Klüver-Bucy syndrome: symptoms, causes and associated disorders

The proper functioning of our brain allows our body to function properly and allows us to adapt and respond to the environment that surrounds us. So, we can regulate and organize our behavior so that we make it functional and allow us to satisfy our needs.

However, sometimes it is possible to suffer serious injuries or disorders that can generate the appearance of diverse symptomatology that hinders our functioning and adaptation and that can lead us to situations of great danger. This is the case of Klüver-Bucy syndrome., which we will talk about in this article.

• You may be interested in: "The 15 most frequent neurological disorders"

Klüver-Bucy syndrome

It receives the name of Klüver-Bucy syndrome to a group of symptoms associated with the destruction of certain brain areas, existing in both humans and apes (in fact it was discovered by Heinrich Klüver and Paul Bucy during experimentation with monkeys) and with serious consequences in the daily functioning of those who use it. suffers.

The main symptoms of this disorder are the presence of a lack of fear in the face of stimuli that should generate it,

absence of risk assessment, meekness and obedience together with hypersexuality indiscriminate, hyperphagia (the subject eats excessively and lacks control over intake, and may even ingest inedible objects and materials such as plastics), hyperorality or a tendency to explore everything with the mouth, hypermetamorphosis or a tendency to be overexcited by any visual stimulus and to imitate it, lack of recognition or visual agnosia and memory disturbances.

There may also be language problems or aphasias. It is common for them to show anger or rage, but to express it in a placid and apparently unreactive way.

This set of symptoms causes a great affectation in the daily life of those who suffer from it, affecting your life in spheres such as work, personal relationships, family or even the basic activities of daily living. It is a very limiting syndrome that requires treatment and indicates the existence of lesions or some type of brain involvement.

Causes of the disorder

Unlike other disorders and syndromes, in which the causes of its appearance are not completely clear, the Klüver-Bucy syndrome has been observed as a direct consequence of extirpation or bilateral lesion of the tonsillar complex and part of the temporal lobes (the hippocampus and uncus).

This destruction explains the existence of symptoms linked to affectivity, to the emission or inhibition of emotional responses and the management of aggressiveness and sexuality, among many others. The alteration or destruction of the inferior temporal, where the visual associative areas are located, explains the frequent presence of agnosia at the visual level.

In short, it causes the set of symptoms mentioned above both by the destruction of the area in charge of said functions as well as the cessation of the flow of information that other areas need to integrate.

Disorders in which it appears

Although the causes of Klüver-Bucy syndrome are bilateral brain damage in the tonsillar and temporal complex, said lesion can appear in a large number of situations, medical conditions and disorders in which neuronal degeneration occurs. It may be useful to know some of them, highlighting especially the following.

1. Traumatic brain injury

Depending on the area of ​​the brain affected and the damage that occurs, a head injury can lead to Klüver-Bucy syndrome.

2. stroke

Cerebral hemorrhages and/or ischemia are also a frequent cause of Kluver-Bucy syndrome. The destruction, suffocation or compression of the neurons of the different brain areas, if it occurs bilaterally in the temporal and tonsillar complex, could result in the appearance of said syndrome.

3. herpetic encephalitis

Herpetic encephalitis, an infection of the nervous system caused by retrograde transmission of the herpes virus to brain tissue (frequently said infection affecting the temporal lobe), is also a cause associated with Klüver-Bucy syndrome. In fact, it is considered its most frequent cause.

4. temporary epilepsy

Epilepsy is a disorder that can cause great affectation to those who suffer from it and is also one of the most frequent causes of this syndrome. Hyperactivation of neural bundles It can cause neuronal destruction, and the appearance of this syndrome. Although during a seizure it is possible that it may appear temporarily, the more epileptic seizures, the greater the risk that it will become more permanent.

• Related article: "Epilepsy: definition, causes, diagnosis and treatment"

5. dementias

Kluver Bucy syndrome occurs frequently in neurodegenerative diseases like dementias. In dementia, neurons degenerate, stopping to function correctly and progressively dying. When said affectation damages the temporal, this syndrome frequently appears.

The most prototypical case is that of Alzheimer's disease, in which it is common to find Klüver-Bucy syndrome in phase 3 or final, in which it occurs the maximum cerebral deterioration, with the majority of diminished mental capacities (he no longer recognizes those around him or himself in front of the mirror, he becomes mute slowly and ceasing its movement, its language is profoundly altered) and there is total dependence for survival that ends with the death of the patient. It also appears in frontotemporal dementia or Pick's disease.

6. Anoxic-ischemic encephalopathy

Anoxia or lack of oxygen at the brain level causes neuronal death. What if it occurs in the areas indicated above, can lead to the appearance of Klüver-Bucy syndrome.

7. Meningitis

The meningitis or inflammation of the meninges caused by an infection is another cause of this disorder, due to chemical changes caused by infection and to the understanding of the brain by the meninges. If the brain is inflamed in addition to the meninges, the syndrome is even more likely.

8. surgical injuries

Although it is not frequent at present, Klüver-Bucy syndrome can sometimes appear as a consequence of injuries caused during brain surgery. Especially when it is necessary to carry out the resection of both temporal lobes.

9. tumors

The presence of brain tumors is also a possible cause of Klüver-Bucy syndrome. It can occur with benign tumors if the temporal bones are located or compressed, or with infiltrative malignant tumors. It is also possible that it occurs secondary to the metastasis of a tumor located outside the nervous system.

Treatment

Klüver-Bucy syndrome is a disorder caused by severe brain injury, with little recovery capacity in most cases. There is no cure for this problem. However, it is possible to establish treatments that help control the symptoms, and drugs such as benzodiazepines and anticonvulsants like carbamazepine.

In addition, some patients may show improvement if brain involvement decreases. This is what happens, for example, with some head injuries or cerebrovascular accidents (once the penumbra zone has been recovered ischemic or reabsorbed hemorrhage), in some cases of treated epilepsy or when an infection is eliminated without causing damage permanent.

Bibliographic references:

• Ledo-Varela, M.T.; Gimenez-Amaya, J.M. and Flames, A. (2007). The human amygdaloid complex and its involvement in psychiatric disorders. An. Syst. Sanit. Navar.; 30 (1): 61-74.
• Kluver, H. & Bucky, P. (1997). Preliminary analysis of functions of the temporal lobes in monkeys. 1939. J. Neuropsychiatry Clin. Neurosci. 9 (4):606-620
• Soto-Cabrera, E.; González-Aguilar, A. and Marquez-Romero, J.M. (2010). Klüver-Bucy syndrome secondary to medulloblastoma metastasis. Neurology; 25: 135-136. Mexico.