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Prosopagnosia
Neuropsychologia,
1964, Vol. 2, pp. 237 to 246.
Pcrpamon Press Ltd.Printed inEngland
PROSOPAGNOSIA MALVIN COLE*
and J. PEREZ-CRUETS
(Received 6 February1964) Abstract-The investigation of a patient with prosopagnosia is reported. on intellectual tests were available and were compared with those obtained illness. Classical and instrumental conditioning, using either the patient’s face, were performed. It was thought that the defect was one of recognition intellectual deterioration, perceptual disability or memory loss.
Pre-morbid scores after the onset of or the examiner’s not dependent on
PROSOPAGNOSIAis an unusual but striking disability. Although the syndrome [l, 21 is well established it seldom occurs in isolation.
neuropsychological Nevertheless, the similar in the majority of reported cases while the
prosopagnosic deficit is remarkably associated defects vary. The underlying physiological and psychological basis of the disorder are quite obscure and have been reviewed by HBCAENand his associates [3, 4, 5). As in most of the disputes about the agnosias, the arguments revolve around the presence or absence and significance of other generalized abnormalities of cerebral function, intellectual deterioration, the importance of primary perceptual disorders [6], the significance of associated disorders such as inattention, memory loss etc. and psychological concepts such as those of “Gestalt” and “Ocula” etc [7]. The following case is presented not only because of the rarity of this syndrome but also because of the existence of pre-morbid psychological tests and the use of conditioning techniques in an attempt to carry the investigation of this disorder further than is possible by clinical testing alone. Cusepresentation. J. J. S. age 38 (WRAH No. 6012002) was transferred to Walter Reed Army Hospital on June 17, 1961 with a history of headache and yellowish nasal discharge of approximately three weeks duration and of confusion for several days. On admission he was confused and disoriented and complained of left fronto-temporal headache. He was afebrile and his vital signs were within normal limits. The disc margins were blurred bilaterally. He had a right hemiparesis and stiff neck. There were no other abnormal neurological signs. General physical examination was unremarkable, though previously, at another hospital, the oropharynx and nasal mucosa had been covered with purulent exudate and radiological examination was thought to demonstrate bilateral, frontal, maxillary, and ethmoid sinusitis. The clinical diagnosis of left frontal abscess was supported by the evidence obtained by lumbar puncture, eiectroencephalography and left carotid arteriography. On the day of admission he suddenly became unresponsive and his pulse decreased to 46 min. A left frontal abscess containing 65 ml of purulent material was aspirated with clinical improvement. Two days later he again became comatose and apneic and 75 ml of purulent material was aspirated from the same region. A third aspiration was necessary on the next day and following this he began to improve. On 18 July 1961 the abscess cavity was excised. This procedure was well tolerated and over the subsequent 6 months he continued to improve. * From the Department of Neurophysiology, Division of Neuropsychiatry, Walter Reed Army Institute of Research, Walter Reed Army Medical Center, Washington D.C. Present address: Dept. of Neurology, Seton Hall, College of Medicine, Jersey City, N. J. t Present address: Pavlovian Laboratory, Department of Psychiatry, Johns Hopkins Medical School, Baltimore, Maryland. 237
238
MAL~IN COU and J. PEREZ-CRUET
The patient came to our attention in February, 1962 complaining of dficulty recognizing faces since awakening after operation. He stated that when he heard the person’s voice or was able to use some feature other than the face he was able to identify the person to whom he was speaking, but was unable to do so by simply looking at the face itself. Neurological abnormalities at that time were contined to the psychological and visual spheres. The remainder of the cranial nerves, motor system, reflexes and sensory systems were normal including joint position sense, vibratory sensation, 2 point sensation, stereognosis, point localization, and double simultaneous visual and tactile sensation. There was no neglect of extra-personal space nor anything to suggest amorphosynthesis[g]. Visual acuity (corrected) was J-4 on the left and J-6 on the right. The fundi were normal. The visual field examination revealed a normal blind spot on the right and an enlarged one on the left (20/1000 white test object). An upper altidudinal defect was present (Fig. 1). Investigations. Hewas always alert, co-operative and, exceptfor the instances to be mentioned, consistent in his responses. During the period of testing (4 months) his condition remained unchanged. Orientation for time, place, and person were intact. His major disability was in the recognition of faces. When approached, he gazed at the person’s face and moved his head in various directions in a puzzled manner. He stated that he first looked at the chin and mouth, then carefully inspected the sides of the face, nose, eyes, and fore-head but according to his own statement, “could not put it all together”. At times he commented on the person’s body contour, clothes, specific facial features, scars, eyeglasses, coloring, etc. and used this, occasionally with success, to identify the person in front of him. Even when he was successful in the identification he stated that he was never really certain whether he was correct. Tliere was no metamorphopsia except for the fact that faces appeared blurred. He had difficulty differentiating white from colored people and when successful it was by closely examining facial contour and hair texture rather than color. This disability prevented visual recognition of those who were familiar to him including his wife. Recognition by ear was in general instantaneous though after a lapse of several weeks without speaking to a relatively unfamilii person he was not certain of their identity even after a conversation of minutes. When he looked into the mirror he stated that his own face appeared blurred and strange to him. When asked to identify his ward nurse in a group of three nurses wearing the same insignia and uniforms he chose the wrong one; but when each said the single word “hello” to him he immediately corrected himself. The contrast between this instantaneous recognition of voices and his puzzlement and confusion when visual recognition alone was demanded was most striking. The patient was presented with a single picture of himself and asked to identify it. He thought that it “possibly” was himself but was uncertain. He was then handed a pictnre of the examiner (M. C.) and though we had spent many hours together and were now face to face with the pictnre in front of him he was unable to identify the picture. He could not tell whether a picture of one of us (J. P. C.) represented a male or female face and when presented with two and three copies of our pictures, (the patient, M. C., J. P. C.) arranged on the table in front of him was unable to choose the similar photographs. When asked specifically if there were any pictures of the examiner (M. C.) in the group he said that he was uncertain and when pressed for a decision said, “no”. At the end of the test session hew& again shown a single photograph of himself and asked. “Have YOUever seen this fellow ?,’ He replied. “hmm. welldown through the chin there’s a groove, but I can’t bay who it is”. This was rep&e& a&n and again with the same reply. Finally he was asked to guess at the identity and he replied, “I don’t know”. During one examination he was able to identify President Kennedy, Mr. Krushchev, President Roosevelt, and Prime Minister Churchill from photographs but not President Eisenhower. When these pictures were immediately repeated he could not say whom they represented. On other examinations he was unable to identify any of the pictures. He identified a picture of Hitler by the swastika and mustache (according to his own statement) but immediatly afterwards when the arm bu& was covered he H’U~~nublc to do so. Nine out of ten animals were correctiy identified but a horse was thought to be a mule. Prior to becoming ill the patient had been given standard army psychological tests on three separate occasions. In 1950 he attained an Army Standard Score (General Technical Score, GT) of 77 and 90. In 1956 he attained a score of 91 on the same test. Table 1 shows the supposed correlation of these scores Table I. Relationship between Army Standard Scores, U. S. Army percentile equivalent Wechsler-Bellevue, and Wechsler Adult Intelligence Scales [14]. Percentile equivalent u. s. Army
Army Standard (GT) Score 70
7 16 31 50
80 90 100 --
Wechsler Bellevue 77 a6 94 101 - ------
WAIS ?8 85 92 100
JJS
Keeler
I
--
BS 20/1000
while
Left
3mm
March
3,5, IO. 15,20,30 1000
Scale
DATE
30, ‘1000
-o white
while
eye
=I”
I, 1962
JJS
Keeler MC
J6@r!ec!ed!
Juler Scotem unable to diff.
VISION
NAME
BS 20/1000
while
-
a
I
,”
-
.1 _._ _ .” .,
-, _
-._.“I”.-^_.
.._^.^-_l.^
YI
FIG. 1. Upper attitudinal defect. Blind spots (20/1000 white) normal on the right, enlarged on the left. Fields to: 3, 5, 10, IS, 20/1000 white. (dense area). 30/1OCO white seen in cross hatched area. Note sparing of macular area, left temporal crescent and small area near Right horizontal meridian. (Juler Scotometer).
VISION J4 (corrected) Juler Scolom unable to dift
NAME
_->-11-
1000
3,5,to,15,20,30
,600
,~__
Right
white
eye
Mqrch I, 1962 DATE Stole 3 mm = I0
MALVIN COLE and J. PEREZ-CRUET
240
Table 2. Wechsler Adult Intelligence Scale Scores obtained in 1962 Raw Score Information Comprehension Arithmetic Similarities Digit Span Vocabulary Digit Symbol Picture Completion Block Design Picture Arrangement Object Assembly
11 16 9 6 8 28 16 7 32 12 11 Verbal Score Performance Score Full Scale Score
Scaled Score 8 9 8 6 6 8 3 6 10 6
Age Corrected Score 7 9 s 7 6 7 4 6 11 7 4
45 IQ 485 29 IQ 78 74 .IQ 81
with the army percentile rating, Wechsler-Bellevue (W-B) and Wechsler Adult Intelligence Scale (WAIS). Table 2 indicates the subtest and calculated scores on the WATS in February, 1962. In addition in March, 1962 his score on Raven’s Standard Progressive Matrices Test was 42 or approximately the 56th percentile. He was able to recognize facial expressions mimicking anger and surprise. He was also able accurately to recogmze military rank and branch insignia and the American and British flags. Similarly, Picture Absurdities, Aesthetic Pictures, Missing Parts Pictures, Dissected Sentences and Interpretations of Pictures such as the “Telegraph Boy” from the Terman Merri!l Scale were done very well. Facial expressions in cartoons were often missed and incorrect interpretations of the cartoons were thus made. When shown a “soap opera” on television with the sound turned off, he did not recognize that the characters were women, was unable to identify d mechanical robot even when this was specifically pointed out, and did not recognize very obvious and exaggerated fear portrayed by the actresses when specif;.calIy queried on this point. He was unable to ascertain the overall plot of the story; this appeared to be largely due to the difficulty in the recognition of faces. There was no difficulty in pointing to objects, whether singly or in groups, or pictures of those objects, when the examiner named them; nor was there difficulty in naming the objects or pictures when the examiner pointed to them. Although there was no difficulty recognising objects, pictures or words when exposure was unlimited, there was a definite rise in temporal threshold of recognition as measured tachistoscopicaily at times in the range of 1, 5, 10, or even 15 sec. Temporal threshold was in general longer for pictures (i. e. Telegraph Boy) and scattered objects than for one or two words. Single objects were usually recognized more rapidly although occasionally unlimited exposure was required even for these. The abnormalities were not confined to any particular field of vision or area of extra personal space. He was able to state accurately the colors of common objects and claimed to be able to revisualize colors and shades. Under most conditions, however, he was unable to state the color of objects shown to him, calling a red pen “black” and yellow objects “light” and usually referring to objects as “light or dark” close to black or white”. This depended to some degree on the exact conditions of stimulus presentation. During one examination, in bright daylight, he was unable to name the colors of bright patches of cloth, reporting them as stated above. Nevertheless, when asked to point to the red, green, blue, yellow patch of cloth he did so correctly, but was uncertain that he was correct. He stated that he was able to revisualize his parents and other relatives, friends, his present house and childhood home. He did not think that he could revisualize his wife’s face as clearly. Dreaming was reported as unchanged and stereoscopic vision was normal. Optokinettc nystagmus using a reversible motor driven drum showed directional preponderance to the right, absent downward verttcal nystagmus and intact upward vertrcal nystagmus. When asked to draw from memory he often, but inconsistently, drew a small figure in the upper left comer of the page (Fig. 2), this was the only evidence of the “closing in” phenomenon [9]. He at times, complained that he was unable to visualize the figure in order to draw it. This was especially true when he was asked to draw geometrical figures such as triangles or cubes. The di.iZculty with drawing was inconsistent and at times he was able to do figures quite well which at other times were impossible. A similar deficit was noted when he attempted to make fi,oues out of match sticks. During one examination he was asked to make a triangle from match sticks. He said that he was unable to recall the form of a triangle, performed the task correctly, but was unsure that he had, in fact, made the requested figure.
PROSOPAGNOSIA
241
FIG. 2. Drawings are made in the upper left hand corner of the page. The triangle in this instance was well drawn but the cube (to request without a model) was extremely poor. The figures are quite smaIl Copying of zig-zag lines was quite satisfactory as was the copying of figures by joining points on the paper. Although he had some difficulty copying, precisely, three dimensional figures, such as cubes (Fig.2). his ability to copy a figure was far superior to his ability to draw it from memory. He had no difbculty in choosing the best of several three dimensional figures or in choosing the correct figure (triangle, cube, rectangle, etc.) from a group shown to him. His subject score on the WAlS Block Design Test was 10. The dil?lculty in drawing geometrical figures contrasted with his preserved ability to draw more complex figures such as a house, 5 pointed star (drawing or with match sticks), daiiy, bicycle, faces and various fire arms (Fig. 3) The Stanford-Binet “Paper Cutting” test was performed well as were simple Abelson’s figures: more complex Abelson’s figures were done poorly. Due to his dif8culty with colors the Weigl-GoldsteinScheerer Color Form Test could not be given. Horizontal and vertical meridians were indicated accurately with the eyes open or closed. There was no visual disorientation or n&reaching. A map of the United States was recognized and various cities located without difhculty. A plan of his ward and hospital wing were drawn accurately. Nevertheless, he was unable to find his way to and from our laboratory, a short distance from his ward, after many attempts and instructions and was unable to describe various routes around the hospital. Writing, throwing, cutting, using tools, kicking and sighting down a gun barrel were all done dextrahy, There was no known family sinistrality except perhaps for one brother; this was ind. Articulation was normal except that when he was tired some slurring was noted. Syntax was normal and there was no telegraphic style. A mild nominal and receptive dysphasia were present. He was able to rhyme words and find their synonyms and antonyms. Writing was intact; reading was accurate but slower than normal; there was no gross defect in spelling. There was no speci6c alcalulia and mathematical problems were done in Proportion to his overall functioning on the other tests (WAIS subtest score of 8). The clock test was intact as was Head’s Hand Eye Ear Test and there was no Right-Left disorientation or finger agnosia.
242
IMALVIN COLE and J. PEREZ-CRUET
He was able to imitate postures and there was no evidence of dressing, motor, or ideational apraxa. There was no evidence of disconnection inter or intra-hemispherically when this was specifically tested for [IO, 1I]. The memory quotient (Wechsler Memory Scale) was 77 which was comparable to his total IQ, though 8 points lower than the Verbal IQ. Memory for names was extremely poor though after days or even weeks he was able to describe the various tests given to him in detail including the Raven’s Matrices. Memory for sentences was intact on the first try in the Terman Merrill Scale (year XI, 5) level but he fai!ed the Babcock sentence on the eleventh attempt. There was no defect in Memory for Designs but he was unable to perform the Rey-Davis Test correctly on the ninth attempt, the errors being similar to those reported previously [12].
FIG. 3. Complex figures were relatively well done to request.
No model used.
An electro-encephalog in February, 1962 was normal with a 10 c/s well formed symmetrical basic rhythm which responded well to eye opening. Evoked responses to a bright flashing visual stimulus with electrodes placed over the calcarine cortex (I 50 flashes integrated) revealed no right-left or superior-inferior preponderance (courtesy of Dr. Robert Cohn, National Naval Medical Center). Intravenous injection of 0.5g of sodium amytal did not change the disability. Conditioning experiments were performed in order to further investigate the inability to recognize faces according to the following design. A: C/assical conditioning. A picture of the patient’s own face was always paired with a briefelectric shock (22) V.) to the hand; pictures of the experimenters (M. C. and J. P. C.) were never paired with the shock. Changes in heart rate, skin resistance, finger pulse volume and respiration during the presentation of the pictures were used as indices of autonomic conditioning; withdrawal of the hand or gross body movements before the shock were used as evidence for motor conditional reflexes. The results indicated that the patient was able to establish autonomic conditional reflexes to his own picture, but at times also showed some autonomic conditioning to the pictures of the examiners; thus, differentiation was judged as poor. No motor conditional reflexes were observed. B: Avoidnnce (inrtrrrmenrrrl) con~fitfoninp. The paiient was then instructed to press a lever every tune his own picture and only when his own picture was presented to him. A correct response avoided the electric shock, but an incorrect lever response was followed by a shock. In these experiments the patient always pressed the lever to the presentation of his own picture and never made an incorrect response to the other pictures. In another avoidance experiment the patient was instructed to press the lever every time a picture of one examiner (M. C.) was presented but not to do so to his own picture or to a picture of the other examiner (J. P. C.). The avoidance behavior was correct on the fifth and sixth presentations of the positive stimulus
243
PROSOPAGNOSIA
(M. C.), that is, on the eleventh and sixteenth trials. Nevertheless it was not established, as there was failure on the eighteenth trial (seventh presentation of the positive stimulus). There were no incorrect lever responses to the other pictures. The autonomic components of the avoidance behavior were more pronounced with the positive picture i.e. where instrumental avoidance contingencies were imposed (Table 3). Table 3. Results on kJStrUmeIJtal avoidance experiment (C). M. C. and 3. P. C. were examiners. J. J. S. was the patient. Positive stimulus: M. C. HR : Heart Rate: PGR: psychogalvaniic response; RR: respiratory response; FPV: finger pulse volume. Motor was the pressing of a Key. Unconditional Responses Conditional Responses HR
PGR
RR
FPV
HR
PGR
+1 +I +1 +I +2 +1 0 0 +1 +I +3 +1 +2 iI +1 -t4 +2 +I +3 0 +-2
0 0 0 0 0 0 0 0 0 0
0 0 t-2 +2 +1 +2 0 +1 +1 +1 +2 +1 +2 +2 +2 i-2 +2 $2 1-2 +I i-2
0 +3 +3 +2 0 +2 t-1 +3 +2 +2 +4 +2 +4 +3 0 +4 +2 +3 +3 +3 +4
14 +2 +2 +2 .+3 -
+3 0 +2 t-2 -
RR
FPV
Motor
+3 t-3 +3 +3 -l-4 -
$4 +3 -k4 +4 +4 -
0 0 0 0 0 0 0 0 0 0 +
M.C. J.P.C. J.P.C. M.C. J.J S J.J.S. J.J.S. MC. M.C. J.P.C. M.C. J.P.C. J.P.C. J.J.S. J.P.C. hl.C. J.P.C. M.C. M.C. J.J.S. M.C.
iI 0 0 0 0 +3 +1 0 +I 0 i-3
-+4 -
: 0 0 + 0 0 + 0 +
Discussion
In this case, as in all the cases reported to date, prosopagnosia was not an isolated disability. Some cases [l, 21 have shown fewer associated deficits; others were quite comparable [3, 131. The inability to recognize faces by sight alone, with recognition when hearing the spoken voice, and the compensatory methods used by the patients to overcome the dil?iculty have been strikingIy similar from case to case, including the present one. The patient reported here was always very co-operative, in general consistent, was aware of and spontaneously complained of his difficulty? and showed no general confusion, disorientation or catastrophic reactions. The presence of intelluctual loss and the degree to which it may contribute to prosopagnosia is a major question. In this patient the first Army G. T. Score (1950) was 77, comparable to the fifteenth percentile of the U. S. Army, a Wechsler-Bellevue (W-B) of 85 and a Wechsler Adult Intelligence Scale (WAIS) of approximately the same level [14]. The second and third G. T. scores given in 1950 and 1956 were equivalent to the thirty-first U. S. Army Percentile, or scores of 93 on the W-B and WAIS. We have been unable to ascertain the basis foi the discrepancy between the first and subsequent scores; it may have been a practice effect, but it would appear certain that the patient’s premorbid level was no higher than the latter tests. It will be assumed, for the purpose of this discussion, that these latter tests represented the patients’ baseline. This assumption will throw a subsequent dementing process into greater relief. The WAIS IQ scores obtained in 1962 showed some decrease from the previous supposed levels. The mild aphasia would explain, in part at least, the decrease of Verbal
244
MALVIN COLE and J. PEREZ-CRUET
IQ to 85. The more severe decrease in Performance IQ to 78 would be consistent with the visuo-spatial deficits found on other tests, but is also a sensitive indicator of any generalized dementing process. By contrast, the 1962 score on the Raven’s Standard Progressive Matrices of42, or the sixty-fifth percentile, would be evidence against any severe intellectual loss. It is realized that conclusions on the basis of these scores are open to question. It cannot be concluded from these scores that generalized intellectual loss was not present; nevertheless it is suggested, especially in view of the score on Raven’s Test that if it existed it was minor in degree. The attempts, often successful, at compensation by non-visual means or by visual clues other than the physiognomy support this argument. Visual perception was abnormal as indicated by the prolonged exposure times required for recognition. Object agnosia, however, was not present, multiple objects were recognised, as were single ones. Similarly “symbols” such as military insignia, and pictures of objects were no more difficult for the patient than common objects. When the exposure was of sticient duration for complex pictures to be described interpretation was accurate 1151. Recognition of all objects and pictures, except for faces and colors was intact, when exposure was unlimited. This selective difficulty with recognition (with unlimited exposure) implies that the perceptual disability cannot fully explain the prosopagnosia. It is also evidence for the concept that prosopagnosia is not necessarily part of a more inclusive visual agnosia. BAY [6] has suggested that the combination of perceptual disability plus intellectual loss is sufficient to explain the prosopagnosic defect. He also pointed out that any patient with severe “high grade field contraction” including those with glaucoma or retinal lesions will be prosopagnosic. He considered that faces were especially difficult to recognise because of their fine shades and weakly contrasting details. Nevertheless, these points do not explain why patients such as the one reported here show this discrepancy (i. e. selectivity) between faces on the one hand and other single objects, multiple objects, maps, animals in black and white, shaded drawings, and colored pictures with shadows, and complex pictures, of all sizes on the other. One would expect that small pictures of faces would be more easily recognized than large ones, using BAY’Sanalogy of peeping through a pin hole [6]. We have not found this to be the case. There was no evidence that the area about the eyes was more easily recognized than other areas. In fact, repeated questioning revealed that this patient usually began at the chin, describing first the furrows in and about that area, then describing the general contour of the face and zygomatic areas and then the eyebrows and eyes. In addition to the difficulty recognising faces other deficits were noted. One was the superior performance in drawing complex figures as opposed to simple geometrical ones. Revisualization for faces, places, and colors was reported as intact while revisualization of simple geometric figures, in order to draw them, was often defective. The superior performance in copying figures in comparison to drawing them from memory, the ability to choose correct figures from a group shown to him, results on the paper cutting test and Raven’s Progressive Matrices mark this as a “constructional apraxia” [ 161. Topographical disabilities often accompany prosopagnosia which has been considered to represent an “agnosia for faces and places” [17] but according to H&AEN et al. this association is not invariable [3]. These latter authors found, in general, a dissociation between the patients with spatial and topographical disabilities and those with more general disorders including object agnosia and dyslexia. The patient reported here falls into the first category manifesting the not uncommon dissociation between : (1) being unable to describe routes or find his way around the hospital and (2) being
PROSOPAGNOSIA
245
able to draw floor plans and to find places on a map. ‘The difficulty with color recognition was similar, in many respects, to those of drawing geometrical figures to command and to the inability to recognise faces. These consisted of (1) an inability to do a task to command (i.e. name a color, draw a triangle); (2) he could indicate the correct response to a stimulus presented by the examiner (i.e. naming a figure drawn by the examiner or pointing to a color named by him). (3) He was always uncertain that he had, in fact, given the correct response. This was not explained by his mild aphasia since naming was, in general, better than performance. It appeared to be a difficulty in initiating a response to a command, the performance of which was facilitated by presentation to the patient of the objects (triangle, color of cloth) rather than the command alone. In this sense it was an apraxia although the uncertainty when the act As is true of topographical disability, was accomplished implies something in addition. difficulties with colors are commonly associated with prosopagnosia. The conditioning studies revealed that the patient was able to form poorly differentiated autonomic conditional responses to his own picture and he was capable of instrumental avoidance of a shock paired with it. The conditional responses were much more difficult to establish when he had to discriminate pictures of other faces than his own. Under these conditions even on the trials with successful avoidance behavior autonomic differentiation was poor. Questioning immediately after each experimental session revealed that he was still unable to say whom each picture represented even when he and the examiner (M. C.) were face to face with the picture of the latter between them. He was able to learn which picture was associated with the shock using the same characteristics as in his non-experimental behavior. That is, avoidance was based on the use of specific facial lines, the presence or absence of spectacles, and small variations in the angle at which the photograph was taken rather than the overall physiognomy. From the data, perceptual disability, intellectual deterioration and memory impairment cannot be completely excluded as contributory factors in prosopagnosia. However, the points presented suggest that these do not account for prosopagnosia and that the defect depends on an inability to synthesize the individual features of a face into a specific identifiable whole. The patient never mistakes an individual feature for something else nor does he mistake a facefor something else. He sees and notes small furrows, scars or other markings and, in his attempt to compensate for his difficulty, appears to pay more attention to these features than many normal persons. He tries to deduce from these features, the identity of the person in front of him and is, at times, successful. The tendency to concentrate on one component of the face is not due to the release of a cerebral automatism such as found in tonic innervation of gaze [I& 19, 20, 21, 221 but is, rather. a compensatory mechanism. It is a defect in recognition, not in that a face is taken for something else, but that a speczjic face is not identified. This is in agreement with the position taken by FAUST [23] HECAEN et al. [3] and BEYN and KNYAZEVA [17]. The compensatory mechanisms used by the patient, plus the inability to “put it all together” suggest a defect in recognition in the Gestalt sense 1241. The case reported here, due to the lack of pathological data, does not contribute to the localization of the lesion in this syndrome. At most, the visual fields, the directional preponderance of optokinetic nystagmus [25], the constructional apraxia, difficulty with colors and topographical agnosia lend support to the concept that it was due to a bilateral parieto-occipital lesion though, in other cases, the right parieto-occipital area alone has
246
MALVIN COLE and J. PEREZ-CRL;ET
been emphasized. Again, without proof, it may only be suggested that the immediate pathogenesis was infarction secondary to posterior cerebral artery compression during episodes of uncal herniation. Ackr.owledgements-The
authors wish to thank D. McK. RIOCH, M.D. ; Professor R. A. CHAMBERS,M.D. : W. NAU CA,M.D. ; and E. A. WEINSTEIN,M.D. for reviey.ving and criticizing the manuscript. We are most grateful to H. H. BREWINGTON,B.S.; 0. LYERLY,X.B.. and M. KALIS for technical help and the Neurosurgical staff for their co-operation.
REFERENCES 1. PALLIS,C. A. Impaired identification
of faces and places with agnosia for colors. J. Nerrrol. Neuro-
surr. Psychiul. 18, 218-224, 1955. 2. B~~RNSTEIN, B. and KIDRON, D. P. Prosopagnosia. J. Neuroi. Nuero.turg. Psychint. 22, 124-131, 1959. 3. HBCAEN,H., ANGELERGUES,R., BERNHARDT,C. et CHIARELLI,J. Essai De Distinction Des Modalites Cliniques De L’Agnosie Des Physionomies. Rev. NeNrol. 96, 125-144, 1957. 4. HBCMN, H. Less Agnosies Visuelles Pour Les Objets Animes. in Les Grandes Activities Du Lobe Occipital. Ed. ALAJOUANINE,T. (pp. 281-284) Paris, Masson Et Cie. 1960. 5. DE AJURIAGUERRA, J. et H~~CAEN, H. Le Corte.r Cerebral. Ed. 2, Masson. Paris 1950. 6. BAY, E. Disturbances of Visual Perception and Their Examination. Brain 76, 515-550, 1953. 7. CRIrCHLEY, M. The ParietaI Lobes. (pp 291-298) London, Edward Arnold, 1053. 8. DENNY-BROWN,D., MEYER,J. S., and HORENSTEIN,S. Bruin 75, 433471, 1952. 9. MAYER-GROSS,W. Proc. Roy. Sac. Med. 25, 1203-1212, 1935. 10. GFSCHWIND, N. and KAPLAN, E. .4 Human Split Brain Syndrome: A Preliminary Report. Personal Communication Based on a Paper Presented at the Boston Society for Psychiatry and Neurology, December 14, 1961. II. GESCHWIND,N. Disconnection of The Cerebral Hemispheres in Man. An Address Delivered at the Seventh Annual Veterans Administration Conference. Cincinnati, Ohio. March 27, 1962. 12. ZAUGWILL,0. I,. Some Clinical Applications of The Rey-Davis Performance Test. J. Ment. Sci. 92, 19-34, 1949. 13. MACRAE, D. and TROLLE,E. The Defect of Function in Visual Agnosia. Bruin 79, 94-110, 1956. 14. HEDLUND,J. L. Army Classification Scores: Their Meaning and Clinical Implication. From a Paper prepared for The Conference of Army Clinical Psychologists, Sponsored by The Surgeon General, Department of the Army, Washington, D. C. September, 1959, page 7. 15. ETTLIXGER,G. The Description and interpretation of Pictures in Cases of Brain Lesion. J. Metlt. Sri. 106, 1337-1346, 1960. 16. MCFIE, J. and ZANGWILL,0. L. Brain 83, 243-260, 1960. 17. BRYN, E. S. and KNYAZEVA,G. R. The Problem of Prosopagnosia. J. NeuroI. Neurnalug. P.sychiat. 25, 154-158, 1962. 18. DENNY-BROWN,D. Arqui. Neuro-Psiquiatria 10, 399-404, 1952. 19. HBCAEN.H. and De AJURIAGUERRA,J. Bruin 77, 37340, 1954. 20. DENNY-BROWS,D. The Nature of Apraxia. J. Nerv. Ment. Dis. 126, 9-32, 1958. 21. DEWY-BROWN, D. and CHAMBERS,R. A. The Parietal Lobe and Behavior in Proc. A.R.N.M.D. The Rrain and Human Behavior. 36,35-l 17, 1355. 22. DEhWY-BROWN, D. T/X Basal Gar~,&k. Oxford University Press, London, 23. FAUST, C. Nerl.enaitz 18, 291-297, 1947. 24. KOHLER,W. Gestuft P.qychology. New York. Horace Liveright, 1929.
1962.
25. CAR~CHAEL, E. A., DIX, M. R., and HALLPIKE,C. S. Lesions of The Cerebral Hemispheres and Their Effects upon Optokinetic and Caloric Nystagmus. Brain 77, 345. R&um&L’obscrvation d’un sujet prisentant une prosopagnosie est rapportee. Les scores pr& morbides aux tests intellectuels purent &re retrouv& et furent compart% avec ceux obtenus apr&s le d8but de la maladie. L.e cooditionnemenl classique et instrumental en utilisant la physionomie du malade ou ceile de l’observateur fut realis& On admet que le deficit Porte sur la reconnaissance et ne dtpend de la dtt&ioration intellectuelle, ni d’un trouble perceptif ni d’un dCficit mntsique. Zusammenfassung-Bericht iiber die Untersuchungsergebnisse bei einem Kranken mit ProsopAgnosie. Dadurch, dass Intelligenztests aus der Zeit vor Krankheitsbeginn zur Verfiigung standen, konnte man Vergleiche mit spdteren Untersuchungen anstellen. Ausser denklassischen Verfahren wurden spezielle angewandt. Man bediente sich dabei sowohl der Physiognomie des Kranken als such der des Untersuchers als Testobjekt. Man kam zu dem Ergebnis, dass der nachweisbare Defekt ein rein gnostischer war, und dass Abhangigkeit von einer allgemeinen Tntelligenzminderung, von einer Wahrnehmungsst8rung oder von mnestischen Mtigeln nicht vorlag.
1964, Vol. 2, pp. 237 to 246.
Pcrpamon Press Ltd.Printed inEngland
PROSOPAGNOSIA MALVIN COLE*
and J. PEREZ-CRUETS
(Received 6 February1964) Abstract-The investigation of a patient with prosopagnosia is reported. on intellectual tests were available and were compared with those obtained illness. Classical and instrumental conditioning, using either the patient’s face, were performed. It was thought that the defect was one of recognition intellectual deterioration, perceptual disability or memory loss.
Pre-morbid scores after the onset of or the examiner’s not dependent on
PROSOPAGNOSIAis an unusual but striking disability. Although the syndrome [l, 21 is well established it seldom occurs in isolation.
neuropsychological Nevertheless, the similar in the majority of reported cases while the
prosopagnosic deficit is remarkably associated defects vary. The underlying physiological and psychological basis of the disorder are quite obscure and have been reviewed by HBCAENand his associates [3, 4, 5). As in most of the disputes about the agnosias, the arguments revolve around the presence or absence and significance of other generalized abnormalities of cerebral function, intellectual deterioration, the importance of primary perceptual disorders [6], the significance of associated disorders such as inattention, memory loss etc. and psychological concepts such as those of “Gestalt” and “Ocula” etc [7]. The following case is presented not only because of the rarity of this syndrome but also because of the existence of pre-morbid psychological tests and the use of conditioning techniques in an attempt to carry the investigation of this disorder further than is possible by clinical testing alone. Cusepresentation. J. J. S. age 38 (WRAH No. 6012002) was transferred to Walter Reed Army Hospital on June 17, 1961 with a history of headache and yellowish nasal discharge of approximately three weeks duration and of confusion for several days. On admission he was confused and disoriented and complained of left fronto-temporal headache. He was afebrile and his vital signs were within normal limits. The disc margins were blurred bilaterally. He had a right hemiparesis and stiff neck. There were no other abnormal neurological signs. General physical examination was unremarkable, though previously, at another hospital, the oropharynx and nasal mucosa had been covered with purulent exudate and radiological examination was thought to demonstrate bilateral, frontal, maxillary, and ethmoid sinusitis. The clinical diagnosis of left frontal abscess was supported by the evidence obtained by lumbar puncture, eiectroencephalography and left carotid arteriography. On the day of admission he suddenly became unresponsive and his pulse decreased to 46 min. A left frontal abscess containing 65 ml of purulent material was aspirated with clinical improvement. Two days later he again became comatose and apneic and 75 ml of purulent material was aspirated from the same region. A third aspiration was necessary on the next day and following this he began to improve. On 18 July 1961 the abscess cavity was excised. This procedure was well tolerated and over the subsequent 6 months he continued to improve. * From the Department of Neurophysiology, Division of Neuropsychiatry, Walter Reed Army Institute of Research, Walter Reed Army Medical Center, Washington D.C. Present address: Dept. of Neurology, Seton Hall, College of Medicine, Jersey City, N. J. t Present address: Pavlovian Laboratory, Department of Psychiatry, Johns Hopkins Medical School, Baltimore, Maryland. 237
238
MAL~IN COU and J. PEREZ-CRUET
The patient came to our attention in February, 1962 complaining of dficulty recognizing faces since awakening after operation. He stated that when he heard the person’s voice or was able to use some feature other than the face he was able to identify the person to whom he was speaking, but was unable to do so by simply looking at the face itself. Neurological abnormalities at that time were contined to the psychological and visual spheres. The remainder of the cranial nerves, motor system, reflexes and sensory systems were normal including joint position sense, vibratory sensation, 2 point sensation, stereognosis, point localization, and double simultaneous visual and tactile sensation. There was no neglect of extra-personal space nor anything to suggest amorphosynthesis[g]. Visual acuity (corrected) was J-4 on the left and J-6 on the right. The fundi were normal. The visual field examination revealed a normal blind spot on the right and an enlarged one on the left (20/1000 white test object). An upper altidudinal defect was present (Fig. 1). Investigations. Hewas always alert, co-operative and, exceptfor the instances to be mentioned, consistent in his responses. During the period of testing (4 months) his condition remained unchanged. Orientation for time, place, and person were intact. His major disability was in the recognition of faces. When approached, he gazed at the person’s face and moved his head in various directions in a puzzled manner. He stated that he first looked at the chin and mouth, then carefully inspected the sides of the face, nose, eyes, and fore-head but according to his own statement, “could not put it all together”. At times he commented on the person’s body contour, clothes, specific facial features, scars, eyeglasses, coloring, etc. and used this, occasionally with success, to identify the person in front of him. Even when he was successful in the identification he stated that he was never really certain whether he was correct. Tliere was no metamorphopsia except for the fact that faces appeared blurred. He had difficulty differentiating white from colored people and when successful it was by closely examining facial contour and hair texture rather than color. This disability prevented visual recognition of those who were familiar to him including his wife. Recognition by ear was in general instantaneous though after a lapse of several weeks without speaking to a relatively unfamilii person he was not certain of their identity even after a conversation of minutes. When he looked into the mirror he stated that his own face appeared blurred and strange to him. When asked to identify his ward nurse in a group of three nurses wearing the same insignia and uniforms he chose the wrong one; but when each said the single word “hello” to him he immediately corrected himself. The contrast between this instantaneous recognition of voices and his puzzlement and confusion when visual recognition alone was demanded was most striking. The patient was presented with a single picture of himself and asked to identify it. He thought that it “possibly” was himself but was uncertain. He was then handed a pictnre of the examiner (M. C.) and though we had spent many hours together and were now face to face with the pictnre in front of him he was unable to identify the picture. He could not tell whether a picture of one of us (J. P. C.) represented a male or female face and when presented with two and three copies of our pictures, (the patient, M. C., J. P. C.) arranged on the table in front of him was unable to choose the similar photographs. When asked specifically if there were any pictures of the examiner (M. C.) in the group he said that he was uncertain and when pressed for a decision said, “no”. At the end of the test session hew& again shown a single photograph of himself and asked. “Have YOUever seen this fellow ?,’ He replied. “hmm. welldown through the chin there’s a groove, but I can’t bay who it is”. This was rep&e& a&n and again with the same reply. Finally he was asked to guess at the identity and he replied, “I don’t know”. During one examination he was able to identify President Kennedy, Mr. Krushchev, President Roosevelt, and Prime Minister Churchill from photographs but not President Eisenhower. When these pictures were immediately repeated he could not say whom they represented. On other examinations he was unable to identify any of the pictures. He identified a picture of Hitler by the swastika and mustache (according to his own statement) but immediatly afterwards when the arm bu& was covered he H’U~~nublc to do so. Nine out of ten animals were correctiy identified but a horse was thought to be a mule. Prior to becoming ill the patient had been given standard army psychological tests on three separate occasions. In 1950 he attained an Army Standard Score (General Technical Score, GT) of 77 and 90. In 1956 he attained a score of 91 on the same test. Table 1 shows the supposed correlation of these scores Table I. Relationship between Army Standard Scores, U. S. Army percentile equivalent Wechsler-Bellevue, and Wechsler Adult Intelligence Scales [14]. Percentile equivalent u. s. Army
Army Standard (GT) Score 70
7 16 31 50
80 90 100 --
Wechsler Bellevue 77 a6 94 101 - ------
WAIS ?8 85 92 100
JJS
Keeler
I
--
BS 20/1000
while
Left
3mm
March
3,5, IO. 15,20,30 1000
Scale
DATE
30, ‘1000
-o white
while
eye
=I”
I, 1962
JJS
Keeler MC
J6@r!ec!ed!
Juler Scotem unable to diff.
VISION
NAME
BS 20/1000
while
-
a
I
,”
-
.1 _._ _ .” .,
-, _
-._.“I”.-^_.
.._^.^-_l.^
YI
FIG. 1. Upper attitudinal defect. Blind spots (20/1000 white) normal on the right, enlarged on the left. Fields to: 3, 5, 10, IS, 20/1000 white. (dense area). 30/1OCO white seen in cross hatched area. Note sparing of macular area, left temporal crescent and small area near Right horizontal meridian. (Juler Scotometer).
VISION J4 (corrected) Juler Scolom unable to dift
NAME
_->-11-
1000
3,5,to,15,20,30
,600
,~__
Right
white
eye
Mqrch I, 1962 DATE Stole 3 mm = I0
MALVIN COLE and J. PEREZ-CRUET
240
Table 2. Wechsler Adult Intelligence Scale Scores obtained in 1962 Raw Score Information Comprehension Arithmetic Similarities Digit Span Vocabulary Digit Symbol Picture Completion Block Design Picture Arrangement Object Assembly
11 16 9 6 8 28 16 7 32 12 11 Verbal Score Performance Score Full Scale Score
Scaled Score 8 9 8 6 6 8 3 6 10 6
Age Corrected Score 7 9 s 7 6 7 4 6 11 7 4
45 IQ 485 29 IQ 78 74 .IQ 81
with the army percentile rating, Wechsler-Bellevue (W-B) and Wechsler Adult Intelligence Scale (WAIS). Table 2 indicates the subtest and calculated scores on the WATS in February, 1962. In addition in March, 1962 his score on Raven’s Standard Progressive Matrices Test was 42 or approximately the 56th percentile. He was able to recognize facial expressions mimicking anger and surprise. He was also able accurately to recogmze military rank and branch insignia and the American and British flags. Similarly, Picture Absurdities, Aesthetic Pictures, Missing Parts Pictures, Dissected Sentences and Interpretations of Pictures such as the “Telegraph Boy” from the Terman Merri!l Scale were done very well. Facial expressions in cartoons were often missed and incorrect interpretations of the cartoons were thus made. When shown a “soap opera” on television with the sound turned off, he did not recognize that the characters were women, was unable to identify d mechanical robot even when this was specifically pointed out, and did not recognize very obvious and exaggerated fear portrayed by the actresses when specif;.calIy queried on this point. He was unable to ascertain the overall plot of the story; this appeared to be largely due to the difficulty in the recognition of faces. There was no difficulty in pointing to objects, whether singly or in groups, or pictures of those objects, when the examiner named them; nor was there difficulty in naming the objects or pictures when the examiner pointed to them. Although there was no difficulty recognising objects, pictures or words when exposure was unlimited, there was a definite rise in temporal threshold of recognition as measured tachistoscopicaily at times in the range of 1, 5, 10, or even 15 sec. Temporal threshold was in general longer for pictures (i. e. Telegraph Boy) and scattered objects than for one or two words. Single objects were usually recognized more rapidly although occasionally unlimited exposure was required even for these. The abnormalities were not confined to any particular field of vision or area of extra personal space. He was able to state accurately the colors of common objects and claimed to be able to revisualize colors and shades. Under most conditions, however, he was unable to state the color of objects shown to him, calling a red pen “black” and yellow objects “light” and usually referring to objects as “light or dark” close to black or white”. This depended to some degree on the exact conditions of stimulus presentation. During one examination, in bright daylight, he was unable to name the colors of bright patches of cloth, reporting them as stated above. Nevertheless, when asked to point to the red, green, blue, yellow patch of cloth he did so correctly, but was uncertain that he was correct. He stated that he was able to revisualize his parents and other relatives, friends, his present house and childhood home. He did not think that he could revisualize his wife’s face as clearly. Dreaming was reported as unchanged and stereoscopic vision was normal. Optokinettc nystagmus using a reversible motor driven drum showed directional preponderance to the right, absent downward verttcal nystagmus and intact upward vertrcal nystagmus. When asked to draw from memory he often, but inconsistently, drew a small figure in the upper left comer of the page (Fig. 2), this was the only evidence of the “closing in” phenomenon [9]. He at times, complained that he was unable to visualize the figure in order to draw it. This was especially true when he was asked to draw geometrical figures such as triangles or cubes. The di.iZculty with drawing was inconsistent and at times he was able to do figures quite well which at other times were impossible. A similar deficit was noted when he attempted to make fi,oues out of match sticks. During one examination he was asked to make a triangle from match sticks. He said that he was unable to recall the form of a triangle, performed the task correctly, but was unsure that he had, in fact, made the requested figure.
PROSOPAGNOSIA
241
FIG. 2. Drawings are made in the upper left hand corner of the page. The triangle in this instance was well drawn but the cube (to request without a model) was extremely poor. The figures are quite smaIl Copying of zig-zag lines was quite satisfactory as was the copying of figures by joining points on the paper. Although he had some difficulty copying, precisely, three dimensional figures, such as cubes (Fig.2). his ability to copy a figure was far superior to his ability to draw it from memory. He had no difbculty in choosing the best of several three dimensional figures or in choosing the correct figure (triangle, cube, rectangle, etc.) from a group shown to him. His subject score on the WAlS Block Design Test was 10. The dil?lculty in drawing geometrical figures contrasted with his preserved ability to draw more complex figures such as a house, 5 pointed star (drawing or with match sticks), daiiy, bicycle, faces and various fire arms (Fig. 3) The Stanford-Binet “Paper Cutting” test was performed well as were simple Abelson’s figures: more complex Abelson’s figures were done poorly. Due to his dif8culty with colors the Weigl-GoldsteinScheerer Color Form Test could not be given. Horizontal and vertical meridians were indicated accurately with the eyes open or closed. There was no visual disorientation or n&reaching. A map of the United States was recognized and various cities located without difhculty. A plan of his ward and hospital wing were drawn accurately. Nevertheless, he was unable to find his way to and from our laboratory, a short distance from his ward, after many attempts and instructions and was unable to describe various routes around the hospital. Writing, throwing, cutting, using tools, kicking and sighting down a gun barrel were all done dextrahy, There was no known family sinistrality except perhaps for one brother; this was ind. Articulation was normal except that when he was tired some slurring was noted. Syntax was normal and there was no telegraphic style. A mild nominal and receptive dysphasia were present. He was able to rhyme words and find their synonyms and antonyms. Writing was intact; reading was accurate but slower than normal; there was no gross defect in spelling. There was no speci6c alcalulia and mathematical problems were done in Proportion to his overall functioning on the other tests (WAIS subtest score of 8). The clock test was intact as was Head’s Hand Eye Ear Test and there was no Right-Left disorientation or finger agnosia.
242
IMALVIN COLE and J. PEREZ-CRUET
He was able to imitate postures and there was no evidence of dressing, motor, or ideational apraxa. There was no evidence of disconnection inter or intra-hemispherically when this was specifically tested for [IO, 1I]. The memory quotient (Wechsler Memory Scale) was 77 which was comparable to his total IQ, though 8 points lower than the Verbal IQ. Memory for names was extremely poor though after days or even weeks he was able to describe the various tests given to him in detail including the Raven’s Matrices. Memory for sentences was intact on the first try in the Terman Merrill Scale (year XI, 5) level but he fai!ed the Babcock sentence on the eleventh attempt. There was no defect in Memory for Designs but he was unable to perform the Rey-Davis Test correctly on the ninth attempt, the errors being similar to those reported previously [12].
FIG. 3. Complex figures were relatively well done to request.
No model used.
An electro-encephalog in February, 1962 was normal with a 10 c/s well formed symmetrical basic rhythm which responded well to eye opening. Evoked responses to a bright flashing visual stimulus with electrodes placed over the calcarine cortex (I 50 flashes integrated) revealed no right-left or superior-inferior preponderance (courtesy of Dr. Robert Cohn, National Naval Medical Center). Intravenous injection of 0.5g of sodium amytal did not change the disability. Conditioning experiments were performed in order to further investigate the inability to recognize faces according to the following design. A: C/assical conditioning. A picture of the patient’s own face was always paired with a briefelectric shock (22) V.) to the hand; pictures of the experimenters (M. C. and J. P. C.) were never paired with the shock. Changes in heart rate, skin resistance, finger pulse volume and respiration during the presentation of the pictures were used as indices of autonomic conditioning; withdrawal of the hand or gross body movements before the shock were used as evidence for motor conditional reflexes. The results indicated that the patient was able to establish autonomic conditional reflexes to his own picture, but at times also showed some autonomic conditioning to the pictures of the examiners; thus, differentiation was judged as poor. No motor conditional reflexes were observed. B: Avoidnnce (inrtrrrmenrrrl) con~fitfoninp. The paiient was then instructed to press a lever every tune his own picture and only when his own picture was presented to him. A correct response avoided the electric shock, but an incorrect lever response was followed by a shock. In these experiments the patient always pressed the lever to the presentation of his own picture and never made an incorrect response to the other pictures. In another avoidance experiment the patient was instructed to press the lever every time a picture of one examiner (M. C.) was presented but not to do so to his own picture or to a picture of the other examiner (J. P. C.). The avoidance behavior was correct on the fifth and sixth presentations of the positive stimulus
243
PROSOPAGNOSIA
(M. C.), that is, on the eleventh and sixteenth trials. Nevertheless it was not established, as there was failure on the eighteenth trial (seventh presentation of the positive stimulus). There were no incorrect lever responses to the other pictures. The autonomic components of the avoidance behavior were more pronounced with the positive picture i.e. where instrumental avoidance contingencies were imposed (Table 3). Table 3. Results on kJStrUmeIJtal avoidance experiment (C). M. C. and 3. P. C. were examiners. J. J. S. was the patient. Positive stimulus: M. C. HR : Heart Rate: PGR: psychogalvaniic response; RR: respiratory response; FPV: finger pulse volume. Motor was the pressing of a Key. Unconditional Responses Conditional Responses HR
PGR
RR
FPV
HR
PGR
+1 +I +1 +I +2 +1 0 0 +1 +I +3 +1 +2 iI +1 -t4 +2 +I +3 0 +-2
0 0 0 0 0 0 0 0 0 0
0 0 t-2 +2 +1 +2 0 +1 +1 +1 +2 +1 +2 +2 +2 i-2 +2 $2 1-2 +I i-2
0 +3 +3 +2 0 +2 t-1 +3 +2 +2 +4 +2 +4 +3 0 +4 +2 +3 +3 +3 +4
14 +2 +2 +2 .+3 -
+3 0 +2 t-2 -
RR
FPV
Motor
+3 t-3 +3 +3 -l-4 -
$4 +3 -k4 +4 +4 -
0 0 0 0 0 0 0 0 0 0 +
M.C. J.P.C. J.P.C. M.C. J.J S J.J.S. J.J.S. MC. M.C. J.P.C. M.C. J.P.C. J.P.C. J.J.S. J.P.C. hl.C. J.P.C. M.C. M.C. J.J.S. M.C.
iI 0 0 0 0 +3 +1 0 +I 0 i-3
-+4 -
: 0 0 + 0 0 + 0 +
Discussion
In this case, as in all the cases reported to date, prosopagnosia was not an isolated disability. Some cases [l, 21 have shown fewer associated deficits; others were quite comparable [3, 131. The inability to recognize faces by sight alone, with recognition when hearing the spoken voice, and the compensatory methods used by the patients to overcome the dil?iculty have been strikingIy similar from case to case, including the present one. The patient reported here was always very co-operative, in general consistent, was aware of and spontaneously complained of his difficulty? and showed no general confusion, disorientation or catastrophic reactions. The presence of intelluctual loss and the degree to which it may contribute to prosopagnosia is a major question. In this patient the first Army G. T. Score (1950) was 77, comparable to the fifteenth percentile of the U. S. Army, a Wechsler-Bellevue (W-B) of 85 and a Wechsler Adult Intelligence Scale (WAIS) of approximately the same level [14]. The second and third G. T. scores given in 1950 and 1956 were equivalent to the thirty-first U. S. Army Percentile, or scores of 93 on the W-B and WAIS. We have been unable to ascertain the basis foi the discrepancy between the first and subsequent scores; it may have been a practice effect, but it would appear certain that the patient’s premorbid level was no higher than the latter tests. It will be assumed, for the purpose of this discussion, that these latter tests represented the patients’ baseline. This assumption will throw a subsequent dementing process into greater relief. The WAIS IQ scores obtained in 1962 showed some decrease from the previous supposed levels. The mild aphasia would explain, in part at least, the decrease of Verbal
244
MALVIN COLE and J. PEREZ-CRUET
IQ to 85. The more severe decrease in Performance IQ to 78 would be consistent with the visuo-spatial deficits found on other tests, but is also a sensitive indicator of any generalized dementing process. By contrast, the 1962 score on the Raven’s Standard Progressive Matrices of42, or the sixty-fifth percentile, would be evidence against any severe intellectual loss. It is realized that conclusions on the basis of these scores are open to question. It cannot be concluded from these scores that generalized intellectual loss was not present; nevertheless it is suggested, especially in view of the score on Raven’s Test that if it existed it was minor in degree. The attempts, often successful, at compensation by non-visual means or by visual clues other than the physiognomy support this argument. Visual perception was abnormal as indicated by the prolonged exposure times required for recognition. Object agnosia, however, was not present, multiple objects were recognised, as were single ones. Similarly “symbols” such as military insignia, and pictures of objects were no more difficult for the patient than common objects. When the exposure was of sticient duration for complex pictures to be described interpretation was accurate 1151. Recognition of all objects and pictures, except for faces and colors was intact, when exposure was unlimited. This selective difficulty with recognition (with unlimited exposure) implies that the perceptual disability cannot fully explain the prosopagnosia. It is also evidence for the concept that prosopagnosia is not necessarily part of a more inclusive visual agnosia. BAY [6] has suggested that the combination of perceptual disability plus intellectual loss is sufficient to explain the prosopagnosic defect. He also pointed out that any patient with severe “high grade field contraction” including those with glaucoma or retinal lesions will be prosopagnosic. He considered that faces were especially difficult to recognise because of their fine shades and weakly contrasting details. Nevertheless, these points do not explain why patients such as the one reported here show this discrepancy (i. e. selectivity) between faces on the one hand and other single objects, multiple objects, maps, animals in black and white, shaded drawings, and colored pictures with shadows, and complex pictures, of all sizes on the other. One would expect that small pictures of faces would be more easily recognized than large ones, using BAY’Sanalogy of peeping through a pin hole [6]. We have not found this to be the case. There was no evidence that the area about the eyes was more easily recognized than other areas. In fact, repeated questioning revealed that this patient usually began at the chin, describing first the furrows in and about that area, then describing the general contour of the face and zygomatic areas and then the eyebrows and eyes. In addition to the difficulty recognising faces other deficits were noted. One was the superior performance in drawing complex figures as opposed to simple geometrical ones. Revisualization for faces, places, and colors was reported as intact while revisualization of simple geometric figures, in order to draw them, was often defective. The superior performance in copying figures in comparison to drawing them from memory, the ability to choose correct figures from a group shown to him, results on the paper cutting test and Raven’s Progressive Matrices mark this as a “constructional apraxia” [ 161. Topographical disabilities often accompany prosopagnosia which has been considered to represent an “agnosia for faces and places” [17] but according to H&AEN et al. this association is not invariable [3]. These latter authors found, in general, a dissociation between the patients with spatial and topographical disabilities and those with more general disorders including object agnosia and dyslexia. The patient reported here falls into the first category manifesting the not uncommon dissociation between : (1) being unable to describe routes or find his way around the hospital and (2) being
PROSOPAGNOSIA
245
able to draw floor plans and to find places on a map. ‘The difficulty with color recognition was similar, in many respects, to those of drawing geometrical figures to command and to the inability to recognise faces. These consisted of (1) an inability to do a task to command (i.e. name a color, draw a triangle); (2) he could indicate the correct response to a stimulus presented by the examiner (i.e. naming a figure drawn by the examiner or pointing to a color named by him). (3) He was always uncertain that he had, in fact, given the correct response. This was not explained by his mild aphasia since naming was, in general, better than performance. It appeared to be a difficulty in initiating a response to a command, the performance of which was facilitated by presentation to the patient of the objects (triangle, color of cloth) rather than the command alone. In this sense it was an apraxia although the uncertainty when the act As is true of topographical disability, was accomplished implies something in addition. difficulties with colors are commonly associated with prosopagnosia. The conditioning studies revealed that the patient was able to form poorly differentiated autonomic conditional responses to his own picture and he was capable of instrumental avoidance of a shock paired with it. The conditional responses were much more difficult to establish when he had to discriminate pictures of other faces than his own. Under these conditions even on the trials with successful avoidance behavior autonomic differentiation was poor. Questioning immediately after each experimental session revealed that he was still unable to say whom each picture represented even when he and the examiner (M. C.) were face to face with the picture of the latter between them. He was able to learn which picture was associated with the shock using the same characteristics as in his non-experimental behavior. That is, avoidance was based on the use of specific facial lines, the presence or absence of spectacles, and small variations in the angle at which the photograph was taken rather than the overall physiognomy. From the data, perceptual disability, intellectual deterioration and memory impairment cannot be completely excluded as contributory factors in prosopagnosia. However, the points presented suggest that these do not account for prosopagnosia and that the defect depends on an inability to synthesize the individual features of a face into a specific identifiable whole. The patient never mistakes an individual feature for something else nor does he mistake a facefor something else. He sees and notes small furrows, scars or other markings and, in his attempt to compensate for his difficulty, appears to pay more attention to these features than many normal persons. He tries to deduce from these features, the identity of the person in front of him and is, at times, successful. The tendency to concentrate on one component of the face is not due to the release of a cerebral automatism such as found in tonic innervation of gaze [I& 19, 20, 21, 221 but is, rather. a compensatory mechanism. It is a defect in recognition, not in that a face is taken for something else, but that a speczjic face is not identified. This is in agreement with the position taken by FAUST [23] HECAEN et al. [3] and BEYN and KNYAZEVA [17]. The compensatory mechanisms used by the patient, plus the inability to “put it all together” suggest a defect in recognition in the Gestalt sense 1241. The case reported here, due to the lack of pathological data, does not contribute to the localization of the lesion in this syndrome. At most, the visual fields, the directional preponderance of optokinetic nystagmus [25], the constructional apraxia, difficulty with colors and topographical agnosia lend support to the concept that it was due to a bilateral parieto-occipital lesion though, in other cases, the right parieto-occipital area alone has
246
MALVIN COLE and J. PEREZ-CRL;ET
been emphasized. Again, without proof, it may only be suggested that the immediate pathogenesis was infarction secondary to posterior cerebral artery compression during episodes of uncal herniation. Ackr.owledgements-The
authors wish to thank D. McK. RIOCH, M.D. ; Professor R. A. CHAMBERS,M.D. : W. NAU CA,M.D. ; and E. A. WEINSTEIN,M.D. for reviey.ving and criticizing the manuscript. We are most grateful to H. H. BREWINGTON,B.S.; 0. LYERLY,X.B.. and M. KALIS for technical help and the Neurosurgical staff for their co-operation.
REFERENCES 1. PALLIS,C. A. Impaired identification
of faces and places with agnosia for colors. J. Nerrrol. Neuro-
surr. Psychiul. 18, 218-224, 1955. 2. B~~RNSTEIN, B. and KIDRON, D. P. Prosopagnosia. J. Neuroi. Nuero.turg. Psychint. 22, 124-131, 1959. 3. HBCAEN,H., ANGELERGUES,R., BERNHARDT,C. et CHIARELLI,J. Essai De Distinction Des Modalites Cliniques De L’Agnosie Des Physionomies. Rev. NeNrol. 96, 125-144, 1957. 4. HBCMN, H. Less Agnosies Visuelles Pour Les Objets Animes. in Les Grandes Activities Du Lobe Occipital. Ed. ALAJOUANINE,T. (pp. 281-284) Paris, Masson Et Cie. 1960. 5. DE AJURIAGUERRA, J. et H~~CAEN, H. Le Corte.r Cerebral. Ed. 2, Masson. Paris 1950. 6. BAY, E. Disturbances of Visual Perception and Their Examination. Brain 76, 515-550, 1953. 7. CRIrCHLEY, M. The ParietaI Lobes. (pp 291-298) London, Edward Arnold, 1053. 8. DENNY-BROWN,D., MEYER,J. S., and HORENSTEIN,S. Bruin 75, 433471, 1952. 9. MAYER-GROSS,W. Proc. Roy. Sac. Med. 25, 1203-1212, 1935. 10. GFSCHWIND, N. and KAPLAN, E. .4 Human Split Brain Syndrome: A Preliminary Report. Personal Communication Based on a Paper Presented at the Boston Society for Psychiatry and Neurology, December 14, 1961. II. GESCHWIND,N. Disconnection of The Cerebral Hemispheres in Man. An Address Delivered at the Seventh Annual Veterans Administration Conference. Cincinnati, Ohio. March 27, 1962. 12. ZAUGWILL,0. I,. Some Clinical Applications of The Rey-Davis Performance Test. J. Ment. Sci. 92, 19-34, 1949. 13. MACRAE, D. and TROLLE,E. The Defect of Function in Visual Agnosia. Bruin 79, 94-110, 1956. 14. HEDLUND,J. L. Army Classification Scores: Their Meaning and Clinical Implication. From a Paper prepared for The Conference of Army Clinical Psychologists, Sponsored by The Surgeon General, Department of the Army, Washington, D. C. September, 1959, page 7. 15. ETTLIXGER,G. The Description and interpretation of Pictures in Cases of Brain Lesion. J. Metlt. Sri. 106, 1337-1346, 1960. 16. MCFIE, J. and ZANGWILL,0. L. Brain 83, 243-260, 1960. 17. BRYN, E. S. and KNYAZEVA,G. R. The Problem of Prosopagnosia. J. NeuroI. Neurnalug. P.sychiat. 25, 154-158, 1962. 18. DENNY-BROWN,D. Arqui. Neuro-Psiquiatria 10, 399-404, 1952. 19. HBCAEN.H. and De AJURIAGUERRA,J. Bruin 77, 37340, 1954. 20. DENNY-BROWS,D. The Nature of Apraxia. J. Nerv. Ment. Dis. 126, 9-32, 1958. 21. DEWY-BROWN, D. and CHAMBERS,R. A. The Parietal Lobe and Behavior in Proc. A.R.N.M.D. The Rrain and Human Behavior. 36,35-l 17, 1355. 22. DEhWY-BROWN, D. T/X Basal Gar~,&k. Oxford University Press, London, 23. FAUST, C. Nerl.enaitz 18, 291-297, 1947. 24. KOHLER,W. Gestuft P.qychology. New York. Horace Liveright, 1929.
1962.
25. CAR~CHAEL, E. A., DIX, M. R., and HALLPIKE,C. S. Lesions of The Cerebral Hemispheres and Their Effects upon Optokinetic and Caloric Nystagmus. Brain 77, 345. R&um&L’obscrvation d’un sujet prisentant une prosopagnosie est rapportee. Les scores pr& morbides aux tests intellectuels purent &re retrouv& et furent compart% avec ceux obtenus apr&s le d8but de la maladie. L.e cooditionnemenl classique et instrumental en utilisant la physionomie du malade ou ceile de l’observateur fut realis& On admet que le deficit Porte sur la reconnaissance et ne dtpend de la dtt&ioration intellectuelle, ni d’un trouble perceptif ni d’un dCficit mntsique. Zusammenfassung-Bericht iiber die Untersuchungsergebnisse bei einem Kranken mit ProsopAgnosie. Dadurch, dass Intelligenztests aus der Zeit vor Krankheitsbeginn zur Verfiigung standen, konnte man Vergleiche mit spdteren Untersuchungen anstellen. Ausser denklassischen Verfahren wurden spezielle angewandt. Man bediente sich dabei sowohl der Physiognomie des Kranken als such der des Untersuchers als Testobjekt. Man kam zu dem Ergebnis, dass der nachweisbare Defekt ein rein gnostischer war, und dass Abhangigkeit von einer allgemeinen Tntelligenzminderung, von einer Wahrnehmungsst8rung oder von mnestischen Mtigeln nicht vorlag.