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Klüver-Bucy Syndrome

NORD is very grateful to Robert C. Bransfield, MD, DLFAPA, PC, Clinical Associate Professor at Robert Wood Johnson UNDNJ Medical School and Associate Director of Psychiatry, Riverview Medical Center, Red Bank, New Jersey, for assistance in the preparation of this report.

Synonyms of Klüver-Bucy Syndrome

  • bilateral temporal lobe disorder

Disorder Subdivisions

  • No subdivisions found.

General Discussion

Klüver-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 idiosyncratic behaviors.

Symptoms

Major symptoms may include excessive oral tendencies with an urge to put all kinds of objects into the mouth, hypermetamorophosis (a need to explore everything), memory loss, emotional changes, extreme sexual behavior, indifference, placidity, visual distractibility and visual agnosia (difficulty identifying and processing visual information). An almost uncontrollable appetite for food may also be noted. There may also be other symptoms associated with dementia (loss of reason) as well.

Causes

Klüver-Bucy syndrome is the result of damage to the temporal lobes of the brain. This may be the result of trauma to the brain itself, or the result of other degenerative brain diseases, tumors, or it can be caused by some brain infections, most commonly herpes simplex encephalitis (a viral brain infection).

Affected Populations

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

Related Disorders

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

Pick disease, or frontotemporal dementia, is a very rare progressive neurological disease initially predominately affecting the frontal and temporal lobes of the brain. It is characterized by progressive deterioration of intellect with changes in behavior and personality. The memory is usually intact in the early stages of the disease and there is less disorientation than in Alzheimer's disease. However, in later stages there is loss of motor control as well as confusion and severe dementia. (For more information on this disorder, choose "Pick" as your search term in the Rare Disease Database.)

Alzheimer's 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's degenerate and disrupt the passage of electrochemical signals between the cells. Affected individuals become increasingly forgetful. As memory losses increase, 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's syndrome is a deficiency of vitamin B-1 that leads to cardiovascular, central and peripheral nervous system disturbances. 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. (For more information on this disorder, choose "Korsakoff" as your search term in the Rare Disease Database.)

Standard Therapies

Treatment of Klüver-Bucy syndrome is supportive and psychotropics that may be effective for some of the associated symptoms.

Investigational Therapies

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: prpl@cc.nih.gov

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

Klüver-Bucy Syndrome Resources

Organizations:

References

TEXTBOOKS
Adams RD, et al., eds. Principles of Neurology, 6th ed. New York, NY: McGraw-Hill Companies; 1997:450,517.

JOURNAL ARTICLES
Carroll BT, et al., Anatomic basis of Klüver-Bucy syndrome. J NeuropsychiatryClin Neurosci. 1999;11:116.

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

Pradhan S. et al., Klüver Bucy syndrome in young children. Clin Neurol Neurosurg. 1998;100:254-58.

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

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

INTERNET
NINDS Klüver-Bucy Syndrome Information Page
http://www.ninds.nih.gov/disorders/kluver_bucy/kluver_bucy.htm
Last updated July 2, 2008. Accessed February 9, 2012.

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

Report last updated: 2012/03/08 00:00:00 GMT+0

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