Skip to main content

Klüver-Bucy syndrome

Medical Professionals

Professional Reference articles are designed for health professionals to use. They are written by UK doctors and based on research evidence, UK and European Guidelines. You may find one of our health articles more useful.

Continue reading below

What is Klüver-Bucy syndrome?1

Klüver-Bucy syndrome is a neuro-behavioural syndrome associated with bilateral lesions in the anterior temporal horn or amygdala. Heinrich Klüver and Paul Bucy first described the syndrome in 1937 after experimental work where they removed rhesus monkeys' temporal lobes.2 They found that the monkeys developed:

  • Visual agnosia - they could see, but were unable to recognise familiar objects or their use.

  • Oral tendencies - they would examine their surroundings with their mouths instead of their eyes.

  • Hypermetamorphosis - a desire to explore everything.

  • Emotional changes - emotion was dulled and facial movements and vocalisations were far less expressive. They lost fear where it would normally occur. Even after being attacked by a snake, they would casually approach it again. This was called 'placidity'.

  • Hypersexuality - a dramatic increase in overt sexual behaviour, including masturbation. They may even attempt copulation with inanimate objects.

The syndrome in humans is due to bilateral destruction of the amygdaloid body and inferior temporal cortex, most commonly due to herpes simplex encephalitis (HSE). It shares visual agnosia and loss of normal fear and anger responses in common with the monkey model but one also sees loss of memory with dementia, distractibility and seizures. The hypersexuality tends to be less overt than in the monkeys but may be public and unacceptable.

How common is Klüver-Bucy syndrome? (Epidemiology)3

It is a very rare disorder and most of the literature relates to animal models rather than human cases:

  • Most literature relating to humans is isolated case reports and few papers report more than a small number of cases.

  • It is likely to become more common as a consequence of greater survival following HSE, as antiviral agents improve and are readily available.

Risk factors

The most common cause is HSE but it has also been associated with other infections such as:

Other causes include:

It has occasionally been described in children.8

Continue reading below

Clinical features

NB: we rarely, if ever, see the full syndrome in humans.

In adults

  • Emotional blunting: there is a flat affect and poor response to emotional stimuli (placidity).

  • Hyperphagia: there is a strong compulsion to place objects in the mouth, probably to gain oral stimulation and to explore the object to counteract the visual agnosia, rather than due to hunger. Nevertheless, there is bulimia and there will be marked weight gain unless diet is restricted. Actions may include socially inappropriate licking or touching.

  • Visual agnosia: there is an inability to recognise objects or faces visually. This is also called 'psychic blindness' and may account for the oral compulsion.

  • Increased sexual behaviour: individuals with Klüver-Bucy syndrome lack social sexual restraint with profuse and inappropriate sexual activity.

In children8

  • It usually follows HSE and develops on regaining consciousness and activity.

  • Altered emotional behaviour, changes in dietary habits, hyperorality and hypersexuality have been reported as present in all, while psychic blindness and hypermetamorphosis occurred in only a few.

  • Marked indifference and lack of emotional attachment towards their family.

  • Apathy and easy distractibility are rare.

  • Bulimia and a strong urge to put items other than food into the mouth are common.

  • Hypersexuality presents as frequent holding of genitals, intermittent pelvic thrusting movements and rubbing of genitals to the bed on lying prone. Usually sexually inappropriate behaviour in children is taken as indicative of sexual abuse. There was no suggestion reported that they had been abused and it is thought that their ignorance of sex led to a different pattern from adults.

Diagnosis3

KBS is diagnosed clinically by the presence of its characteristic symptoms. Brain MRI is used to confirm the diagnosis by demonstrating bilateral temporal lobe mutilation. Other investigations will be directed at establishing the underlying cause.

Continue reading below

Differential diagnosis

The differential diagnosis usually relates to pinpointing the actual site of the lesion(s) and to cause.9 Where psychiatric symptoms are predominant, the presence of Klüver-Bucy syndrome suggests a primarily organic cause.

Management of Klüver-Bucy syndrome

General points

  • Patients need careful monitoring to prevent bulimia and consequent obesity but also to prevent uninhibited and inappropriate sexual activity, which has been reported as leading to criminal conviction for at least one patient.10

  • Sudden behavioural or emotional changes after HSE treatment may be indicative of a relapse and should prompt a longer course of aciclovir.11

Pharmacological

Selective serotonin reuptake inhibitors (SSRIs) have been shown to be of value but carbamazepine may be better.8

Prognosis

Cognitive and behavioural disturbances after HSE are often severe but improvement can occur over a long time and residual disabilities vary from major to fairly mild.12 13 The loss of memory is consistent with the hypothesis that medial temporal lobe structures mediate memory consolidation.

Prevention of Klüver-Bucy syndrome

A paper from India concluded that HSE is often misdiagnosed, leading to late treatment.14 Important factors influencing mortality and morbidity are early aciclovir therapy, age, the immune status of the patient, duration of illness and consciousness level before initiation of therapy.

Further reading and references

  • Klüver-Bucy Syndrome; National Institute of Neurological Disorders and Stroke
  • M Das J, Siddiqui W; Kluver Bucy Syndrome. StatPearls, Jan 2023.
  • Kar SK, Das A, Pandey S, et al; Kluver-Bucy Syndrome in an Adolescent Girl: A Sequel of Encephalitis. J Pediatr Neurosci. 2018 Oct-Dec;13(4):523-524. doi: 10.4103/JPN.JPN_70_18.
  • Cho AR, Lim YH, Chung SH, et al; Bilateral Anterior Opercular Syndrome With Partial Kluver-Bucy Syndrome in a Stroke Patient: A Case Report. Ann Rehabil Med. 2016 Jun;40(3):540-4. doi: 10.5535/arm.2016.40.3.540. Epub 2016 Jun 29.
  1. Costa R, Fontes J, Mendes T, et al; Kluver-Bucy Syndrome: A Rare Complication of Herpes Simplex Encephalitis. Eur J Case Rep Intern Med. 2021 Jul 22;8(7):002725. doi: 10.12890/2021_002725. eCollection 2021.
  2. Kluver H, Bucy PC; Psychic blindness and other symptoms following bilateral temporal lobectomy in rhesus monkeys. Am J Physiol 1937;119:352-3
  3. Al-Attas AA, Aldayel AY, Aloufi TH, et al; Kluver-Bucy syndrome secondary to a nondominant middle cerebral artery ischemic stroke: a case report and review of the literature. J Med Case Rep. 2021 Jul 15;15(1):346. doi: 10.1186/s13256-021-02932-0.
  4. Cohen MJ, Park YD, Kim H, et al; Long-term neuropsychological follow-up of a child with Kluver-Bucy syndrome. Epilepsy Behav. 2010 Dec;19(4):643-6.
  5. Leesch W, Fischer I, Staudinger R, et al; Primary cerebral Whipple disease presenting as Kluver-Bucy syndrome. Arch Neurol. 2009 Jan;66(1):130-1.
  6. Kile SJ, Ellis WG, Olichney JM, et al; Alzheimer abnormalities of the amygdala with Kluver-Bucy syndrome symptoms: an amygdaloid variant of Alzheimer disease. Arch Neurol. 2009 Jan;66(1):125-9.
  7. Naito K, Hashimoto T, Ikeda S; Kluver-Bucy syndrome following status epilepticus associated with hepatic encephalopathy. Epilepsy Behav. 2008 Feb;12(2):337-9. Epub 2007 Nov 5.
  8. Jha S, Patel R; Kluver-Bucy syndrome -- an experience with six cases. Neurol India. 2004 Sep;52(3):369-71.
  9. Lippe S, Gonin-Flambois C, Jambaque I; The neuropsychology of the Kluver-Bucy syndrome in children. Handb Clin Neurol. 2013;112:1285-8. doi: 10.1016/B978-0-444-52910-7.00051-9.
  10. Devinsky J, Sacks O, Devinsky O; Kluver-Bucy syndrome, hypersexuality, and the law. Neurocase. 2010 Apr;16(2):140-5. Epub 2009 Nov 18.
  11. D Ku B, Sang Yoon S; Relapsing herpes simplex encephalitis resulting in kluver-bucy syndrome. Intern Med. 2011;50(7):763-6. Epub 2011 Apr 1.
  12. Pascual-Castroviejo I, Pascual-Pascual SI, Viano J; Kluver-Bucy syndrome. Seven year follow-up of one patient. Neurologia. 2008 Mar;23(2):114-8.
  13. 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 Aug 1;35(3):254-60. Epub 2002 Jul 10.
  14. Jha S, Patel R, Yadav RK, et al; Clinical spectrum, pitfalls in diagnosis and therapeutic implications in herpes simplex encephalitis. J Assoc Physicians India. 2004 Jan;52:24-6.

Article history

The information on this page is written and peer reviewed by qualified clinicians.

flu eligibility checker

Are you protected against flu?

See if you are eligible for a free NHS flu jab today.

symptom checker

Feeling unwell?

Assess your symptoms online for free