BRAIN INJURY,
1992, VOL. 6 , NO. 5 , 469476
Case Report
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Delusional reduplication following closed-head injury MARCUS J . C. ROGERS a n d M I C H A E L D . FRANZENt Department of Behavioral Medicine and Psychiatry, West Virgnia University, Morgantown, WV 26505, USA
(Received 26 August 1991; accepted 23 September 1991) Somatic delusions following brain injury are not uncommon, and have been well documented in the literature. This study documents a case of somatic delusion which was seen in a patient following a head injury secondary to a motorcycle accident. Although perhaps not typical it serves to illustrate an interesting example of a somatic delusion following head trauma. O n recovery from coma this patient reported the existence of a ‘third arm’ adjacent to the limb that had received the greatest impact in the accident. The patient was unreceptive to any counter-persuasions and in fact remained largely unconcerned about t h s addition to his anatomy. A thorough neuropsychologcal evaluation was carried out in an attempt to seek an explanation for this phenomenon. The results suggest that the phenomenon has at least a partly psychiatric aetiology rather than a purely neurological foundation.
Introduction Significant brain impairment may result in aberrant belief systems, many of which can be accurately classified as delusions. A number of different forms of somatic delusions have been noted following brain impairment. Perhaps one of the more common is anosagnosia, or denial of illness. Here the patient insists that some body part is unimpaired and working, even with strong objective evidence to the contrary. Anosagnosia is most commonly seen in those patients suffering hemiparesis or blindness, but the denial can be of cognitive problems such as amnesia as well as of physical incapacities. It can also involve total unilateral neglect, which is often the case with hemiparesis. In reviewing this area, McGlynn and Schacter [l] critique a number of different interpretations of the phenomenon and attempt to formulate an ‘integrative theoretical framework‘ to account for it. Their idea is consistent with the majority of theorists who agree that anosagnosia results from damage to the right hemisphere and more specifically to the right parietal lobe and possibly the frontal regions. One theory is that damage to these areas can disturb awareness of the body scheme, or that t h s representation becomes lost to conscious awareness [2]. Capgrass Syndrome is a more bizarre delusion following insult to the brain. It involves the belief that significant others have been duplicated, and that their place has been taken by impostors sharing all the same features as the originals [3]. This disorder has been
t To whom correspondence should be addressed 0269-9052/92 $3.00 0 1992 Taylor 81 Francis Ltd
M.J . C . Rogers and M . D . Franzen
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observed following a variety of neurological impairments including hypothyroidism, cerebral haemorrhage and temporal lobe epilepsy [4-61. Weston and Whitlock (51 observed this phenomenon following head trauma. Damage to the right hemisphere has been reported most often in these patients [7] but, unlike anosagnosia where the lesions are found primarily in the parietal lobe [I], these patients generally show damage to the frontal lobe. Banks et al. [S] describe the cases of two patients; one of these had bilateral damage to the medial frontal white matter, to the corpus callosum, right basal ganglia, internal capsule and thalamus. The other had a ruptured anterior communicating aneurysm with subsequent resection of the right frontal gyms rectus. These patients began to exhibit ‘automaton-like’ movements of their left hands. These limbs would, at times, act independently grasping for objects, and at times actively impeding the functioning of the other arm. This phenomenon has been called ‘alien hand syndrome’. The authors postulate that the syndrome results from the ‘combination of a partial callosectomy and mesial frontal lesions’. Here the patient denies control over the limb and may even insist that the limb belongs to a stranger. A related disorder, and one perhaps more relevant to the present case, is the perception after an amputation that the limb still exists, the ‘phantom limb syndrome’. The most salient sensation for these people is the pain that they perceive origmating from the ‘limb’. Carlen et al. [Y] found that, of 73 amputees, 67% developed phantom limb pain immediately or soon after surgery. I n explanation of this phenomenon they suggest that cpinal cord cells that have been ‘released from inhibitory control through loss of afferent impulses’ produce the sensation of a phantom limb. They emphasize the spinal cord as opposed to more rostra1 structures, because of the fact that paraplegics who have lost a greater amount of efferent impulses often do not develop any phantom sensations until months after spinal cord section. Weinstein et (11. [lo] discussed the existence of phantom limbs in hemiplegia. They called this phenomenon ‘delusional reduplication’. In the cases that they discuss, the phantom limb does not actually take the place of an amputated limb, but rather supplements the normal complement, in other words an extra limb is perceived to be present. The tern1 delusional reduplication can be said to describe the following case fairly accurately. Case study
C. was admitted to the trauma service of a regional hospital centre following a motor cycle accident where he left the road and collided with a telephone pole. The resulting injuries were predominantly confined to the right side of his body and included a fracture of the first rib, scapula and zygoma. Specific injuries to his head as revealed on a CAT scan 2 days post-trauma showed multiple craniofacial fractures involving the right temporal bone, right orbital roof and petrous portion of the temporal bone on the right. There was mild effacement of the right lateral ventricle and parenchymal haemorrhage and oedema were found in the right subfrontal area. His right arm suffered considerable trauma nccessitating skin grafts (unsuccessful) plus occlusion of the right subclavian artery, avulsed right clavicle and extensive nerve damage. On admission to the hospital he had a Glasgow Coma Scale score of 7 (eyes = 1, motor = 1, verbal = 5). His mother reported (unconfirmed) that he was able to obey the command to squeeze her hand after 2-3 days, but it was approximately 13 days until he could recognize her. As the patient became niorc anm-c, he began to report the idea that he had an extra (‘third’) arm. He stated that this arm was located underneath his existing right arm and had its origin at his right
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Delusional reduplication following closed-head injuvy
47 1
shoulder. He believed that the pain he was experiencing originated in this third arm, and constantly requested that the arm be searched for and moved into more comfortable positions. He stated that at first his doctors did not believe that he had an extra arm until the surgeons found it while performing surgery. The first time he noticed it himself was when it came to his assistance while in the bathroom. H e described this extra arm as having the same proportions as his real arms but being slightly smaller in scale. H e perceived this third arm as being extremely hot, and believed that his bed contained a hot water system that was responsible for the arm’s high temperature. At other times he would describe the sensation in the hand of the third arm as being like that of ‘having a gold wedding ring melt into my palm’. He believed that this extra limb was there to help him in tasks that had now become difficult as a consequence of the loss of function in his right arm. It was his belief that as function returned in his injured limb his extra arm would atrophy and disappear. H e would also make reference to his real arm, on the same side as the third arm, as being totally out of proportion to his body. On one occasion he stated he thought people in a 200-yard radius would be frightened by his arm because of its extraordinary size. If asked to indicate his right arm, he would point to the real one, and he was able to identify the various parts of the real arm without inaccuracy or distortion. The staff continually referred to his ‘third arm’ as his ‘phantom arm’ and in time he himself adopted this descriptor. Ths change of name, however, did not reduce h s references to the arm or alter his frequent wish for it to be found or moved. If his requests were ignored or challenged he quickly seemed to forget that his request had not been met. O n first evaluation there was some evidence of mild delusional thinking. H e believed ‘mentally I have become stronger since the accident’, and he felt he had become more perceptive of his surroundings. For instance, he believed his olfactory sense had become enhanced. H e said he knew through his sense of smell when people entered the room and stood behind him. H e had to be turned in his chair before he could be convinced that t h s was not the case. H e also complained of visual problems with h s right eye that had a significantly dilated pupil. An evaluation did not uncover any clinically significant problems with vision. He was slightly disinhibited in both manner and speech, although it was not clear from his family if this was also the case pre-morbidly. He would make sexually explicit remarks to his wife in front of the examiner, as well as making inappropriate remarks to the examiner himself It was clear, though, from his history, that sex took a very dominant role in his life, and so this behaviour may not have been a significant change from previous behaviour patterns or a signal of greatly decreased inhibition. H e graduated from high school at 18, having been a relatively good student. Upon graduation he joined the army and served in a tank crew and in the airborne rangers. H e left the army before his accident because he reportedly found it increasingly difficult to accept orders. Upon leaving the army he worked odd jobs and then set up a construction business. His medical history is significant for two previous motorcycle accidents. The first accident occurred in 1979 when he fell from his bike injuring his leg and hitting his head with such force that it split his helmet. H e denied any significant long-term physical or psychological effect from this incident. His second accident occurred a year later. H e fell from his bike and suffered a severe impact to the back of this head which he reported resulted in a ‘blood clot from my skull to my spine’. From his description, it appears he suffered a haematoma across his occipital lobes. H e was hospitalized for approximately 3 months.
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As a consequence of this latter accident C. became more short-tempered-reported by both himself and his family. H e became involved in frequent fights; his nickname reflected his prowess a t this activity-‘Mule’. It is unclear to what extent his belligerent tendencies existed before the accident, but it seems the behaviour following the second accident was an exacerbation of pre-morbid inclinations. Along with his short temper he began to suffer extremely painful headaches, which he admitted at times would be so bad as to make him cry. He experienced headaches approximately every 2 days.
Evaluation results The Luria Nebraska Neuropsychologlcal Battery Form I1 [ l l ] and a number of short tests were administered to gauge the patient’s general level of neuropsychological functioning and to attempt to identify any possible neurologcal cause for his behaviour. The short procedures included the Trail Making Test I121 Digit Span [13], Stroop Color Word Test 1141, Knox Cube Test 1151, Benton’s Visual Form Discrimination Test [16], Benton’s Line Orientation Test [ 161 and the Semmes Body Orientation Test [17]. Cognitive efficiency was found to be two standard deviations below normal. A test measuring cognitive flexibility and speed (Trails A-D) also revealed a moderate level of impairment. However, both tasks required good manual dexterity, which was compromised in this patient. Auditory verbal sequential memory as measured by D i g t Span was in the normal range. Specifically he was able to recall eight diglts forward and four backwards. His performance on this task was somewhat above average, but the discrepancy between the two tasks suggests some problems with mental control and concentration. His visual spatial sequential memory, as measured by the Knox Cube Test [15], revealed some impairment. He was only able to repeat a series of five movements, and his overall performance was inconsistent, indicating some attentional problems. Parietal lobe functioning was assessed because of the observed relationship between parietal dysfunction and anomalies of bodily experience [2]. The Judgement of Line Onentation Test, the Visual Form Discrimination Test and the Semmes Body Orientation Test were used. His performance on these tests was almost perfect, which would tend to rule out any major involvement of the parietal lobes in his presenting problem. These test findings are consistent with the CT scan results, which did not uncover any significant parietal involvement. The majority of neuropsychological functions, as measured by the Luria Nebraska Neuropsychologd Battery, were intact. Significant elevations were noted on both clinical and factor analytic scales indexing tactile and motor functions. These elevations, though, were a direct result of the injuries to his right arm. The Intermediate Memory scale was also elevated. This appeared to result from his incidental memory being somewhat weak, but information that he deliberately encoded was well consolidated and retrievable. All other neuropsychological functions measured on this test--such as reading, writing and arithmetic, as well as receptive and expressive speech-were all intact. The patient endorsed items on the MMPI consistent with individuals who tend to report feelings of unreality, odd thinlung, and possibly delusions or hallucinations. The dcgee of elevation of the scales was in the mild to moderate range. It can largely be assumed that the significant elevations found with this inventory are a consequence of his responding to his current experiences, although it cannot be ruled out that some existing idiosyncratic personality factors may have predisposed him to his current somatic condition. There were no signs of overt psychosis or severe psychopathology.
Delusional reduplication following closed-head injury
473
Table 1 . Results ofshort tests. Trai/ Making Test-Halstead-Reitan
Trials A B
c
D
96s 177s 110s 180s
Line Orientation Test 28/30 trials correct Brain Inj Downloaded from informahealthcare.com by University of British Columbia on 10/29/14 For personal use only.
Visual Fom Disrrimination 15/16 trials correct
Knox Cube Test Seven trials correct Mmmum of five sequential moves recalled Norm 11 years Stroop Colov Word Test
Word Color Color/Word
60
44 24
(26) (26) (29)
Digit Span Subtest of W A I S - R
Maximum digits forward Maximum digits backwards Scale Score
8
4 10
Personal Orientation Test
Touch parts of body named by examiner-8/8 correct Names parts of body touched by examiner-8/8 correct Touch named parts of examiner’s body-7/8 correct Touch own body according to schematic diagram-5/8 correct
Discussion The results of the evaluation tend to suggest the delusional reduplication of the right arm is partly a psychiatric manifestation rather than one singularly resulting from any central nervous system lesion. There was no evidence of major parietal involvement from either the neuropsychological results or the CT scan. However, the bodily injuries were on the right side where the greatest impact occurred, and it is possible that subtle, mild injuries may have occurred in the right hemisphere. Similarly, the parietal lobes have been implicated in immediate recall, and the relatively intact performance on the verbal task (Dig-lt Span) compared with the relatively poor performance on the visual spatial immediate recall task (Knox Cube) indicates the possibility of subtle right parietal involvement, although comparisons between these two tests have not been previously validated as a measure of laterality. In light of the above findings a number of theoretical explanations can be suggested to try to explain this phenomenon. The existence of a psychotic-type process would be an obvious consideration in the case. The symptoms did have a definite delusional quality, particularly as the ideas were held with such conviction (at least initially), and there was little doubt in the patient’s mind the arm did exist. The phenomenon also could be a product of an acute confusional state secondary to the brain insult. However, this hypothesis is doubtful given the length of time after the injury that the symptoms were present, and the fact that he was generally well oriented. Furthermore, a search of the
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literature did not reveal any published observations regarding a relation between delusional reduplication and confusional states. The frequent lack of concern he demonstrated for his injured a r m , and the emphasis he put on his third arm, suggested a form of neglect. He ncver came to acknowledge the scriousncss of his injury or make rcference to the possibility of lifelong incapacity. H e was able to watch his arni being biopsied and dressed, with absolutely no display of concern, a fact that remains hard to understand g v c n the open nature of the wounds. Neglect has traditionally been associated with lesions to the right parietal lobe, although recent research has suggested that this form of awareness is mediated more diffusely involving subcortical structures [Is]. O n formal testing using the Semmes Body Orientation Test there was no indication that there was any neglect of his body. Tests to gauge the integnty
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Delusional reduplication following closed-head injury
475
of the parietal lobes revealed no deficits in any visual spatial hnctioning or somatosensory discrimination. Therefore the standard interpretation of neglect does not appear to be sufficient to explain this situation. Because of the variety of patients seen by Weinstein et al. [lo] exhibiting reduplication of limbs, they doubt it can be ‘attributed to destruction of any specific area such as the parietal lobe’, or ‘the corticothalamic and reticulothalamic activating and inhibitory system’. Rather, they postulate ‘the lesion initiates a reorganization of brain function in which any member that the patient conceives of as damaged may be reduplicated, no matter what the cause of the incapacity’. Weinstein [19] further states that reduplicative delusional states are dependent upon psychological variables. When a limb remains attached to the body but is essentially divorced from it as a result of nerve damage, the nervous system may not be able to reconcile the continued existence of the limb with the lack of somatosensory efferentation. Input from the damaged nerve ends may be distorted and misinterpreted in an effort to make sense of new and unfamiliar input. O n the other hand the phenomenon may reflect a simple refusal to come to terms with a distressing event, a frequent cause of many bizarre forms of pathology. In time the patient came to use the term ‘phantom arm’ to refer to his previously named ‘third arm’, more to placate the hospital staff than as a sign of a change in his own perceptions. His expressed belief that in time his ‘third arm’ would atrophy and eventually disappear as his right arm healed is consistent with the interpretation that his belief in the third arm was a pathological psychological coping mechanism. O n latest contact, approximately 4 months post-injury, the patient’s right arm had begun to show signs of spasticity and to draw up towards his chest. He spent some time in a rehabilitation unit but was unhappy away from his family and so signed hmself out. His family stated he no longer makes reference to his ‘third arm’ but experiences episodes of depression and dependency. H e is currently being medicated for his depression and has not been able to return to work.
Conclusion Given the seriousness of his accident the neuropsychological evaluation revealed that the patient’s cognitive functioning was relatively unimpaired except in a few specific areas. Cognitive efficiency was found to be slightly compromised, which may have resulted in him being unable to deal with changing situations in an effective manner. His limited capacity in this area could have impaired his ability to handle the psychological and physical upheavals experienced after the accident. Besides his lowered cognitive efficiency, his ability to handle his traumatic situation is likely to have been further aggravated and compromised by some apparent, and previously mentioned, personality idiosyncrasies, w h c h resembled in many respects an antisocial personality disorder. These symptoms appear to have been exacerbated by this head injury. These factors, in combination, may have resulted in his misinterpretation of his situation in a way that can only be described as a pathological form of wishkl thinking. Although there were subtle signs of right parietal involvement in the Knox Cube results, other procedures which tap right parietal functions, especially body awareness functions, were found to be unimpaired limiting the utility of strictly neurological aetiology for his somatic delusion. The neurological damage may have predisposed the patient to develop his somatic delusion, but the degree of manifest damage is insufficient to explain completely the clinical presentation. This case serves to illustrate the importance of considering not only neurologcal factors, but also pre-morbid personality, as well as emotional and situational factors
476
M . J . C . Rogers and M . D . F r a n z e n
following the injury, when attempting t o evaluate patients with definite neurological damage. It is likely another individual with identical injuries t o those suffered by this patient would respond in a very different manner, almost purely o n the strength of pre-morbid personality and learning experience. It would seem advisable, therefore, t o evaluate aspects of history and personality if o n e is t o understand and accurately conceptualize post-traumatic changes.
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Acknowledgement T h e authors would like t o thank Marc Haut, PhD for his helpful comments on a n earlier draft of this manuscript.
References 1. MCGLYNN,S. M. and SCHACTER, D. L.: Unawareness of deficits in neuropsychologcai syndromcc.Journal of Clinical arid Experimtital Xeirropsychology, 11: 143-205, 1989.
2. GEKSTMANN, J.: Problem of imperception of disease and of impaired body temtories with organic lcsions: relation to body scheme and its disorder. Archives of Neurology and Psychiatry, 48: 89it-913, 1942. 3. DOKAN, J. M.: The Capgrass syndrome: neurologd/neuropsychological perspectives. Xeiiropsychology, 4: 29-42, 1990. 1. MADAKASIKA, S. and HALL,T.: Capgrass syndrome in a patient with myxedema. American Joirrrial ofPsyrhiatry, 138: 150G1507, 1981. 5. WESTON,M. J . and WHITLOCK, F. A,: The Capgrass syndrome following head injury. British Joirrnal ojPsychiatry, 119: 25-31, 1971. 6. CHKISTODOULOU, G. N . : Course and prognosis of the syndrome of doublcs. Journal of Nervous Merital Disease, 166: 68-72, 1978. 7. ALEXANDER, M. P., Si-vss, D. T. and BENSON,D. F.: Capgrass syndrome: a reduplicative phenomenon. .YeirroloXy, 29: 334-339, 1979. 8 . BANKS, G., SHOKT, P., JGLIO MARTINEZ, A,, LA.TCHAW, R., RATCLIFF, G. and BOLLER, F.: The alien hand syndrome, clinical and postmortem findings. Archives of Neurology, 46: 456-460, 1989. 9. CAKLEN, P. L., WALL,P. D., NALWORNA, H. and STEINBACH, T.: Phantom limbs and related phenomena in recent traumatic amputations. h'eurofogy, 28: 211-217, 1978. 10. WEINSTEIN, E. A., KAHN,l i . L., MALITZ, S. and ROZANSKI, J.: Delusional reduplication of parts of the body. Brain, 77: 43-60, 1954. C. J., MOSES,J. A. and HAMMEKE, T. A.: L~iriaNebraska Neuropsychological Battery: 11. GOLDEN, Form I and II Maniral. (Western Psychologcal Services, Los Angeles), 1985. D.: f i e Halstead Reitati Neuropsychological Battery: f i e o r y and 12. EVEITAN,R . M . and WOLFSON, Clinical Ititwpretarion (Neuropsychology Press, Tucson, AZ), 1985. D.: WAIS-R Manrial (Psychological Corporation, New York), 1981. 13 WECHSLER. C . J.: Stroop Color and Word Test: A Mariitalfor Clinical and Experimental Use (Stoelting, 14. GOLDEN, Chicago), 1978. 15. STONE,M. H. and WKIGHT, B. D.: Ktiox Cube Test-Instruction Manual (Stoelting, Chicago), 1980. 16. BENTON,A. L., HAMSHEK, K. DES.,VARNEY, N . R . and SPREEN, 0.:Contributions to Neuropsychological Asressriient (Oxford University Press, New York), 1983. 17. SEMMES, J., WEINSTEIN, S., GHENT,L. and TEUBEK,H. L.: Correlates of impaired orientation in personal and extrapersonal space. Brain, 86: 747-772, 1963. 18. HEALTON, E. B., NAVOKRO,C., BKESSMAN, S. and BKUST,J. C. M.: Subcortical neglect. Srurology, 32: 776-778, 1982. 19. WEINSTEIN, F. A.: Anosahmosia and denial of illness. In G. P. Prigatano and S. Schacter (Eds), Awarerresx of Dejcit ofter Braiti hjiq: Clinical arid Theoretical Issues (Oxford University Press, New York), pp. 240-257, 1991.
1992, VOL. 6 , NO. 5 , 469476
Case Report
Brain Inj Downloaded from informahealthcare.com by University of British Columbia on 10/29/14 For personal use only.
Delusional reduplication following closed-head injury MARCUS J . C. ROGERS a n d M I C H A E L D . FRANZENt Department of Behavioral Medicine and Psychiatry, West Virgnia University, Morgantown, WV 26505, USA
(Received 26 August 1991; accepted 23 September 1991) Somatic delusions following brain injury are not uncommon, and have been well documented in the literature. This study documents a case of somatic delusion which was seen in a patient following a head injury secondary to a motorcycle accident. Although perhaps not typical it serves to illustrate an interesting example of a somatic delusion following head trauma. O n recovery from coma this patient reported the existence of a ‘third arm’ adjacent to the limb that had received the greatest impact in the accident. The patient was unreceptive to any counter-persuasions and in fact remained largely unconcerned about t h s addition to his anatomy. A thorough neuropsychologcal evaluation was carried out in an attempt to seek an explanation for this phenomenon. The results suggest that the phenomenon has at least a partly psychiatric aetiology rather than a purely neurological foundation.
Introduction Significant brain impairment may result in aberrant belief systems, many of which can be accurately classified as delusions. A number of different forms of somatic delusions have been noted following brain impairment. Perhaps one of the more common is anosagnosia, or denial of illness. Here the patient insists that some body part is unimpaired and working, even with strong objective evidence to the contrary. Anosagnosia is most commonly seen in those patients suffering hemiparesis or blindness, but the denial can be of cognitive problems such as amnesia as well as of physical incapacities. It can also involve total unilateral neglect, which is often the case with hemiparesis. In reviewing this area, McGlynn and Schacter [l] critique a number of different interpretations of the phenomenon and attempt to formulate an ‘integrative theoretical framework‘ to account for it. Their idea is consistent with the majority of theorists who agree that anosagnosia results from damage to the right hemisphere and more specifically to the right parietal lobe and possibly the frontal regions. One theory is that damage to these areas can disturb awareness of the body scheme, or that t h s representation becomes lost to conscious awareness [2]. Capgrass Syndrome is a more bizarre delusion following insult to the brain. It involves the belief that significant others have been duplicated, and that their place has been taken by impostors sharing all the same features as the originals [3]. This disorder has been
t To whom correspondence should be addressed 0269-9052/92 $3.00 0 1992 Taylor 81 Francis Ltd
M.J . C . Rogers and M . D . Franzen
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observed following a variety of neurological impairments including hypothyroidism, cerebral haemorrhage and temporal lobe epilepsy [4-61. Weston and Whitlock (51 observed this phenomenon following head trauma. Damage to the right hemisphere has been reported most often in these patients [7] but, unlike anosagnosia where the lesions are found primarily in the parietal lobe [I], these patients generally show damage to the frontal lobe. Banks et al. [S] describe the cases of two patients; one of these had bilateral damage to the medial frontal white matter, to the corpus callosum, right basal ganglia, internal capsule and thalamus. The other had a ruptured anterior communicating aneurysm with subsequent resection of the right frontal gyms rectus. These patients began to exhibit ‘automaton-like’ movements of their left hands. These limbs would, at times, act independently grasping for objects, and at times actively impeding the functioning of the other arm. This phenomenon has been called ‘alien hand syndrome’. The authors postulate that the syndrome results from the ‘combination of a partial callosectomy and mesial frontal lesions’. Here the patient denies control over the limb and may even insist that the limb belongs to a stranger. A related disorder, and one perhaps more relevant to the present case, is the perception after an amputation that the limb still exists, the ‘phantom limb syndrome’. The most salient sensation for these people is the pain that they perceive origmating from the ‘limb’. Carlen et al. [Y] found that, of 73 amputees, 67% developed phantom limb pain immediately or soon after surgery. I n explanation of this phenomenon they suggest that cpinal cord cells that have been ‘released from inhibitory control through loss of afferent impulses’ produce the sensation of a phantom limb. They emphasize the spinal cord as opposed to more rostra1 structures, because of the fact that paraplegics who have lost a greater amount of efferent impulses often do not develop any phantom sensations until months after spinal cord section. Weinstein et (11. [lo] discussed the existence of phantom limbs in hemiplegia. They called this phenomenon ‘delusional reduplication’. In the cases that they discuss, the phantom limb does not actually take the place of an amputated limb, but rather supplements the normal complement, in other words an extra limb is perceived to be present. The tern1 delusional reduplication can be said to describe the following case fairly accurately. Case study
C. was admitted to the trauma service of a regional hospital centre following a motor cycle accident where he left the road and collided with a telephone pole. The resulting injuries were predominantly confined to the right side of his body and included a fracture of the first rib, scapula and zygoma. Specific injuries to his head as revealed on a CAT scan 2 days post-trauma showed multiple craniofacial fractures involving the right temporal bone, right orbital roof and petrous portion of the temporal bone on the right. There was mild effacement of the right lateral ventricle and parenchymal haemorrhage and oedema were found in the right subfrontal area. His right arm suffered considerable trauma nccessitating skin grafts (unsuccessful) plus occlusion of the right subclavian artery, avulsed right clavicle and extensive nerve damage. On admission to the hospital he had a Glasgow Coma Scale score of 7 (eyes = 1, motor = 1, verbal = 5). His mother reported (unconfirmed) that he was able to obey the command to squeeze her hand after 2-3 days, but it was approximately 13 days until he could recognize her. As the patient became niorc anm-c, he began to report the idea that he had an extra (‘third’) arm. He stated that this arm was located underneath his existing right arm and had its origin at his right
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Delusional reduplication following closed-head injuvy
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shoulder. He believed that the pain he was experiencing originated in this third arm, and constantly requested that the arm be searched for and moved into more comfortable positions. He stated that at first his doctors did not believe that he had an extra arm until the surgeons found it while performing surgery. The first time he noticed it himself was when it came to his assistance while in the bathroom. H e described this extra arm as having the same proportions as his real arms but being slightly smaller in scale. H e perceived this third arm as being extremely hot, and believed that his bed contained a hot water system that was responsible for the arm’s high temperature. At other times he would describe the sensation in the hand of the third arm as being like that of ‘having a gold wedding ring melt into my palm’. He believed that this extra limb was there to help him in tasks that had now become difficult as a consequence of the loss of function in his right arm. It was his belief that as function returned in his injured limb his extra arm would atrophy and disappear. H e would also make reference to his real arm, on the same side as the third arm, as being totally out of proportion to his body. On one occasion he stated he thought people in a 200-yard radius would be frightened by his arm because of its extraordinary size. If asked to indicate his right arm, he would point to the real one, and he was able to identify the various parts of the real arm without inaccuracy or distortion. The staff continually referred to his ‘third arm’ as his ‘phantom arm’ and in time he himself adopted this descriptor. Ths change of name, however, did not reduce h s references to the arm or alter his frequent wish for it to be found or moved. If his requests were ignored or challenged he quickly seemed to forget that his request had not been met. O n first evaluation there was some evidence of mild delusional thinking. H e believed ‘mentally I have become stronger since the accident’, and he felt he had become more perceptive of his surroundings. For instance, he believed his olfactory sense had become enhanced. H e said he knew through his sense of smell when people entered the room and stood behind him. H e had to be turned in his chair before he could be convinced that t h s was not the case. H e also complained of visual problems with h s right eye that had a significantly dilated pupil. An evaluation did not uncover any clinically significant problems with vision. He was slightly disinhibited in both manner and speech, although it was not clear from his family if this was also the case pre-morbidly. He would make sexually explicit remarks to his wife in front of the examiner, as well as making inappropriate remarks to the examiner himself It was clear, though, from his history, that sex took a very dominant role in his life, and so this behaviour may not have been a significant change from previous behaviour patterns or a signal of greatly decreased inhibition. H e graduated from high school at 18, having been a relatively good student. Upon graduation he joined the army and served in a tank crew and in the airborne rangers. H e left the army before his accident because he reportedly found it increasingly difficult to accept orders. Upon leaving the army he worked odd jobs and then set up a construction business. His medical history is significant for two previous motorcycle accidents. The first accident occurred in 1979 when he fell from his bike injuring his leg and hitting his head with such force that it split his helmet. H e denied any significant long-term physical or psychological effect from this incident. His second accident occurred a year later. H e fell from his bike and suffered a severe impact to the back of this head which he reported resulted in a ‘blood clot from my skull to my spine’. From his description, it appears he suffered a haematoma across his occipital lobes. H e was hospitalized for approximately 3 months.
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As a consequence of this latter accident C. became more short-tempered-reported by both himself and his family. H e became involved in frequent fights; his nickname reflected his prowess a t this activity-‘Mule’. It is unclear to what extent his belligerent tendencies existed before the accident, but it seems the behaviour following the second accident was an exacerbation of pre-morbid inclinations. Along with his short temper he began to suffer extremely painful headaches, which he admitted at times would be so bad as to make him cry. He experienced headaches approximately every 2 days.
Evaluation results The Luria Nebraska Neuropsychologlcal Battery Form I1 [ l l ] and a number of short tests were administered to gauge the patient’s general level of neuropsychological functioning and to attempt to identify any possible neurologcal cause for his behaviour. The short procedures included the Trail Making Test I121 Digit Span [13], Stroop Color Word Test 1141, Knox Cube Test 1151, Benton’s Visual Form Discrimination Test [16], Benton’s Line Orientation Test [ 161 and the Semmes Body Orientation Test [17]. Cognitive efficiency was found to be two standard deviations below normal. A test measuring cognitive flexibility and speed (Trails A-D) also revealed a moderate level of impairment. However, both tasks required good manual dexterity, which was compromised in this patient. Auditory verbal sequential memory as measured by D i g t Span was in the normal range. Specifically he was able to recall eight diglts forward and four backwards. His performance on this task was somewhat above average, but the discrepancy between the two tasks suggests some problems with mental control and concentration. His visual spatial sequential memory, as measured by the Knox Cube Test [15], revealed some impairment. He was only able to repeat a series of five movements, and his overall performance was inconsistent, indicating some attentional problems. Parietal lobe functioning was assessed because of the observed relationship between parietal dysfunction and anomalies of bodily experience [2]. The Judgement of Line Onentation Test, the Visual Form Discrimination Test and the Semmes Body Orientation Test were used. His performance on these tests was almost perfect, which would tend to rule out any major involvement of the parietal lobes in his presenting problem. These test findings are consistent with the CT scan results, which did not uncover any significant parietal involvement. The majority of neuropsychological functions, as measured by the Luria Nebraska Neuropsychologd Battery, were intact. Significant elevations were noted on both clinical and factor analytic scales indexing tactile and motor functions. These elevations, though, were a direct result of the injuries to his right arm. The Intermediate Memory scale was also elevated. This appeared to result from his incidental memory being somewhat weak, but information that he deliberately encoded was well consolidated and retrievable. All other neuropsychological functions measured on this test--such as reading, writing and arithmetic, as well as receptive and expressive speech-were all intact. The patient endorsed items on the MMPI consistent with individuals who tend to report feelings of unreality, odd thinlung, and possibly delusions or hallucinations. The dcgee of elevation of the scales was in the mild to moderate range. It can largely be assumed that the significant elevations found with this inventory are a consequence of his responding to his current experiences, although it cannot be ruled out that some existing idiosyncratic personality factors may have predisposed him to his current somatic condition. There were no signs of overt psychosis or severe psychopathology.
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Table 1 . Results ofshort tests. Trai/ Making Test-Halstead-Reitan
Trials A B
c
D
96s 177s 110s 180s
Line Orientation Test 28/30 trials correct Brain Inj Downloaded from informahealthcare.com by University of British Columbia on 10/29/14 For personal use only.
Visual Fom Disrrimination 15/16 trials correct
Knox Cube Test Seven trials correct Mmmum of five sequential moves recalled Norm 11 years Stroop Colov Word Test
Word Color Color/Word
60
44 24
(26) (26) (29)
Digit Span Subtest of W A I S - R
Maximum digits forward Maximum digits backwards Scale Score
8
4 10
Personal Orientation Test
Touch parts of body named by examiner-8/8 correct Names parts of body touched by examiner-8/8 correct Touch named parts of examiner’s body-7/8 correct Touch own body according to schematic diagram-5/8 correct
Discussion The results of the evaluation tend to suggest the delusional reduplication of the right arm is partly a psychiatric manifestation rather than one singularly resulting from any central nervous system lesion. There was no evidence of major parietal involvement from either the neuropsychological results or the CT scan. However, the bodily injuries were on the right side where the greatest impact occurred, and it is possible that subtle, mild injuries may have occurred in the right hemisphere. Similarly, the parietal lobes have been implicated in immediate recall, and the relatively intact performance on the verbal task (Dig-lt Span) compared with the relatively poor performance on the visual spatial immediate recall task (Knox Cube) indicates the possibility of subtle right parietal involvement, although comparisons between these two tests have not been previously validated as a measure of laterality. In light of the above findings a number of theoretical explanations can be suggested to try to explain this phenomenon. The existence of a psychotic-type process would be an obvious consideration in the case. The symptoms did have a definite delusional quality, particularly as the ideas were held with such conviction (at least initially), and there was little doubt in the patient’s mind the arm did exist. The phenomenon also could be a product of an acute confusional state secondary to the brain insult. However, this hypothesis is doubtful given the length of time after the injury that the symptoms were present, and the fact that he was generally well oriented. Furthermore, a search of the
M .J . C . Rofers and M . D . Franzen
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literature did not reveal any published observations regarding a relation between delusional reduplication and confusional states. The frequent lack of concern he demonstrated for his injured a r m , and the emphasis he put on his third arm, suggested a form of neglect. He ncver came to acknowledge the scriousncss of his injury or make rcference to the possibility of lifelong incapacity. H e was able to watch his arni being biopsied and dressed, with absolutely no display of concern, a fact that remains hard to understand g v c n the open nature of the wounds. Neglect has traditionally been associated with lesions to the right parietal lobe, although recent research has suggested that this form of awareness is mediated more diffusely involving subcortical structures [Is]. O n formal testing using the Semmes Body Orientation Test there was no indication that there was any neglect of his body. Tests to gauge the integnty
Brain Inj Downloaded from informahealthcare.com by University of British Columbia on 10/29/14 For personal use only.
Delusional reduplication following closed-head injury
475
of the parietal lobes revealed no deficits in any visual spatial hnctioning or somatosensory discrimination. Therefore the standard interpretation of neglect does not appear to be sufficient to explain this situation. Because of the variety of patients seen by Weinstein et al. [lo] exhibiting reduplication of limbs, they doubt it can be ‘attributed to destruction of any specific area such as the parietal lobe’, or ‘the corticothalamic and reticulothalamic activating and inhibitory system’. Rather, they postulate ‘the lesion initiates a reorganization of brain function in which any member that the patient conceives of as damaged may be reduplicated, no matter what the cause of the incapacity’. Weinstein [19] further states that reduplicative delusional states are dependent upon psychological variables. When a limb remains attached to the body but is essentially divorced from it as a result of nerve damage, the nervous system may not be able to reconcile the continued existence of the limb with the lack of somatosensory efferentation. Input from the damaged nerve ends may be distorted and misinterpreted in an effort to make sense of new and unfamiliar input. O n the other hand the phenomenon may reflect a simple refusal to come to terms with a distressing event, a frequent cause of many bizarre forms of pathology. In time the patient came to use the term ‘phantom arm’ to refer to his previously named ‘third arm’, more to placate the hospital staff than as a sign of a change in his own perceptions. His expressed belief that in time his ‘third arm’ would atrophy and eventually disappear as his right arm healed is consistent with the interpretation that his belief in the third arm was a pathological psychological coping mechanism. O n latest contact, approximately 4 months post-injury, the patient’s right arm had begun to show signs of spasticity and to draw up towards his chest. He spent some time in a rehabilitation unit but was unhappy away from his family and so signed hmself out. His family stated he no longer makes reference to his ‘third arm’ but experiences episodes of depression and dependency. H e is currently being medicated for his depression and has not been able to return to work.
Conclusion Given the seriousness of his accident the neuropsychological evaluation revealed that the patient’s cognitive functioning was relatively unimpaired except in a few specific areas. Cognitive efficiency was found to be slightly compromised, which may have resulted in him being unable to deal with changing situations in an effective manner. His limited capacity in this area could have impaired his ability to handle the psychological and physical upheavals experienced after the accident. Besides his lowered cognitive efficiency, his ability to handle his traumatic situation is likely to have been further aggravated and compromised by some apparent, and previously mentioned, personality idiosyncrasies, w h c h resembled in many respects an antisocial personality disorder. These symptoms appear to have been exacerbated by this head injury. These factors, in combination, may have resulted in his misinterpretation of his situation in a way that can only be described as a pathological form of wishkl thinking. Although there were subtle signs of right parietal involvement in the Knox Cube results, other procedures which tap right parietal functions, especially body awareness functions, were found to be unimpaired limiting the utility of strictly neurological aetiology for his somatic delusion. The neurological damage may have predisposed the patient to develop his somatic delusion, but the degree of manifest damage is insufficient to explain completely the clinical presentation. This case serves to illustrate the importance of considering not only neurologcal factors, but also pre-morbid personality, as well as emotional and situational factors
476
M . J . C . Rogers and M . D . F r a n z e n
following the injury, when attempting t o evaluate patients with definite neurological damage. It is likely another individual with identical injuries t o those suffered by this patient would respond in a very different manner, almost purely o n the strength of pre-morbid personality and learning experience. It would seem advisable, therefore, t o evaluate aspects of history and personality if o n e is t o understand and accurately conceptualize post-traumatic changes.
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Acknowledgement T h e authors would like t o thank Marc Haut, PhD for his helpful comments on a n earlier draft of this manuscript.
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2. GEKSTMANN, J.: Problem of imperception of disease and of impaired body temtories with organic lcsions: relation to body scheme and its disorder. Archives of Neurology and Psychiatry, 48: 89it-913, 1942. 3. DOKAN, J. M.: The Capgrass syndrome: neurologd/neuropsychological perspectives. Xeiiropsychology, 4: 29-42, 1990. 1. MADAKASIKA, S. and HALL,T.: Capgrass syndrome in a patient with myxedema. American Joirrrial ofPsyrhiatry, 138: 150G1507, 1981. 5. WESTON,M. J . and WHITLOCK, F. A,: The Capgrass syndrome following head injury. British Joirrnal ojPsychiatry, 119: 25-31, 1971. 6. CHKISTODOULOU, G. N . : Course and prognosis of the syndrome of doublcs. Journal of Nervous Merital Disease, 166: 68-72, 1978. 7. ALEXANDER, M. P., Si-vss, D. T. and BENSON,D. F.: Capgrass syndrome: a reduplicative phenomenon. .YeirroloXy, 29: 334-339, 1979. 8 . BANKS, G., SHOKT, P., JGLIO MARTINEZ, A,, LA.TCHAW, R., RATCLIFF, G. and BOLLER, F.: The alien hand syndrome, clinical and postmortem findings. Archives of Neurology, 46: 456-460, 1989. 9. CAKLEN, P. L., WALL,P. D., NALWORNA, H. and STEINBACH, T.: Phantom limbs and related phenomena in recent traumatic amputations. h'eurofogy, 28: 211-217, 1978. 10. WEINSTEIN, E. A., KAHN,l i . L., MALITZ, S. and ROZANSKI, J.: Delusional reduplication of parts of the body. Brain, 77: 43-60, 1954. C. J., MOSES,J. A. and HAMMEKE, T. A.: L~iriaNebraska Neuropsychological Battery: 11. GOLDEN, Form I and II Maniral. (Western Psychologcal Services, Los Angeles), 1985. D.: f i e Halstead Reitati Neuropsychological Battery: f i e o r y and 12. EVEITAN,R . M . and WOLFSON, Clinical Ititwpretarion (Neuropsychology Press, Tucson, AZ), 1985. D.: WAIS-R Manrial (Psychological Corporation, New York), 1981. 13 WECHSLER. C . J.: Stroop Color and Word Test: A Mariitalfor Clinical and Experimental Use (Stoelting, 14. GOLDEN, Chicago), 1978. 15. STONE,M. H. and WKIGHT, B. D.: Ktiox Cube Test-Instruction Manual (Stoelting, Chicago), 1980. 16. BENTON,A. L., HAMSHEK, K. DES.,VARNEY, N . R . and SPREEN, 0.:Contributions to Neuropsychological Asressriient (Oxford University Press, New York), 1983. 17. SEMMES, J., WEINSTEIN, S., GHENT,L. and TEUBEK,H. L.: Correlates of impaired orientation in personal and extrapersonal space. Brain, 86: 747-772, 1963. 18. HEALTON, E. B., NAVOKRO,C., BKESSMAN, S. and BKUST,J. C. M.: Subcortical neglect. Srurology, 32: 776-778, 1982. 19. WEINSTEIN, F. A.: Anosahmosia and denial of illness. In G. P. Prigatano and S. Schacter (Eds), Awarerresx of Dejcit ofter Braiti hjiq: Clinical arid Theoretical Issues (Oxford University Press, New York), pp. 240-257, 1991.
