Kluver Bucy Syndrome


Article Author:
Joe M Das


Article Editor:
Waquar Siddiqui


Editors In Chief:
David Wood
Andrew Wilt
Hajira Basit


Managing Editors:
Avais Raja
Orawan Chaigasame
Khalid Alsayouri
Kyle Blair
Radia Jamil
Erin Hughes
Patrick Le
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Hassam Zulfiqar
Navid Mahabadi
Hussain Sajjad
Steve Bhimji
Muhammad Hashmi
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Abbey Smiley
Sarosh Vaqar
Mark Pellegrini
James Hughes
Beenish Sohail
Hajira Basit
Phillip Hynes
Sandeep Sekhon


Updated:
6/16/2019 12:22:38 PM

Introduction

Kluver-Bucy syndrome (KBS) is a neuro-psychiatric disorder due to lesions affecting bilateral temporal lobes, especially hippocampus and amygdala.[1]

The following clinical features characterize the syndrome:

  1. Hyperorality
  2. Hypermetamorphosis (excessive attentiveness to visual stimuli with a tendency to touch every such stimulus)
  3. Hypersexuality
  4. Bulimia
  5. Placidity
  6. Visual agnosia and
  7. Amnesia.

Patients having a combination of three or more different elements listed above are described as having partial KBS.[2][3]

The clinical features of KBS were initially reported by Sanger Brown and Edward Albert Sharpey-Schäfer in 1888.[4] But the complete syndrome was described later by Heinrich Kluver (neuropsychologist) and Paul Bucy (neurosurgeon) in 1939, unaware of the previous reporting.[5] They described the behavioral syndrome which occurred in a Rhesus monkey (named Aurora) three weeks after bilateral temporal lobectomy. The first description of KBS in humans came from Dr. Terzian and Dr. Ore in 1955 in a 19-year-old man who underwent bilateral temporal lobectomy for seizures.[6] The first identified and reported case of KBS was in a 22-year-old male patient with bilateral temporal damage due to herpes simplex meningoencephalitis by Marlowe et al.[7]

Etiology

KBS is known to be associated with several pathologies. There are many case reports of several conditions (listed below) ranging from infections like shigellosis to methamphetamine withdrawal. But how KBS occurred in these conditions remains unclear.

  1. Herpes simplex encephalitis (HSE)[8]
  2. Stroke (temporal lobe infarction - usually bilateral)[9][10]
  3. Listeria meningoencephalitis[11]
  4. Traumatic brain injury[12]
  5. Central nervous system tuberculosis[2]
  6. Primary cerebral Whipple disease[13]
  7. Alzheimer disease[14]
  8. Pick disease [15]
  9. Hypoglycemia[16]
  10. Acute sporadic porphyria[17]
  11. Huntington disease[18]
  12. Juvenile neuronal lipofuscinosis[19]
  13. Toxoplasmosis
  14. Epilepsy[20]
  15. Parkinson disease
  16. Heat stroke[21]
  17. Shigellosis[22]
  18. Methamphetamine withdrawal[23]
  19. Systemic lupus erythematosus[24]
  20. Anoxic-ischemic encephalopathy[25]
  21. Neurocysticercosis[25]
  22. Non-Hodgkin lymphoma[26]
  23. Mycoplasmal bronchitis.[27]
  24. Methotrexate leukoencephalopathy[28]
  25. Subdural hygroma[29]

The most common pathologies leading to the development of KBS are head injury and stroke in adults and herpes simplex encephalitis in children.

Epidemiology

Human KBS is more or less restricted to case series and reports. Hence the exact prevalence is difficult to estimate.

Pathophysiology

The significant clinical symptoms of KBS are produced by the destruction of either the temporal neocortex or the amygdala bilaterally. The full syndrome is rarely seen in humans because the anterior temporal lobe dysfunction is usually less severe in humans when compared to that following total temporal lobe resection in monkeys.[30]

The exact anatomical basis of KBS is still controversial. KBS is thought to occur due to the disturbances in the temporal portions of limbic networks that connect with multiple cortical and subcortical circuits to modulate emotional behavior and affect.[30] A sine qua non for KBS is the involvement of medial temporal lobe regions along with bilateral lesions of the Ammon horn.[31] Even though KBS is always thought to follow bilateral malfunctions of the temporal lobes, it is important to note that the amygdala, uncus, hippocampus, orbitofrontal and cingulate gyri, and insular cortex have an important role in its pathogenesis. 

Theories regarding the etiology of KBS:

  1. Norman Geschwind theory: Interruption of visual input to limbic circuit leading to disconnection syndrome produces KBS.[32]
  2. Muller theory: Disconnection of the pathways connecting the dorsomedial thalamus with the prefrontal cortex and other limbic areas lead to KBS. These pathways are essential for memory and emotional regulation.[33]

The origin of various symptoms of KBS are explainable as follows:

  • Rage is produced by the involvement of the ventromedian nucleus of thalamus and amygdala.[34]
  • A permanent "hypersexed state," produced by discrete bilateral lesions of the lateral amygdaloid nucleus. Temporal lobe seizures may produce a transient state.
  • Visual agnosia results from bilateral ventral temporal ablations and temporal lobectomies. 

History and Physical

Clinical features:

In adults:

  1. Hyperorality - Socially inappropriate lickings and a strong compulsion to place objects inside the mouth
  2. Hypersexuality - Lack of social restraint in terms of sexuality, with inappropriate sexual activity and attempted copulation with inanimate objects
  3. Eating disorder - Objects are placed in the mouth and explored with the tongue to counteract visual agnosia. Bulimia, which is an eating disorder characterized by binge eating, followed by purging, is also markedly seen and may cause weight gain.
  4. Placidity - Flat affect and reduced response to emotional stimuli
  5. Visual agnosia (Psychic blindness) - Inability to recognize familiar objects or faces presented visually

Placidity, hyperorality, and dietary changes are the most commonly occurring symptoms in KBS.

In children:

KBS in children usually occurs secondary to HSE with classic features occurring only in a few.

  1. Marked indifference
  2. Bulimia and hyperorality
  3. Lack of emotional attachment towards the family
  4. Hypersexuality:
    • The frequent holding of genitals
    • Intermittent pelvic thrusts
    • Rubbing of genitals to the bed after lying prone

Evaluation

The diagnosis of KBS is mainly clinical. Once diagnosed, proper evaluation to find out the underlying pathology will be helpful in the overall management.

Magnetic resonance imaging of the brain is useful in identifying the extent of temporal lobe damage.

Electroencephalogram is also useful to identify seizures originating especially from the temporal lobe.

In head injury and other conditions producing a long duration of loss of consciousness, the appropriate staging of the consciousness is possible with the Modified Innsbruck Remission Scale, which includes the Kluver Bucy phase as well.[35]

Treatment / Management

The treatment of KBS can be challenging due to the facts that there is no specific treatment for the condition, and the clinical course will vary from patient to patient. Most of the treatment focuses on managing the symptoms. The main drugs used in the management are:

  1. Mood stabilizers
  2. Antidepressants (selective serotonin reuptake inhibitors)
  3. Antipsychotic drugs
  4. Carbamazepine
  5. Leuprolide

Carbamazepine and leuprolide are used to reduce the sexual behavioral abnormality, whereas haloperidol and anticholinergics are useful in treating behavioral abnormalities associated with KBS.[36]

Differential Diagnosis

KBS requires differentiation from the following conditions:

  1. Frontotemporal degeneration - Progressive deterioration of intellect associated with behavioral and personality changes.
  2. Alzheimer disease - Memory loss, personality changes
  3. Korsakoff syndrome - Poor memory, irritation, personality changes 
  4. Conditions causing hyperphagia - Prader-Willi syndrome and Kleine-Levin syndrome

Prognosis

Some KBS features (i.e., hyperorality, placidity, hypermetamorphosis) persist indefinitely, whereas others gradually resolve over several years.

The clinical course of the disease varies among the case reports.

KBS occurring secondary to epileptic seizures, infections or post-infectious, and traumatic brain injuries may have a better prognosis as many of the damages would be reversible if recognized early and managed appropriately.

Complications

Due to hyperorality and hypermetamorphosis, the patient may try to put whatever objects he comes across into his mouth, which can be dangerous.

Due to hypersexuality, he may try to engage in sex with others whom he does not even know, leading to criminal procedures against the patient there is no awareness of the diagnosis.

Bulimia can cause weight gain, electrolyte disturbance, and poor oral hygiene.

Deterrence and Patient Education

Patients' relatives should be educated about the condition and counseled that treatment may not always be successful. They should receive information that situations may arise, which require physical patient restraint. 

Enhancing Healthcare Team Outcomes

A close interaction between the treating neurologist, psychiatrist, neurosurgeon, and radiologist is necessary for coming to the final diagnosis of KBS. Careful monitoring of diet is required if they have symptoms consistent with eating disorders. Staff members, including nurses, should be cognizant about hypersexual behaviors in these patients.

The outcomes for these patients are poor; they often require medications to suppress abnormal behavior, and often, physical restraints are needed. Many end up in psychiatric institutions where they remain for life.


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Kluver Bucy Syndrome - Questions

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A 12-year-old boy is seen in the clinic exhibiting extreme docility, hyperorality, and hypersexuality. The child does not appear to recognize his parents. Which of the following is the most probable diagnosis?



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Kluver Bucy syndrome is associated with lesions of which area of the brain?



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A 7-year-old boy is brought to the clinic with excessive oral manipulation of objects, bulimia, and frequent manipulation of genitals. Which of the following is the most common cause of his underlying problem?



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A 21-year-old man is brought to the clinic for excessive oral manipulation of objects, bulimia, and frequent manipulation of genitals, two months after a severe head injury. He tries to misbehave with ladies in the public and engages in frequent masturbation. Which of the following drugs is most useful for the treatment of his condition?



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A 50-year-old man is diagnosed with Kluver Bucy syndrome following a stroke affecting bilateral middle cerebral artery territories. Which of the following symptoms is least likely to be diagnostic of his condition?



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A 16-year-old boy from India is brought to the clinic for excessive oral manipulation of objects, bulimia, and frequent manipulation of genitals. He tries to misbehave with ladies in the public and engages in frequent masturbation. He is also complaining of frequent episodes of headache for the past one month. Which of the following cranial nerves is most likely to be affected by the patient's condition?



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A 50-year-old male is diagnosed with Kluver Bucy syndrome following a stroke affecting bilateral middle cerebral artery territories. Which of the following clinical features is most likely to improve with treatment in this patient?



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Kluver Bucy Syndrome - References

References

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