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Traumatic transient klüver-bucy syndrome
Traumatic Transient Kl iver.Bucy Syndrome Tyrone U Hardy, M.D., and Jeffrey Aldridge, M.D.
A case of transient Klfiver-Bucy syndrome after a gunshot wound through the head is presented. We have found no other case of posttraumatic Kliiver-Bucy syndrome described in the literature. The original criteria for the syndrome are reviewed and compared with findings in previously reported human cases.
The syndrome following bilateral temporal lobe resection in the rhesus monkey was described in 1938 by Kliiver and Bucy [3]. Postoperative changes in the animals included decrease in aggressive behavior, increased sexuality, excessive attention to visual stimuli, changes in appetite, visual agnosia, and a strong tendency to oral exploration. Others (see [1]) have reviewed and expanded on these original findings. Elements of the syndrome have also been described in a few human patients having various lesions of the lateral temporal and frontoorbital cortices [ 1, 2, 4, 5]. In this paper, we present a case of the syndrome following a gunshot wound through the temporal lobes. Case Report A 16-year-old Caucasian boy was rushed to the emergency room after shooting himself through the head with a 25caliber handgun. Upon arrival, he was agitated, combative, and disoriented, but responded appropriately to simple verbal commands. Examination in the emergency room revealed a 3 mm penetrating injury approximately 4 cm posterior to the lateral orbital rim in the right temporal area. A similar exit wound was noted in an analogous location on the left side. The patient reported blindness in the right eye, and a total right ophthalmoplegia was evident. Examination of the left eye was normal. Testing of all other cranial nerves revealed no deficit. Good strength and tone were demonstrated in all four extremities, and no sensory impairment was detected. Deep tendon reflexes were active and symmetrical with plantar responses downgoing. The remainder of the physical examination was unremarkable. Appropriate laboratory studies were within normal limits. Roentgenograms of the From the Universityof New Mexico School of Medicine, Division of NeurologicalSurgery,Albuquerque,NM. Address reprint requests to Dr. Tyrone L. Hardy, University of New MexicoSchoolof Medicine,Divisionof NeurologicalSurgery,Albuquerque, NM 87131. Key words: Kliiver-Bucysyndrome;temporallobe; gunshotwound; transient symptoms. 338
skull revealed a small stellate lucency at the right temporal entrance site, without any evidence of intracranial bone or metal fragments. An emergency computerized tomographic scan of the head demonstrated bilateral, high-density, patchy temporal lobe lesions consistent with moderate contusion having no mass effect (Figure). The patient was taken to the intensive care unit, where he was managed expectantly. A dense left hemiparesis developed 24 hours after injury. A repeat computerized tomographic scan demonstrated that the previously noted right temporal lobe lesion had enlarged, with slight mass effect becoming discernible. After the administration of dexamethasone and mannitol, the patient was taken to the operating room where a right frontotemporal craniotomy was made. An intracerebral hematoma was evacuated, and a small subdural polyethylene catheter was placed for postoperative monitoring of intracranial pressure. During the immediate postoperative period, the intracranial pressure was noted to fluctuate markedly, with values up to 58 tort. In spite of the marked variability of the patient's intracranial pressure, it was possible--with the judicious use of mannitol, ventilatory hyperventilation, and intravenous lidocaine boluses--to maintain the pressure between 20 and 34 torr. The patient's other medications during the postoperative period included chloram. phenicol, as well as phenobarbital and phenytoin for seizure prophylaxis. The patient gradually improved and, by the first postoperative day, he had been successfully extubated and was responding appropriately to simple verbal commands. He did, however, have evidence of an anomic aphasia and also failed to recognize members of his immediate family. This aphasia, and the patient's previously mentioned right ophthalmoplegia, resolved by the third postoperative day. Improvement of the patient's left hemiparesis continued, and he was ambulatory by the end of the first postoperative week. His prosopagnosia also resolved. During this time, indiscriminate verbal sexual advances were noted by the nurses, the patient's sisters, and a female medical student caring for the patient. He also spoke to his mother explicitly about his sexual relationships with his girlfriend. All of the family members agreed that this was extremely atypical for the patient. The sexual content of his speech was predominantly heterosexual, and his sexual advances were never noted to be aggressive in nature. In addition, frequent overt masturbation was noted. The patient was
0090-3019/81/050338-03501.25 © 1980 by Little, Brown and Company (Inc.)
Hardy and Aldridge: Kltiver-Bucy Syndrome in Man
339
memory quotient of 100. This discrepancy was felt to be due primarily to the patient's severe difficulty in assimilating new, verbally presented information. Deficits in personality functioning included impaired frustration tolerance, poor impulse control, hyperactivity, and emotional lability. At the time of discharge, the patient's appetite was normal and he was making no blatantly sexual comments in interviews. The complexity of his language and his verbal inflection had improved. Blunting of his affect was still evident, and the patient's obsessive insistence on exact routine persisted.
CT scan dem~mstrating bilateral high-density, patchy anterior temporal lobe lesions.
also observed to have a ravenous appetite, but no inappropriate tendency to oral exploration was noted. Concurrent physical and occupational therapy resulted in improvement of fine motor skills and activities of daily living. During this time, the patient was also noted to be obsessive about keeping to an orderly daily routine, and he insisted that visitors, therapists, and family members all abide by a strict timetable. Personal belongings had to be kept in their designated places at all times. Deviation from this compulsive routine did not cause the patient to become angry but he did insist upon more regimentation. Attention to even the most trivial visual stimuli provoked strong verbal responses. For example, a light turned on in a neighboring room or a small child quietly passing down the hallway triggered loud, demanding questions about the significance of these events. On the fifteenth day after injury, the patient underwent psychiatric evaluation. Lack of emotion, impaired immediate and short-term memory, blunted affect, and a giddy mood were noted by the consultant. Minimal inappropriate sexuality was evident at this late stage of the patient's course. Steady improvement continued, and plans were made for the patient's transfer to a long-term rehabilitation facility. Prior to discharge, extensive psychological testing was performed. Evaluation was made using the adult Halstead-Reitan neuropsychological test battery. The most notable deficit was information gaps in his long-term memory. His full-scale IQ was 121, with a
Discussion Pilleri [4] presented 3 cases of the Kltiver-Bucy syndrome. However, all 3 of these patients suffered from diffuse degenerative diseases of the central nervous system. In only 1 of their patients does the case description seem characteristic of the syndrome described by Kltiver and Bucy. This patient was a 61-year-old man with Alzhe~mer's disease, who was noted to display emotional dullness, a tendency toward grasping, and strong oral behavior. Terzian and Dalle Ore [5] described a 19,year-old man who underwent bilateral temporal lobectomy for treatment of temporal lobe epilepsy. Postoperatively, the patient was unable to recognize close relatives, reacted excessively to visual stimuli, and ate voraciously. Hypersexuality, memory difficulties, and absence of emotion were also noted. The duration of these changes was not mentioned. Other reported cases in humans [1] have also been a result of deliberate surgical therapy for epilepsy, as well as psychosis and mental retardation. The findings in these cases were somewhat variable, but nevertheless, a majority of the original criteria of the Kltiver-Bucy syndrome were noted. It seems as though the tendency toward hyperoral activity--particularly coprophagia, uriposia, and indiscriminate oral exploration--is less frequently seen in humans. This was evident in our patient, in whom only a ravenous appetite was observed. On the other hand, docility or diminished aggressive behavior is a very consistent finding. For this reason, the use of bilateral amygdalotomies has been advocated in social-affective aggressive personality disorders. The patient presented here exhibited several of the psychological and physiological characteristics of the syndrome of Kl{iver and Bucy. Memory difficulty, hypersexuality, and placidity were marked. A voracious appetite, absence of any aggressive behavior, inappropriate attention to visual stimuli, and homeostatic instability completed the transient, but characteristic, constellation of changes previously described in human cases of bitemporal brain lesions. References 1. Kling A: Effects of amygdalectomyon social-affectivebehavior in non-human primates, in EleftheriouB (ed): Advances in Behavioral
340
Surgical Neurology Vol 15 No 5 May 1981
Biology. Vol 2, The Neurobiologyof Amygdala. New York, London: Plenum, 1972, pp 511-536 2. KliiverH: "The temporal lobe syndrome" produced by bilateral ablations, in WolstenholmeGEW, O'Connor CM (eds): The Neurologic Basis of Behavior (A Ciba Foundation Symposium). Boston: Little, Brown, 1958, pp 175-182 3. KltiverH, Bucy PC: An analysisof certain effectsof bilateral temporal
lobectomy in the rhesus monkey, with special reference to "psychic blindness." J Psychol 5:33-54, 1938 4. Pilleri G: The KRiver-Bucysyndrome in man: a clinico-anatomical contribution to the function of the medial temporal lobe structures. Psychiatr Neurol (Basel) 152:65-103, 1966 5. Terzian H, Dalle Ore G: Syndrome of Kltiver and Bucy. Neurology 5:373-381, 1953
Editorial: What Happened? In the course of reviewing manuscripts for publication in
paralysis (which is true) and that that paralysis is perma-
Sur~cal Neurology one of the greatest defects which is en- nent (which often is not true). Similarly, destruction of the countered is the failure of the authors to present the course of the patient subsequent to treatment. It is important that readers be told what the postoperative course has been over as long a period of time as possible. Both patients and physicians have a much greater interest in the long-term results than they have in the immediate ones. This is true in evaluating any form of treatment. For example, the immediate results of removal of a glioblastoma of the brain are often very good. Yet those same patients commonly are dead within two years after the operation. Tumors which have been incompletely removed almost always continue to grow. It is important that we know how rapidly or how slowly they grow. This can be determined only if we are informed regarding the patients' condition years after the operations were performed. Similarly, the results of lesions of the central nervous system are often temporary. The injured brain has a great capacity for the restitution of function. For example, if the pyramidal tract is destroyed, the immediate result is a paralysis of the parts of the body which were innervated by that pyramidal tract. For many years it was assumed, therefore, that destruction of the pyramidal tract leads to
cerebellum results in the incoordination and ataxia which is typical of cerebellar lesions. Yet with the passage of time most of that disability may disappear. This article by Hardy and Aldridge is about a patient in whom a gunshot wound destroyed much of both temporal lobes and produced the symptoms that have come to be known as the Klfiver-Bucy syndrome. No one would doubt that the destruction of the temporal lobes caused by that bullet was a permanent one. Yet the typical symptoms of bilateral temporal lobectomy which that patient developed were transitory. Many other examples of change in the patients' condition with the passage of time, both for better and for worse, could be cited. Any article which is published is incomplete unless it describes the patients' condition over as long a period of time as possible. The failure to do so is encountered so frequently that the editors of Surgical Neurology are taking this opportunity to ask their contributing authors to make their reports as complete as possible.
Paul C. Bucy, M.D., Editor
A case of transient Klfiver-Bucy syndrome after a gunshot wound through the head is presented. We have found no other case of posttraumatic Kliiver-Bucy syndrome described in the literature. The original criteria for the syndrome are reviewed and compared with findings in previously reported human cases.
The syndrome following bilateral temporal lobe resection in the rhesus monkey was described in 1938 by Kliiver and Bucy [3]. Postoperative changes in the animals included decrease in aggressive behavior, increased sexuality, excessive attention to visual stimuli, changes in appetite, visual agnosia, and a strong tendency to oral exploration. Others (see [1]) have reviewed and expanded on these original findings. Elements of the syndrome have also been described in a few human patients having various lesions of the lateral temporal and frontoorbital cortices [ 1, 2, 4, 5]. In this paper, we present a case of the syndrome following a gunshot wound through the temporal lobes. Case Report A 16-year-old Caucasian boy was rushed to the emergency room after shooting himself through the head with a 25caliber handgun. Upon arrival, he was agitated, combative, and disoriented, but responded appropriately to simple verbal commands. Examination in the emergency room revealed a 3 mm penetrating injury approximately 4 cm posterior to the lateral orbital rim in the right temporal area. A similar exit wound was noted in an analogous location on the left side. The patient reported blindness in the right eye, and a total right ophthalmoplegia was evident. Examination of the left eye was normal. Testing of all other cranial nerves revealed no deficit. Good strength and tone were demonstrated in all four extremities, and no sensory impairment was detected. Deep tendon reflexes were active and symmetrical with plantar responses downgoing. The remainder of the physical examination was unremarkable. Appropriate laboratory studies were within normal limits. Roentgenograms of the From the Universityof New Mexico School of Medicine, Division of NeurologicalSurgery,Albuquerque,NM. Address reprint requests to Dr. Tyrone L. Hardy, University of New MexicoSchoolof Medicine,Divisionof NeurologicalSurgery,Albuquerque, NM 87131. Key words: Kliiver-Bucysyndrome;temporallobe; gunshotwound; transient symptoms. 338
skull revealed a small stellate lucency at the right temporal entrance site, without any evidence of intracranial bone or metal fragments. An emergency computerized tomographic scan of the head demonstrated bilateral, high-density, patchy temporal lobe lesions consistent with moderate contusion having no mass effect (Figure). The patient was taken to the intensive care unit, where he was managed expectantly. A dense left hemiparesis developed 24 hours after injury. A repeat computerized tomographic scan demonstrated that the previously noted right temporal lobe lesion had enlarged, with slight mass effect becoming discernible. After the administration of dexamethasone and mannitol, the patient was taken to the operating room where a right frontotemporal craniotomy was made. An intracerebral hematoma was evacuated, and a small subdural polyethylene catheter was placed for postoperative monitoring of intracranial pressure. During the immediate postoperative period, the intracranial pressure was noted to fluctuate markedly, with values up to 58 tort. In spite of the marked variability of the patient's intracranial pressure, it was possible--with the judicious use of mannitol, ventilatory hyperventilation, and intravenous lidocaine boluses--to maintain the pressure between 20 and 34 torr. The patient's other medications during the postoperative period included chloram. phenicol, as well as phenobarbital and phenytoin for seizure prophylaxis. The patient gradually improved and, by the first postoperative day, he had been successfully extubated and was responding appropriately to simple verbal commands. He did, however, have evidence of an anomic aphasia and also failed to recognize members of his immediate family. This aphasia, and the patient's previously mentioned right ophthalmoplegia, resolved by the third postoperative day. Improvement of the patient's left hemiparesis continued, and he was ambulatory by the end of the first postoperative week. His prosopagnosia also resolved. During this time, indiscriminate verbal sexual advances were noted by the nurses, the patient's sisters, and a female medical student caring for the patient. He also spoke to his mother explicitly about his sexual relationships with his girlfriend. All of the family members agreed that this was extremely atypical for the patient. The sexual content of his speech was predominantly heterosexual, and his sexual advances were never noted to be aggressive in nature. In addition, frequent overt masturbation was noted. The patient was
0090-3019/81/050338-03501.25 © 1980 by Little, Brown and Company (Inc.)
Hardy and Aldridge: Kltiver-Bucy Syndrome in Man
339
memory quotient of 100. This discrepancy was felt to be due primarily to the patient's severe difficulty in assimilating new, verbally presented information. Deficits in personality functioning included impaired frustration tolerance, poor impulse control, hyperactivity, and emotional lability. At the time of discharge, the patient's appetite was normal and he was making no blatantly sexual comments in interviews. The complexity of his language and his verbal inflection had improved. Blunting of his affect was still evident, and the patient's obsessive insistence on exact routine persisted.
CT scan dem~mstrating bilateral high-density, patchy anterior temporal lobe lesions.
also observed to have a ravenous appetite, but no inappropriate tendency to oral exploration was noted. Concurrent physical and occupational therapy resulted in improvement of fine motor skills and activities of daily living. During this time, the patient was also noted to be obsessive about keeping to an orderly daily routine, and he insisted that visitors, therapists, and family members all abide by a strict timetable. Personal belongings had to be kept in their designated places at all times. Deviation from this compulsive routine did not cause the patient to become angry but he did insist upon more regimentation. Attention to even the most trivial visual stimuli provoked strong verbal responses. For example, a light turned on in a neighboring room or a small child quietly passing down the hallway triggered loud, demanding questions about the significance of these events. On the fifteenth day after injury, the patient underwent psychiatric evaluation. Lack of emotion, impaired immediate and short-term memory, blunted affect, and a giddy mood were noted by the consultant. Minimal inappropriate sexuality was evident at this late stage of the patient's course. Steady improvement continued, and plans were made for the patient's transfer to a long-term rehabilitation facility. Prior to discharge, extensive psychological testing was performed. Evaluation was made using the adult Halstead-Reitan neuropsychological test battery. The most notable deficit was information gaps in his long-term memory. His full-scale IQ was 121, with a
Discussion Pilleri [4] presented 3 cases of the Kltiver-Bucy syndrome. However, all 3 of these patients suffered from diffuse degenerative diseases of the central nervous system. In only 1 of their patients does the case description seem characteristic of the syndrome described by Kltiver and Bucy. This patient was a 61-year-old man with Alzhe~mer's disease, who was noted to display emotional dullness, a tendency toward grasping, and strong oral behavior. Terzian and Dalle Ore [5] described a 19,year-old man who underwent bilateral temporal lobectomy for treatment of temporal lobe epilepsy. Postoperatively, the patient was unable to recognize close relatives, reacted excessively to visual stimuli, and ate voraciously. Hypersexuality, memory difficulties, and absence of emotion were also noted. The duration of these changes was not mentioned. Other reported cases in humans [1] have also been a result of deliberate surgical therapy for epilepsy, as well as psychosis and mental retardation. The findings in these cases were somewhat variable, but nevertheless, a majority of the original criteria of the Kltiver-Bucy syndrome were noted. It seems as though the tendency toward hyperoral activity--particularly coprophagia, uriposia, and indiscriminate oral exploration--is less frequently seen in humans. This was evident in our patient, in whom only a ravenous appetite was observed. On the other hand, docility or diminished aggressive behavior is a very consistent finding. For this reason, the use of bilateral amygdalotomies has been advocated in social-affective aggressive personality disorders. The patient presented here exhibited several of the psychological and physiological characteristics of the syndrome of Kl{iver and Bucy. Memory difficulty, hypersexuality, and placidity were marked. A voracious appetite, absence of any aggressive behavior, inappropriate attention to visual stimuli, and homeostatic instability completed the transient, but characteristic, constellation of changes previously described in human cases of bitemporal brain lesions. References 1. Kling A: Effects of amygdalectomyon social-affectivebehavior in non-human primates, in EleftheriouB (ed): Advances in Behavioral
340
Surgical Neurology Vol 15 No 5 May 1981
Biology. Vol 2, The Neurobiologyof Amygdala. New York, London: Plenum, 1972, pp 511-536 2. KliiverH: "The temporal lobe syndrome" produced by bilateral ablations, in WolstenholmeGEW, O'Connor CM (eds): The Neurologic Basis of Behavior (A Ciba Foundation Symposium). Boston: Little, Brown, 1958, pp 175-182 3. KltiverH, Bucy PC: An analysisof certain effectsof bilateral temporal
lobectomy in the rhesus monkey, with special reference to "psychic blindness." J Psychol 5:33-54, 1938 4. Pilleri G: The KRiver-Bucysyndrome in man: a clinico-anatomical contribution to the function of the medial temporal lobe structures. Psychiatr Neurol (Basel) 152:65-103, 1966 5. Terzian H, Dalle Ore G: Syndrome of Kltiver and Bucy. Neurology 5:373-381, 1953
Editorial: What Happened? In the course of reviewing manuscripts for publication in
paralysis (which is true) and that that paralysis is perma-
Sur~cal Neurology one of the greatest defects which is en- nent (which often is not true). Similarly, destruction of the countered is the failure of the authors to present the course of the patient subsequent to treatment. It is important that readers be told what the postoperative course has been over as long a period of time as possible. Both patients and physicians have a much greater interest in the long-term results than they have in the immediate ones. This is true in evaluating any form of treatment. For example, the immediate results of removal of a glioblastoma of the brain are often very good. Yet those same patients commonly are dead within two years after the operation. Tumors which have been incompletely removed almost always continue to grow. It is important that we know how rapidly or how slowly they grow. This can be determined only if we are informed regarding the patients' condition years after the operations were performed. Similarly, the results of lesions of the central nervous system are often temporary. The injured brain has a great capacity for the restitution of function. For example, if the pyramidal tract is destroyed, the immediate result is a paralysis of the parts of the body which were innervated by that pyramidal tract. For many years it was assumed, therefore, that destruction of the pyramidal tract leads to
cerebellum results in the incoordination and ataxia which is typical of cerebellar lesions. Yet with the passage of time most of that disability may disappear. This article by Hardy and Aldridge is about a patient in whom a gunshot wound destroyed much of both temporal lobes and produced the symptoms that have come to be known as the Klfiver-Bucy syndrome. No one would doubt that the destruction of the temporal lobes caused by that bullet was a permanent one. Yet the typical symptoms of bilateral temporal lobectomy which that patient developed were transitory. Many other examples of change in the patients' condition with the passage of time, both for better and for worse, could be cited. Any article which is published is incomplete unless it describes the patients' condition over as long a period of time as possible. The failure to do so is encountered so frequently that the editors of Surgical Neurology are taking this opportunity to ask their contributing authors to make their reports as complete as possible.
Paul C. Bucy, M.D., Editor