Kluver-Bucy syndrome as a result of minor head trauma.

ABSTRACT

Kluver-Bucy syndrome (KBS) has been described as a disconnection of the temporal lobes from the remainder of the brain. Its presence in minor head trauma has not been previously reported. We therefore report what we believe to be the first case of KBS due to mild head trauma and unilateral injury to a temporal lobe.

**********

KLUVER-BUCY SYNDROME (KBS) has been described as a disconnection of the temporal lobes from the rest of the brain. Findings include blunted affect, bulimia, hypersexuality, and aggressive behavior. (1) Although this condition has been described in severe head trauma, (2,3) its occurrence after minor trauma has not been reported. We report a case of spontaneously resolving posttraumatic KBS in a patient with minor head trauma and describe the subtle findings on magnetic resonance imaging (MRI).

CASE REPORT

A 24-year-old Korean woman was a restrained driver involved in a high-speed motor vehicle collison. She came to the emergency room with stable vital signs and was able to move all extremities. The primary and secondary surveys were negative with the exception of a Glasgow Coma Scale (GCS) score of 14 and a laceration on the medial aspect of the left ankle. Radiographic evaluation including computed tomography (CT) of the head, blood tests, and urinalysis showed no abnormalities except for a blood alcohol level of 0.08%. The patient was admitted to the hospital for observation.

Throughout her hospital stay, the patient continued to have a mildly depressed level of consciousness. She was excessively somnolent, and her affect was blunted. She was easily irritable but expressed no sad or happy emotions under appropriate circumstances. Her mother and sister found her speech incomprehensible in her native Korean tongue, and the patient herself reported some difficulty finding words. She began eating voraciously, often 5 to 6 meals a day. Additionally, she began exhibiting mild signs of amnesia, often forgetting things that had been mentioned to her earlier in the day. At no point was there any difficulty in motor activities, loss of consciousness, or seizure activity.

Over the ensuing days, the increased appetite and blunt affect progressed, and neurologic and psychiatric evaluations were requested. It was discovered through a Korean translator that the patient was responding to questions in broken English, though her intention was to respond in Korean. Additionally, her sentences seemed premeditated and lacked a certain spontaneity, as if she had to concentrate to produce them. The patient even reported the same difficulty of finding the right words and staying within the correct language.

During her hospital stay, the patient was reported to have outbursts of irritation with the nurses and doctors. Additionally, she was seen to run around the ward naked. In one instance, she began moving her foot against the leg of a physician. She also began complimenting one doctor as "being very handsome and tall and every doctor should be tall and handsome" in a blunt tone.

Results of neurologic evaluation were normal except for the odd behavior. Psychologic evaluation ruled out depression and obsessive-compulsive disorder with no need for pharmaceutical intervention. Repeated CT of the head showed no abnormalities. An electroencephalogram revealed no seizure activity.

Ultimately, MRI was done. Multiple small plaques of increased signal intensity on T2 weighted images were found in the frontal, temporal, and parietal lobes, reminiscent of demyelination plaques. One large plaque occupied the white matter of the left temporal lobe (Figure). No lesions were seen in the gray matter or the amygdala of the temporal lobes. No overt lesions were seen in the right temporal lobe. Because the patient's personal and family history did not include demyelinating diseases such as multiple sclerosis, the diagnosis was focal axonal injury to the left temporal lobe. Neither CT nor MRI showed evidence of overt hemorrhage or contusion. Additionally, diffusion-weighted scan did not show any infarct.

During the next few days, the patient's affect improved, and she was noted to smile and converse in a more normal tone. Subsequently, she slept less, and her irritation subsided. Her eating habits also improved. She was discharged to a rehabilitation facility where she continued to show signs of improvement.

DISCUSSION

In 1937, Heinrich Kluver and Paul Bucy described a distinct, reproducible behavioral syndrome after bilateral temporal lobectomy in rhesus monkeys. (4) The animals developed a need to examine objects orally rather than with their hands, loss of normal anger and fear responses, and increased sexual activity, seen up to 4 weeks after temporal lobe resection. The animals were no longer able to recognize the objects of their surroundings. They developed an "irresistible" impulse to touch every object in sight and to examine all objects by mouth. (2) Kluver and Bucy believed this oral behavior was the result of a visual agnosia--an inability to recognize objects by sight.

Kluver-Bucy syndrome was first recognized in humans in 1955, when a patient with refractory seizures had bilateral temporal lobectomy. (5) He had symptoms similar to those seen in the animal model, without the need to examine objects by mouth. Since then, a number of features have been described as being part of this syndrome. Aphasia and amnesia have been described in nearly all patients, and most will exhibit one or more of the following: blunted affect, apathy, prosopagnosia or "psychic blindness" (the inability to distinguish among friends, relatives, and strangers), hypermetamorphosis manifested by consistent exploration of the environment and placement of objects into the mouth, bulimia, hyperactive oral behavior, and altered sexual behavior such as frequent sexual overtures and attempts at physical contact. (1) This cluster of symptoms was present in our patient who demonstrated both aphasia and amnesia, as well as blunted affect, increased oral activity, and a change in sexual behavior manifested by a ggressive, flirtatious behavior toward male physicians in contact with her.

Although the classic syndrome was described after bilateral temporal lobe resections, a number of nontraumatic and traumatic causes have been linked to the syndrome, including temporal lobe epilepsy, (5) herpes temporal encephalitis, (1,2) Alzheimer's and Pick's dementia, (1,6,7) cerebrovascular disease, (2) metabolic disturbances, (2,8) multicentric glioblastoma. (9) and traumatic brain injury. (1-3,10-13) Since certain aspects of human oral, affective, and sexual behavioral patterns have been localized to the temporal lobes, the etiology is thought to be significant damage to temporal lobes bilaterally. (9) More specifically, the sensory agnosia results from disruption of the temporal neocortex, while the oral behavior and the hypersexuality are caused by disturbances in the amygdala. (6) Classically, KBS has been associated with bitemporal lesions, though unilateral left temporal lobe resection has been noted to produce the syndrome as well. (9)

Posttraumatic KBS occurs as a consequence of severe head trauma to bilateral temporal lobes. It typically occurs in patients with prolonged loss of consciousness and has been observed during the recovery or remission phase of traumatic brain injury. (2,3,10,13) There is even a suggestion that the occurrence of KBS is a positive prognostic factor, associated with a favorable outcome after severe head trauma. (13)

The natural history of KBS is not known, but evidence suggests that in trauma, its course is temporary, ranging from 7 days to 1 year. (2, 13) For this reason, therapy in traumatic cases may not be indicated. Saltuari and Gerstenbrand (13) noted that quick recovery from KBS correlated significantly with good prognosis. In nontraumatic cases, treatment with carbamazepine, (10,11) leuprolide, (7) and selective serotonin reuptake inhibitors (12) have all been used with various degrees of success.

Perhaps the most striking aspect of our case is the mildness of the patient's presentation, as evidenced by her initial score of 14 on the Glasgow Coma Scale. Virtually all case reports have noted severe head injuries (GCS score of 3 to 7), with many patients requiring neurosurgical intervention. (3,10,12) We found no previous report of the combination of minor head trauma, unilateral temporal lobe involvement, and KBS in the literature. Furthermore, our patient's spontaneous recovery is consistent with the self-limiting nature of the syndrome.

CONCLUSION

Kluver-Bucy syndrome is an exceedingly rare occurrence due to severe injury to bilateral temporal lobes. Its association with mild head trauma and a unilateral temporal lobe lesion has not been described before. It is a self-limiting phenomenon in traumatic cases, and when present, may suggest a favorable neurologic outcome.

References

(1.) Lilly R, Cummings JL, Benson F, et al: The human KluverBucy syndrome. Neurology 1983; 33:1141-1145

(2.) Gerstenbrand F, Poewe W, Aichner F, et al: Kluver-Bucy syndrome in man: experiences with posttraumatic cases. Neurosci Biobehav Rev 1983; 7:413-417

(3.) Hardy TL, Aldridge J: Traumatic transient Kluver-Bucy syndrome. Surg Neurol 1981; 15:338-340

(4.) Kluver H, Bucy PC: An analysis of certain effects of bilateral temporal lobectomy in the rhesus monkey, with special reference, to psychic blindness. J Psychol 1938; 5:33-54

(5.) Terzian H, Dalle Ore G: Syndrome of Kluver and Bucy reproduced in man by bilateral removal of temporal lobes. Neurology 1955; 5:373-380

(6.) Cummings JL, Duchen LW: Kluver-Bucy syndrome in Pick disease: clinical and pathologic correlations. Neurology 1981; 31:1415-1422

(7.) Ott BR: Leuprolide treatment of sexual aggression in a patient with dementia and the Kluver-Bucy syndrome. Clin Neuropharmacol 1995; 18:443-447

(8.) Guidotti TL, Charness ME, Lamon JM: Acute intermittent porphyria and the Kluver-Bucy syndrome. Johns Hopkins Med J 1979; 145:233-235

(9.) Ghika-Schmid F, Assal G, De Tribolet N, et al: Kluver-Bucy syndrome after left anterior temporal resection. Neuropsychologia 1995; 33:101-113

(10.) Goscinski I, Kwiatkowski S, Polak J, et al: The Kluver-Bucy syndrome. Acta Neurochir 1997; 139:303-306

(11.) Hooshang H, Sepdham T, Vries JK: Kluver-Bucy syndrome: successful treatment with carbamazepine. JAMA 1974; 229:1782

(12.) Slaughter J. Bobo W, Childers MK: Selective serotonin reuptake inhibitor treatment of post-traumatic Kluver-Bucy syndrome. Brain Inj 1999; 13:59-62

(13.) Saltuari FR, Gerstenbrand F: Presence of Kluver-Bucy syndrome as a positive prognostic feature for the remission of traumatic prolonged disturbances of consciousness. Acta Neurol Scand 1995; 91:54-57

RELATED ARTICLE: KEY POINTS

* Kluver-Bucy syndrome occurs in association with bilateral temporal lobe injury.

* Findings include blunted affect, bulimia, hypersexuality, and aggressive behavior.

* Self-limiting phenomenon with favorable neurologic outcomes.

From the Department of Surgery, Division of Trauma and Critical Care, and the Los Angeles County--University of Southern California Medical Center, Los Angeles; and the Department of Neurosurgery, University of Southern California Keck School of Medicine, Los Angeles (Dr. Kim).

Reprint requests to Juan A. Asensio, MD, LAC+USC Medical Center, Division of Trauma Surgery/Critical Care, 1200 N State St, Room 10-750, Los Angeles, CA 90033.