Klüver-Bucy syndrome is a very rare disease of the brain in which there is damage bilaterally to the temporal lobes of the cerebral cortex. Individuals with this syndrome often experience a number of different symptoms including issues with short-term memory, significant issues with appropriate social and sexual function, and myriad other idiosyncratic behaviors. At this time, there is no treatment available to stop or reverse the symptoms of this syndrome. Treatment is based on treating the ameliorating symptoms.
This syndrome was first described in 1937 by Heinrich Klüver (1897—1979) and Paul Bucy (1904—1992) after they performed temporal lobectomies on rhesus monkeys and looked at the postsurgical effects. Symptoms exhibited by the monkeys very closely resemble those seen in humans today including (1) docility, (2) hyperorality (the tendency to put things in the mouth to identify them), (3) visual agnosia (lack of the ability to recognize the names or functions of familiar objects), (4) disconnection of the visual system from the limbic system, and (5) hypersexuality demonstrated through an increased sex drive and inappropriate sexual behavior toward others.
The first case in humans was reported in 1975 in a 22-year-old male who had been diagnosed with bilateral temporal lobe damage after contracting herpes simplex meningoencephalitis. Other human cases have been reported in patients diagnosed with Todd’s palsy (neurocysticercosis), neurotuberculosis, temporal atrophy, cerebral atrophy, or hydrocephalus. While there is no known treatment at this time, and this disease appears to be irreversible, managing symptoms, particularly those that are behavioral in nature, appears to have some positive effects for patients.
Very little is known about the pathophysiology of this disease. Some studies reported by Jha and Patel (2004) suggest that lesions of Ammon’s horns, parts of the hippocampi, as well as the medial lobes of the temporal lobe may be contributing to the symptoms seen in this syndrome. In addition, some evidence exists that compression of the hippocampi bilaterally may play a role. But it is important to stress that at this time, there is not a clear understanding of the relationship between injury/damage to the temporal lobes and the symptoms observed with this syndrome.
There are a number of disorders that are very similar to Klüver-Bucy syndrome and are easily confused with this disease. They include Pick disease (frontotemporal dementia), which is another very rare progressive nervous system disease that affects primarily the frontal and temporal lobes. While early symptoms of this disease do not mimic Klüver-Bucy syndrome well, as the disease progresses, its symptoms more closely resemble Klüver-Bucy syndrome. Korsakoff’s syndrome is the result of a deficiency in vitamin B1. This leads to cardiovascular issues as well as problems with both the peripheral and central nervous systems. Again, the early symptoms of this disease including fatigue, irritation, memory loss, and poor appetite do not necessarily closely resemble those of Klüver-Bucy syndrome, but as the disease progresses, symptoms more readily mimic Klüver-Bucy syndrome. There are a number of other diseases that also share similarities with Klüver-Bucy syndrome including Alzheimer’s disease, Huntington’s disease, tuberculosis, meningitis, shigellosis, and acute intermittent porphyria. Care must be taken to accurately make this diagnosis.
Charles A. Ferguson
See also: Agnosia; Brain Anatomy; Limbic System; National Organization for Rare Disorders; Proprioception Deficit Disorders; Sacks, Oliver Wolf; Visual System
Deginal, Amaresh, & Siddling Changty. (2011). Post traumatic Kluver-Bucy syndrome: A case report. Indian Journal of Neurotrauma, 8(1), 41—42.
Jha, Sanjeev, & R. Patel. (2004). Klüver-Bucy syndrome—an experience with six cases. Neurology of India, 52(3), 369—371.
National Organization for Rare Disorders (NORD). (2015). Klüver-Bucy syndrome. Retrieved from http://rarediseases.org/rare-diseases/kluver-bucy-syndrome/
Weisberg, Leon A. (2002). Kluver-Bucy syndrome after minor brain injury. Southern Medical Journal, 95(8), editorial.