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Kluver-Bucy Syndrome

Klüver-Bucy Syndrome

Kluver-Bucy syndrome is a very rare behavioral disorder triggered by damage to both temporal lobes of the brain.


Presentation

This paragraph shall merely serve as an orientation for symptoms that may be experienced by KBS patients. As has been mentioned before, humans typically do not present all of them at once [7].

KBS may be associated with emotional changes, particularly with placidity and affective indifference. Patients may even be apathetic and may not react accordingly to external stimuli. Facial expressions, gestures and speech are dull.  This emotional emptiness becomes most clear when these patients are exposed to fear-triggering objects and situations. In some cases, fear may, however, be expressed in non-dangerous situations.

The term hyperorality is often used in relation with KBS and describes an excess need to explore one's surroundings with the mouth [8]. Such behavior may not only be harmful, but is socially not accepted. KBS patients may suddenly start to lick objects in public. Some patients present a general, enhanced urge to explore with any sense available. This may be as much of a social problem as the aforementioned oral tendencies since people in public are not welcome to touch anything in view.

There may be a relation between these oral tendencies and dietary changes. Affected individuals eat in excess, they may place anything edible and non-edible into their mouth. The former will lead to obesity in the short run; the latter may cause life-threatening damage to the gastrointestinal tract or may lead to intoxication.

Visual agnosia may either occur due to failures in physiological processing of sensory inputs or because of the inability to associate objects viewed with pre-existing memories. Thus, the eyes of KBS patients may see an object, but the respective information may never be assembled or connected with memories. Visual agnosia enhances hyperorality.

Memory loss is experienced in some cases.

The increased sexual urge associated with KBS may be a major problem for affected people. They may engage in overt, public sexual behavior and criminally relevant activities such as uninvited attempts to have sex [9].

Overeating
  • Over 36 hours of hospitalization in a district hospital she developed unusual neuropsychiatric disorders: hypersexuality, hyperorality, absence, visual agnosia, sensory aphasia, amnesia, and depression typical of KBS.[ncbi.nlm.nih.gov]
  • Although cognitive and behavioral disturbances following herpes encephalitis are often severe, improvement can occur over an extended period, and chronic residual sequelae may be relatively mild.[ncbi.nlm.nih.gov]
  • Since the age of 2, he had experienced clusters of three or four daily complex partial seizures over 2-3 days in a month, followed by a seizure-free interval of 3-4 weeks.[ncbi.nlm.nih.gov]
  • Due to lack of environmental learning of sex and also, due to emotional and physical dependence on parents, the manifestations in young children showed modification over those of adults.[ncbi.nlm.nih.gov]
  • ., hyperorality, placidity, hypermetamorphosis) persist indefinitely, whereas others gradually resolve over several years. Klüver and Bucy were initially unaware of an earlier report of KBS by Sanger Brown and Edward Schäfer in 1888.[ncbi.nlm.nih.gov]
Polydipsia
  • Other unusual features included narcolepsy, polydipsia, and polyuria. Virologic studies failed to incriminate the etiologic agent, including herpes simplex virus. Brain biopsy of the frontal lobe demonstrated Alzheimer type II astrocytosis.[ncbi.nlm.nih.gov]
  • Other unusual features included narcolepsy, polydipsia, and polyuria. Virologic studies failed to incriminate the etiologic agent, including herpes simplex virus.[hub.hku.hk]
Apathy
  • A 70-year-old man with progressive behavioral symptoms of hyperorality, hypersexuality, hypermetamorphosis, visual agnosia, hyperphagia, and apathy who died at age 77 of asphyxiation on a foreign object.[ncbi.nlm.nih.gov]
  • A couple days after admission, our patient became alert and presented hypersexuality, hypermetamorphosis, increased oral tendency, behavior changes including apathy with loss of anger and fear, and a very increased appetite, transient visual agnosia and[ncbi.nlm.nih.gov]
  • Apathy and easy distractibility were rare. Bulimia and strong urge to put non-food items into the mouth were common.[ncbi.nlm.nih.gov]
  • Although medical management of ketoacidosis was uncomplicated, she developed hyperphagia, hyper-sexuality, hypermetamorphosis, visual agnosia, dull affect, rage alternating with apathy, and a mild right hemiparesis.[nature.com]
  • Apathy and easy distractibility are rare. Bulimia and a strong urge to put items other than food into the mouth are common.[patient.info]
Irritability
  • During the seizure-free period, the patient exhibited hyperorality, sniffing, irritability alternating with placidity, anxiety, unsolicited sexual gestures, and unusual calmness after eating.[ncbi.nlm.nih.gov]
  • Hyperorality was in form of an attempt to put non-edible substances in mouth, while abnormal behavior manifested as hostility, irritability or placidity.[content.iospress.com]
  • Early symptoms of Korsakoff’s syndrome include fatigue, irritation, poor memory, difficulty sleeping, chest pain, abdominal discomfort, poor appetite and constipation. Later symptoms are principally cardiovascular and neurological.[lazoi.com]
  • Early symptoms of Korsakoff’s syndrome include fatigue, irritation, poor memory, difficulty sleeping, chest pain, abdominal discomfort, poor appetite and constipation. Later symptoms are principally cardiovascular and neurological. 5.[slideshare.net]
  • […] lesions – jacksonian motor seizures Lesions of premotor areas – less important motor deficit Irritative lesions in premotor areas – “adversive” seizures, or supplementary motor area seizures Supplementary motor area seizures: cessation of all activity[slideplayer.com]
Abulia
  • […] fluency (verbal & nonverbal), reasoning problems, reduction of spontaneous responses inhibition, perseveration, attention deficits Orbitofrontal cortex lesions – desinhibition, anosmia Mezial frontal cortex, anterior cingullary cortex lesions – apathy, abulia[slideplayer.com]
Somnolence
  • At the moment of second hospital admission, the patient was found somnolent and confused at home, with traumatic signs of biting of the tongue and urine emission.[ncbi.nlm.nih.gov]

Workup

KBS as a behavioral disorder may be diagnosed based an the patient's medical history and clinical examination. It may, however, be very challenging to diagnose the underlying disease [8]. Symptoms not characteristic for KBS may be of great value to this end.

Depending on the list of most likely differential diagnoses, distinct measures need to be taken. Herpes simplex encephalitis is often associated with headaches and fever and may be diagnosed after analyzing a sample of liquor cerebrospinalis. Imaging diagnostics may be the approach of choice to identify several of alternative, less frequent causes of KBS. Magnetic resonance imaging and computed tomography scans of the brain may reveal signs of inflammation, ischemia, neoplasms and other pathologies. An electroencephalogram may be helpful in identifying the cause of seizures.

Amyloid Plaque
  • Neurofibrillary tangles and beta-amyloid plaques have been observed in the amygdala in Alzheimer disease. A disproportionate abundance of this abnormality in the amygdala may cause behavioral symptoms similar to Klüver-Bucy syndrome.[ncbi.nlm.nih.gov]
Neurofibrillary Tangle
  • Neurofibrillary tangles and beta-amyloid plaques have been observed in the amygdala in Alzheimer disease. A disproportionate abundance of this abnormality in the amygdala may cause behavioral symptoms similar to Klüver-Bucy syndrome.[ncbi.nlm.nih.gov]

Treatment

In general, patients need to be monitored to prevent they harm themselves, e.g., by ingesting non-edible objects. Dietary controls are required to avoid bulimia, subsequent overweight and obesity. Inappropriate, public behavior should be evaded. There is at least one case report where such behavior lead to criminal conviction [9].

Additionally, an adequate treatment of the underlying disease is required. In case of herpes simplex encephalitis it mainly consists in prolonged application of anti-viral drugs. Drug resistances might be encountered and indicate administration of another compound. Even after successful treatment of herpes simplex encephalitis, relapses may occur and manifest in form of sudden behavioral changes. In these patients, the anti-viral therapy needs to be re-initiated as soon as possible [10]. Other primary disease require distinct therapeutic approaches.

KBS itself has been treated with antidepressants and anticonvulsants, notably with selective serotonin reuptake inhibitors and carbamazepine [8].

Prognosis

Prognosis varies largely [6]. It improves significantly if the underlying disease can be treated. In this context, more effective anti-viral medication may change the average outcome of herpes simplex encephalitis. This may be associated with less mortality of the latter, an higher incidence of KBS, but a better prognosis for patients that do develop this behavioral disorder.

Etiology

As has been indicated in the previous paragraph, temporal lobes and amygdalas fulfill a variety of tasks regarding processing and association of visual and auditory input, memory and emotions. Distinct parts of the aforementioned brain structures may account for all these functions. Complete malfunction would thus require extensive lesions that comprise large parts of the temporal lobes and amygdalas, and this situation is rarely given in humans. However, partial injury may trigger some of the symptoms traditionally associated with KBS.

This "partial injury" may result from different cerebral disorders such as inflammation due to infections or autoimmune responses, vascular diseases, traumas, metabolic disorders, neoplasms and neurodegeneration: The most common trigger for KBS is an infection with the herpes simplex virus that lead to herpes simplex encephalitis. Tuberculosis may also be associated with KBS [2]. Cerebral infarction may occur in this part of the brain and cause functional impairment of the temporal lobes. Any other condition involving cerebral ischemia, hypoxia and hypoglycemia may have similar consequences as a stroke. Traumatic insults are another possible cause of KBS [3], although these most frequently involve injury to other cerebral areas. Of note, trauma may be understood in the broader sense of the word and does also include tissue damage or resection during surgical interventions. Niemann-Pick disease and porphyria may serve as examples for metabolic disorders triggering KBS. The disease has been described in relation with Alzheimer disease [4]. Neuronal ceroid lipofuscinosis is another neurodegenerative disease possible associated with KBS; it may affect children and adolescents.

Epidemiology

KBS is a very rare disease. A literature review reveals several descriptions of isolated cases but no larger studies on the disease. It is tempting to speculate that the age distribution of KBS reflects that of its underlying diseases, but this hypothesis has not yet been confirmed. KBS can occur at any age. It has been described in children [5]. Men and women are equally affected.

Sex distribution
Age distribution

Pathophysiology

The main functions of the temporal lobe are processing of visual and auditory sensory inputs, associating this input with pre-existing memories and connecting it with an emotional component, and creating new, long-term memory. For instance, the healthy rhesus monkeys used by Klüver and Bucy reacted with fear to snakes, i.e., they saw the snake, possibly associated this visual input with a previous, unpleasant memory or just reacted on instinct and labeled the corresponding object as dangerous. This chain of events was not any longer present in rhesus monkeys that underwent bilateral lobectomy. They remained placid, possibly even interested in front of the snake.

Lobectomies may be required in humans after severe accidents, due to epilepsy or neoplasms, but most KBS patients suffer from partial temporal lobe damage. The above mentioned functions are fulfilled by neurons located in different layers of the temporal lobe and depending on the precise site of lesion, some KBS symptoms will be developed and others will not. Presentation of three typical symptoms is considered diagnostic for KBS and in humans, most frequently manifested symptoms are affective indifference and a tendency to explore one's surroundings with the mouth. The latter may be related with an abnormally high demand for comestibles. Impaired association of sensory input with pre-existing memories may show in form of the inability to recognize objects and persons.

Prevention

The early diagnosis of primary diseases may allow for therapy in a timely manner and may thus diminish lesions to the temporal lobes and amygdalas. Herpes simplex encephalitis, for instance, may easily be misdiagnosed [11].

Summary

Heinrich Klüver and Paul Bucy were both neuroscientists that practiced in the United States during the first half of the 20th century. They worked with rhesus monkeys and conducted experiments in order to shed light on the function of their temporal lobes. In this context, they described behavioral changes that resulted from bilateral lesion of the temporal lobes of these animals and designated the whole of these symptoms as Kluver-Bucy syndrome (KBS) [1]. In detail, they observed the following:

  • Visual agnosia. Monkeys were able to see, i.e., there were no obvious lesions to their eyes, but they were unable to recognize what they were seeing.
  • A tendency to explore their surroundings by using other senses may be explained by the lack of vision. Interestingly, monkeys principally used their mouths to do so. Eating behavior changed.
  • Affective indifference and apathy. Animals significantly reduced expression of emotions. Loss of fear was the most striking observation to this end.
  • An increased sexual urge. Masturbation and attempts to mate with inanimate objects were interpreted as an enhanced sex drive.

Today, the function of the temporal lobe is known in much more detail. For instance, this part of the brain is involved in visual processing. Visual agnosia may thus be perfectly explained by temporal lobe lesions.

The amygdala is located within the medial part of the temporal lobe and fulfills important functions in emotional assessment and association. Here, sensory impressions may be evaluated as stemming from a possibly dangerous object and protective fear is generated. Therefore, if temporal lobe injury involves the amygdala, the affective indifference previously observed in monkeys may develop. It has been proposed that the amygdala affects the sexual urge of a mammal.

In humans, a complete KBS is very rarely seen. Bilateral lesions of the temporal lobes that comprise the respective amygdalas may, however, be observed in patients suffering from systemic diseases associated with encephalitis. In practice, such patients almost exclusively suffer from herpes simplex encephalitis. Visual agnosia, affective indifference and, to a limited degree, an increased sexual urge are observed in humans. Patients also show signs of dementia, which may be ascribed to temporal lobe functions regarding memory. Seizures may be experienced. It has to be noted that the clinical presentation of these patients may be dominated by symptoms triggered by other, more severe neural lesions.

Patient Information

Kluver-Bucy syndrome (KBS) describes a behavioral disorder resulting from bilateral lesions to temporal lobe and amygdala. The latter are part of the cerebral cortex and fulfill a variety of functions comprising processing of visual and auditory sensory inputs, associating this input with pre-existing memories, connecting it with an emotional component, and creating new, long-term memory.

Causes

Any cerebral disorder causing damage to both temporal lobes and amygdalas may trigger KBS. The most common cause of this disease is an encephalitis due to infection with herpes simplex virus. But trauma, cerebral infarction, a neurodegenerative disease such as Alzheimer disease, certain metabolic disorders and brain tumors may also account for temporal lobe lesions and subsequent KBS.

Symptoms

  • Placidity and affective indifference. KBS patients are emotionally dull and may not react in an adequate manner to external stimuli. Since the connection between an object in sight and the knowledge of danger is made in the temporal lobe, damage to this brain structures renders affected individuals rather fearless.
  • Patients may show a tendency to explore their surroundings with their mouth. They may suddenly start licking at any object in view and this behavior may cause social problems. Some patients are also demonstrating an increased need to explore by using their other senses.
  • The urge to put anything in their mouth also manifests in form of eating disorders. Individuals suffering from KBS may ingest a variety of edible and non-edible objects, may gain considerable weight in short periods of time and may harm themselves by swallowing harmful substances.
  • Although KBS does not affect the eyes, patients may show psychological blindness, i.e., their eyes see, but their brain is unable to process the sensory input.
  • Hypersexuality is often shown. Patients engage in sexual behavior in public and may even commit criminal activities.

Diagnosis

Diagnosis is based on the medical history of the patient and the results of a clinical examination. Laboratory analyses of blood and cerebrospinal fluid as well as imaging diagnostics, mainly magnetic resonance imaging and computed tomography scans are applied to identify the cause of KBS. More specific examinations may follow depending on the results of the former.

Treatment

If the underlying disease has been identified, it should be accordingly treated. Prognosis of KBS improves significantly if the primary disorder can be cured.

Treatment of KBS itself may consist in administration of antidepressants and anticonvulsants.

References

Article

  1. Kluver H, Bucy PC. Psychic blindness and other symptoms following bilateral temporal lobectomy in rhesus monkeys. Am J Physiol 1937; 119:352-353
  2. Misra UK, Phadke RV, Seth PK. Kluver- Bucy syndrome: Sequelae of tubercular meningitis. Neurol India 1994; 42:29-31.
  3. Gerstenbrand F, Poewe W, Aichner F, Saltuari L. Kluver-Bucy syndrome in man: experiences with posttraumatic cases. Neurosci Biobehav Rev. 1983; 7(3):413-417.
  4. Kile SJ, Ellis WG, Olichney JM, Farias S, DeCarli C. Alzheimer abnormalities of the amygdala with Kluver-Bucy syndrome symptoms: an amygdaloid variant of Alzheimer disease. Arch Neurol. 2009; 66(1):125-129.
  5. Pradhan S, Singh MN, Pandey N. Kluver Bucy syndrome in young children. Clin Neurol Neurosurg. 1998; 100(4):254-258.
  6. Raschilas F, Wolff M, Delatour F, et al. Outcome of and prognostic factors for herpes simplex encephalitis in adult patients: results of a multicenter study. Clin Infect Dis. 2002; 35(3):254-260.
  7. Lippe S, Gonin-Flambois C, Jambaque I. The neuropsychology of the Kluver-Bucy syndrome in children. Handb Clin Neurol. 2013; 112:1285-1288.
  8. Jha S, Patel R. Kluver-Bucy syndrome -- an experience with six cases. Neurol India. 2004; 52(3):369-371.
  9. Devinsky J, Sacks O, Devinsky O. Kluver-Bucy syndrome, hypersexuality, and the law. Neurocase. 2010; 16(2):140-145.
  10. Ku BD, Yoon SS. Relapsing herpes simplex encephalitis resulting in Kluver-Bucy syndrome. Intern Med. 2011; 50(7):763-766.
  11. Jha S, Patel R, Yadav RK, Kumar V. Clinical spectrum, pitfalls in diagnosis and therapeutic implications in herpes simplex encephalitis. J Assoc Physicians India. 2004; 52:24-26.

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Last updated: 2019-07-11 21:08