5. Pupillary reactions in patients with optic nerve disease. The solid lines represent the right pupil and the broken lines the left pupil in each case. A: Unilateral optic atrophy due to multiple sclerosis. Five years before examination a 38-year-old woman suddenly noticed that vision in her left eye became poor. lt recovered without treatment but was lost entirely a few months later. Vision in her right eye continued normal. Ncurologic examination showed asymmetric deep tendon reflexes and impaired abdominal reflexes. The left optic nerve was atrophic. The pupils reacted normally when the right eye was stimulated (Al, A3) but not at all when the left eye was stimulated (A2, A4). The pupils remained equal at all time . B: " Low-intensity" light reflexes with good visual acuity after unilateral retrobulbar neuritis. A 25-year-old man deve loped pain on lateral movement of his right eye, with rapid loss of vision. Within 2 days papillcdema developed and the eye was blind. The blindness cleared within days, and 8 days before examination acuity had returned to 20/25, with a normal visual field. The right knee jerk was hyperactive and the left lower abdominal reflex was abolished. Despite the apparently full recovery of vision the pupillary reactions to light and to darkness elicited from the right eye had marked "low intensity" features (Bl, 83). Stimulation of the left eye produced normal reflexes (B2, B4), and the near vision contractions were normal in both eyes (BS). C: Unilateral optic atrophy after benign meningitis (adhesive arachnoiditis). The patient wa a 32-year-old woman. At the age of 17 she had had a febrile disease with headaches, a stiff neck. vertigo, and somnolence. A few days after the onset of fever, she had lost her peripheral vision in the right eye and her sense of smell on both side . At examination she was neurologically normal, aside from the lo s of smell and visual impairment. Acuity was 20/15 on the right and 20/20 on the left side. The fundus and field were normal on the left side, but in the right eye there was optic atrophy, and the peripheral field was lost except for one small island (see diagram CS). The behavior of the pupils agreed well with this: all reactions were normal except for reduced afferent conduction from the right eye (Cl and C3, see also Figure 17-6). The ""'hr ui...si.on. patient was followed over a 22-year period with our further neurologic, vi ual, or pupillary changes. D: Bilateral optic atrophy due to multiple sclerosis. The patient, a 21-year-old college student, began to lose vision about 5 months before examination. He wa found to have bilateral optic neuritis. Within 4 months he had lo I his central vision on both ides, and a short time later he became completely blind. During the following 4 weeks there was some recovery. At the time of examination his acuity was 20/200 in each eye, with bilateral temporal pallor and fields as shown in diagram DS. Neurologic examination showed bilaterally clonic patella and ankle reflexes, with the left patella reflex more active than the right. Abdominal reflexes were weak and quickly exhau ted. There was an extensor plantar response on the left side and intention tremor in both hands. Note that the pupils reacted fairly extensively to light despite the complete loss of central and reduction of peripheral vision. E: Bilateral afferent deficit because of multiple sclerosi . The patient was 25 years old when he was examined. At the age of 15 he had had an episode of pain on eye movements, followed by bilateral loss of vision within 3 to 4 days, with edema of the discs and scattered hemorrhages surrounding the discs. His vision gradually returned within 2 months. and he was well until 12 days before examination, when vision again became blurred and then lost in the left eye and reduced in the right eye. There was gradual improvement, and on the day of examination acuity was 20/200 on the left and 20/ 100 on the right side. The deep tendon reflexes were hyperactive, with the right knee jerk more active than the left, and with bilateral ankle clonus. There was a positive Hoffmann's sign on the right. Because of the bilateral afferent impairment the pupillary reflexes were reduced when either eye was stimulated by light (El and E2). The darkness reflexes also showed marked "low intensity" form (E3 and E4). The patient could sec well enough to do the near-vision test, and an extensive pupillary contraction resulted (ES). Comment: Note that there was no anisocoria at any time in these patients with m1co111plicated afferent deficit. (From 0. Lowenstein, Arch. Ophthal. , Chicago, 52 [1954]:385; 1954, American Medical Association) 921 922 I IV. Pupillary Pathology: Symptomatology Table 17-2. The assumption of separate pupillary and visual fibrils in the optic nerve and tract YEAR AUTHOR 1880 1883 1885 1886 1887 1888 1888 1889 1889 1889 1889 1890 1891 1891 1893 1894 1894 1894 1894 1895 1896 1RQ6 1897 1897 1898 1898 1898 1899 1899 1899 1900 1900 1901 1902 1903 1903 1903 1903 1903 1904 1904 1906 1906 1907 1908 1908 1908 1910 1912 1913 1913 1913 1913 1917 1918 1919 1921 1922 1923 1923 Hedda eus Bechterew v . Gudden Moebius v . Gudden Moebius Snitzka Bernhardt Heddaeus Magnus Mendel Michel Hall Jessop Heddaeus Bechterew Heddaeus Henschen Krliger Lacqueur Lewis SiemerlinPBrixa Schirmer Baas Ine:elrans Schmidt-Rimoler Bach Bernheimer Dimmer Bechterew Levinsohn Kooczynskj Ruge Bodinski Kreuzfuchs Majano Marburg Thomas Bach Reichardt Ferrier Posev & Soiller Perier Lacqueur Mandelstamm Marx Weiler Goldflam Behr A. Lowenstein Magitot Morax Levinsohn Lutz Abelsdorff Heine Bielschowskv Bielschowsky Rowland fiber thiclmess resistance YEAR to damage thicker greater thicker greater thicker greater 1923 1924 1924 l::,,,q, l"UChS TaJ<anasni Dams & Coppez de Freitas Ribero liassin .mg-var Terrien & Hudelo Foerster Nemlicher Tieri Favory & al. Okuvama Soriano Spiller Bartels Gifford & Meyer Holzaptel ::ioriano Jaensch v . Bahr Hirata Jaenscn Voss Editorial, J. AMA Lodato .Parsons Schaeffer Behr Lagrange Stower YeaJ Janct Monbrun Rea Dollfus Duhamel & Umbdenstock Matteucci Parsons & Duke-Elder Dollrus Duke-Elder Pirot Francois & Deewer Bonnet Thiebaut & Helle Kyrieleis Schwebel ::sKoroaumova Cogan Miani Colombo Bolla Yoshioka Larmande & al . Schweitzer & Chior ino Guillaumat & al . Skorodumova Huber thicker greater greater greater thicker thicker thicker thinner thicker greater greater less greater greater less less greater thicker thicker thicker thicker thicker greater greater thicker thicker greater less less greater greater greater greater less greater greater greater 1927 1928 1928 1928 1929 1929 1930 1930 1931 1931 1931 1931 1932 1933 1933 19JJ 19;:!:3 1934 1934 1934 1934 1935 193 5 1937 11 937 1938 1938 1942 1948 1948 1948 1948 1948 1949 1949 1949 1950 1951 1953 1954 1954 1\154 1956 1956 1957 1957 1958 1958 1959 1959 1961 fiber thiclmess Wick Alexander Bramwell 1924 19;:;o 1927 1927 lv"', thicker thicker AUTHOR resistance to damage greater greater greater iess 1,rreater less greater PTeater greater greater thicker greater less greater less thinner thicker greater greater less greater less less e:reater less tmcker greater greater less less less 1,rreater greater thicker greater greater 17. Afferent Lesions Table 17-3. Supposedly blind eyes with intact pupil reflexes A. IMPERFECT - BLINDNESS Nagel 1871 1874 Schon Heddaeu s 188 0 188 3 Jackson Laqueur 1895 1896 Saenger 1898 Uhthoff 18 99 Piltz Levinsohn 1900 Cruchet 1903 1903 Roux Marburg 1903 1904 Reichardt 1914 Magitot 1919 Abelsdorff 1921 Heine 1924 Bramwell Behr 1925 Hirata Jaensch Duhamel& Umbdenstock B . OTHER CAUSES Baumeister 1873 1895 Gould 1896 Harlan 1897 Brixa Reichardt 1904 Liebrecht 1907 ~ Weiler 191 3 Magitot TIT4 Lonmann 1919 Abelsdorff 1924 Marquez 1933 Jaensch 1937 Stower Adrogue 1939 1942 Dollfus 1933 1933 1948 1954 1954 Schwebel Skorodumova 1933 rR . CORTICAL BLINDNESS Frohmilller 1876 18 91 Jesso2 Uhthoff 1898 Goldflam 1912 ,___ Behr 1913 Magitot 1913 >---Heine 1913 Adrogue 1926 D. MALINGERING & 1926 Danis &Coe:eez HYSTERIA Bodenheimer 1928 Wernicke 1894 &Korbsch 1897 Antonelli Foerster 1928 1902 Paggi .1WL Korbsch 1903 Roux Favory &al . 1929 ,___. Bielschowsky 1931 1922 Best 1924 Wiegmann Duhamel& 1948 Gifford &Mayer 1931 Umbdenstock C . SHORT TIMING Heoctaeus 1880 Roux 1903 Laqueur 1908 1908 Marx Bielschowsky 1923 1929 Favory &al . Jaensch 1933 ~ 198 5, Wertenbakeretal.: (?;n.r. 198 8 ). Table 17-4. The assumption of separate pupil fascicles in the optic nerve YEAR AUTHOR LOCATION OF PUPIL FASCICLE 1883 Bechterew 1888 1888 1889 Bechterew Spitzka Heddaeus 1890 1893 1894 1894 1896 --1900 Michel Heddaeus Henschen Kruger Bechterew 1901 1904 1907 1908 1920 1922 1923 1927 1927 1928 1931 1933 1933 1937 1949 1950 1956 1956 1957 Kopc zyanski Heddaeus Pe rier Marx Brown Terrien &Hudelo Wick Ingvar Terrien & Hudelo Ingvar Soriano v. Bahr Voss Yealland Dollfus Fram;ois & Deewer Cogan Miani Colombo Bolla from posterior chiasmal surface into walls of third ventricle (uncrossed} now believes fibers run in optic tract cited after Bechterew {1883} axial in optic nerve -+ walls of third ven tricle (uncrossed, after Bechterew) in papITio - macuiar bunale oi optic nerves same as 1889 media-dorsally in optic tract cited after Bechterew (1883 ) from cliiasm to wall oi third ventricle, but with chiasmal hemi-decussation "completes" his theory : fibers hemi-decussate in optic chiasm and travel in optic tract not describe d {"puEillary nerves"} as before "special fiber bundle" in optic nerve in papillo - macular bundle of optic nerves dorso - lateral inferior in optic nerve and chiasm cited after Bechterew (1883 ) on surface of optic chiasm and tracts inferior 172 of optic nerve same as 192 7 "dorsal position" in optic tract superficially in optic nerve cited after Ingvar not from nasal retinal half peripherally in optic nerve in nasal periphery of optic nerve predominantly nasal in optic nerve nasally in optic nerve nasally in optic nerve (in optic canal) Bechterew Jaensch / 923 924 / IV. Pupillary Pathology: Symptomatology Table 17-5. YEAR 1853 1863 1880 1886 1888 1889 1893 1896 1898 1898 1900 1901 1903 1905 1907 1909 *· Unilateral blindness or amblyopia as a cause ofanisocoria AUTHOR Seifert Billod Heddaeus Moebius Magnus Mag:nus Knies Baas Preston Schmidt-Rimpler Dejerine Baquis Marburg Babinski Perier Behr *+ + + + YEAR 1900 1913 1914 1916 1922 0 1924 0 ( ) 1924 _Q_ 1925 1927 0 + ( ) 1935 ( ) 1936 + 1939 + 1942 ( ) 1945 1949 + AUTHOR Weiler Behr zur Nedden Lenoir Bielschowsk:y Behr Fuchs Behr Velter & Tournal Behr Jaensch Adrog;ue Meesmann Berg Jones *0 + + + + + YEAR 1949 1950 1951 1954 1956 1958 0 + + + + + + + 0 1960 1961 1963 1964 1966 1969 1970 1972 AUTHOR * Duke-Elder + ,_ Bonnet ,++ Bonnet I~ Lowenstein ,+ Cogan Kadlecova & 0 Peleska Harms ,_Q_ + Huber + Jaeger + Duke-Elder I~ ThomEson Walsh & Ho;yt _Q_ Charles &Hamasaki 0 Rutkowski & 0 Thompson +==yes; O== no. uppo ed facicles never were demonstrated a natomically. (2) Pupillary reflexes cou ld be obtained by stimulation with mall beams of light directed to any part of the retina, not on ly to areas corresponding to the retinal representation of the various " pupil fascicles." Further, in patients with lesions in the retina optic nerve, or optic tract the visual and the pupillary field defects always matched. And pupillary reflexes could be elicited from residual islands of vision in any part of the field. The e facts showed that visual and pupillary fiber -if they were separate-had to be intermingled, so that for each retinal area pupillary and visual fibers ran closely together in their afferent course. The assumption of a discrete bundle of afferent fibers for the pupil thus did not stand up to scrutiny. Physiologic findings in recent years about retinal organization and fiber projections provided a clue to the probable explanation. As usual , things were more complex than had been thought in the past. It was established that there are at least three chief types of retinal gangl ion cell s. These differ in phylogenetic age, in size of cell bodies, thickness of axon fibers, and spread of dendritic fields; in thresholds of excitability, axonal conduction speed, and properties of receptive fields; in discharge patterns of impulses and in central projections. One class, the X or beta cells, project mostly to the forebrain. A second class, the W or gamma cells, pass thei r axon fibers to the midbrain; and the third class, the Y or alpha cells, supply both these areas. These different types of cells serve visual and pupillary reactions in different proportions. The main afferent input for the pupils is derived from the W cells. They do not serve vision in higher species that have cortical visual representation. In these species conduction of visual impulses has been taken over by the phylogenetically younger X and Y systems. Their fibers, chiefly directed to the lateral geniculate ganglion, have, however collateral branches to the midbrain; and some of these appear to serve the pupil. The vexing question of whether the pupillary fibers are separate and distinct from the visua l ones or whether they are collaterals of visual neurons in the retina was thus solved by the realization that both types of fibers exist (see details in Chapter 3). (b) Pupillary Equality Many authors will swear that the pupil of a blind eye is always larger than its normal fellow pupil (Table 17-5). For primates this is not true. In the absence of additional pathology affecting other parts of the pupillary reflex pathways, the pupils in a healthy eye and in its blind fellow eye remain equal at all times, at least within the limits of clinical observation. There are several reasons for the widespread belief that unilateral or asymmetric damage to the retina or optic nerve will cause anisocoria. (1) When the pupils are tested in Galen 's way by covering the two eyes alternately, as advocated by Kestenbaum and others, each pupil can be seen only when it reacts directly. Therefore the pupil in the good eye always looks small and that in the bad eye always looks large. It is easily forgotten that (behind the cover) the consensually reacting pupil behaves in exactly the same manner as the directly reacting one: the pupil of the bad eye constricts just as much as that in the healthy eye when the normal eye is stim ul ated; and the pupil in the good eye reacts as poorly as the bad one when the bad eye is exposed to light. Simultaneous records of the movements of the two pupils prove that the responses in the two eyes arc alike, and that no anisocoria is present (Figures 17-3 to 17-7). No doubt the catchy terms "pseudo-anisocoria " (Kestcnbaum) and "amaurotic mydriasis" have helped to spread the erroneous impression that damage to the retina or optic nerve will dilate the pupil of the affected eye. 17. Afferent Lesions / 925 (2) There is a time-honored belief that unilateral illumination will cause anisocoria in normal subjects. The pupil of the eye that receives more light is said to become smaller than the other pupil. This is indeed true in cat whose uncrossed fibers in the optic chiasm and in the midbrain hemidecussation of the pupillary pathway are incompletely developed. But in primates-as described more fully in Chapters 3 and 18-each pretectum distributes the afferent impulses it receives symmetrically to the ipsilateral and contralateral oculomotor nuclei , no matter which eye is illuminated. Since the intercalated midbrain neuron thus determines the distribution of all afferent input, lesions limited to the primary optic pathways that cause this input to be asymmetric do not lead to anisocoria in primates. (3) Only few cases with afferent pupil pathology are, however, uncomplicated, since often there is additional impairment of other pupillary neurons, caused by the same disease process or unrelated. Without graphic records it is difficult to understand the more complex reaction patterns, and the anisocoria is blamed on the afferent lesion because it is the patient s presenting condition. ln reality, however, the anisocoria may be due to -a third nerve defect on the same or on the other side (Figure 17-8); -Homer's syndrome on the same or on the other side; -damage to the iris or its nerve or blood supply, for example, by pressure in acute glaucoma, by inflammation, by ocular or orbital tumors, by ischemia, or by other local or systemic vascular conditions (cf. especially Charles and Hamasaki, 1970; and Rutkowski and Thompson, 1972); -other common (and often benign) pupillary anomalies that may be present in addition to the afferent lesion, such as consensual deficit (Chapter 18), or "simple, central" ("see-saw") anisocoria (Chapter 28; see Figure 17-9). O. lsec.➔ Figure 17-6. Pupillary reactions to light of low intensity in the same patient as in Figure 17-5,C. A: Light stimulation of the normal left eye. B: simulation of the affected right eye. The solid Jines represent the movements of the right pupil, the broken lines those of the left pupil. For each reaction the dens ity of the Wratten neutral grey filter used is indicated. The pupillary thresho ld was reach~d with 8 log uni!s of grey fi(ter for the left eye (A' and A'), an_d with only 6 lo~ units for the nght eye (B 1). With increasing bnghtnes of the hght fla hes, the latent periods of the reflexes became shorter and the contractions faster and more extensive. Comment: Note that no anisocoria developed at any time in this patient with u11complica1ed afferent damage. Stimulation of either eye produced the ame extent and shape of reflexe , provided that 2 extra log units of light intensity were g.ive n to the bad eye. Figure 17-7. Unilateral afferent deficit mistaken for hysteria. The patient was a 30-year-old woman. She had been nervous tired, and easily upset since she had been ill about a year bef~re with "asiatic virus." One month before examination she complained of pain behind her right eye, especially on looking around. In darkness she saw light flashes in her right eye that looked like "_a network of fibers." Shortly thereafter, she Jost all vision in her r~ght eye. Three days later vision began to return gradually. At the time ?f examination she was neurologically normal ; but she was c~ot1onally upset because of her vision and because of pending divorce procedings. Her complaints sounded somewhat bizarre. Sl~e said t~ings looked about equally bright with each eye, but with he;, nght ey~ everything looked "a horrible green, like a corpse. The patient could see red with either eye through red goggles, and ophthalmologic examination was entirely negative. Therefore her eye doctor thought that her complaints must be ~sychogenic in nature. The pupillogram, however, showed a distmct afferent defect on the right side: with bright light the reflexes ~ere slightly smaller, and had a longer latent period, when the nght then when the left eye was stimulated (A and B). Further, the pup11lary threshold was raised about I log unit in the right eye, c_ompared with the left: when the right eye was stimulated with light of low intensity (l.r.), the reactions were almost extinguished by a~ 8-l?g Wratten grey filter (C2 ), while for the left eye (I.I.) the fu~Ils still r!a_cted well (D 2). Filter d~nsity is given by the numbers 0 and 10 111 C and D. The nght-s1ded afferent impairment was confirmed by visual flicker fusion tests. 926 8 I --- IV. Pupillary Pathology: Symptomatology ----\ 6 ----~ I , 1-----1-\- .1---t--------------- -\-- 5 ~--------1--,- \ -----------•-----, ---,,,---,-\~-,-- ,_1 - -1' , _,,-~~-,,-----;" _\_ , -/'--'--------I I , ___ --,---"-----------1 lfl-----1 3 o.,sec.~ Figure 17-8. Damage to the optic nerve and to the third nerve on the same side (A) or on the opposite side (B). A: The patient, a 15-year-old boy, was struck by a car and lost consciousness immediately. When he was admitted to the hospital in deep coma his right pupil measured 5 mm and reacted to light, but the left pupil was dilated and fixed. There was right-sided plantar extension, and in response to pain stimuli, he moved his left extremities more than the right ones. His spinal fluid was bloody, but skull X-rays and bilateral carotid arteriograms were negative. He remained comatose for 38 days and then gradually improved. It became evident that he had right hemiparesis, left facial and oculomotor weakness, and left optic nerve involvement. On examination 7 weeks after the accident the tendon reflexes were increased and muscle strength decreased on the right side. The gait was atactic and association movements were poor. Vision was 20/40 on the right and 20/100 on the left side. The right optic disc was normal, the left pale but clearly outlined, and there were no hemorrhages. There was a central scotoma on the left side. Extraocular movements and corneal sensitivity were not impaired, and the EEG was normal. In room light the pupil of the left (bad) eye seemed dilated. But the pupillogram showed that the anisocoria was only apparent; the two pupils were equal in darkness, but the left pupil (broken lines) lagged behind the right pupil (solid lines) in all reactions to light and during near vision because of a residual oculomotor defect. In addition, both pupils showed only small, "low intensity" reactions when the left eye was stimulated. B: The patient was 15 years old. Four months before examination her vision was found defective in a routine check at school. She had a history of gonococcal vaginitis at the age of 4. Blood tests for syphilis were negative, and cerebrospinal fluid tests were normal. There was an apical heart murmur and enlargement of the left auricle and ventricle. She had never had any episode of motor, sensory, or other neurologic difficulty. She did well in school and got along with other children, was not nervous, and had only an occasional headache. The tendon reflexes were symmetrically increased, and abdominal and plantar reflexes were absent. Her neurologist thought that CNS lues was unlikely despite the early venerial infection. Ophthalmologic examination showed pallor of the left disk with marked constriction of the retinal arteries. Her right fundus was normal. Central vision was 20/20 in each eye, and the extraocular movements were full. The visual field of the left eye was constricted, especially in the upper nasal area. In room light the right pupil was larger than the left, but in darkness the pupils were equally large. The apparent anisocoria was due to right-sided parasympathetic deficit: the right pupil (solid lines, on the side of the normal optic nerve) reacted less extensively to light and to near vision than the left eye (broken Jines). Both eyes showed reduced reactions of "low-intensity" form when the left eye was stimulated. Comment: In both patients the pupils became unequal during contractions to light stimulation of either eye and during near vision. The anisorcoria was caused by efferent parasympathetic deficit, not by the afferent impairment. 17. Afferent Lesions / 927 In Table 17-6 we have listed such complications of the pupillary reflex pattern among one hundred patients with afferent defects picked at random (alphabetically) from our group of eighteen hundred unselected clinical cases. Note that only about 10% of these patients had no pupillary defects at all aside from their afferent impairment. Many of the complications were not of clinical significance; but each of them could cause anisocoria. 2. Lesions in the Optic Chiasm or Tract When the optic chiasm or tract is damaged "lowintensity" pupillary reactions to light and to darkness are found when either eye is stimulated. Depending upon the site of the lesion the residual reactions will be more or less symmetric when they are elicited from the right or from the left eye. But the direct and consensual light reflects are equal, and anisocoria will not develop as long as the pretectal decussation and efferent pathways remain intact and, of course, in the absence of other related or unrelated pathology (Figures 17-10 and 17-11). 3 '-- 2 --1-•------- (a) Behr's Pupillary Phenomena Behr (1909 and later) examined patients with optic tract lesions, and said their pupils were unequal, whereby the pupil opposite the tract lesion always was larger than the pupil on the side of the lesion. To explain this asymmetry, Behr assumed that normally all impulses from the nasal side of the retina served the direct light reflex alone (Figure 17-12,A'), and all impulses from the temporal retina served the consensual reflex alone (Figure 1712,A2). Only the foveal fibers reached both pupils. According to Behr, the afferent fibers reached the oculomotor nuclei without pretectal synapse. And since there were more crossed than uncrossed fibers in the optic chiasm, an optic tract lesion interrupted more fibers from the contralateral eye than from the ipsilateral eye. Consequently, Behr said, optic tract damage caused the pupil opposite the tract lesion to "dilate." Others agreed with Behr (Table 17-7,A), and his assumptions have survived as "Behr's sign of optic tract disease." However, they are incorrect. As described in more detail in Chapter 3, all afferent fibers of the optic tracts 1 ----1-------•---·l-------l---·I--------I .r. l.r. l.r. l.r. Ill II --c ~ ~l~J __________ ~D-~ II 1111 5 ,__ 4E 3 ,__ E 2 t ---1--------1---1-------·•--- ~,- [I] ______ , :--:/-''.'.'.::::::::==::::..J ------- --1--L ,,,,, ..... --~ ....,,,..,,,,..,,,,. -d--t--------1---1-------'l--c--:--t-------l-;--;-·t-------~ d.r. .r. II 0.1sec ---~~----l- 1 --1------ Ii d.l. 11111 \.,._,,,... d.l. 111 1111 ...... Fit,>ure17-9. Asymmetric afferent impairment with anisocoria, due to multiple sclerosis. The patient was 35 years old. Four years before examination, she had had an episode of left-sided visual loss, lasting a few days. Three years later there was a second attack, leading to blindness in her right eye within a week, and in her left eye more gradually. Again there was improvement, and within 4 months she saw well enough to read. Six weeks before examination vision went down again; it was lost entirely in the right eye and was reduced to finger counting to the left eye. There was bilateral optic atrophy, but no other ocular defect. Serologic tests of blood and spinal fluid were negative. Neurologic examination showed symmetrically overactive tendon reflexes, left-sided plantar. extension, a positive Oppenheim sign on the left, absent abdo.minal reflexes on the right, and a positive Hoffmann sign in the nght hand. Sensory and motor functions were intact. There were coarse nystagmoid movements of both eyes and active pupillary unrest. No reflexes to light or darkness could be elicited from t?e right eye (A and C), and only inextensive "low-intensity" react10ns from the left eye (Band D). The right (worse) eye had a larger pupil (solid lines) than the left (broken lines). Comment: Note that the anisocoria was more marked in darkness than when the pupils contracted to light. It was not due to the afferent asymmetry, but was a coincidental "simple, central" (see-saw) anisocoria (see Chapter 28). Table 17-6. Complications of pupil syndrome in 100 randomly selected cases with afferent deficit CASE MAL'! DISEASE IAFFE- 1.Bfilf!:. R syphilis; cerebral hemorrhage L CNS lues; 2° glaucoma 2 ~55 -3-i"25°" OU basal arachnoiditis -4-53 R congenital syphilis L CNS lues c:?52 5 L retinal vascular disease 6 ~50 L 31 retinopathy 7 OU MS ~rain stem involved) c:?26 8 L glaucoma; psychoneurosis 9 ~43 1() 26 MS, leading to death OU OU 11 cno embolus R middle cerebral A. R 12 0"52 MS L acute glaucoma attack OU 13 0"29 R head trauma 1!_&13 diabetes insip.; autonomic fits R 15 22 <Su 16 c:?63 chronic simple glaucoma OU retinitis p!gmentosa; deaf mute 17 0"42 R l&_Q-12._ meningitis serosa _R_ _!_LQ..1§___MS OU luetic optic atrophy 20 0"'61 21 0"'20 CNS lues, optic neiritis OU 22 0"31 hypertension; cerebral hemorrJ:J. OU L 23 0"32 MS 4 64 CNS lues OU -- chronic simple glaucoma OU ---RMS; narrow angle glaucoma parasellar mening:ioma n__gj5 28 20 glaucoma; hypertension L ~ -4retinitis pigmentosa -----y:;prrmary srmple glaucoma 30 0"5ll lues nervosa R n..__~28 32 39 meningioma tubercul. sellae OU food poisoning ~ 38 R --L,v,,... 34 ~24 -L25 MS 36 0"'44 tabes dorsalis; optic atroph,r: MS cITJ E_i46 38 53 MS OU °39"" 58 head trauma R 40 <Y.31 septic brain embolus OU 41 0"38 MS 42 cn,5 pinealoma OU* 43 0"'52 chronic simple glaucoma 44 0"'21 MS OU 45 CJ'27 Fuchs's heterochromic cyclitis R 46 0"'26 meningitis (filariasis) OU L il..._8 48 lues cerebri 1§__ 40 MS R vascular hypertension R il.__~~ --R50 32 retrobulbar neuritis 1 0"50 fi=ffi= ar- iris ou ou ou ou ou -- -- COMPLICATIONS ACA effer. ~ ~ -++ + -++ -++ + -+-+- OU R ou -R- --OU '""au -- + + NON~---NONE =1 + 1==1 NONE OU -- -R- y- --- -- --++ -- -- -- -- I+ --- ----- -- --+- -- -- + -+-+-+-+-+- ------- ---+----------------irNO"1E ________ --+- ---- --+- -- -- -- ------- -+------NOl -- -- -R- -- '""au -'""au -------E-- -- --- --- --------- --+- --+ -+--+-- I I I I + NONE R°"" -+- --?- = ou + ---+-- -- -- --- + OU==== ----NON_E__ --- ACA = alternating contraction anisocoria; effer. = efferent (parasympathetic) symp. = sympathetic deficit; supr.= supra-nuclear deficit; OU= bilateral. + -+--- MAIN DISEASE glaucoma; hypertension CNS lues; 20 g:iaucoma syphilis; hypertension MS §!__i2 55 43 MS 56 63 essential hypertension ~ -8MS ~~6 narrow angle glaucoma 59 4 MS head trauma 60 0"'57 61 0"54 aneurysm ant. communic. A. 62 0"24 MS MS ~g.32 CNS lues; 3 strokes 64 71 MS §.§_&41 66 44 syphilis; optic atrophy 67 0"63 pituitar,r: adenoma , 68 0"42 acute glaucoma OD MS 69 ~38 70 43 chronic irido·<!_yclitis 71 0"53 L orbital pseudotumor 72 0"'30 tapeto-retinal degeneration congenital sypnilis 73 0"17 74 0"23 schizophrenia chrom. pituitary adenoma ~l60 76 36 retinitis pigmentosa 7'7 47 MS ~ 54 primary simple glaucoma retinitis pigmentosa; deaf 22_~6 80 45 basal meningioma \tract) ~ -;;r- lues cerebri retinitis pigmentosa; epilepsy ~g.4o 83 52 lues cerebri, optic atropliy 84 ~38 head trauma; choroiditis L §_§___8.n._ head trauma 86 21 MS (junction scotoma) 87 <).55 CNS lues 88 0"56 hypertension; glaucoma thromoos1s post. cerebral A. narrow angle simple glaucoma 91 36 epileps,r: 92 0'57 brain stem g:lioma (hemianops.) 93 0"53 CNS lues 94 0'25 MS 95 0"36 post virus encephalitis 51 52 53 ou ___QQ_--OU CASE --- + + --deficit; ~~ 0"53 0"'51 0"'58 f;! ~9-1L 97 98 99 100 0"32 0"'60 c:?38 <:;;24 AFFECOMPLICATIONS RENT iris ACA effer. ~ supr. + R --OU -R-R ou -- -- OU OU R L R OU R OU -R- -L- R OU OU ou -R- _L_ OU L OU L R* OU OU OU R OU = --- ------ ou -- NONE __+_ --+ ==== --- --- -- --R- -- -- -+- --- -- -- -- -R- ~ -- -- + -- --- -- --- --L- --- + --+- NON_E__ --- --- -- -+- - -____ -- --- ____ -L--- ou -- OU --- -- -- -- -- -- ----L-~ -- ------ --L-L- -- ou ou -R- ~ L L ou ou ou OU -L- L°" -+- + -+- ou -ou ____-- R ====== L OU -OU? ------NON_E__ ---+---+- ----- ~ -- + + -+---+--+- -- -+- --- 7TIJ ----- --- --- --- 75u --- --- --+- -- --- --- ou --- -- --- --- -- -+- + + + + __ --____NON_E ou ____--------____ ou ____-+- -- ----- -R -OU* -MS L -syphilis; retinal c,r:st R -meningio-vascular brain svohilis OU -MS OU --------y;r-post virus infection -- -ua- ---- -+--+-+- NON_E__ ----+--+- ~ ---- --- --- ou --- -- -+- -- ou -ou ------ --- -- -- + 17. Afferent Lesions / 929 t t t .s i.. ~ 5 ~ t 'f -~ ~ 7 ~6 ~ .~ ..., 5 Q... i 'f Tc.me i.n. 0.1 .second. ➔ Figure l 7-10. Chiasmal lesions. A: Pituitary adenoma. The patient was 61 years old. He had been well until 2 years before examination, when he began to have trouble with vision when driving at night. Four months before examination reading became difficult. He had no headaches, and the neurologic examination was negative. Ophthalmologic examination showed slight paling of the papillae, with deep physiologic cupping and bitemporal hemianopsia for 1/IOO0white targets. The retinal vessels were normal, and there were no signs of increased intracranial pressure. X-rays showed the sella to be enlarged, with a thinned dorsum. The pupils were equal. When either eye was stimulated by light or darkness, the pupillary renexes had "low-intensity" form, while the reactions to near vision and to sensory stimulation were normal (not shown). On surgery, a huge pituitary adenoma with a thick capsule was excized. Postoperatively, vision improved in the temporal fields. The patient was left with a bilateral upper quadrantic field cut. Comment: In this patient the tumor had not expanded into the cavernous sinus, and the oculomotor nerves remained intact. There was no anisocoria. B: Retrobulbar neuritis with junction scotoma. The patient, a 19-year-old college student, was well until 3 weeks before she was admitted to the hospital. She first noticed a queer sensation, beginning in her feet and gradually ascending to her waist. When she stepped into her bath she did not feel the heat of the water and was surprised how hot it was when it reached above her waist. She developed pain in her left eye on lateral movement. Within about 5 days vision became blurred, and in another 2 days the left eye was blind. During the last 2 days before examination she had an ache in her right eye during eye movements. The tendon reflexes were hyperactive (R > L), with bilateral ankle clonus. There was bilateral reduction of vibration sense from the toes to the iliac crest. Otherwise sensory and motor functions were intact, and there were no meaningeal signs. The spinal fluid contained 72 WBC on admission and 20 WBC 5 days later, but it was normal otherwise. The left disc was paler than the right, and blurred in the upper nasal part. The right disc was within normal limits. Vision was reduced to light perception in the left eye, and to 20/50 in the right eye, with a junction scotoma, indicating a lesion just anterior to the chiasm on the left side, catching the crossed optic fibers from the right eye together with all optic fibers from the left eye (BS). The pupils showed "low-intensity" reflexes to light and to darkness when the right eye was stimulated, and only traces of reactions when the left eye was stimulated (stray light to the right eye?). Comment: Note that the right and left pupils remained equal at all times. Junction scotoma is due to tumors in the majority of cases. In this patient, however, the onset was sudden, with signs of optic neuritis. Further, there were additional neurologic signs which receded during the next months. And while vision failed to improve, no further deficit developed during the next 2 years. (From O. Lowenstein, Arch. Ophrhal., Chicago, 52 [1954]: 385; 1954, American Medical Association) 930 / IV. Pupillary Pathology: Symptomatology synapse with intercalated neurons in the pretectal area, and these (in primates) distribute the afferent messages symmetrically to the oculomotor nuclei on the two sides (Figure 17-12 B). In fact, Behr's dogma was not even supported by his own cases, nor by those mentioned by others who agreed with him, as was pointed out by Weve in 1919 (Table 17-7,B-C). Behr emphasized that the anisocoria he oberved wa much more marked in dim light than in bright light (Table 17-7,B). This would be quite impo sible if the inequality were due to damage in the light reflex arc. This feature raised the suspicion that the sympathetic fibers on the side of the tract lesion might have been injured in their intracranial course in the ophthalmic branch of the fifth nerve, or within the central sympathetic neuron. Indeed, the palpebral fissure of Behr's ca e numbered ] (1913) wa found to be smaller on the side of the lesion than on the other side. In tead of realizing the implication of this telltale finding, Behr adopted "an enlarged palpcbral fissure opposite the lesion" as an additional sign of optic tract disease. There were several other reasons why anisocoria could have been found in Behr's patients, who had brain syphilis with sluggish pupils; or tumors, or trauma with high intracranial pressure and signs of large areas of destruction, or distortion and rotation of the brainstem. Table 17-7,B lists all cases with adequate detail which we could find that were published to support Behr's theory. In light of this meager and faulty material, it is surprising that "Behr's sign of optic tract disease" has been accepted so widely up to the present (see Chapter 3). Table 17-7. A. "BEIIR'S "Behr's signs" SIGN" OF OPTIC TRACT DISEASE ( ANISOCORIA) YEAR AUTHOR * YEAR 1909 1910 1910 1910 1911 1911 1913 1913 1914 1918 1919 1920 1922 1924 1924 1925 192(3 1927 1927 Behr Best Krusius Weiler Behr Bumke (B} Behr Schlesinger Schlesinger Weve Weve Best Oloff Behr Parsons @ Behr Behr Pastore Wilbrand & Behr Bunge Dieter Schlesinger Weiss Engel Lutz Bing + + 0 0 + + + + + 0 0 + + + + + + 0 1931 1928 1928 1928 1928 1930 1930 1931 + + +** + + + 0 0 AUTHOR Best Noromann 1931 1931 Soriano Weill & 1933 Nordmann von Bahr 1933 Behr 1935 Jaensch 1936 1936 Meller Demmler 1938 Duke-Elder 1949 Lowenstein & al. 1950 """IT5ir Lowenstein Skovodumova 1954 1956 Cogan Smith 1962 1966 Tliom12son Herman 1975 Bell & 1978 Thompson Savino & al. 1978 ~ O'Connor & al, (a&b} 1982 Thom12son Loewenl'eld 1983 Newman & 1983 Miller --* -0- + 0 0 0 + + + + + 0 0 0 + + 0 + 0 0 0 0 0 0 In columns *, + means yes and O, no; ** seemed to agree with Behr, but his own case did~show the sign; (B) = cited after Behr ( not sure of own opinion). Figure 17-11. Optic tract lesions. A: Pituitary tumor. The patient, a 41-year-old man, had blurred vision for 5 years, and recently had started to "faint." These periods of unconsciousness lasted about 5 minutes. He had no headaches or other complaints but his face and extremities looked distinctly acromegalic. Ophthalmologic examination revealed bilaterally pale discs and left homonymous hemianopsia. Neurologically, there was a left central seventh neive paresis, and the abdominal reflexes were absent. Skull X-rays showed the sella to be enlarged and the anterior and posterior clinoid processes destroyed. The pupils reacted poorly to light, with "low-intensity" reflexes. On operation, an eosinophilic pituitary adenoma was found. Comment: Despite the large size of the mass the third neives were normal. Extraocular movements were full and there was no anisocoria. B: Syphilitic basal meningitis. The patient was 59 years old. At the age of 19 he had been infected with syphilis and had been treated incompletely. A few days before examination he suddenly noticed that "his left eye was almost blind." He had increased deep tendon reflexes on the right side, with slight weakness in his right leg. The cerebrospinal fluid was positive for neurosyphilis. The patient had left-sided hemianopsia, splitting both maculae. The pupils were equal, and the reflexes to light and darkness reduced, with "low intensity" shape. There was no anisocoria. C: Right parietal tumor. The patient, a 53-year-old man, had undergone surgery for a large parietal tumor several years before examination. He had left hemianopsia with macular sparing. His pupils were equal and reacted normally to light and to darkness. Comment: In this patient the lesion was supra-geniculate, and it did not affect the pupillary reactions to bright light. (From 0. Lowenstein, Arch. Ophthal., Chicago, 52 [1954]: 385; "1954, American Medical Association) 17. Afferent Lesions / 931 Table 17-7 (continued) B. CASES SAID TO PP0VE THE EXISTENCE OF "BEHR'S SIGN" (ANISOCORIA) OF OPTIC TRACT DISEASE LESION CLINICAL DETAILS PATIENT PUPILS YEAR AUTHOR the the patient had lues cerebri and very high intracranial pressure; R o" 49 R<L 1909 Behr right pupil was sluggish the pupil and palpebral fissure were R) L;; anisocoria was more L cJ' 29 R>L 1913 Behr #1 marked in dim than in bright light; 5D papilledema OU, and}50 mm intracranial pressure; on autopsy, a huge tumor was found between the pons and the temporal lobe left", and the brain stem was rotated and distorted #-2-cf' 21 the left eye was blind, with finger counting right in the nasal ~ield; the L)R R>L sella was enlarged, the clivus destroyed; 450 mm intracran1al pressure and bilateral proptosis (worse on the left side); divergent squint; the anisocoria was much more marked in dim than in bright light lucs cercbri, 450 mm intracranial eressure, and eaeilledem_a OU . 1914 Schlesine:er#l cJ' 32 R L>R normal fundi with macular sparing (8-year history); L hem1anesthesia, !til a'~ R L>R L hemiearesis and hemiataxia, left 7th nerve earesis bullet into soft palate, stuck in left parietal lobe near midline; L pyra #3 (? 25 R L>R L midal signs, L sensory loss with hemiathetos is and hemiataxia; hand was coe,ler than the right; sweating R < L, and R vascular reflexes abolished 1922 Oloff both eu2ils were sluggish; no details (2atient was shot in the head) 7Jf37 R L>R review of previous findings and opinions on pupil and palpebral fissure 1924} Behr 1926 bullet entered the soft palate, R of midline -+ lesion included R Behr #1 cJ' 37 L>R R peduncle, R optic tract and rostral midbrain; had hemiplegia and sensory deficit on the left side, left hemianopsia, left 3rd nerve earesis and left 7th nerve earesis #2 R) L L> R the left eye was blind, the right had temporal field loss; signs of ~ 40 hypophyseal tumor; the clinoid was destroyed, and there was 450 mm intracranial pressure; the pupil reflexes were abolished on the left side and hemianoeic on the right #3 aiter stroke, had left hemiplegia and 7th nerve paresis, pupillary R L>R ~ 53 hemiano2ia but no visual hemianoeia 1928 Dieter cf' 54 500 mm intracranial pressure, vascular hypertension, and myocarR=L R ditis; sudden L hemianopsia and pupil hemianopsia; the pupillary defect disappeared then the intracranial pressure was dropped, while the visual defect 2ersisted 1928 Weiss R(L '?J' 16 stab wound via right orbit and superior orbital fissure, with hematoma; R right hemianopic pupillary deficit and visual defect, left pyramidal and sensory impairment - D =diopters; OU = both eyes; also text). - Note that Schlesinger's case #2 and Dieter's Another feature of optic tract lesions mentioned by Behr has, however, been substantiated since (Table 17-7,D). In patients with optic tract hemianopsia, stimulation of the eye with the functioning nasal retina causes more vigorous pupillary reflexes than stimulation of the eye with the intact temporal retina. This is not difficult to understand, first, because the temporal visual field (nasal retina) is larger than the nasal visual field (temporal retina); second, because the concentration of receptors and of ganglion cells is denser in the nasal than in the temporal retinal half; and third, because more afferent fibers cross in the chiasm than remain uncrossed, not only in lower forms but even in primates (Figure 17-13, and Chapter 3). (b) ''Wernicke's Sign" or Pupil Hemiakinesia According to Sachs, von Graefe first noticed that the pupils failed to react to light stimuli directed upon the blind part of the retina in patients with optic tract lesions. Wilbrand (1881) and others mentioned this, and Wernicke ( 1883) brought it to general attention (Table 17-8). Thereafter, may authors observed the hemianopic case undoubtedly had cerebral blindness (see pupil defect due to optic tract lesions; but others could not find Wemicke's sign. This was due mostly to technical difficulties: when the test was done with bright light, there was sufficient scatter within the eye to cause good pupillary contractions even when the light beam was directed upon the blind retinal half. Those who did careful studies with less intense stimuli had no trouble demonstrating hemiopic pupillary field defects. 3. Lesions in the Afferent Pupillary Fibers beyond the Optic Tract The pupillary fibers do not run with the optic fibers that enter the lateral geniculate nucleus. Instead, they pass to the pretectal area by way of the superior collicular brachia. Therefore Knies in 1893 suggested that damage in one brachium should cause pupillary hemiakinesia without visual loss. This idea agrees well with the fact that cutting both collicular brachia in cats and monkeys abolished the light reflex (and unilateral lesions caused hemiopic pupillary deficit) while the animals could see well in all parts of the visual field 932 / IV. Pupillary Pathology: Symptomatology Table 17-7 (continued) C.CLI ICAL OBJECTIONS TO "BEHR'S YEAR AUTHOR STATEMENTS 1881 Wilbrand 1 1910 Martius Krusius 1910 Weiler 191 Weve 1919 1927 Weve 1930 Lutz 1931 Nordmann 1931 Soriano 1933 von Bahr 1931} 1933 Weill & ordmann 1954 Lowenstein 1978 Bell & Thompson Savino & al. 197 19 3 19 3 Pastore !Newman Miller DUE TO OPTIC TRACT DISEASE the optic tract and third nerve may be involved in the same lesion; in such cases the pupil becomes large and sluggish a eatient with a tract lesion and a third nerve lesion on the same side has an enlarged, sluggish eueil could not corroborate Behr's statements; an enlarged pupil and palpebral fissure opposite the lesion are Jl2l related to tract hemiano2ia the pupil edge is difficult to see in darkness; hence, when the eye on the side of a tract lesion is illuminated, the oeposite pupil may seem to be the larger one in 23 normal subjects with alternate stimulation of the nasal and I.he temporal retina the direct and the consensual pupil reactions were equal 13 times, the direct reaction was greater than the consensual one 7 times and smaller 3 times normally the reactions to nasal and to tem2oral retinal stimulation remain R =L in patients with optic tract disease "Behr's phenomenon" of a larger pupil opposite the lesion is NOT alwavs found <f 21 with bin as al hemianopsia: both pupils reacted to stimulation of either eye ( Behr had claimed the temporal retina supplied only the onnosite pupil and the nasal retina only the ipsilateral oupil, saw 5 cases witn nypopnyseal tumor and one temporal retina functioning; according to Behr, this area supplied the consensual light reflex only, and light stimulation should cause the pupil of the blind eye to become smaller than the one in the seeing eye. Three of the patients showed this, but in the fourth the pupils remained equal, and in the fifth the opposite happened: the pupil 2.LJllll. seeing; e:i,::ewas smaller than that of the blind one thought anisocoria, if present, Could NOT find Behr's anisocoria in a single case of tract hemianopsia: to be due to some other mechanism found that both the temporal and the nasal hemiretina elicit both direct and consensual light reflexes; and stimulation of either hemiretina alone did not 11roduce anisocoria among 3 cases of verified optic tract lesion the larger pupil was opposite the lesion in 2, and on the side of the lesion in one; among 5 additional cases without autopsy, 2 agreed and 3 disagreed with Behr's theorv lesions in the optic chiasm or tract do not cause anisocoria in man , in the absence of additional impairment of other pupillary neurons 4 patients with isolated optic tract among 21 patients with optic tract and 2 had a larger eueil oeeosite & of 10 patients Loewenfeld D "BEHR' S PUPIL": ANISOCORIA" summary with optic tract on "Behr's" PREPONDERANCE lesions did not have Behr's anisocoria lesions only 3 had anisocoria; the tract lesion lesions one of these had posterior synechiae, none had anisocoria signs. OF NASAL COMPARED TO TEMPORAL RETINA YEAR AUTHOR FINDINGS 1913 Behr 1924 Behr, pp 64-65 1926 Behr 1928 Schlesinger 1933 von Bahr 1935 0sterberg 1963 Van Buren stimulation of the nasal hemiretina elicited more extensive pupillary contractions than stimulation of the temwral hemiretina (patients with optic tract disease) in patients with optic tract lesions, stimulation ot the eye on the side ol the lesion (crossed chiasmal fibrrs surviving) elicited more extensive contractions of both pupils than stimulation of the o:Qposite eye (uncrossed chiasmal fibers surviving) $? 53 with apoplexy (lesion R): when light was on the left eye (uncrossed chiasmal fibers surviving) both pueils were larger than when the light was on the right eye ( crossed chiasmal fibers) agreed with Behr: when the eye with the greater field loss ( due to an optic tract lesion) was stimulated (opposite the lesion), the pupillary threshold was lower than when the eye on the side of the lesion ( with less field loss) was stimulated both pupils were contracted more effectively by stimulation of the nasal hemiretina than by stimulation of the temeoral hemiretina ( but the eueils remained equal) found both rod and cone densities somewhat greater in the superior nasal than in the inferior temporal retina (man) found that the human retinal ganglion cell layer reaches nearly twice as far on the nasal side of the fivea than temporal to the fovea 1967 Kupfer, Chumbley &Donovan Bell & Thompson 1978 1982 1982 Savino, Paris, Schatz, Orr & Corbett Thomeson O'Connor & al 1983 Newman & Miller 1978 found that more fibers cross in the human chiasm than remain uncrossed 4 cases with isolated optic tract hemianopia:. the pupils contracted more readily when the eye on the side of the lesion was stimulated (larger temporal visual field, and crossed ehiasmal fibers) than when the other eye was stimulated of 21 patients with optic tract lesion, 14 had substantially asymmetric or unilateral loss of acuity; these patients had less extensive pupil contractions upon stimulation of the eye with the JX>Orer acuity, but the eupils remained equal in most cases discussion of O'Connor and co-workers' eXQeriment (1982; see Cha2ter 3) 3 patients with partial optic tract hemianopia due to thalamic lesions ( 2 after vascular accident, and 1 with an infective cyst) had relative afferent defect when the eye opposite the lesion was stimulated 10 patients with optic tract lesions and homonymous bemianopia: 8 had relative afferent defect when the eye opposite the lesion was stimulated; none had anisocoria 17. Afferent Lesions Figure 17-12. Behr's theory about the effect of optic tract lesions. Behr assumed that all pupillary fibers in the optic tractfrom the nasal retina of the contralateral eye and the temporal retina of the ipsilateral eye-were funneled to the contralateral oculomotor nucleus without a central synpase (A' and A2). Only the foveal fibers (indicated by the thin broken lines) were said to bifurcate in the midbrain and to reach both phincter nuclei. This was a sumed to answer the question of why in patients and animals with sagittal ection of the optic chiasm both direct and conensual light reflexes could be obtained when either eye was stimulated alone. After damage to one optic tract (as indicated by the double-lines) more afferent impulses would therefore reach the B Right Left Right r ,/ - ,--✓ Time 111 Seconds Left / 933 sphincter nucleus on the side of the lesion than the nucleus on the opposite side. The pupil on the side of the lesion (that is, on the side of the good half-field) would therefore become smaller than the other pupil, no matter which eye was stimulated. Behr's scheme is erroneous because the fibers of the optic tract synapse with intercalated neurons in the pretectal area of the midbrain; and in primates the fiber distribution of the pretectal neuron to the ipsilateral and the contralateral third nerve nuclei is symmetric (B). Optic tract lesions do not affect this pretectal fiber di tribution, and for this reason they do not cau e anisocoria in normal monkeys and man. Figure 17-13. Pupillary effects of complete optic tract lesion. A: Normal visual field measurements with a planimeter showed the area within the temporal field to be larger than the area within the nasal field by the following percentages: isopter 4, 71%; isopter 3, 72%; isoptcr 2, 61%; and isopter I, 68%. Note that the nasal field was smaller even with the smaller i opters, where screening part of the field by the nose could play no role. B: Pupillogram of a 26-year-old man with complete right hemianopia, that is, lo s of the temporal visual field of the right eye and the nasal visual field of the left eye. The lesion was due to surgical injury to the left optic tract during biopsy for a low-grade astrocytoma in the left diencephalon. The patient's remaining temporal lield in the left eye (nasal retina and crossed optic fibers) was 75% larger than the nasal field in the right eye (temporal retina and uncrossed optic fibe~s) with the ] 4 isopter, and 60% larger with the 12 isopter. The pup11la:Yr_esponsescorresponded to this finding: both the left pupil (solid lme) and the right pupil (broken line) constricted each time the st!mulating light was shifted from the right to the left eye, and th_eydilated when the light was shifted back to the right eye. The ~1ffcrence could be compensated for by inserting a 0.4-log grey filter 11110 the stimulus light path to the left eye. Comme111: Ac~ordmg to Behr's theory the patient's right pupil (opposite the !es1on) should have_bcen "enlarged" and its contractions to light 1mpa1rc?. Actually 11was smaller than the left pupil. Further, it was obvious that the anisocoria in this patient had nothing to do wnh afferent ~onduction, since it was slightly more pronounced ~hen the pupils were large due to reduced afferent input (light nght) than when they were small due to increased afferent input (light left). (From R.A. Bell and 1-1.S.Thompson, Amer. J. Ophlhal., 85 l 1978]: 538; published with permission from The American Journal of Ophthalmology, " The Ophthalmic Publishing Company) 934 JV. Pupillary Pathology:Symptomatology / Table 17-8. YEAR --1879 1881 1881 1882 1883 1883 1885 1885 1885 1885 18 6 1 7 1887 1888 1888 1888 1890 1890 1890 1890 1891 1891 1892 1891 1891 1893 1893 1893 1893 1894 1894 1894 1894 1894 1895 1895 1895 1895 1896 1896 1896 1896 1897 1897 1898 1898 1898 1899 1899 1899 1899 1899 1899 1899 1899 1900 1900 1900 Pupil hemiakinesia in patients with optic tract impairment AUTHOR v. Graefe Leeser Wilbrand Schueller Wernicke Heddaeus Bernhard Hirschberg Seguin Uhthoff Seguin Seguin de Schweinitz Leyden Magnus Martius Dercum Henschen Remak Wilbrand Jessop v. Leyden v. Leyden Oliver Story Knies Samelsohm Uhthoff Wernicke Henschen Muschin Peretti Rothmann Samelsohn Eales Evetsky Knies Thomas Baas Broadbent Eskridge 022enheim Goldscheider Rudier Ahlstrom Preston Rudnier Kem12ner Knotz Koster Liebrecht Saenger Schwartz Weiss Wolff Dejerine Dercum Kipp * : += yes; - +* -+ - + + + _Q_ _Q_ + + + + + ,_Q_ + YEAR AUTHOR 1900 1900 1901 1901 1901 1902 Salomonson + Silex 0 Ballaban + Thompson + Vossius + Hirschberg + ~ Nonne + 1903 Marburg + mi3 Stoewer + -+ 1903 Vossius Friedlaender & 1904 + Kempner -+ 1904 &Iiwartz 1904 Wolff + 1905 v. Monakow 0 1'9'oo"""Ferrier + 1906 Morax + 1906 Posey &Spiller + Dupuys -Du temps 1907 + & Lejonne 1907 Lavastine + 1907 Morax + 1907 v. Rad &Neuberger + 1907 Sachs + ms Best + I9o9 Benr + ,_ 1909 Hesse + 1910 Best + 1910 Krusius + 1910 Sachs - ++ 1910 Weiler 1911 Bumke & + Trendelenburg -+ 1911 Bumke 1911 Jess ,-+ 1911 Vogt + 1912 Goldflam + 1912 Jess + 1912 Schlesinger + 1912 Sulzer &Chappe ,_+ 1913 Beiir ,-++ 1913 Clausen 1913 Jess ,_+ 1913 Morax ,-++ 1913 Schlesinger 1913 Walker ,_+ 1913 Wood ,_+ 1914 van Bouwdijk & + Bastinaase 1914 Dejerine ,-+ Dejerine & 1914 + Jumentier 1914 Heed &Price + 1914 Schlesinger + IQ 1914 Ulbrich ,1914 Walker + 1917 Klauber + 1917 Wilbrand &Saenger + + + + 0 + + ,_ + ,- + ,_ + + ,_ ,- + ,- ++ I~ -+ - + + --;- -+ - + + + + + I~ ,- + + -- + ,- '7) + I~ + () -+ + ,- + + - -- - ++ -+ -+ -_Q_ -+ + * - -- YEAR AUTHOR 1918 1918 1919 1920 1920 1920 1921 1921 Lutz Wevt: Jeliffe &White Best Levinsohn Oloff Black Groethuysen !Mr Heine 1922 Bielschowsky 1922 Fuchs 1922 di Marzio 71J2"2"Oioir 1922 Vogt ~ Lillie "T923 iJlitiioU 1924 Bussy 1925 Behr 1925 Lenz 1925 Hessberg 1926 Danis & Coppez 1926 Behr 1926 Favory 1926 Schlesinger 1927 Bozzoli ""I9'27" Lutz 1927 Lauber 1927 Pastore ""I9'27" Seguini l92"i" Wilbrand &Saenger 1928 Bu!!&e 1928 Dieter 1928 Lutz 1928 Magnus 1928 Sciiies mger 1928 Suva 1928 Weiss 1929 Barbieri 1929 Igersheimer 1929 Jess 1929 Mackenzie 1929 Plaza & Luque 1930 Engel 1930 Lutz 1930 Mylius 1930 Soriano 1931 Braun 1931 Esser 1931 Nordmann 1931 Pellathy 1931 Schmelzer 1931 Soriano 1932 Braun 1933 Weill &Nordmann 1933 Wessely 1934 Biffis 1934 Parsons 1935 Behr ,_* ,_+ ,_+ ,_+ ,_+ ,_+ ,_+ ,- ? ,_+ ,_+ ,_+ ,- + YEAR AUTHOR 1935 1936 1936 1936 1936 1937 1938 1938 1938 1938 1938 1938 S¢'rensen Harms Jaensch Meller Pomfret Yealland Alaimo Frensberg Monbrun Rea Strebel Terrien + '-=i='"~ Adrogue ,1939 Muller ,-++ """i940"" Frydrychovicz & Harms ,-+ 1941 Le Gros Clark ,_ + 1942 Meesman + 1945 Berg ,_ + 1943 Berens &Zuckerman + 1949 Duke-Elder + 1949 Sautter + ·1950 Sautter + ·+ 1951 Bonnet 0 ~ Harms 1951 Smirnov + 1953 Sautter + 1954 Dubois -Poulsen &al. + 1954 Kyrieleis + + "TI54"" I:owenstern Skovodumova + 1954 + 1954° Tamura Yosnitomi + 1954 1956 Cogan + + -n'56 Samojiov +'"' """TI5lr Sokolowski &Segal - + 1958 Samojloff & Shak:hnovich + + 1959 Guillaumat 1959 Samojlov + 1960 Battistini + 1960 Morone + '+ 1000 Samojiov '+ ~ Siialrniiov1cii '+ ,_ 1961 Battistini ,_0 1962 Kornyansky & Shakhnovich ,_+ Bodechtel ,- + 1963 ,_+ 1964 Bodian ,_+ 1965 Harms 1969 Bresky &Charles + '+ 1970 Sugita & al. ,_ ,_+ 1971 Jay le & al. 1972 Harms & al. ,_ + 1973 Narasaki & al. ,_ + 1973 Narasaki & Noguchi ,_ + 1962 Smith ,_ + + 1979 Corbett & al. - - - * + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + -- + + -- + -- + + + + + + + + + + 0 = no. Additional reports: von Bahr, 1933; Bell and Thompson, 1978; Savino et al., Newman and Miller, 1983. 1978; Thompson, 1982; O'Connor et al., 1982; 17. Afferent Lesions Table 17-9. - CONDITION, SIGNS SHOWING IMPAIRMENT VISION AND PUPILS 1872 1883 1885 1887 1889 1891 Peltzer Wernicke Pflliger Bouveret Oulmont Hirsch 1891 v. Leyoen d"60, stroke; blind with fixed pupils ~ occiEital tumor with EU[>il liemial<inesia 0"16, shot through occip. lobes; blind, poor pupils C/'72, emboli; blind, EUEils "not Ve!J'. sensitive" d'67, stroke; blind with large, fixed pupils ()_24, occip. tumor; R blind, fixed pupil; L hemianopic with pupil hemiakinesia <:;?6 9, stroKe; !iemianopsia & !iemiaKinesia 1896 1896 1900 1901 1902 1902 1911 Kohn Rossolimo Purtscher Willet Josserand Pauly Strauss #1 CJ'62, lues; R hemianopsia & L fixeo pupil pupils almost rixed CJ'48, infarcts; hemianoesia, 0"'60, CO Eoisoning, hemiano12ic P&V deficit 0"48, trauma, hemianopsia & hemiakinesia Q, occip. infarcts; blind with fixed pupils ~6, occ. infarcts; blind with fixed pupils CJ'26, brain abscess, hemianopic V&P deficit #2 Dejerine & Jumentier Walker 1914 Wilbrand 1917 T92o"" Olo:H 1922 Oloff 1924 Strebel #8 1926 Behr 1928 Bunge 1928 Dieter 1928 Foerster 1929 Stern & Lehmann 1933 Toka;):'. Riley #1 1943 TI'f.r in1949 1951 1951 935 Cases with central visual defects and impaired pupil reactions to light YEAR AUTHOR 1947 / Lutt Harms F13 Fl4 F15 F16 F17 Harms F2 F6 F7 F9 Fl0 1952 1954 Harms ~) Harms Harms 1956 Harms F12 Fl3 F3 F6 F28 #1 #5 F4 c1'25, occip. abscess; hemianopia & hemiakinesia Q 51, brain hemorrhage; hemiopic V&P deficit cases thought to be "12osterior", with hemiakinesia p. 97; cerebral blindness with fixed pupils 0"37, occiE. snot wound, hemianoEic V&P defect 18, dim vision with (2Uf>ilhemiakinesia , occi12, shot wound; hemiO[>iC V&P deficit 0"37, shot wound, hemianopsia & hemiakinesia 0"55, OCCi)2, tumor, hemianO[>iC V&P deficit 0"54, occip. infarcts; hemiopic V&P deficit 0"26, collicular tumor; blind with fixed 2u12ils 0"22, occip. lobectomy (tumor) - 3 recurrences; hemiano12sia with EUQil hemiakinesia d'l9, occi)2, microzyria; V = 1.)2,; P = sluggish 64, Ca metastases; blind & large, poor pupils 42, strol<e; macula spared, pupils sluggisli O"i::8 , stroi<es; Elina willi tixeo EU[>ilS O':'grenade splinter, lower quad ran tic V&P defect O':'bomb splinter; upper quadrantic V&P deficit occi12. shot wound; lower guadr. V&P deficit 0"73, stroke; hemianopsia with pupil hemiakinesia ~ occi)2. grenade s12linter; V&P hemiano12ic defect P&V deficit ~ OCCiE. shot wound; guadrantic O':'occi.12. grenade splinter; paracentral V&P defect 0-:occiJ2. shot wound, 12aracentral V&P deficit sector P&V defect ~ shot wound O':'grenade splinter, quadrantic V&P defect Fi~res 4, 5, 8, 9 same as #14, 15, 13, 16 in 1949 same as Fig. 14, 1949 O':'bomb S[>linter, sector V&P defect brain abscess, hemiano2ic V&P defect ~ mine S[>linter, u.1212erfield cut for P&V same as Fig. 17, 1949 same as Fig. 17, 1949 same as F:!g 3, 1949 "occipital lesion'' with hemianopic V&P deficit & 8. or BEYOND OCCIPITAL AREA basal artery embolus; both thalami and colliculi involved optic tract pinc!iea oy taut vessel running across it oilateraI papITieoema; ihea soon Ke[>t e;):'.eSroIIea U[>; oolli 12ost.cereorals occiuaea; eaema huge lesion occip. lobes, extended to temporal &parietal lobes large, cystic tumor; very high ICP; 01ea soon papilleoema; hemiplegia, pain, ptosis, gaze palsy; lesion in pes pedunculi, & optic tract EOM involveo; lesion in L occ. lobe & R 3ro nerve softened areas in occ. lobe and midbrain fundi "almost I normal incongr. fields, fracture extended to base & parietal bone basilar allieroma, both post. cerebrals also occluaeo admitted in deep coma & died 8 hours later hemianesthesia; int. hydroceph. optic neuritis, hemiparesis, abscess fairl;):'. anterior in occipital lobe ankle clonus, 7th n. palsy; abscess extended to base; pressure ; signs insula, putamen, optic tract large lesion incl. pyramids, no anatomic evidence that 012tic tract was spared atheroma of both post cerebrals and basilar arte!J'. bullet stuck in brain close to OEtic tract tumor deep in temp. lobe; pressure lesion of optic tract no macular sparing; huge lesion in parieto-occip. area 7tli & 3rd n. palsy; lesion in peouncle, optic tract &miooram 5 cm g:lioma, brain eoema, t!ialami & coilicu!i com2resseo 500 mm ICP: when ICP was dropped, pupils normal, vision not on removal of tumor 12u12ils recovered, vision not gaze palsies; pupil defect recurred each time tumor grew (with hight ICP), and vanished each time Eost-012erativel;):'. anterior colliculi involved in defect 200 mm ICP; pupils reacted poorlr due to dense cataracts many brain stem signs; basilar vessels fibrosed oi12lo12ia, liemi[>Iegia; foci in occ. lobes, mioorain ano pons splinter in parieto-occip. area, reaching beyond midline splinter ill R cheek next to nose, stuck in post. temporal area R no details given assumed "hemorrhrure in upoer visual path" foreign bodies & brain substance removed !brain prolapse via aerectJ de12ressed bone fracture with SJ2linters removed from brain bone splinters removed from brain deEressed fracture 12arieto-occi12. de12ressed fracture, incongruous fields parietal inju!)'.;( pupil affected less than vision) no details given see above fronto-basal wound plus parietal fracture R macula not spared severe trauma with intracran. bone s12linters at "R rim of occ.bone" see above see above see above lno data given 1917 Wilbrand: should read Wilbrand &Saenger; occ. = occipital; ICP = intracranial CO= carbon monoxyde (lighting gas); V = vision; 1.p.= light perception; P = pupil. pressure; EOM = extraocular muscles; 936 / IV. Pupillary Pathology: Symptomatology Table 17-9 YEAR 1956 1958 1958 1959 1959 1960 1960 1962 1965 1957 1965 1969 -1970} 1972 1972 1972 1973 1973 1975 (continued) AUTHOR CONDITION, VISIO AND PUPILS SIGNS I DICATING DAMAGE BEYOND OCCIPITALAREA Shakbnovich Samojlov & Cases with cerebral hemianopsia: no clinical data published Shakbnovich reacted normally to bright light in western language. Note that Shakhnovic h stimuli but subnormally to weak the pupil defects consisted of Samojlov light stimuli; these were considered slight raising of threshold, not Samojlov & "orienting" reflexes, involving cefield loss Shakhnovich rebral function, in contrast to reflex Samojlov "defensive" (subcortical) reactions to Shakhnovich strong stimulation Kornyanski & Shakhnovich Shakhnovich Blegen _9air in vertebral A.-+ 3 d blind & slow 12u12ils man:\'.:brain stem signs during eeisode (recovered) Harms Fl6 same as Fig.3 1 1951 b see above Fl7 same as Fig. 4 1 1956b see above Fl8 _1i• occip. tumor ; hcmianoeic V&P deficit incongr . field loss; large tumor (no details) F20 occiQ. shot wound; hemianoeic V&P defect incongr . fields, no macular sparing (no details) F22 0"38, sudden hemianopsia & hemiakinesia assumed "aneurvsmal bleeding in lelt occipital brain Bresky & 0"39, occip. gunshot, hemianopic V&P deficit had cerebral bematoma which had been aspirated Charles Sugita 113 _9,65, sudden field loss with ,eu,eil defect lesion suspected in temporo-parietal lobe; hemiparesis & al. if:r -~34, "subcortical meningioma", V&P deficit lesion suspected in parieto-temporal area -g45 1 occiQ. a~ioma; hemianoeic V&P defect several episodes of subaracbnoid bleecling Bor~ann ~ occipital eorencephall'. eale papillae Harms #1 0"46 1 trauma, hemianoeic V&P defect acute occiQ. EEG focus; but later normal occiE EEG O";transverse shot wound; hemiano,eic V&P defect bone defect L occipital & R base at petrous border ~ 0"50 1 occ. grenade sElinter I hemianoeic V&P def. no macular s12aring (no clinical details given) Koerner #1 0"41, tangential missile, large V&P defect R post. parietal wound, bone fragm. in parietal lobe; & Teuber much necrotic brain & blood clots removed; bemif!aresis #2 0"39 1 temporo-occ. shell fragm. ;9.uadr. V&P loss wound behind r. ear, occieito-parietal fracture & s:elinters 115 0'13, shell fragment; large V&P field defect 12arietal area included; aehasia, pyr. signs, 7th n. Earesis #6 0"43 1 shell fragmenti incongruous field defect V&P wound 3-5 cm deep; purulent meningitis; hemiparesis #8 0"43, occie. bullet wound; large V&P deficit continuecl visual attacl<s wiili nausea &clizziness #9 0"38, occip. shell fragment, large V&P field loss bone fragments in R hemisphere; fractured lesser sphenoid wing; retinal hemorrh~es; grossly incongruous fields Narasaki#3 <5'36, fall; upper quadrantic V&P defect contused parietal lobe, epilepsy; enlarged 3rd &4th Ventr; & al. central vestibular damage; hand tremors & numbness #4 _Q31, stroke; hemianopsia &hemiakinesia occlusion of ant. choroidal A; numb hand & foot #5 0"68, stroke i hemianoesia & QUQilhemiakinesia bloocl + for lues; temporal EEG defect; hemiEaralysis #1 0"68. stroke; hemianopsia &pupil hemiakinesia Cibis no details given #2 & al. 0"55 1 occiQ. shell fragment; 12aracentral V&P loss scotoma selitting macula #3 0"53, stroke; homonymous field loss for V&P no details given #4 0"58 1 bernianoesia with QUQildeficit aiter narcosis at first diElOEia (no further details) fragment in parieto-occip. area -+ seizures; deaf, mentally #5 0"53, grenade fragm.; quadrantic V&P deficit slow -w- A.= artery; occ.,or occip.= occipital; fragm.= fragment; pyr.= pyramidal;+= positive; V = vision; P = pupil. ADDITIONS: 1955, Tamura; 1955, Yoshitomi; 1974, Campos; 1978, 1979, Hellner & co-workers; 1979, Alexandridis, Krastel&Reuther; 1979, Haman, Hellner, MUller-Jensen&Zschocke; 1981, Reuter, Alexandridis&Krastel; 1981, Reuter, Krastel & Alexandridis; 1983, Alexandridis, Krastel & Reuter; 1985, Tychsen & Hoyt. See also Table 3 -30, B. (Karp Ius and Kreidl, 19 l2, l 913). Moreover, in patients with pineal or collicular tumors the light reflex may be abolished without visual impairment. This is often blamed on pressure exerted upon the midbrain tegmentum, involving the sphincter nucleus and efferent oculomotor fibers. But in the early stages of such tumors the nucleus and efferent fibers are still intact, as witnessed by prompt, extensive pupillary contractions to near vision. It is, of course, not possible to decide in such cases whether the defect is located in the superior collicular brachia, the pretectal neurons, or their fibers to the Edinger-Westphal nucleus. The area is small, and all of these structures (and the motor nucleus as well) will eventually by injured by an expanding mass. Ellis (1984) described a patient with a pineal tumor whose pupils showed the characteristic defect seen in optic tract lesions but who had no visual field loss; and Forman, Odell, and Behrens (1988) saw a man with a tiny aterio-venous malformation involving the right posterior choroidal artery, clearly demonstrated by high resolution magnetic scan. He also had pupillary deficit without visual field loss. Perhaps such pupillary hemianopia without visual hemianopia-now thought extremely rare-might be found more often if it were looked for carefully in patients with pinealoma, collicular tumors, or diencephalic tumors projecting caudally and pressing upon the pretectum and colliculi, or in patients with unilateral Argyll Robertson syndrome. 17. Afferent Lesions / 937 4. Lesions in the Supra-geniculate Visual Pathways (Optic Radiations and Cortex) Some authors think that "Wernicke's pupillary hemiakinesia" is worthless for diagnosis: it is said that hemianopic pupillary deficit is found not only after optic tract lesions but in patients with supra-geniculate lesi~ns as well. Harms and his co-workers have taken a leading role in this school of thought, with publications stretching over more than four decades. Harms said that "it is certain that our views about the course of the afferent pupillary path in man are erroneous ... [and that] the cortex must be a link in the light reflex pathway." Harms dismissed all opposing findings as based on faulty experimental technique. Obviously, this statement cannot be reconciled with the findings just cited, namely that pupillary hemianopia, or total loss of the light reflex with bilateral lesions, can be produced by damage to the superior collicular brachia. We disagree with Harm's view for several reasons. (1) To begin with, in a question of cerebral localization, von Gudden's dictum applies: "First anatomy, then physiology; but if physiology is first, then not without anatomy." In other words, if you challenge accepted 89-=~-l ~, ___ -A./ .,-~ 7~'---" 6 / '- ·I·1r+I, ..1-_ 9f-ES~ 8 ,=-- 7c.- d. r. t E E .. "'=.--------- -D ---~~ ', ideas of neuroanatomic relations, you must furnish anatomic verification. Neither Harms nor any of the authors who agreed with him (Bresky and Charles, 1969; Sugita et al., 1970; Koerner and Teuber, 1973; Narasaki et al., 1973; Cibis et al., 1975; AJexandridis et al., 1979) have presented cases with detailed autopsy results which proved the lesion to have been confined to the optic radiations and calcarine cortex, and which ruled out the possibility of direct or secondary damage to the optic tract, the midbrain, or both. I have collected all cases l could find in the literature with lesions in the central visual pathways and with abormal pupils (Table 17-9 and Figure 17-14).3 Among these there was none that conflicted with the traditional views about the afferent light relex path. Either the clinical signs or the autopsy findings indicated that the damage included far more than the supra-geniculate visual radiations and the calcarine cortex. 3. Only authors cited in the text agreed assumptions about the afferent path. ------ ~--53-~-l ~\ ,,,,., ... - ',~.._--;-,"" ... --' I I - rl. r. I :\,, -C ,,,,,,--............ -........ with Harms's --~ - 1.1. Il I ----,----:r-·------------- " Id. I. -E --------------- '"' 2.'v - -.._ ..-- .._______ --8 ~~-------------· 7 F 6~.-.-♦-+-+-+ * .. -.- ...- ...- ...- ...- ...- •• - ...-+-+-♦-♦-♦- 0. lsec. ➔ Figure 17-14. Cerebral blindness after cerebrovascular accident. The patient, a 43-year-old physician, had been under treatment for essential hypertension for 5 years. He had narrow and irregular retinal arterioles and electrocardiographic evidence of left ventricular hypertrophy. Two months before examination he was found unconscious and incontinent. He remained semistuporous for 36 hours, with frequent episodes of generalized rigidity. Rotatory nystagmus was present. Spinal fluid, sampled 3 days after the stroke, was deeply xanthochromic. It was negative on smear and culture. The patient improved gradually, and at the time of examination had only mild dysarthria, but he was blind. The loss of vision was shown to be organic by electroencephalographic studies. It was the neurologist's opinion that the patient had suffered bilateral po terior cerebral artery thrombosis with hemorrhagic infarction of both occipital lobes. During the pupillographic examinations the patient was alert and there was no apparent disturbance of mental function. He said he could not see the light flashes used (after dark adaptation). His pupils were very large and equal, and they constricted well on bilateral light stimulation (A). Unilateral light flashes elicited reduced reflexes (Band C). ln addition, the darkness reflexes were inextensive and sluggish in all phases; and there was distinct unilateral consensual deficit (B, D, and F). Both pupils were unable to follow short light flashes at faster then two per second rates (F). Commelll: Considering the patient's blindness, the pupillary reflexes to bilateral light stimulation were extensive. The reduced reflex amplitudes upon unilateral stimulation are compatible with optic tract involvement. The consensual deficit indicated additional damage in the rostral midbrain, that is, the collicular brachia or pretectal region. And pupillary sluggishness usually is brought about by nuclear or efferent oculomotor impairment. The rotatory nystagmus during the early stage after the stroke also indicated midbrain involvement. The damage was thus not confined to the occipital lobes. 938 / IV. Pupillary Pathology: Symptomatology Clinically, these patients were not merely blind: there were visual, verbal, and motor agnosias and apraxias, di orientation, and confusion. Many patient were in prolonged coma, or had high intracranial pre sure, with papilledema and retinal hemorrhages. In three patients (with tumors) the pupil re urned normal light reflexes-with persistent blindnes -when the pressure was relieved surgically, and lost them anew when the pressure ro e again because the tumor recurred. There were hemiparesis and hemiplegia, hemianesthesia, ataxia, diplopia and gaze palsies, third and eventh nerve pareses, and other brainstem signs; and the visual field defects were incongruous, including the macula. Anatomically there was damage by concussion, laceration, infection, edema, and pressure, including the temporal and parietal lobes, the basal ganglia, the midbrain, the pyramidal tracts and internal cap ule, and the optic tract. Skull fractures extended to the ba e, the temporal and parietal bones, and the lesser sphenoid wing. Bone fragments and mangled brain substance were removed, or were extruded (due to brain edema) through the bone defect. Hematomas and subarachnoid hemorrhages occurred; and emboli occluded the posterior cerebral, the middle cerebral, the basilar, and the veterbral arteries. These arteries supply a large area, including both brainstem and forebrain structures (Figure 17-15). In fact, even the territory irrigated by the posterior cerebral arteries exceeds the occipital lobe (Figure 17-16). The only proof offered for the integrity of the optic tracts by Harms and his school was that the fundi looked normal to clinical inspection in many of the patients, sometimes years after the injury. However, as is well known, the fundi may appear grossly normal and yet there may be fiber loss in the optic nerve and tract. Without special studies with red-free light it is extremely difficult to be sure that no such damage exists (see Hoyt and co-workers, 1972, 1973). (2) We found reports on more than 400 cases with incomplete cerebral blindness or with cerebral hemianopsia, most of them documented surgically or by autopsy, in whom no matching pupillary field defects could be discovered (Table 17-10; see also Figure 17-18,B). Harm's objections that this was due merely to experimental error-especially stray light reaching the good retinal half-does not address the point that most of these authors had no trouble finding hemianopic pupil defects in patients with optic tract lesions (Table 17-8). ) Post. cerebral a. Figure 17-16. Territory irrigated by the posterior cerebral artery (sagittal view). Occlusion of this vessel will affect large areas of the brain in addition to the suprageniculate optic pathways. (From J. de Ajuriaguerra and H. Hecaen, Le Cortex Cerebral: Etude Neuro-P.lychio-Pathologique[Paris: Masson et Cie, 1960)) Figure J 7-15. Blood supply of the visual pathways. Occlusion of the posterior cerebral artery or of the rostral basilar artery will affect a much larger area than the optic radiations and visual cortex. (Slightly modified from F.B. Walsh and G.W. Smith, J. Neurosurg., 9 [1952]: 517) 17. Afferent Lesions 939 / Table 17-10. Incongruous visual and pupillary deficits due to supra-geniculate lesions A.CASES WITH INCOMPLETE No YEAR AUTHOR 1877 1885 1885 1892 1892 1893 1893 1894 1896 1896 1897 1905 1905 1906 1911 1915 1911 1918 CEREBRAL --V* v-v- -v- = Marie &Leri -7i3 Wilbrand & Saenger - #1 m _y__ V Tr No YEAR AUTHOR 1 1 -v2 V 1 V 1 V 1 V 1 ~ 1 -v1 1 -v1 1 -11 """"To 1 1 ~ #1 ___I!:_ -7i2 ___I!:_ #7 ___I!:_ #8 ___I!:_ #2 _v_ Fuerstner v. Monakow Reinhard Groenouw Muller Dejerine &Vialet Schmid-Rim2ler M~nus Peters All;yn Dejerine &Vialet Chaillous Lewin &Guillen'.: Ra;ymond & al Manzutto Marie & Chatelin BLINDNESS AND NORMAL PUPILS 1920 Monbrun & Gautrand- #1 ~ - ---iF3 1941 1943 1 Wesmhal 1 Lenz & Schwab 1 vom Hofe #1 WorsterDrought -72 #1 Alfanda!:J'. 1 Terrien Walsh & 1 Goldberg #1 Hem2hill #4 Riley & al 1943 1947 1950 1952 1953 1953 1954 1955 McDonald Walsh Biemond Lawrence Courville Foley Reese Kearns &al 1920 1925 1928 1928 1936 1938 1940 -72 1 1 1 --*V 'fr° Tr I ....J!_ V V V -I- YEAR AUTHOR 1955 1957 1961 1961 1962 Kearns &al Symonds & Mackenzie Mones &al Silverman & a Gloning &al -vAE -v-- 1962 V V ~ 1966 Weinberger &al Smith &al 1969 Walsh & Hoyt_ 1970 1968 Rasmussen Sabah 'I V _l _ _S!_ 1 1 #2 #2 _I_ __Q_ 0 '1 No * 4H #3 #4 #5 6 #2 #3 #4 #8 #9 #10 #14 #1 #2 #1 #5 #1.7 #6.12 E 2343 1 #1 #2 #3 V V V V A V V V V V V V 0 0 D D V D 0 Ve 0 0 0 No: plain numbers indicate number of patients described; # numbers show case numbers of individual patients; p2343 = page in book: several children. *: V = cerebrovascular accident (hemorrhages and occlusions); Tr= trauma; To= toxic condition; AE = air embolus; O = anoxia, including accidental, cardiac arrest; A= angiogram; Ve= ventriculogram; D = developmental, including obstructive hydrocephalus, microcephaly; I= infectious condition. B,CASES WITH CEREBRAL HEMIANOPSIA OR OTHER FIELD DEFECTS WITHOUT MA TC HING PUPIL No YEAR AUTHOR 1866 1877 1881 1883 1883 1885 1885 1885 1886 1887 1888 1889 1889 1890 1890 1891 Levick Fuerstner Wilbrand Richter Wernicke Reinhard 1 --- #1 #2 - Wernicke Wilbrand Se~in Wilbrand Reinhard Schmid- Rim2ler Siemerlinll" Foerster v. Limbeck Galezowski Numbers and abbreviations ADDITIONAL ·t 1 h CASES: · 1 · gem aem1p egia; --I* YEAR AUTHOR No * YEAR AUTHOR No 1891 1 AE V V 1914 1915 #2 v. Leyden I, V .....h..Y1891 1891 var s 1892 1 11 1 ~ 1893 1893 --1t.L V #7 --5!.__ 1894 #13 --5!.__ 1896 #14 V 1896 #15 -3!._ 1 --5!.__ 1897 1 1899 V 1 Tr 1900 1 v-" 1900 1 Tr 1901 1 Tr 1 V 1904 1 _:f__ 1906 1 Tr 1909 1 1911 " as above; DEFICIT --1- v.Monakow Wilbrand Mliller Vialet Uhthoff Henschen Mills& de Schweinitz Peters Ktistermann Silex Jacgueau Me;yer Ballaban Beevor Tschuida Raviart &Cannac Jess T = tumor; var = various; #1 1 #2 3 #13 36 7 #2 #2 1 1 #3 1 5 1 1 1 #3 '1 T _y_ _y_ ~ var -- _!_,_Y V V V -I-TV _y_ 'I Tr ~ s = several; 1924 1927 Schlesinger Marie&: Chatelin Heilig Wilbrand & Saenger Fleischer & Einsinger Strebel Pastore 1927 1924 1933 1938 1943 1952 1952 1956 Bunge Plaza Hilgard &Wendt Terrien Rile;y & al Hecaen &al Lawrence Samojlov 1916 1918 1920 m #24 * -D- -- -- Tr 1 -67 --1--1- 11 7 38 1 1 #1 #6 7 1 1 Tr Tr Tr Tr _:f_ V ~ var -I- T V T T V -T- m = many. 1922, Scarlett &Ingham: 9 cases • trauma· • 1972 , Ho yt & a 1• .• 3 cases consee a1so Hellner & al., 1978; Tychsen & Hoyt, 1985; and Hamann & al., 1979 . ' 940 Table 17-11. rv. Pupillary Total cortical blindness with pupillary reaction to light YEAR AUTHOR 1855 1 75 1877 1883 1884 1885 1 85 von Graefe Samelsohn Jodko Wernicke Schoel er&: Uhthoff Berger v. Monakow il Reinhard #2 #3 1886 1 7 1 8 1891 1 91 1 94 1896 197 1897 1 97 1 9 1900 1900 1901 1902 1905 1905 1906 1909 1911 1911 1913 1914 1916 1917 Pathology: Symptomatology CONDITION PUPIL when blind d"'14, fell on forehead no l.E.; OS fixed <.;? 73, intestinal hemorrhages -sincoee anoxia during anesthesia 18, tem~rallobetumor:lesion Rocc. lobc, Ltract m. old microce12h. child, closed fontanels O" 71, cerebrovascular accident tr 70 3 auuo ect1c strokes <.r 60 aooolectic cysts in occipital lobes 48, infarct; masses in bothocciEit.al lobes ~ _ 71, several strokes with e12isodes of blindness ~ O" 86, cerebrovascular disease #10 cr64, cerebrovascular accident Bouveret <:772, thrombosis of both 2ost. cerebral arteries Chauffard O" 74, com11Ietelr blind after stroke Moeli #3 ~ 44, atheromalous softening of occiEital lobes Wilbrand 63, cerebrovascular accident Jocas 58, severe stroke 4 years earlier Anton 56 1 cerebrovascular accident I leinersdorff 52, seetic abscess ln both occipital lobes Kiistermann #1 O" 51 1 ccrebrovascular occlusion Lunz #l! O" 36, CNS lues i imeroved after treatment Uhthoff child after cerebrosoinal meniru,-itis Jacaueau #2 O" 70, cerebrovascular disease Meyer #1 O" 49, diabetic, cerebrovascular accident #2 O" 64 1 infarctionofbothocci)2ital lobes Ballaban 6 cases cortical!.}'. blind, normal fundi Christiansen 30, self-inflicted bullet in occipital brain #1 Lewin & 67, CO poisoning GuilleE,Y ~ oY 47, lowered in well -+ unconscious Wehrli ~ 50, thrombosis both calcarine arteries Schmidt 54, eeileesl in grand mal seizures Raviart&Cannac 58, ccrebrovascular ace ident Marie & Leri infarct Wich rn ~ 53, pernicious anemia -+cerebrovasc. occlusion (Bielschows~) Behr Q 1. 5 y. , convulsions with whoo12ing cough Uhthoff 13 m. child, congen. or ve!:Y early ence2halitis #20 Heilig O"33, transverse bullet in both occipital lobes Berger 1 case war inju!:}:'.to ootli occiEital lo6es 2 cases transverse occipital shot wouncis ~ -8 ~ ~ g. plain numbers denote number normal fundi: m = months; Tr= trauma; Tu= tumor; OD reacted reactea well reactive reacted well normal reactive reactive reactive reacted well R=L reactive R-L reactive reactive reactive reactive reactive normal normal normal reactive very prompt normal reacted well reacted well normal normal 2rom12t normal reactea well reactive normal reactive quite good reactive prompt, good BEST FINAL VISION blind repeatca bouts 6linan. "( none up to death none contmuea @ma none iilfer 3ra s'frolce none ue to aeatli none up to death homon. hemiano12ia hemiano12ia hemianoeia none none none none macular fields none l.p. small field liemianoeia none none hemiano2sia macular fields none poor, hemiano12ic none i.e. near bl 1. E. within 3 months regained in 30 hours none none none PROOF OF SITE - cer. signs, course cer. s1121cs autopsy course autopsy aufopsy autoesi autopsy autoesr auto12sr autoesr auto12si auto.ES.}'. autopsy autoesy course, n.f. autoEsl autopsy autopsy cer. si~, n.L course, n.i. course, n.f. cer. Sis;!!s, n.f. autopsy Cer. s!gns z funQl autopsy cer. s1~s, n.L cer. signs, n.f. aut0)2S.}'. cer. signs autopsi auto12sy autopsy ICODE Tr u 0 Tu D V V V V V V V V V V V V V I V I I V V V 7 Tr To To V 0 V V V I 500 mm Hg ICP reactive recovered I autopsi normal none Tr autopsy normal none Tr liem1anops la veniiea wouna normal ~ aufopsy 6Ima unHI aealli normal of cases; numbers markf'd # are individual case numbers; l.p. = light perception; cer. signs= cerebral signs; n.f. = f.c. = finger counting; CO= carbon monoxide; ICP = intracranial pressure. CODE: V = vascular cause; I= infection; To= toxic condition; 0 = anoxia; D = developmental defect; ? not reported. (3) We collected an additional 173 case reports on patients who were totally blind when their pupils were examined and found to respond briskly (Table 17-11). The objection of stray light obviously does not apply to these cases. Harms dispatched them by saying that small islands of functioning cortex may have smvived and were responsible for the reactions, and that the patients may have been too ill to be certain of their blindness. But a substantial number of these patients were neither terminally ill nor confused when they were tested, and visual examinations were done carefully and repeatedly. Especially in patients who had suffered from anoxia, brought on by cardiac arrest or other causes, the occipital cortex was sometimes damaged virtually selectively. This is to be expected, since this cortical area is especially vulnerable to drops in aterial perfusion pressure because it is supplied by the most distal branches of its arterial tree. Finally, absence of visual evoked potentials, or a flat occipital electroencephalogram, testified to total cortical loss, and autopsy confirmed this diagnosis in many cases. Even patients with complete "brain death," that is, an isoelectric electroencephalogram, occasionally showed pupilary reactions to light. This is not surprising because the midbrain is much more resistant to anoxia than are the cerebral hemispheres. Two patients described by Brierley and co-workers (1971) had a flat electroencephalogram for 5 months after cardiac arrest, but their pupils continued to react to light. Dr. Brierley was kind enough to inform us about the anatomic condition of the visual system of these patients. "The optic chiasma and tracts were normal, the lateral geniculate bodies were slightly reduced in size; they showed normal lamination; within each lamina a small proportion of cells were dark-staining and slightly shrunken. There was a general increase in small astrocytes .... The most important point concerns the calcarine cortex itself. This was totally destroyed on each side in the two cases. Thus, a band of thickened pia-arachnoid was separated from the sub-arachnoid white matter by a loose network of collagen and glial fibers, within which no neurones were recognizable." (4) These pathologic findings agree wtih experiments 17. Afferent Lesions / Table 17-11 941 (continued) YEAR AUTHOR CONDITION BEST FINAL VISION PUPIL when blind normal prom12t normal normal normal R L normal normal normal perfect blind until death 2 cases grenade splinter in both occipital lobes Berger to tally blind Wilbrand & ~ 1 case 6ilateral occipital Infarcts none Saenger o. 98 1 case apoplectic lesions in occipital cortex according to lesion ~ several cases cortical blincl.ness remainecl. 6Iincl. )2.309 cases with occipit.a1 war wounds remarneo @ma 1918 Morax O" 30 1 occipital shell fragment almost norma.t m 3m 1919 Guillain &Barre O"'25 1 4 d blind (occi12ital shell fr~ment) -totally blind 3 years 1919 Saenger O"'25, mine splinter in occiput totally blincl. 4 months 1920 Monbrun & U" 41, shell lragment in occipital brain Gautrand 114 recovered in 6 months prompt 1920 Schlacpfer O"'34, air embolus after lung puncture 2 days blind-recov. R L prompt 1924 Fejer ~ CO poisoning amaurosis reactive 1924 Rathe!)'. &Gournay ~ CO eoisoning, admitted in coma small hemiopic field normal 1924 Strebel /16 ~ transverse shot through both occipital lobes remained blind reactive 1928 Foerster O"'26, removed large occipital tumor . deep coma 4 months reactecl well 1929 Ford 20 m. pertussis rigidity & convuls1~1lS none normal 1929 Lagrange & al. 65, occlusion both ~st. cerebral arteries norinal-none 1931 Weber O" 2 1 ether narcosis convulsions decerebrate none after 2nd stroke reactive 1932 Rimbaud &.al. O" 58, 2 cerebrovascular episodes, 7 months aeart full fielcl in 14 clays 1!)33 Masson #1 reactive 24, 1 2 d blind after ventriculogram (tumor) recoverecl reactive 29, 24 hours blind after ventriculogram ~eoceph.) 115 very poor m 2 years reactive 1933 Wechsler O" 13, overcome by smoke - coma seizu~·es recoverecl. increasecl 1934 Caussade O", CO QQisoning delirium - clear bui blrno l. E. and color reacted well 1934 Grimsdale O"32, gas poisonin11,- prolonged coma . in coma to death reactive 1936 Courville #3 Cl' 42, deep coma &convulsions after NO narc?s1s 2;200 with scotoma reacted well 1937 Ford & al. Cl' 33 1 respiraton:failure 36 h. convuls1~ns QiOl none normal 1941 llemehill #2 Cl' 63, occlusion of both post. cerebral arteries foveal fields reactive 1942 Frarn;:ois 49 1 CO P2iSoning 24 liours blino none normal 1943 Hamby 24 1 (Qacterial) occipital brain abscess recovereo reacted well 1945 Yaskins &Soaeth CJ'48 1 subarachnoid hemorrhage (Jr vertebral a. soasm on neck injection (occ. lobe) 1947 Thiebaut & al. none normal 1947 Walsh poor in central 40 normal p.32 lt'l 1 0 1 blind 3 months after shrapnel wound 1948 Aubertine &.al. recovereo in 4 montlis reacted well 2 1 coma-convulsions &anpnoea after lighti~g~ 1949 Wiedersheim recovered in 10 days normal 27 1 air embolus (2neumothorax) 6 da:ys blmd 1950 Ferrante recovered in 9 days normal CJ'24 1 air embolus (lmeumothorax) 1 day blind 1950 Velzeboer normal none ~60, cerebrovascular accident 1950 Woywitka & Ill recovered in 5 months normal* 3, whoo12ingcough ence12haI!t!s-1 month bl!nd Riches #2 _ 9 1 whooping cough encephalitis - 1 week blrnd normal* recovered in 3 months 1950 Biemond n 45 hemorrha<TP to both occinital lobes normal almost blind abbreviation as above; p = p;ge in book; NO= nitrous oxide; h = hours; d = days; a= artery; CODE: AE = air embolus;IO anoxia direct cause of blindness; Ven =ventriculography; * =after control of convulsions. 1917 1917 g. ~ & 8. -8 on this system in animals (see Chapter 3). Uni- or bilateral occipital lobectomy as well as complete removal of the forebrain in many monkeys, dogs, cats, and rabbits was never observed to abolish the pupillary light reflex. Further, electric stimulation of the lateral geniculate nucleus or in the suprageniculate visual path never caused pupillary constriction, while stimulation of the brachia of the superior colliculi under the same experimental conditions did so readily; and-as already mentioned-interruption of both collicular brachia without damage to the forebrain abolished the light reflex. (5) From a general physiologic point of view the concept of a "cortical reflex" is a contradiction in terms. In all systems the cortex is that part of the nervous system concerned with conscious perception and further elaboration of ensory input. I find it extremely difficult to entertain the idea that the pupillary reaction to light, a primitive, entirely unconscious reflex, should be conducted by way of a "cortical reflex arc" to the mi db rain motor center. Certainly, nothing in the reports up to now would justify such a revolutionary notion. As seen in Table 17-9 and Figure 17-17, Samojlov, - PROOF OF SITE CODE autopsy autopsy autOESY autopsy autopsy or surgery surgery surgery; n. I. surgery surgery Tr V V V Tr Tr --rr--rr~ AE clinical course To coma To clinical course Tr surgery surgery 7oautopsy ~ autopsy 0 decerebrate 2. 5 y V autopsy Ven surgery Ven surgery course, cer. signs ---ro~ cerebral signs To cer. SY:l:ns, n.f. no cortical function 0 0 course autopsy V To course --y--autopsy course ~ autopsy V surgery Tr course course, cer. si~s AE AE course cerebral signs V cer. Si!1,!!S,course IO cer. signs, course IO autonsv V ---rr- --ro- = rnfect1ous d1Sease, but Shakhnovich, and their co-workers (1956--1962) noticed in patients with cerebral hemianopsia that the pupils appeared to react quite normally when bright light stimuli were used. But with light close to the pupillary threshold no reaction occurred when the blind retinal half was stimulated, while small, short-lasting contractions resulted from stimulation of the good retinal half. Samojlov and Shakhnovich offered the explanation that there were two kinds of light reflexes: (1) "defensive" reflex movements, transmitted subcortically,and requiring strong stimuli, and (2) "orienting" reflexes, due to the "newness" of the stimuli, and elicited by weak light. "Orienting reactions" were said to run over cortical paths, and therefore to be lost when suprageniculate lesions destroyed these. We cannot agree with this explanation (Chapters 3 and 13). But Samojlov and Shakhnovich demonstrated clearly an important fact which explains the discrepancies in the literature: while strong light stimuli continue to elicit brisk light reflexes in patients with suprageniculate lesions, weak light stimuli can lose their effectiveness after such damage (see below). In other words, the 942 Table 17-11 YEAR -1951 IV. Pupillary Pathology: Symptomatology (continued) AUTHOR CONDITION Fink Schildge Wagman Dufour Reese #1 #3 PUPIL when blind ~2 days coma after lightin~ gas poisoning active blind after occipital electro-shoe!< reactive-O" 77, cerebrovascular accident reacted well 8 1 long coma after CO poisoning reacted well .}:'.earold child I born c.i::anotic reac led well 0"33, 3-5 minutes tracheal obstruction - blind normal #4 38, c.i::anotic 15 minutes after food asoiration /decerl reacted well 1956 Cordier & al. 43 1 air embolism after pneumothorax active 1957 Bergmann #1 O" 44, hypertension & uremia, fluctuating blindness active -,r-35 1 sudden coma & cerebral Tiemorrli~e - blind normal ~ _ 19 1 lighting gas poisoning -..guadruparesis & blind normal ~ ~ 15, depressed fracture both occip. &R parietal bono normal _11_5 __ O"45 1 cerebrovascular accident normal #6 O" 10, deaf, blind, mentall.i:: deteriorated - died normal 49, completely blincl after second stroke normal i/8 52 occlusive CNS disease lhnth calcarines \ reacted weII #10 0"72, several strokes blind reactive ~ 60, completel;)' blind after cerebrovascular accident normal ~ 43, Hodgkin's disease I blind wiili loss oiup-gaze normal 1957 Symonds I'; #2 69, occlusive cerebrovascular disease normal Mackenzie #6 O"7 5, cerebrovascular infarct normal #7 74 2 strokes normal 70 2 strokes ~ normal 1958 lloyt &.Walsh 39, cardiac arrest after ice pick stabbing in chest brisk TI59 Samson!, iil. 58, cere6rovascular acc1aen[ normal 1961 Cremieux & al. 68, 2 strokes: ischemic necrosis in both occ. lobes normal 1961 Huber #1 rav necrosis of both occipital lobes /temp. tumor\ normal #2 sudden blindness after occipital ventriculogram normal 1961 Silverman &.al. ::!cases without light perception after vertebral angio1<r. normal 1962 Cloning & al,_!!__ O" 58, cerebrovascular accident normal #6 prompt _SI!69, leg U1rombosis - stroke ace iclenl ~ O" 53 1 cerebrovasciiiar normal #12 0"80, parklnsonism -sudden stroke normal #13 (Jr 56, cardiac condition -sudden stroke normal 1962 Rinaldi #1 (Jr 21, 3rd ventricle tumor decompression prompt &al. ~ 25, 3rd ventricle tumor, high ICP, ventriculogram very goocl ~ very goocl 4, tumor 4th ventricle, ventriculogram reactive ~ 32, frontal melanoma, ventricular decom2ression 1962 Weinberger & al #3 3, cardiac arrest -deep coma _.blind reactive 1952 1952 1954 1954 -? -8. w- 8. -i -- 1 =~ abbreviations as above; CODE: Ven= venlriculogram; VA= vertebral BEST FINAL VISION some vision recovered blind 1 year 8 months o::TTn3 monilis none h.m. in 2 1/2 ~ears im roved to 20 15in 7w recovered in 8 da.i::s recovered poor in bemiopic field . poor in small field regained fair in foveal field none to deaili none to death none until cleath none until death none none h. m. in small field R inf. quadrants 1. p. L inf. quadrant some central field 6/330 in periphery small lower field-none until death blind until cleath regained vis ion regained vision ...!..:..E.:_ 011 4th day regained some Vision regained some vis~ regarnea vis10n hemianopsia poor vision to tally bl incl 2 years regainecl vis ion Eilincluntil aealli (2m) almost normal -- PROOF OF SITE ICODE cer. signs, n.f. To course Tr '-Vautopsy I~ course, cer. signs no proof course, n. I. course, n.f. course, fundi AE To cer. si~ns, EEG cer. signs, EEG V I~ cer. signs, EEG X ra.i::, su~e!',l:'.,EEGTr cer. signs, EEG '-VM cer. signs, EEG V EEG, autops.i:: '-VEEG, atops.i:: ,_ EEG, autopsy ,_:f___ EEG, pneumoenc. V '--.YEEG, course V autops.i:: n.f., cer.signs f.n., cer. Si[;nS I~ '-Vn.f., cer. s!gns I~ n.f., cer. signs ,_ V EEG, cer. signs '-Vaut0J2S,)'. I~ autops;):'. Ven course VA course cer. signs, n.f. V cer. signs, n.f. EEcl, cer. signs ~ course V course Ven autopsy ven surgery ·-ven surgecy ven autOQSY course, cer. signs g ·~ angiogram. pupillary threshold is raised in many of these patients. The same was actually found by Harms and by those who agreed with him; but they exaggerated these findings by speaking of pupillary akinesia, pupillary hemianopsia, or Lossof pupilla,y function in the affected field, instead of the hypokinesia which actually was observed. Recently a number of authors of the Hamburg school re-investigated this question, using Harm's perimeter and Jensen's TV-pupillograph, combined with VER studies (Hellner, Jensen, Hamann, Miiller-J ensen, Zschlocke ). These studies, together with the earlier findings, furnish a solid foundation for the following conclusions. (1) Infrageniculate impairment of the optic pathway, that is, lesions in the optic nerve, chiasm, or tracts, brings about congruent loss of visual and of pupillary function, both in regard to the area of the field affected and to the severity of the damage (Figure 17-18,A). (2) Congenital suprageniculate lesions and other damage of long standing, limited to the visual cortex and optic radiations-such as occipital porencephalic cysts- cause visual loss and a raised pupillary threshold in the affected field. The pupillary dysfunction is due to transsynaptic degeneration in the primary optic path, as demonstrated by fundus changes ("bow-tie atrophy"), visible with red-free light (Hoyt and co-workers, 19721973 and 1985; Borgmann, 1972; Hamann et al., 1979). (3) Lesions acquired later in life that affect the occipital cortex and optic radiations-such as pressure by tumors or by surgical excision of the temporal or occipital lobe-can leave the pupils normal for considerable periods (Figure 17-18,B). More often there are variable degrees of pupillary hypokinesia in the blind area of the field. These are usually incongruous with vision, that is, they are less extensive than the patient's visual loss (Figure 17-18,C and D). (4) The pupillary disturbances in patients with suprageniculate lesions often are due to the fact that the damage is not confined to the visual radiation and calcarine cortex. Especially after vascular occlusions or trauma, larger areas of the brain usually are affected: occipital strokes seldom interfere with the calcarine ·~ -v-v- -~ 17. Afferent Lesions / 943 Table 17-11 (continued) YEAH AUTHOR Lawrence 1963 Chakravortx 1964 McAulev 1965 Acers & Cooper Ill #2 1966 Kooi & Sharbrough 1966 Smith & al #4 1967 Garland & Pearce 1967 Grilfith & Dodge 1969 Treister 1969 Walsh & case 1. 6 II Hoyt 11.83 II 7.15 II 13.28 II 13.29 1969 Leenstra Borsje & Bookstra 1970 Bigorg:ne &al 1970 Carmola & Harris 1970 Ilugonnier 1970 Khunadorn & Kalumpaheti 1970 Olurin l 952 - CONDITION 45 , infarct to both occioital lobes 4 1/2 months, high fever alter triple vaccine 2v7m cardiac arrest rdrowninirl <:Jr5m, bacterial menin11:itis r..r4m, bacterial meningitis normal normal reactive normal reactive BEST FINAL VISION l.p. in I.periphery 14 m no apparent vis. regaineil in !l monilis recovered vision remained bl ind ~33, normal gross l.p. Oat occip. EEG Tr intact normal normal reacted well normal normal reactive good good none recovered recovered homon,l'.mous fielcl higb thresh.cent.fielil l.o. none regained vis ion regained vis ion occipital EEG cerebral s ii2:s EEG, cer. signs CSF, cer. signs surgeri course, n.l'. autoesy coma decerebr. cerebral s i!2:S D To Tr I Tr M M 0 0 t trauma-. 2 w coma+semicoma 0""8, congen. h,l'.droceehalus-+seizures-+blind CO intoxication+coma+blind ~31 2 children alter head injury 25 mold child, acute meningo-enceehalitis 0..- occioital shraonel wound O"'1 hemolytic transfusion reaction 16m, diffuse cortical degeneration 37, cerebral hypoxia-.coma ~ 38, trac!ieal o6struction-.coma ~ 1 patient with isoelectric EEG+death - Br ierle.l:'.& al. Davis & al. Hermans & al. Huber Klotz & al. Lloyd #3 --;n- PROOF OF SITE I CODE autopsy V normal fundi 0 l'Iat occip. EEG 0 I course, n. f. course, n.f. I reactive coma until death EEG flat chronic respiratory distress (emphysema) 26 automobile accident blind 3.5 hours ~ 21, occ ieital trauma reactive brisk reactive central field recovereCI blind EEG, cer. course surgery 0""54, cmbolic infarct of both occipital lob s normal none until death 2 cases 6irt!i trauma or aspliyxia at birth 1 case postmeningitic hyilroceplialus 1 case missile injury 1 adult, 2 children severe hcail trauma (trrufic ace.) 1 cliIIil, enceplialitis Irom tl'lple vaccine 1 child, measles encephalitis 59 1 cariliac arrest Hl hours coma child, whooping cough encephalitis prolongeil convulsions ij cliililren with malaria} __.. coma 3 cases other fever 2 cases, cardiac arrest comelete cortical death 25, mumes blind & ataxic from 4th to 10th day 64, 9 hours blind alter air embolus (lung euncture) 1 eatient acute occi11ital edema (112st-ventriculogram) 60, post-operative hypotensive crisis 22, ilrug overilose ileep coma witliout cere23, ilrug overilose }bra! function or measurable pulse normal normal normal normal normal normal normal normal normal normal reactive brisk normal reactive norma; normal normal -? 1971 1971 1971 1971 1973 1973 DTTDTT i B abbreviations as above; y =year; w = week. CODE: M = unclassified condition. See discussion of later cases in text. Note: After 1975, I no longer searcbed blood supply only. More often they include areas served by more proximal branches of the posterior cerebral artery, or even the posterior communicating artery and its branches, or the basilar and vertebral arteries (Figure 17-15; see also Chapter 44). How soon after a suprageniculate lesion retrograde trans-synaptic degeneration develops in the adult human optic tract remains unknown. It is a very gradual process (see Brierley's description above). Most patients described in the literature were seen many years after their injury. Hellner, Jensen, and Muller-Jensen (l 978) saw a patient with cortical hemianopsia due to a tentorial mcningioma, arising from the left transverse sinus, that pressed upon the occipital pole. One year after beginning of symptoms the tumor was excised and the patient recovered; but 3 years later the tumor recurred and the visual loss returned. However, the pupiUary field, recorded at that time, was entirely normal (Figure 17-18,B). In contrast, patients with occlusion of the posterior cerebral artery or its branches had pupillary field none none some vision regameil some v1s,on regameil v1swn regained vis ion regained in 1 month regaineil vision regaineil vis ion in } 2 weeks to 2 months none to death recovered recovered regained vision eartial li~ht sense recovere recovered ? signs autopsy 0 Tr Tr V cer. signs, n.fundi 0 course, n. Iuna:1 course, n.fw1di Tr course, n. Iuncl, ~ prolongeil convulI -I} sions coma course, cer.signs 0 I cer. signs' n. r. course, neuroI -I} 11sychiatric si~s 0 autoes.l'. mening:itic si!!:!.1B I AE course, norm,f, Ven course cer.sii2:s, EEG 0 low core tempera~ y-ture; no signs of } cerebral activity for such cases. defects much earlier. These ranged from barely perceptible to marked; and they were always incongruous with the visual fields in the same patients more restricted than the pupiUary fields (Figure 17-18,C and D). Without early observation and follow-up it cannot be said with certainty whether the degree of a patient's pupillary deficit is related to the duration or to the extent of the lesion. The dysgenesis which follows injuries early in life is more marked than is degeneration of adult structures after later damage. Van Buren (1963) found extensive atrophy of retinal ganglion ceUs in monkeys 4 years after occipital lobectomy (Figure 11-15). Further, in animals the extensiveness of retrograde degeneration after occipital lesions depends partly on their phylogenetic development. In lower mammals, with relatively little cortical and extensive midbrain projections of the retinal neurons, the optic tract and retinal ganglion cells do not show as much degeneration after suprageniculate injuries as they do in primates. -r- 944 I IV. Pupillary Pathology: Symptomatology IIIIIIIIIIIIIIIIUIIIIII 111111111111 111111111111111111111111 11111111111 11111111111 11111111111 11111111111 m::::n:mmm::::: 111111111111111111111111 Figure 17-17. Pupillary reactions in a patient with suprageniculate hemianopia. Light reflexes were recorded as described in Figure 15-52. Pupillary movements are shown as changes in the white area on top of each graph. Timing of light timuli is marked by the mall vertical bars. With bright light (first two lines) the reactions elicited from the blind and from the seeing half-retina were equally extensive. But with weaker light (last two lines) no reactions could be obtained from the blind half-retina, while stimulation of the healthy half-retina produced inextensive, shortlasting contraction . timulu in ten ity i indicated in log units above visual threshold. ommem: Note that the "pupillary field loss" was really an increased thre hold and not in ensitivity of the blind half-retina. (From AR. Shakhnovich, Vop. Nelrokhir., 24 [1960]: 20) I ... 2,0 ii1iiih 1111111111111111 IIIIUIIIIII 1111111111n11m11111111111111111111111 1111111111111111IIIUIIIIII CUI/I/lb 111111111111uu 11111111111 111a111111 II lllllllll1a1 IIIIIIIIIIMIIIH 111111111111111 NIIIIII 1111111111111 NI IIIUIIIIII I1111111111111111 IIIIIIIIIUIIII NIIIIII llllllhtk. :mmmttm~mmmmmm::::: 1,0 0,25 lll/1/1 iiiiiii, .... iiiiiiH ::mum:: 111111111111 umm 111111111111 111111111111 11111111 11111111 11111111 tiiiii,.•• 111111110 HIIIUI un11111 UUIIINI IIUIIIIII C DEGREES OF VISUAL FIELD <l > I- iii zw Figure 17-18. Pupillary and visual functions in patients with lesions in the visual pathways. Visual thresholds are shown as black dot , and pupillary thresholds of the consensually reacting eye as circles. In each patient the meridian was chosen that contained the defect. Light stimuli were white, measured 27 minutes of arc, and lasted 0.2 second. The surrounding field was white, with intensity of IO asb. The fixation spot was red, central, and 10 minutes of arc in size (intensity 400 asb). Visual threshold was determined by having the patient activate a buzzer when the light was seen. Pupillary threshold was defined as the intensity needed to elicit a pupillary contraction for half the stimuli. The method allowed demonstration of the blind spot in normal individuals, ruling out stray light as a major experimental error. A: Congruent loss of vision and of pupillary reactions in a patient with chiasmal defect. B. Normal pupillary reactions in a 40-year-old man with cortical hemianopsia due to a tentorial meningioma that pressed upon the occipital pole. Postoperatively, vision was regained, but was lost again due to recurrence of the tumor. The pupillogram was recorded at this time, showing active responses in the blind half-field. C: Slight depression of pupillary function in the blind field of a 62-year-old man who had suffered a stroke ( occlusion of the posterior cerebral artery, about 5 millimeters distal to its junction with the posterior communicating artery). D: Noncongruent, increased pupillary threshold in the blind field of a 22-year-old woman with homonymous left upper quadrantic visual loss, encroachment upon the left lower quadrant, and no macular spar- 1rt I~ 102 DEGREES OF VISUAL FIELD ing. The defect was due to partial occlusion of the posterior cerebral artery, and total occlusion of the calcarine and the parietooccipital arteries. E: Noncongruently raised pupillary threshold in the blind field of a 23-year-old woman with a 6-year history of visual impairment. Homonymous quadrantanopsia of the upper right field, extension to the lower right field, and sparing of the fovea, due to occlusion of the posterior cerebral artery, just distal to its junction with the posterior communicating artery. (From K.A. Hellner, W. Jensen, and A. Muller-Jensen, Klin. Mb!. Augenheilk., I 72 [1978]:731)