$Unique_ID{BRK03908} $Pretitle{} $Title{Kluver-Bucy Syndrome} $Subject{Kluver-Bucy Syndrome Bilateral Temporal Lobe Disorder Pick's Disease Alzheimer's Disease Korsakoff's Syndrome } $Volume{} $Log{} Copyright (C) 1992 National Organization for Rare Disorders, Inc. 872: Kluver-Bucy Syndrome ** IMPORTANT ** It is possible that the main title of the article (Kluver-Bucy Syndrome) is not the name you expected. Please check the SYNONYM listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Bilateral Temporal Lobe Disorder Information on the following diseases can be found in the Related Disorders section of this report: Pick's Disease Alzheimer's Disease Korsakoff's Syndrome General Discussion ** REMINDER ** The Information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. Kluver-Bucy Syndrome is a very rare cerebral neurological (brain disorder. Major symptoms may include an urge to put all kinds of objects into the mouth, memory loss, extreme sexual behavior, placidity, and visual distractibility. Symptoms Kluver-Bucy Syndrome is characterized by loss of memory. There may also be indiscriminate sexual behavior. However, other emotional behavior in response to stimuli is usually lacking (indifference or placidity). An almost uncontrollable appetite for food is also noted. There may also be other forms of dementia (loss of reason) as well. There is an excessive tendency to put all sorts of objects in the mouth, and easy extractability by external stimuli (particularly visual) is usually present. Causes Kluver-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, or it can be caused by some forms of herpes simplex encephalitis (a viral brain infection). Affected Population Kluver-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 Kluver-Bucy Syndrome. Comparisons may be useful for a differential diagnosis: Pick's Disease is a very rare progressive neurological disease affecting the lobes of the brain. Major symptoms may include changes in intellect, behavior and personality. 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 degenerates and disrupts 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. Judgement, 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 which causes 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). Therapies: Standard Treatment of Kluver-Bucy is symptomatic and supportive. Therapies: Investigational This disease entry is based upon medical information available through April 1992. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Kluver-Bucy Syndrome, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 NIH/National Institute of Neurological Disorders & Stroke (NINDS) 9000 Rockville Pike Bethesda, MD 20892 (301) 496-5751 (800) 352-9424 Alzheimer's Disease and Related Disorders Association, Inc. National Headquarters 919 N. Michigan Ave., Suite 1000 Chicago, IL 60611 (312) 335-8700 (800) 272-3900 References CECIL TEXTBOOK OF MEDICINE, 19th Ed.: James B. Wyngaarden, and Lloyd H. Smith, Jr., Editors; W.B. Saunders Co., 1990. Pp. 2080-2087. PRINCIPLES OF NEUROLOGY, 4th Ed.; Raymond D. Adams, M.D. and Maurice Victor, M.D., Editors; McGraw-Hill Information Services Company, 1989. Pp. 360-363, 451-452. THE MERCK MANUAL OF GERIATRICS, 1st Ed.; William B. Abrams, M.D. and Robert Berkow, M.D., Editors; Merck Sharp & Dohme Research Laboratories, Rahway, NJ, 1990. Pp. 946. KLUVER-BUCY SYNDROME WITH SEVERE AMNESIA SECONDARY TO HERPES ENCEPHALITIS, Conlon, P. et al.; Can J Psychiatry, November, 1988, (issue 33, (8)). Pp. 754-756. KLUVER-BUCY SYNDROME. A CASE REPORT., Fragassi, NA, et al.; Acta Neurol, April, 1990, (issue 12 (2)). Pp. 138-142.