High performer to hyper former: A brief overview of Klüver–Bucy syndrome‬

While I was researching a previous blog on coprophagia (eating faeces), I came across a finding that coprophagia was prevalent in those with Klüver-Bucy Syndrome (KBS). I have to be honest and say I had never heard of KBS until that point so I thought I would investigate a little further. Back in 1937, Dr. Heinrich Klüver and Dr. Paul Bucy described an unreported behavioural syndrome in rhesus monkeys following removal of the bilateral temporal lobe in the brain, and described by Klüver himself as “the most striking and apparent alteration ever observed in consequence of surgical experiments performed on animal brains”. The surgical procedure resulted in (i) psychic blindness or visual agnosia (i.e., a deficiency in the ability to recognize visual objects), (ii) strong oral tendencies, (iii) hypermetamorphosis (i.e., an irresistible impulse to notice and react to everything within sight), (iv) decrease in aggressive behaviour, and fear reaction, and (v) hypersexuality.

The first human case was reported in 1955 by Dr. H. Terzian and Dr. G. Ore (in the journal Neurology). They carried out a bilateral removal of the temporal lobes in an adult male that resulted in KBS. The second case (although many papers I have read claim this was the first human case) – a 22-year-old man – was reported by Marlowe and colleagues in a 1975 issue of the journal Cortex. In this individual, KBS occurred following bilateral temporal lobe damage due to herpes simplex meningoencephalitis.

Since those two early cases, KBS has been associated with numerous disorders of the central nervous system. For instance, a treatment study of six KBS cases in a 2004 issue of Neurology India, Dr. Jha and Dr. Patel noted that the range of conditions associated with KBS included Alzheimer’s disease, juvenile neuronal lipofuscinosis, Huntington’s disease, herpes simplex encephalitis, toxoplasmosis, traumatic brain injury, hypoglycemia, acute intermittent porphyria, traumatic head/brain injury, tuberculous meningitis, heat stroke and Shigellosis. Other conditions may also contribute to a diagnosis of KBS including ischaemia, anoxia, progressive subcortical gliosis, Rett Syndrome, Pick’s Disease, porphyria, and carbon monoxide poisoning.

In humans, KBS is a rare behavioural impairment resulting from damage to both of the anterior temporal lobes of the brain. The disorder is not life threatening, but health practitioners can find KBS sufferers difficult to manage. The condition can be caused by either (i) bilateral temporal lobectomy, or (ii) bilateral temporal lobe damage from degenerative disorders, trauma, and encephalitis. At present, there is no known cure for KBS. Research carried out on human case studies have recorded a variety of symptoms including:

• Hyperorality: Typified by KBS sufferers compulsively examining everything by mouth
• Hypersexuality: Typified by KBS sufferers experiencing a heightened sex drive and/or seeking sexual stimulation from unusual or inappropriate items.
• Docility: Typified by KBS sufferers exhibiting low aggressive tendencies and diminished fear responses.
• Dietary changes and/or hyperphagia: Typified by KBS sufferers eating non-nutritive items or substances (i.e., pica) and/or overeating.
• Visual agnosia: Typified by KBS sufferers as the inability to recognize normally familiar objects or people.

Other types of behaviour reported in KBS sufferers include (i) amnesia (i.e., memory loss), (ii) hypermetamorphosis (as noted in rhesus monkeys above), (iii) lack of emotional response and diminished emotional affect amnesia, (iv) dementia, (v) dysphasia (i.e., inability to communicate following brain injury), and (vi) seizures. In humans, the three most common symptoms are docility, hyperorality and dietary changes.

The natural history of KBS is still unknown, but in the case of trauma, a recent paper by Dr. Amaresh Deginal and Dr. Siddling Changty in the Indian Journal of Neurotrauma (2011) reported that the course is temporary, ranging from seven days to one year. They also noted there is no specific treatment apart from oral Carbamazepine (CBZ). CBZ and leuprolides have been used to decrease the hypersexuality in KBS sufferers. Other medications (e.g., anti-cholinergics and haloperidol) have also been used in treating other behavioural consequences associated with KBS as highlighted in the paper by Jha and Patel above. However, as Dr. John Anson and Dr. Donald Kuhlman concluded:

“Klüver-Bucy syndrome remains a fascinating syndrome whose exact neuroanatomicalbasis is unclear. As neurosurgical treatment of seizure disorders increases, the consequences of mesial temporal lobectomy must be considered. Although most patients with intractable seizures improve after surgical intervention, they may develop neuro-behavioural complications such as the Kluver-Bucy syndrome. A more limited surgical resection, particularly one that spares more of the amygdala, may minimize the chance of this type-of complication”.

Dr Mark Griffiths, Professor of Gambling Studies, International Gaming Research Unit, Nottingham Trent University, Nottingham, UK

Further reading

Anson, J.A. & Kuhlman, D.T. (1993). Post-ictal Kliuver-Bucy syndrome after temporal lobectomy. Journal of Neurology, Neurosurgery and Psychiatry, 56, 311-313.

Deginal, A. & Changty, S. (2011). Post traumatic Klüver-Bucy syndrome: A case report. Indian Journal of Neurotrauma, 8, 41-42.

Jha, S. & Patel, R. (2004). Klüver-Bucy syndrome – an experience with six cases. Neurology India, 52, 369-71.

Klüver, H. &  Bucy, P.C. (1937). Psychic blindness and other symptoms following bilateral temporal lobectomy in rhesus monkeys. American Journal of Physiology, 119, 352-353.

Kwiatkowski, S., Starowicz, A., Milczarek, O. & Kawecki, Z. (2011). Neuropsychological characteristic of post-traumatic Klüver-Bucy Syndrome. Archives of Psychiatry and Psychotherapy, 4, 59-65

Lilly, R., Cummings, J.L., Benson, F. & Frankel, M. (1983). The human Klüver‐Bucy syndrome, Neurology, 33, 1141.

Marlowe, W.B., Mancall, E.L. & Thomas J.J. (1975). Complete Klüver-Bucy syndrome in man. Cortex, 11, 53-59.

Ozawa, H., Sasaki, M., Sugai, K., et al. (1997). Single-Photon Emission CT and MR findings in Klüver-Bucy syndrome after Reye syndrome. American Journal of Neuroradiology, 18, 540-42.

Stewart, J.T. (1985). Carbamazepine treatment of patient with Klüver-Bucy syndrome. Journal of Clinical Psychiatry, 46, 496-497.

Terzian, H. & Ore, G.D. (1955) Syndrome of Kluver and Bucy. Reproduced in man by bilateral removal of the temporal lobes. Neurology, 5, 373-80.