Klüver-Bucy syndrome: symptoms, causes and associated disorders.

This set of symptoms particularly affects the regulation of emotions and perception.

The correct functioning of our brain allows our organism to function properly and enables us to adapt and respond to the environment around us. Thus, we can regulate and organize our behavior, we can regulate and organize our behavior in such a way as to make it functional and allow us to satisfy our needs.

However, it is sometimes 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 discuss in this article.which we will talk about in this article.

Klüver-Bucy syndrome

The name Klüver-Bucy syndrome is given to a set of neurological symptoms associated with the destruction of certain brain areas.This disorder, which exists in both humans and apes (in fact it was discovered by Heinrich Klüver and Paul Bucy during experimentation with monkeys), has serious consequences on the daily functioning of the sufferer.

The main symptoms of this disorder are the presence of lack of fear in the face of stimuli that should generate it, absence of risk assessment, meekness and obedience, together with indiscriminate hypersexuality, hyperphagia indiscriminate hyperphagia (the subject eats excessively and with lack of control over intake, and may even ingest inedible objects and materials such as plastics), hyperorality or tendency to explore everything with the mouth, hypermetamorphosis or tendency to become overexcited before any visual stimulus and to imitate it, lack of recognition or visual agnosia and memory disturbances.

Language problems or aphasia may also occur. It is frequent that they present anger or rage, but that they express it in a placid and seemingly non-reactive way.

This set of symptoms causes a great affectation in the daily life of the sufferer, affecting his life in areas such as work, personal relationships, family or even basic life activities. or even the basic activities of daily life. It is a very limiting syndrome that requires treatment and that 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, Klüver-Bucy syndrome has been observed as a direct consequence of the bilateral removal or lesion of the amygdaloid complex and part of the temporal lobes (usually hippocampus and uncus are affected).

This destruction explains the existence of symptomatology linked to affectivity, to the emission or inhibition of emotional responses, and the management of aggressiveness and sexuality, among many others. 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 visual agnosia.

In short, it causes the set of symptoms mentioned above both due to the destruction of the area responsible for these functions and to the cessation of the flow of information that other areas need to integrate.

Disorders in which it appears

While the causes of Klüver-Bucy syndrome are bilateral brain damage in the amygdaloid complex and temporal in the amygdaloid and temporal complexThe 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. Cranio-encephalic traumatism

Depending on the area of the brain affected and the damage caused, a traumatic brain injury can lead to Klüver-Bucy syndrome.

2. Cerebrovascular accident

Cerebral hemorrhages and/or ischemia are also frequent causes of Kluver-Bucy syndrome. The destruction, drowning or compression of the neurons of the different cerebral areas, if it occurs bilaterally in the temporal and amygdaloid complex, could result in the appearance of this syndrome.

3. Herpetic encephalitis

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

4. Temporal epilepsy

Epilepsy is a disorder that can cause great affectation to the sufferer and is also one of the most frequent causes of this syndrome. The hyperactivation of the neuronal bundles can cause neuronal destruction, and the appearance of this syndrome. Although during a crisis it is possible it can appear temporarily, the more epileptic seizures the greater the risk of it becoming more permanent.

5. Dementias

Kluver Bucy syndrome frequently occurs in neurodegenerative diseases such as dementias. such as dementias. In dementias neurons degenerate, stop functioning properly and progressively die. When such affectation damages the temporalis, it is frequent that this syndrome appears.

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

6. Anoxic-ischemic encephalopathy

Anoxia or lack of oxygen at the cerebral level causes neuronal death. This can lead to Klüver-Bucy syndrome if it occurs in the aforementioned areas.

7. Meningitis

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

8. Surgical injuries

Although rare at present, Klüver-Bucy syndrome may occasionally occur as a result of injuries sustained during brain surgery. Especially when it is necessary to resect both temporal lobes.

9. Tumors

The presence of brain tumors is also a possible cause of Klüver-Bucy syndrome. It can occur in the presence of benign tumors if they are localized or compress the temporal lobes, or in the presence of infiltrative malignant tumors. It may also occur secondary to metastasis of a tumor located outside the nervous system.

Treatment

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

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

Bibliographic references:

• Ledo-Varela, M.T.; Giménez-Amaya, J.M. and Llamas, A. (2007). The human amygdaloid complex and its implication in psychiatric disorders. An. Sist. Sanit. Navar.; 30 (1): 61-74.
• Klüver, H. & Bucy, 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 Márquez-Romero, J.M. (2010). Klüver-Bucy syndrome secondary to medulloblastoma metastasis. Neurology; 25: 135-136. Mexico.