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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.
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.
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.
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).
Klüver-Bucy syndrome is a very rare disease that affects males and females equally.
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.)
Treatment of Klüver-Bucy syndrome is supportive and psychotropics that may be effective for some of the associated symptoms.
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:
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Adams RD, et al., eds. Principles of Neurology, 6th ed. New York, NY: McGraw-Hill Companies; 1997:450,517.
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.
NINDS Klüver-Bucy Syndrome Information Page
Last updated July 2, 2008. Accessed February 9, 2012.
Report last updated: 2012/03/08 00:00:00 GMT+0