0022-3018/84/1726-0364$02.00/0 THE JOURNAL OF NERVOUS AND MENTAL DISEASE Copyright © 1984 by The Williams & Wilkins Co. Vol. 172, No. 6 Printed in U.S.A. SINGLE CASE STUDY Dissociated Perception of a Visual Defect ANDREW LEE BENDER, M.D. A case of amaurosis in the right eye with a left homonymous hemianopsia and right superior quadrantanopsia is reviewed. There was marked dissociation in the patient’s perception of his visual defects. The concepts of denial as presented in the literature were reviewed. The lack of denial in children was discussed in association with hemispheric specialization. Denial of a neurological deficit may be the result of improper arbitration of information between the two cerebral hemispheres. Hemianopic visual field defects accompanied by denial occur frequently. Observations on a patient with visual defects of central and peripheral origins occurring almost simultaneously are uncommon. A patient with a right cilioretinal arteriolar occlu- sion, a right homonymous superior quadrantanopsia, and a left homonymous hemianopsia was examined. Shortly after the initial examination, a cerebrovascu- lar accident occurred attended by dense left hemipa- resis and left hemispatial neglect. At the time of the cerebrovascular accident, the patient appeared cen- trally blind but later this resolved to a right homony- mous quadrantanopsia which resolved slowly. The left homonymous hemianopsia and amaurosis in the right eye (OD) gradually resolved over a year. During the period of his illness, the patient manifested marked concern about the monocular blindness but neglected and denied his quadrantanopsia and hemianopsia. A review of the literature failed to reveal any reports of a similar pattern of denial. Case Report A 62-year-old man with a history of cigarette smok- ing, hypertension, and diabetes mellitus was seen. He was taking oral hypoglycemics for his diabetes and beta blockers and diuretics for his hypertension. There was a past history of angina pectoris and a myocardial infarction secondary to arteriosclerotic cardiovascular disease. He was described by his family physician as being a pleasant person who “listened to advice care- fully but then ignored everything he was told.” On the night of admission he awakened to find that he had *The Mount Sinai School of Medicine, New York, New York. Address reprint requests to Dr. Bender, Neurological Service, Pas- cack Valley Hospital, Westwood, New Jersey 07675. The author wishes to thank Edwin A. Weinstein, M.D., for the many helpful suggestions he made. Ms. Margaret De Marrais and Ms. Debbie Michaels were instrumental in obtaining many of the reference materials used in the preparation of this article. 364 lost vision in OD. There was mild pain and tenderness in the right periorbital region, more so superiorly. He immediately sought medical attention, and an ophthalmological consultation disclosed no light per- ception in OD and acuity of 20/25 in the left eye (OS). Intraocular pressures were normal. The ocular fundus of OD disclosed a cherry red spot at the macula, segmentation in the perimacular vessels, and a pale optic disk. A diagnosis of probable cilioretinal arter- iolar occlusion was made. Therapy was instituted immediately. About 12 hours later he regained finger counting vision in OD. About 1 day following his admission an ophthalmolog- ical examination revealed hand movement vision in the temporal visual field of OD. An examination of OS was not performed. The patient complained bit- terly of the loss of vision in OD. A right carotid bruit was noted and a neurological consultation was ob- tained. Neurological examination demonstrated dysphoric behavior. He behaved in a detached, sullen manner during the anamnesis and examination. At times he became hostile and angry. The remainder of the men- tal status examination was normal. By confrontation testing with finger movements and counting fingers there was a dense left homonymous hemianopsia to all but gross movements. The area of macular vision was “split” as demonstrated by confrontation testing. In addition, there was a right superior quadrantan- opsia in OS to all but gross movements. In OD, despite the vascular occlusion, the patient could identify ob- jects such as the examiner’s arm, a sweater, and a book in the right inferior quadrant but not in the right superior quadrant or left hemifield in which only hand movements could be perceived. In the fundus of OD the cherry red spot in the macula persisted and yellow exudate was now noted about the peripapillary blood vessels. Specific tests for a positive or negative sco- toma in OD or OS were negative. Optokinetic nystag- mus was present as the tape passed from right to left DISSOCIATED PERCEPTION OF A VISUAL DEFECT 365 but absent as the tape passed from left to right in the horizontal plane. There were errors in graphesthesia over the left side of the face. The patient denied that there was anything wrong with his vision aside from the loss of vision in OD. The eyes showed no gaze preference. He made errors in directional cutaneous kinesthesis on the left, mislocalized with his left hand in his left visual space, and displayed intermittent left hemi-inattention. There was a drift and pronation sign of the left upper extremity. Gait was somewhat broad based but no lower extremity weakness could be demonstrated. The reflexes were sluggish and both plantar reflexes were flexor. There was a high-pitched right carotid bruit. A lumbar puncture yielded clear acellular, colorless fluid with normal opening pressure and proteins. The initial computerized axial tomography (CAT) scan, done 36 hours after admission, demonstrated a wedge-shaped, right parietal lobe infarction (Figure 1). Anticoagula- tion with heparin was initiated. About 10 hours after the initial neurological exam- ination, the patient suddenly developed a dense left hemiparesis and a neglect of his left-sided extremities. At this time he appeared to be completely blind. He did not identify objects shown to him. He was hostile, abusive, restless, and unable to cooperate for testing. He denied that he was blind or that there was anything wrong with vision in OS even though it was almost certain he could see nothing. He protested the loss of vision in OD. A few hours later the apparent central blindness seemed to recede, leaving the previously noted macular splitting, dense, left homonymous field defect and right superior quadrantanopsia. He denied vigorously that anything was wrong with his visual field but complained bitterly of the loss of vision in OD at the same time. Despite every attempt to dem- onstrate to the patient that he could not see anything in the left homonymous field or in the right superior quadrant, he would defend his visual ability by making up excuses about his lack of vision in the centrally defective fields and would confabulate that he could see. There was a dense left hemiparesis. First this was denied by the patient, who when asked to raise his left arm would raise his right arm. In answer to questions as to why he did not use the left arm he replied, “whenever you use something, it hurts.” There was a hemispatial disorganization so that the patient had no idea where his left extremities were. When asked to localize a moving target in space on his right side he was able to do so but he could not do so on the left side. He would initially not use his right arm to do any tasks in his left visual space. When asked to touch the examiner’s finger on the right, for example, he would correctly go to the finger with his right hand and touch it. When the finger was moved into his left body space, even when he moved his head and eyes to follow it, he would not touch it but would confabulate that he had touched it. About 2 days after the sudden onset of his left hemiparesis, although still irritable, he could cooper- ate enough for testing to obtain the visual field charts shown in Figure 2. Several days later he admitted to marked weakness in the left-sided extremities but insisted that vision in his left eye was “perfect.” Elec- troencephalographic examination disclosed marked reduction in the amplitude of the alpha frequencies on the right side of the head with complete loss of right posterior alpha rhythm. No delta or theta activ- ity was present. The patient was followed for about 6 weeks without change in his visual denial, although he admitted difficulties with mobility and strength of his left-sided extremities to an increasing degree throughout this period. Catastrophic behavior consisting of crying oc- curred to an increasing degree as the denial of hemi- OS. Fic. 2. Visual field examination done 3 days after admission. 0.0. The stippled area indicates vision intact to form and motion; the shaded area indicates amaurosis. The chart is stylized from a bedside examination by confrontation. Fic. 1. CAT scan showing a large right parietal infarction. 366 BENDER paresis waned. An arch aortagram demonstrated a complete occlusion of the right common carotid artery. Literature Review and Discussion This case presents an interesting phenomenon of verbal denial of one form of a visual defect while simultaneously complaining of another form. A dis- tinction between denial and neglect should be made. Verbal denial is either explicit in the sense that it is proclaimed by the patient or passive where it is not specifically mentioned but is alluded to, Neglect is an implicit process generally manifested by evasion, avoidance, or humor. Neglect for one side may be active in the sense of selective performing of tasks on the unaffected side and not performing them on the affected side. Bender et al. (6) described the case of a patient who did not recognize familiar people coming from his defective, left side but had a startle reaction when unfamiliar people approached him from that side. Frequently, as in the case, denial and neglect exist simultaneously, making them difficult to sepa- rate. In 1876, John Hughlings Jackson (19) described a case of left homonymous hemianopsia accompanied by neglect of the left visual space which he termed imperception. Neglect of hemianopsia as a clinical entity was not reported again until 1893, when Eugen Bleuler (7) published a case of hemianopsia, amnestic color blindness, and hemiparesis accompanied by what he termed Seelenlahmung. This German word trans- lates into the English phrase “mind paralysis” and was coined by Bleuler to denote neglect. Denial of hemianopsia has attracted less attention in the literature than denial of complete blindness. Gordon Holmes (18) observed that it was rare for hemianopic patients to complain that they could not see. Critchley (9), studying the patterns of denial in various forms of visual loss, found that visual defects resulting from posterior lesions generally produced more symptoms of denial than visual loss from ante- rior lesions. Critchley noted denial of hemianopsia less frequently in cases where the field defect occurred suddenly and in cases where central vision was pre- served. After Bleuler, there were many reports of denial, including Babinski’s article (3) on anosognosia. Anton attempted explanation by theorizing that there were interruptions of the “pathways connecting the various lobes of the brain” (2, p. 123). Redlich and Bonvincini (27) argued that denial was just a special manifesta- tion of Korsakoff’s psychosis, and Pick (25) stated that there was a lack of representation of the affected area in the brain. This latter view was also advanced by Paul Schilder (28). Schilder expanded Head’s (17) sensory representation theory to include a sense of general body existence, calling denial of illness “or- ganic repression,” a term that introduced more con- fusion than it dispelled. Schilder inferred that disrup- tion of the topographic relationship of soma to brain resulted in a defective cerebral topographic perception of the body and consequent lack of awareness of the affected body part with denial of its existence. Gerstmann (15) studied the problem of denial and its relationship to phantom limb syndrome. He con- cluded that the sudden loss of amputation was neces- sary for the formation of the phantom limb. Drawing a parallel relationship between phantom limb and denial he, like Schilder, used the concept of a disrupted body image as a mechanism for denial. According to Gerstmann, hemi-neglect and anosognosia resulted from focal brain disease involving the pulvinar and the right parietal cortex as well as the thalamoparietal pathway. He postulated that the closer a lesion was to the cortex, the more probable were the complex psy- chological manifestations. On the other hand, Wein- stein and Cole (30) reported that anosognosia did not occur after cortical ablation. This was borne out by the studies of Perenin and Jeannerod (24) and Perenin (23). These authors studied six subjects who were surgically decorticated for intractable seizures. Only one patient of the six developed neglect and that was only of brief duration. It should be noted that although the parietal cortex was removed, other areas of cortex were surgically resected as well. Joynt (20) points out that inattention syndromes in split-brain man are uncommon and that many are partial. Denny-Brown and Chambers (11) believed that an- osognosia was just one of many manifestations of a perceptual distortion resulting from a cortical lesion of one parietal lobe. Assigning the function of syn- thesizing spatial dimensions of contralateral stimuli to the parietal lobe, they termed defects in this mech- anism “amorphosynthesis.” Anosognosia, one mani- festation of amorphosynthesis, was believed to be a defect in handling sensory information derived from one side of the body, a “mental avoiding response.” Cutting (10) studied 100 acute hemiplegic patients. He found apathy, visual field defect, and impaired picture identification to be the most significant cor- relates of anosognosia. He also concluded that the disorder was a failure to integrate information from one side of the body. Some authors favored a strictly motivational etiol- ogy for denial. Lauretta Bender (4) described denial and confabulatory behavior in patients with the dis- figuring effects of Paget’s disease. She believed that denial was chiefly a psychiatric defense mechanism which arose because of damage to the self-image, social isolation by a process mysterious to the patient, and DISSOCIATED PERCEPTION OF A VISUAL DEFECT 367 thwarting of the libido. Goldstein (16), realizing that denial occurred in a substrate of neurological disease, nevertheless felt there was no structural neurological basis for denial and ascribed its manifestation to the its protective role in isolating the patient from the stress of degradation of function. Weinstein and Kahn (31), after an extensive study of denial, concluded that denial fulfilled an expressive requirement set by the premorbid personality. They stressed that an enduring denial of disabilities such as hemianopsia or hemiple- gia never occurred in the absence of organic brain disease. Later, Angelergues et al. (1) pointed out that denial which accompanies blindness is not an isolated phenomenon but occurs as part of a change in person- ality as an adjustment to dealing with a new way of life. Many investigators were able to bring about mental changes, including denial, by injections of barbiturates in patients with preexisting brain damage. Other au- thors report organic mental syndromes as well as denial after electroconvulsive therapy, insulin coma therapy, and episodes of significant hypoglycemia. Large structural lesions of the brain involving more than one lobe, the limbic system, and major portions of the central nuclei also produce denial. These studies lend general support to the theory that denial is pro- duced by a general compromise in cerebral function. M. B. Bender (5) was of the opinion that denial of a neurological defect only occurs in a substrate of or- ganic mental syndrome. Other evidence suggests that hemianopic field de- fects may not be “denied”; rather, they are not per- ceived. Imperception of a field defect may be a clue as to how the visual perceptive mechanism functions. Dimond, Farrington, and co-workers (12-14) fitted special contact lenses to normal subjects. These lenses were designed to restrict vision to a small part of a single visual field. The wearer of these lenses loses foveal vision and there is displacement of perceived vision 20° laterally. No subjects experienced any sub- jective loss of vision. Clinical ophthalmologists see the same syndrome in their patients with glaucoma. These patients, who may lose a large area of their monocular visual field and yet fail to realize it, do not generally have any disease of the brain. Dimond and his co- workers postulated that these phenomena were pro- duced by “completion.” Completion is a visuopercep- tive phenomenon in which the subject “fills in” that which is perceived as missing in a visual stimulus. Thus, the number “3” may be misinterpreted as “8” or the letter “C” might be interpreted as “O.” Completion is not observed in normal subjects un- der any conditions. Tachistoscopic presentation of visual stimuli such as the digit “3” or the letter “C” are not completed by normal individuals; rather the subject says, “It wasn’t there long enough for me to see it.” Poppelreuter (26) studied completion in patients with brain injuries. He believed that completion was the result of a shift in the fixation point of the eye, but later investigation showed that the eye could not move to the new fixation position fast enough for this to be the case. In addition, completion was strongly associated with denial of a visual field defect. Following the work of Trevarthen and Sperry (29), Levy (21) designed an experiment that allowed a chi- meric stimulus to be shown to both hemispheres of patients who had a commissurotomy. They were given a series of pictures in free vision which they matched with the chimeric stimulus in function or appearance. Dissociation occurred in three of the four patients. Levy inferred that the two hemispheres were not only specialized with respect to cognitive ability but also with intentions to act. These intentions seemed to be mediated, at least in part, by somewhat independent neural systems. Levy reasoned that, if this mechanism was not functioning properly, it would be possible for a hemisphere to be activated to solve a problem it was not really designed to solve. Children do not display symptoms of denial with organic central nervous system disease. Several chil- dren I observed with right cerebral lesions as well as a smaller number of children with left cerebral lesions and intact speech all became agitated and complained of the loss of function. Weinstein? has had similar experience with children. Some psychiatrists (8, 22) theorize that denial of illness is a psychiatric symptom of a general neurolog- ical disease and that it is not related to organic neu- rological disease any more than the general symptom, fever, is related to infection. None of these theories explain those cases in which a patient is immediately aware of a hemianopic defect and continues to complain of it. The work on com- missurotomy patients does shed light on those cases of dissociation in which the patient with a symmetrical hemianopsia complains of the defect in one eye and ignores, even denies, the defect in the other eye. Often in these cases the eye in which the defect is temporal in location is the eye in which the patient finds his vision defective and the opposite eye, in which the defective field is nasal, and therefore in the binocular field, is felt by the patient to be normal. Summary A case of visual loss occurring as a result of right posterior cerebral infarction and a right cilioretinal arteriolar occlusion was discussed. Although no satis- 2 Weinstein, E. A. Personal communication to the author. 368 factory explanation exists for the presence of denial, the dissociation of the denial pattern seen in this case and other cases suggests a motivational and perceptual origin. Psychiatric personality studies, especially pre- morbid studies, of those manifesting denial may be ueful in resolving some of the motivational questions raised. The concept of denial as a symptom of disease in the right cerebral hemisphere seems to be improbable. Recent work on commissurotomy patients suggests that hemispheric specialization may prove, in part, responsible for visual denial syndromes. Further psy- chological experimental studies are needed to inves- tigate the mechanism that distributes information to each hemisphere. The lack of denial in children is an aréa that may also yield additional information when it is explored. Perhaps in children, because hemispheric specializa- tion is less determined, the results of improper arbi- tration of information between the hemispheres are not as obvious. The symptoms of denial in adults might also result from a different psychodynamic mechanism for handling the stress of loss of function than is present in children. 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