Kluver-Bucy Syndrome

Disease Overview

Kluver-Bucy syndrome is a very rare cerebral neurological disorder associated with damage to both temporal lobes resulting in abnormalities in memory, social and sexual functioning and unusual (idiosyncratic) behaviors.

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Synonyms

• bilateral temporal lobe disorder
• post-encephalitic Klüver-Bucy syndrome
• post-traumatic Kluver-Bucy syndrome

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Signs & Symptoms

Major symptoms may include excessive oral tendencies with hyperorality (an urge to put inappropriate objects into the mouth), hypermetamorophosis (a need to touch everything), compulsive eating, hypersexuality (extreme sexual behavior), visual agnosia (difficulty identifying objects), docility or plasticity (easily influenced), lack of normal fear, indifference, memory loss and other emotional changes. When associated with temporal frontal dementia, seizures or traumatic brain injury, there may be additional symptoms.

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Causes

Kluver-Bucy syndrome is the result of bilateral damage to the temporal lobes of the brain. This may be the result of trauma to the brain, tumors, degenerative brain diseases or some brain infections, most commonly herpes simplex encephalitis (a viral brain infection).

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Affected populations

Kluver-Bucy syndrome is a very rare disease that affects males and females equally.

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Disorders with Similar Symptoms

Symptoms of the following disorders can be similar to those of Kluver-Bucy syndrome. Comparisons may be useful for a differential diagnosis:

Frontotemporal degeneration is a rare progressive neurological disease initially predominately affecting the frontal and temporal lobes of the brain. It is characterized by an initial predominance of changes in behavior and personality without memory changes. However, in later stages there is loss of motor control as well as confusion and severe dementia. (For more information on this disorder, choose “frontotemporal degeneration” as your search term in the Rare Disease Database.)

Alzheimer disease is a common progressive disorder of the brain affecting memory, thought and language. Groups of nerve endings in the cortex of the brains of people with Alzheimer disease degenerate and disrupt the passage of electrochemical signals between the cells. Affected individuals become increasingly forgetful. As memory loss increases, personality, mood and behavior also tend to change. Judgment, concentration, speech and physical coordination may also be affected. (For more information on this disorder, choose “Alzheimer” as your search term in the Rare Disease Database.)

Korsakoff syndrome is a deficiency of vitamin B-1 that leads to cardiovascular, central and peripheral nervous system disturbances. Early symptoms of Korsakoff syndrome include fatigue, irritation, poor memory, difficulty sleeping, chest pain, abdominal discomfort, poor appetite and constipation. Later symptoms are principally cardiovascular and neurological. (For more information on this disorder, choose “Korsakoff” as your search term in the Rare Disease Database.)

Capgras syndrome is a delusional belief that family members or other significant individuals have been replaced with imposters. In these patients the emotional memory of the significant person(s) is impaired, which results in the delusional belief. Like Kluver-Bucy syndrome, Capgras syndrome is associated with temporal lobe dysfunction.

Predatory aggression is controlled by the central nucleus of the amygdala, while rivalry aggression is controlled by the medial amygdala. Damage to brain areas that inhibit aggression can result in a disinhibition of aggression.

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Standard Therapies

Treatment of Kluver-Bucy syndrome is supportive and psychotropic medications may be effective for some of the associated symptoms.

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Clinical Trials and Studies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. Government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: [email protected]

Some current clinical trials also are posted on the following page on the NORD website:
https://rarediseases.org/for-patients-and-families/information-resources/news-patient-recruitment/

For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com

For information about clinical trials conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/

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References

JOURNAL ARTICLES
Lanska DJ. The Klüver-Bucy Syndrome. Front Neurol Neurosci. 2018;41:77-89. doi: 10.1159/000475721.

Carroll BT, et al. Anatomic basis of Klüver-Bucy syndrome. J NeuropsychiatryClinNeurosci.1999 Winter;11(1):116.

Slaughter J, et al. Selective serotonin reuptake inhibitor treatment of post-traumatic Klüver-Bucy syndrome. Brain Inj.1999;13:59-62.

Hayman LA, et al. Kluver-Bucy syndrome after bilateral selective damage of amygdala and its cortical connections. J Neuropsychiatry ClinNeurosci.1998;10:354-58.

Pradhan S. et al. Kluver-Bucy syndrome in young children. ClinNeurolNeurosurg. 1998;100:254-58.

Goscinski I, et al. The Klüver-Bucy syndrome. J Neurol.1997;41:269-72.

INTERNET
M Das J, Siddiqui W. Kluver-Bucy Syndrome. [Updated 2023 Jun 26]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK544221/ Accessed Oct 25, 2023.

Klüver-Bucy Syndrome. National Institute of Neurological Disorders and Stroke. https://www.ninds.nih.gov/health-information/disorders/kluver-bucy-syndrome#:~:text=What%20is%20Kl%C3%BCver%2DBucy%20syndrome,normal%20fear%20and%20anger%20responses Accessed Oct 25, 2023.

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Programs & Resources

• Assistance Programs
• Patient Organizations
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RareCare^® Assistance Programs

NORD strives to open new assistance programs as funding allows. If we don’t have a program for you now, please continue to check back with us.

Additional Assistance Programs

MedicAlert Assistance Program

NORD and MedicAlert Foundation have teamed up on a new program to provide protection to rare disease patients in emergency situations.

Learn more https://rarediseases.org/patient-assistance-programs/medicalert-assistance-program/

Rare Disease Educational Support Program

Ensuring that patients and caregivers are armed with the tools they need to live their best lives while managing their rare condition is a vital part of NORD’s mission.

Learn more https://rarediseases.org/patient-assistance-programs/rare-disease-educational-support/

Rare Caregiver Respite Program

This first-of-its-kind assistance program is designed for caregivers of a child or adult diagnosed with a rare disorder.

Learn more https://rarediseases.org/patient-assistance-programs/caregiver-respite/

Learn more about Patient Assistance Programs >

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