Journal of Neuro-Ophthalmology, September 1992, Volume 12, Issue 3

Absence of the relative afferent pupillary defect with monocular temporal visual field loss.

We report five patients with monocular temporal visual field abnormalities who did not have clinically detectable relative afferent pupillary defects. The causes for the field defects were posterior ischemic optic neuropathy, craniopharyngioma, pituitary adenoma, pseudotumor cerebri, and traumatic optic neuropathy. We discuss the possible explanations for our observations, considering the known anatomy of the pregeniculate visual pathways and the afferent pupillary pathways.

Journal of Clinical Nellro- ol'hthalmology 12( 3): 181- 191, 1992. Absence of the Relative Afferent Pupillary Defect with Monocular Temporal Visual Field Loss Gregory S. Kosmorsky, D. O., Robert L. Tomsak, M. D., Ph. D., and David K. Diskin, M. D. ,~.' 1992 Raven Press, Ltd., New York We report five patients with monocular temporal visual field abnormalities who did not have clinically detectable relative afferent pupillary defects. The causes for the field defects were posterior ischemic optic neuropathy, craniopharyngioma, pituitary adenoma, pseudotumor cerebri, and traumatic optic neuropathy. We discuss the possible explanations for our observations, considering the known anatomy of the pregeniculate visual path­ways and the afferent pupillary pathways. Key Words: Relative afferent pupillary defect- Monocu­lar temporal visual field loss. From the Department of Ophthalmology ( G. S. K., D. K. D.), Cleveland Clinic Foundation; Division of Neuro­ophthalmology ( R. L. T.), University Hospitals of Cleveland, Case Western Reserve University School of Medicine, Cleve­land, Ohio U. S. A. Address correspondence and reprint requests to Dr. Tomsak at the Division of Neuro- ophthalmology, Lakeside 3200A, Uni­versity Hospitals of Cleveland, 2074 Abington RD, Cleveland, OH 44106, U. S. A. 181 The relative afferent pupillary defect ( RAPD) is the most objective and reliable sign of asymmetric dysfunction of the anterior visual pathways. The depth, or magnitude, of the RAPD is apparently correlated with the amount of visual field loss ( 1). Indeed, monocular visual field defects without an RAPD are considered a sign of hysteria or malin­gering by some authors ( 2- 3). In rare instances, completely monocular temporal visual field defects have been described with mass lesions ( 4-- 8), pos­terior ischemic optic neuropathy ( 9), optic neuritis ( 10), retinal degenerations ( 11), and the empty sella syndrome ( 12- 14). In most of these cases RAPDs were found. We report five patients, with either complete or incomplete monocular temporal visual field de­fects, who did not have clinically detectable RAPDs ( Table 1). This observation led us to reex­amine the traditional thinking about retinotectal afferent pupillary pathways and the relationship between monocular visual field loss and RAPDs. METHODS This study consisted of a thorough evaluation of five patients with monocular temporal visual field defects, either complete or incomplete, not associ­ated with a relative afferent pupillary defect. The visual acuities, visual fields, fundus appearance, RAPD and neuroimaging studies were evaluated. Visual acuity was measured by Snellen optotypes. Visual fields were done kinetically by the Gold­mann technique ( all patients), or by threshold static testing with the Humphrey visual field ana­lyzer as well ( patient 1). The RAPD was tested by using an indirect ophthalmoscope at highest inten­sity while the patients fixated a distance target 182 G. 5. K05MOR5KY ET AL. TABLE 1. Causes of monocular temporal visual field loss Case Cause 1 Posterior ischemic optic neuropathy 2 Craniopharyngioma 3 Pituitary adenoma 4 Pseudotumor cerebri 5 Traumatic optic neuropathy ( 15). Radiologic studies included intravenous dig­ital subtraction angiography ( IV OSA; patient 1), computed tomography ( CT) of the head ( all pa­tients), and magnetic resonance imaging ( 2 pa­tients). CASE REPORTS Case 1 In July 1985, L. P., a 55- year- old man with a his­tory of severe coronary artery disease, had an ep­isode of severe headache with complete blindness lasting 30 minutes and resolving completely. Intra­venous digital subtraction angiography done shortly afterward showed only a small amount of calcification at the origin of the left internal carotid artery. In December 1985, he suddenly and perma­nently lost vision in the entire temporal field of the left eye. Computed tomography of the head was normal. Neuro- ophthalmologic examination re­vealed right eye vision of 20/ 20 + 3 and 20/ 25 left eye vision. A relative afferent pupillary defect was not noted on this or subsequent examinations. Pu­pillary size under direct illumination was equal; that is, Kestenbaum's number ( 16) was zero ( Fig. lA- C). Funduscopy showed a cholesterol embolus in the inferotemporal artery OS; optic atrophy was not noted and the right eye was completely normal except for mild nuclear lens sclerosis. Visual field testing by Humphrey ( Fig. 2A, B) and Goldmann techniques ( Fig. 3A, B) showed a complete monoc­ular temporal hemianopia in the left visual field. Duplex carotid sonography done in November 1986 showed evidence of plaque formation at the origins of both internal carotid arteries but less than 20% stenosis. Visual fields and pupillary ex­amination were unchanged as of April 1987. Case 2 was 20/ 30 with the right eye and 20/ 20 with the left eye. Pupillary examination revealed 1 mm of an­isocoria with brisk direct reactions. There was no relative afferent pupillary defect clinically. Ocular motility was normal. Dilated fundus examination showed bilateral optic atrophy, more marked on the right. The right visual field had a temporal hemianopic defect ( Fig. 4A), whereas the left vi­sual field was within normal limits ( Fig. 4B). On 11/ 3/ 89 he was reexamined and had bitemporal vi­sual field loss due to recurrence of the tumor. A 0.9 log unit RAPD was present 00 ( 17). Case 3 D. C. was a 51- year- old woman last examined in November 1988. She was status postsurgery for removal of a pituitary adenoma. Vision with the right eye was 20/ 20 - 3 and was 20/ 40 with the left eye. Visual fields revealed a left temporal hemian­opic scotoma as well as depression of the left su­perotemporal isopters ( Fig. 58). Visual field testing of the right eye was normal ( Fig. SA), and a rela­tive afferent pupillary defect was not detected. Case 4 A 38- year- old woman ( 0.5.) was followed seri­ally for pseudotumor cerebri, and was last exam­ined in August 1989. Visual acuity was to 20/ 15 with each eye, and color vision was normal by pseudoisochromatic color plates. Numerous visual fields by the Goldmann technique showed consis­tent depression of the inferotemporal isopters on the right only ( Fig. 6A, B). Mild postpapilledema optic atrophy was present in the right eye; the left ~ ptic disk was flat and spontaneous venous pulsa­tions were present bilaterally. An RAPD was not detected on this or any previous examinations. Case 5 A 27- year- old woman involved in an automobile accident on May 27, 1985, suffered a closed head injury with traumatic right optic neuropathy. An mferote. mpora. 1 scotoma has persisted in the right visual field ( Fig. 7A, B). Best corrected acuity was 20/ 30 - 2 with the right eye and 20/ 15 with the left eye, without evidence of a relative afferent pupil­lary defect. Computed tomography of the head was normal. A 19- year- old black man ( K. B.) was first evalu­ated on June 15, 1989 because of difficulty with distance vision. He had a craniopharyngioma op­erated on in early 1988. Best corrected visual acuity , .'.'", '\', · I/ rel · ophlha!," o!. Vol, 12, No, 3. 1992 Discussion Chiasmal visual field defects are typic poral, but may be junctional as describe. ' em­' il- MONOCULAR TEMPORAL VISUAL FIELD LOSS A 183 8 c FIG. 1. ( A) Pupils of patient 1 in room light. ( B) Illumination of right pupil of patient 1 in total darkness. Photographed by electronic flash. ( C) Illumination of left pupil of patient 1 in total darkness. Photographed by electronic flash. brand and Saenger ( 18) and Traquair ( 4,10), and are most often caused by mass lesions. Monocular temporal hemianopic defects, whether complete or incomplete, are equally important because they have the same localizing significance ( 7,8,10,18). The topographic anatomy of the optic nerve fi­bers in the anterior visual pathways, projecting from the retinal ganglion cells to lateral geniculate body, is understood. Macular fibers, especially the papillomacular bundle, in primates and man, are located temporally in the optic disk and distal optic nerve, but come to occupy a large central portion of the more proximal optic nerve near the chiasm ( 19). The upper macular fibers cross dorsally in the chiasm, and the lower macular fibers cross ven-trally. The extramacular fibers from more periph­eral retinal ganglion cells remain peripheral in the optic nerve and cross anteriorly in the chiasm ( 19, p. 387). The more ventrally placed crossed fibers, predominantly from inferior retina subserving su­perotemporal visual field, loop anteriorly in the contralateral distal optic nerve as far as 3 or 4 mm, before turning back toward the lateral geniculate nucleus. This fiber path was initially termed the " commissura arcuata anterior" by Hannover and now is more often referred to as the " anterior knee" of Wilbrand ( 19). This anatomy helps to ex­plain the two types of junctional scotomas seen with anterior chiasmallesions. Wilbrand and Saenger ( 18) noted that damage to JClin Neuro- ophthalmol. Vol. 12. No. 3. 1992 184 G. S. KOSMORSKY ET AL. A FULL . F i ELri 120 PO I NT "'; CREEN 1 r- IG ", ES r ':. r I l'lUl_ U~:, ~ 1 I , . I,., IH I fE. 8CKGNO ' j.\. ' 5 HS8 f:: LHH: 1 ':'. PUT CHECK S. lZE 1 I 1 F l),: t= n l( lt~ T'= IPGET CENTRHL ':; TPl- ITEI,; Y TH~: F.. r ZOt~ F::. 1_ E. d >- l OE: F'E'-: I j · 5 DE:: tl~ r'lE 10 13723 B1RTHO~ TE 12- lB-~ 6 URfE 08- 07- 86 TIME 09: 00= 31 ~ 111 PUPIL OIAl'lETER V!= f 20 · , 20 R:'~ U':, E[ I +;:. <:; 0 Cr..::. [ 11::: DEI..? P J C · II T • • r +- FHL:'::: E pr)' j EPPOF" 3 l) 14­FRL'':, E N£ G EP. PO~; · j l). ~~--~-~~ Cd;+-.;~. L=~~'~ E'~ ~ '~-~~ Jl2J :: I = != lG'':'; IJLIJ fE iJ;::: F~ I: 1 ':-: ~:. ! 20 i. I ~ f-~: 14it~ O ':, F" I: IT .. FIG. 2. ( A) Right visual field of pa­tient 1 by Humphrey machine. ( B) Left visual field of patient 1 by Humphrey machine. the anterior angle of the optic chiasm could result in an ipsilateral central scotoma with a contralater­al upper temporal visual field defect that respects the vertical meridian. In 1920, Traquair ( 4) pub­lished the case of a 28- year- old woman who suf­fered from a large pituitary tumor. A monocular upper temporal scotoma occurred initially only in the right visual field ( 4). Later, in the classic text Clinical Perimetry ( 10) Traquair referred to this type of visual field defect again in a discussion of le­sions of the optic nerve: At the chiasmal termination of the nerve the di­version of the crossed from the uncrossed fibres permits of the predominant involvement of one of these groups, more especially as regards the mac­ular fibres, and the production of unilateral hemi­anopic defects. Scotomata of this type may be r Cli" Ncuro- ophthalmol, Vol. 12, No_ 3, 1992 called " junction scotomata" on account of their site of origin at the junction of the nerve and the chi­asma. ( 10, p. 84) Pathologic verification of the anatomy of temporal hemianopias in man was done by Unsold and Hoyt ( 20) who, at autopsy, obtained the left optic nerve of a patient with a blind right eye and a left temporal hemianopia due to an intracranial aneu­rysm. They showed that nasal segregation of the fibers destined to cross begins before the chiasm and commented that a lesion affecting the nasal side of one optic nerve could exclusively affect the temporal field of vision. Unfortunately, the retinotopic organization of the afferent pupillary fibers is uncertain. The clas­sical teaching is that the afferent limb of the direct pupillary light reflex ( PLR) is subserved by axons MONOCULAR TEMPORAL VISUAL FIELD LOSS B ~ U L L. FIE L D t 20 PO JUT :=.; C · F~ E E tl I , .• C; T E :::; T 185 .:. = F-' Otrnc;. · :. EEr~: ::': t · 120 .: = F · ELATJ' · ... E [ IEFEI~ T · :.: _ 120 • = AE:': · OLI. ITE ( lEFEI~ or ':.: "::-:: 120 6 = E: L 1tJO ':. F'I) T • $ T I t' 1lJLl. l':::. 1 I I. l. lH I TE. E:(. (. tKl Bl. 1tiD -:. F'OT .: HEC t- -:.. I ZE ( IF F F t :<~ T I i)~~ T~ F · t=- E T CEtlTF'HL ST~ · ATE(.' I · THF'EE = nnE: I: EU 34 08 F" EF':: 4 DE: Ft:,: HTtl'lt~ l.( i-:,'-. E · ; ( t ( I FHI. ~, E F · ( I · ~. EFO'ROf;'':. 1 14 FAt ':. E nf (. Ef; · P( IF'-::. ~. J( I lE": · " T It1E 00: I I :~' 4 Hr'H ..;. ra • • • • ::: 1.':. ':": · 8 tIAf" 1E I [ I t · -: c:=- · · ~ F:: 1F'TH( IHT E 1.:: - 1 t · -~',:. ( lATE ( I:~ - I);" _::: 1':. T I r · tE ( I'~: 1 ';': 4 ~ Ht" F'UF'll 0 I Hf'lE TE~' ',:' H .2( 1 .:',:. F<: lJ':. EO + 2'. ~, o [ I~. · 1 .( 11) [ 1I~:: ":) 1) ( IF( · LEFT • • • • FIG. 2. Continued . that partially cross at the optic chiasm and synapse in the pretectal area of the midbrain ( 21). In man approximately 47% ofaxons from retinal ganglion cells remain uncrossed, while 53% of fibers cross in the chiasm ( 22); this crossing asymmetry probably includes pupillary fibers and may partially explain contralateral relative afferent pupillary defects in optic tract hemianopias ( 23), and with lesions af­fecting the pretectal region as reported by Ellis ( 24). He described a right- sided pineal region tu­mor affecting only the pupillary pathways. Pupil­lographically, the responses in the left eye were diminished and the patient had a clinically recog­nizable RAPD as a presenting sign. All other as­pects of visual function were preserved, including visual acuity, visual evoked potentials, color vi­sion, and visual fields. Similar cases have been re­ported subsequently ( 25,26). Pierson and Carpenter ( 27) noted that, in mon­key, the pretectal olivary nuclei ( PON) receive more retinal projections than other pretectal nuclei I'j H t.. 1. E F: I-:~ H r- t r. tot I I " 1 P H ~~ E ' 1" F'F',... ~ F and felt that they play the major role in the papil­lary light reflex. In cat, Distler and Hoffman ( 28) noted bilateral, but predominantly crossed projec­tions from eye to pretectal olivary nuclei with a ratio of crossed to ipsilateral of 17.6: 1. Bilateral pro­jections also occur to the pregeniculate nuclei ( PGN), and the PGN in turn projects to the pre­tecta I olivary nuclei. In primate retinal lesion stud­ies, Polyak ( Ref. 19, pp. 30~ 307; 33~ 353; 376- 385) demonstrated that the majority of fibers projecting to the PGN came from the macular region of the retina, and he concluded that the PGN probably played an important role in the pupillary light re­flex. Pierson and Carpenter ( 27) refuted this pos­sibility by showing that lesions in PGN do not in­hibit the light reflex in monkeys. They ( 27) sug­gested that PGN more likely plays a role in accommodation, since it receives inputs from cor­tical visual and visual association areas as well as from the retina. The pretectal olivary nuclei also receive direct cortical input from cat areas 17, 18, I Clin Neuro- ophthalmol, Vol. 12, No. 3, 1992 186 G. S. KOSMORSKY ET AL. FIG. 3. ( A) Right visual field of pa­tient 1 by Goldmann technique. ( 8) Left visual field of patient 1 by Gold­mann technique. _. ,~, ~:. .." (~ " 11 ~, ." ~ llt> : sph = . ~. I svt>: ~'" __ ' ~ t'h:; c",_. . , I · " e"....... ,". I~ ''''" 0'"°,,'_''''" 11''. ".~_"~..'''' ,- ol'' ll I~~.. "' 0" ll ,,,,••,... .... · - t...._-,-, j, _--- . c. ~:,~, .... ' i:~ J 18', _. 13". , / / . / / / ~ I ./ / /' . I / ,/ f-. j ! i " ( ' 1" ,.~ ' 1' 1- \ \ I 16"). I! _ 18() r' · J · · ,,, , -, \ - ~ 19~"- · . CLEVElAND CLINIC r= OUNOA1IQN 13/ 6",' ­00.0. - Red fjl} 0.$ - Bla. ck / _ IRU," ~" iiII 0 0 - R, O DOS - Black B A 19, and 20a ( 28). The lateral terminal nucleus of the accessory optic system may also playa role in the pupillary light reflex, since it is directly, but con­tralaterally, connected to the pretectal olivary nu­clei ( 29). Whether the fibers that are responsible for the pupillary light reflex are separate from visual fi­bers, or whether they are axon collaterals, has also been debated. Given that there are a group of ret­inal ganglion cells ( W cells) that are luminance­sensitive and project to the pretectal region of cats ( 28,30--- 32), it is possible that separate axons are responsible for the pupillary light reflex. Centrally located W cells in the cat retina differ from the X retinal ganglion cells that most likely subserve vi-sual acuity ( 33). However, Tychsen and Hoyt ( 34) found RAPDs contralateral to congenital occipital hemianopias in two patients. Both had homony­mous hemioptic atrophy, presumably from trans­synaptic degeneration. Their observations would be most consistent with a visual- pupillary axon collateral arrangement. The evidence linking pupillary fibers to visual fibers is based upon the almost constant associa­tion of visual field loss with pupillary abnormali­ties. Furthermore, RAPDs most often occur in le­sions, including amblyopia ( 35), that affect central vision, therefore implying that the bulk of fibers subserving the afferent arc of pupillary light reflex travel with, or are directly a part of, the papillo- I CIII' Nfllro- ophtlwlmol, Vol. 12, No. 3, 1992 MONOCULAR TEMPORAL VISUAL FIELD LOSS 187 FIG. 4. ( A) Right visual field of pa­tient 2 by Goldmann technique. ( 8) Left visual field of patient 2 by Gold­mann technique. DIIotol.,.. ' ...' -- , ":' - rl l \,. " -"- 330 ( yl_ ­.. ell' ." " I") CII,,'" I ' 1','-' r'," Go<_ l1... , ph~_",_ · "__ , ph:_ CVI __ '= ("..... 110.. , ul'I :: ..~ I'I :: 270 · OcN...- lfItllM. '"",,, 1""........ II'lII ' I ... , I .,,,..." 9.,.... 1<; 1•• ... "" Il bod•••• O..'. I\'. Oln. CLEVELAND CLINIC fOUNOATION CLEVELAND CLINIC FOUNDATION No ,= 10.0: 11. : I 0.100 30031• .. ', 00 w~. ..... fo1~ l ~ 1'''' ~:~ .~;~ ~ ~ 2~' , O. OJ" J 0.' 00 t:~~;~ ( l~" 136~' / // \ 00.0.-.... /', go. s. - Bl. ck /" Y / / ,.. I . I ' 00. ,." tiloo. - R•• DO, s. - Black I ' -""' f" '" I : , B A macular bundle. Yet, it is noteworthy that RAPDs occur in many patients with optic neuritis who re­gain normal central visual acuity ( 36- 38). In the series of Kupersmith et al. ( 38) these cases recov­ered to 20/ 20 vision; contrast sensitivity functions were uniformly abnormal, thus raising the possi­bility that afferent pupillary information and con­trast sensitivity information can be selectively damaged in optic neuritis. Wall ( 39) concluded that the functions of P retinal ganglion cells- com­parable to cat X cells- are mainly affected in optic neuritis. Unfortunately, pupillary reactions were not measured in his study. Lastly, in a study cor­relating RAPDs with visual field defects detected by threshold static automated perimetry, Johnson and coworkers ( 40) described four patients with RAPDs but no detectable visual field defects. Con­versely, certain lesions of the central vision, espe­cially those caused by macular disease, do not present with RAPDs as a rule. In cat, the major projections to pretectal olivary nuclei are from ven­tral retinal ganglion cells ( i. e., superior visual field) ( 28). Thompson and coworkers ( 1) reported a linear relationship between the magnitude of the afferent pupillary defect and the amount of unilateral vi­sual field loss measured by Goldmann perimetry; they found a more variable relationship between visual acuity and size of the RAPD. Furthermore, they did not specifically comment on temporal JClin Neuro- ophthalmol, Vol. 12, No. 3, 1992 188 G. S. KOSMORSKY ET AL. FIG. 5. ( A) Right visual field of pa­tient 3 by Goldmann technique. ( B) Left visual field of patient 3 by Gold­mann technique. -.... ­' -.,.., ­......,' n'.... "' j-' ': p ., 0 --,..-.- , r,. 11 ~ ._ .: • . v,-: _ ~ p" : '. pM:::: ., ...''' O~ ,,,. " 0­"''' 11 .""•• " ••~ ,.... ".... · ' C" 0"' ll0'''. I_ ,. ~~ '''< I •• """ 9'"'' "'" ' 20 ~- CLEVELAND CLINIC fOUNOII. T10N rl J= vElANO CLINIC FOUNDATION 16! l, , - lBO:~ o $ i~ I • "', 00 O. - Red fi 0.5. - BI. cl< _ 11;,.- · . · · · " I" iii 0.0 - Red DOS - Black B A hemianopias. Using their template technique on the kinetic visual fields of our patients, we calcu­lated that all should have had clinically observable RAPDs ( Table 2), although these were not noted by us. A similar correlation of visual field loss with the results of automated perimetry was reported by Johnson and coworkers ( 40), and their report strengthens the concept of pupillomotor represen­tation concentrated within the central visual field. Our observations also differ from the predictions indicated by the pupillographic phenomenon of contraction anisocoria observed in normal subjects ( 41,42), wherein stimulation of nasal retina ( i. e., temporal visual field) causes a greater direct and consensual reaction than does stimulation of tem­poral retina, thus suggesting a greater pupillomo­tor drive from nasal retina than temporal retina. The presence of an RAPD contralateral to an optic tract lesion is similarly explained by the predomi­nance of crossed ( i. e., nasal fibers; temporal visual field) versus uncrossed fibers in the optic tract ( 22), in accord with the well- known fact that temporal visual field is larger in area than is nasal visual field ( 23). It could be argued that, if we had used a more sensitive method like pupillography, our patients would have had RAPDs; if so, they clearly would have been of small magnitude. As noted above, Distler and Hoffman ( 28) found that cat ventral I C!,,,'-: e, m" ol'hthalmol. Vol. 12. No. 3. 1992 MONOCULAR TEMPORAL VISUAL FIELD LOSS 189 FIG. 6. ( A) Right visual field of pa­tient 4 by Goldmann technique. ( B) Left visual field of patient 4 by Gold­mann technique. _.. "",' .+ .~...'.. ,- l! o -. pto= __.. ':, 1. _.. ~ pn;:-; • e~ l ,.. _ i . • ~ ph:::; .._,;,, 1 , ph::: :~ t~."' te '~ I "".. ... I~~ 1ft400. '''''' 8 1~ 1. "'" · . t~.~' h•• lOe '''''' lI''' lIl''' Of> g,,,.. Ilt1. • '<) 0 ~, \. \ \ 21U'\ , • O. O~ l' . ',~ " . , 0,'" ", 00 ... ,~:::. CLEVELAND CLINIC FOUNDATION I - 18Q~,~- ' t," \ \ ,.,\ ..":~, \\ o ',' M \ -, • CLEVELAND CLINIC FOUNDATION ... ""'" o ,,.. I:' .,; '; 110', r. 1\ 1 " // "''' f- :'- . 4 110.0. - Rod o 0.5. - alack ; j _ leo,"" ~ o I .\ 00.0.- Red IlO. S. - Black 8 A retinal ganglion cells ( i. e., upper visual field) are responsible for the majority of afferent input to the pretectal olivary nuclei. It is also possible that the use of a test light of lower intensity, as discussed by Borchert and Sa­dun ( 43), might have uncovered RAPDs in our cases. In any event, the ideal conditions for elicit­ing a RAPD are still under discussion ( d. refs 15 and 17 with 43). The dissociation between monocular temporal visual field loss in our patients and absence of clin­ically detectable RAPD is open to a variety of in­terpretations. First, it is unlikely that errors in ob­servation played a major role, since most patients were followed serially and examined by more than one ophthalmologist. Also, we have seen other pa­tients with monocular temporal visual field defects who had RAPDs ( Tomsak and Kosmorsky, unpub­lished). Second, although monocular temporal defects are often considered a sign of nonorganic, or func­tionaL disease, our patients differed in several re­spects. Two of our patients had tumors of the chiasmal region demonstrated by MRI or CT scan­ning, one had optic nerve damage from pseudo­tumor cerebri, one had carotid disease with a visible retinal embolus and presumed posterior isch­emic optic neuropathy ( PION), and one had sud- JClin Neuro- ophthalmol, Vol. 12. No. 3, 1992 190 G. S. KOSMORSKY ET AL. FIG. 7. ( A) Right visual field of pa­tient 5 by Goldmann technique. ( B) Left visual field of patient 5 by Gold­mann technique. ","'" .~... __, 11' 1'_ ~ o"". ' . i rtI - g V - .1./..... w, • Q • e- ~ 3Ci '- I- J 1ft... . c.......... ":~.,, · !.; 1~ / . . ] 30 c,_, ./ '", ~ · ,..... oJ .... c......... J: i,....~ !.;; J.:; '. 11,5 .<> _. / · , c-."... 1d< 1 '.''' Q,_.~ I''''''''' I I · . 0'"'-' _ . ........ W1 ' l'iHM .... -'''-~--'" 1-_._ 4-------.:.---- ",-. ~~~.. .---- n- o -_. I ' i. 210' , 101•••~ o '/ .. - , 0.\ 0( 1 t 3 O~' 1S :-!.. lUO IV tl , .. "'. ,::".::. 22~ CLEVElAND CliNIC FOUNDATiON CLEVELAND CLINIC FOUNDATION B A Calculations done by the method of Thompson et al. ( 1). TABLE 2. Predicted relative afferent pupillary defects den persistent loss of vision following head injury ( traumatic optic neuropathy). Most visual field de­fects were reproducible over time. One of our patients had visual field examina­tions by both Goldmann and Humphrey tech- Case 1 2 3 4 5 Predicted RAPD ( log units) 0.8 0.7 0.3 0.4 0.3 niques ( Case 1). No obvious differences were found, indicating that there was not an unrecog­nized subtle defect in the opposite eye or more generalized defects in the ipsilateral eye. Why some patients with temporal hemianopias, as in our series, do not have RAPDs, whereas oth­ers do ( 7,8,11), implies that the visual pathways and pupillary pathways are functionally separate, and perhaps anatomically variable, especially in the region of the posterior optic nerve and anterior chiasm. In addition, our observations suggest that visual and pupillary pathways can be differentially affected by various types of lesions. In closing, we wish to suggest that patients with monocular temporal visual field loss should not be I Cli" Nellro- ophthalmol. Vol. 12. No. 3. 1992 MONOCULAR TEMPORAL VISUAL FIELD LOSS 191 rejected out of hand as having a functional visual disturbance, even if a RAPD is not present. This visual field defect, which has exquisite localizing value to the ipsilateral and medial prechiasmal portion of the optic nerve, should prompt neuro­radiologic investigation. Furthermore, clearly more remains to be learned about the retinal dis­tribution of ganglion cells involved in the afferent pupillary response to light. Acknowledgment: The authors wish to thank Profes­sor R. W. Guillery for his helpful comments. REFERENCES 1. Thompson H. Montague P, Cox T, Corbett J. The relation­ship between visual acuity, pupillary defect, and visual field loss. 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