Klüver-Bucy Syndrome: A Rare Neurobehavioral Puzzle
Overview
Klüver-Bucy syndrome (KBS) is a rare neurobehavioral disorder characterized by a combination of symptoms including hyperorality (putting objects in the mouth), hypersexuality, visual agnosia (inability to recognize objects), placidity, hypermetamorphosis (excessive visual attentiveness), and changes in dietary behaviour [1,2].
Although the syndrome appears usually in adults, there have been cases reporting children suffering from the syndrome as well.
The prevalence of the full Klüver-Bucy syndrome is not well-documented in scientific studies. However, researchers have documented some particular symptoms of the syndrome in particular populations.
For example, a study by Janszky et. al found that periicital hyperorality, a symptom of KBS, was present in 3% of the investigated epilepsy population, which consisted of 269 patients aged 6-59.
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Symptoms of the Klüver-Bucy Syndrome
The Klüver-Bucy syndrome is usually part of more complex neurological disorders resulting in impaired behaviour such as dementia or seizures [1,2]. The symptoms of the syndrome can vary but typically include:
Hyperorality
Hyperorality refers to a condition characterized by an excessive and abnormal focus on oral activities, such as eating, drinking, and putting objects in the mouth.
Hyperorality can manifest as an increased appetite, preference for sweet foods, stereotypical eating behaviors, and a tendency to put non-food items in the mouth.
It is believed to result from dysfunction in the brain regions involved in the regulation of appetite, reward, and impulse control.
Apart from they KBS, it can be seen in frontotemporal dementia or other neuropsychiatric disorders [3].
Changes in eating behavior
Another manifestation of the Klüver-Bucy syndrome is the change in one's eating behaviour. People with this syndrome change their dietary habits. They showcase an increased appetite or preference for certain foods.
Placidity
Placidity refers to a state of calmness, tranquillity, or lack of emotional reactivity.
It can be seen in various neurological and psychiatric conditions, such as the Kluver-Bucy syndrome [4].
Hypermetamorphosis
Hypermetamorphosis refers to excessive or abnormal attentiveness to visual stimuli.
It is one of the characteristic symptoms of the KBS [5].
Visual agnosia
Visual agnosia is a condition characterized by impaired object recognition despite normal vision.
There are different types of visual agnosia. Apperceptive agnosia involves deficits in early perceptual processing, while associative agnosia involves a failure in recognition despite intact perception [6].
Altered sexual behavior
Individuals suffering the KBS, show an increase in their sexual needs, which we can refer to as hypersexuality.
Interestingly, hypersexuality has been observed in children with no previous sexual experiences. In those, it manifested as repeated holding of genitals, as well as rubbing of the genitals to inanimate objects [7].
Amnesia
Amnesia is a condition characterized by memory loss or the inability to remember past events or form new memories.
Different types of amnesia exist, including retrograde amnesia (difficulty remembering past events) and anterograde amnesia (difficulty forming new memories).
It can be caused by various factors such as brain injury, stroke, certain medical conditions, or psychological trauma [8].
Aphasia
Aphasia is a language disorder that can make it difficult for individuals to read, write, speak, and understand language.
It is caused by damage to the parts of the brain that control language.
The symptoms of aphasia can vary depending on the location and severity of the brain damage.
Treatment for aphasia may include speech-language therapy and other forms of communication support [9,10].
It's important to note that not all individuals with KBS will experience all of these symptoms, and the severity and combination of symptoms can vary[11].
What Causes the Klüver-Bucy Syndrome?
KBS can occur as a result of various neurological conditions such as head trauma, encephalitis, cerebrovascular disease, and certain genetic disorders. Usually, it is associated with bilateral lesions or dysfunction of the temporal lobes, specifically the amygdala.
The amygdala is a brain structure located in the temporal lobe responsible for various functions but most notably stress regulation, emotional regulation and moral decision-making [12,13].
Interestingly this syndrome has been detected in children as well usually in association with acute bitemporal injury or dysfunction [14].
Treatment
The treatment of KBS is challenging. There is no specific cure for the Klüver-Bucy syndrome, rather multiple different approaches in treating the symptom have been proposed.
Pharmacological interventions include antipsychotics, serotonin uptake inhibitors and the prescription of carbamazepine has shown to be effective in some cases [15,16].
Further, behavioural interventions have also been proposed in order to treat KBS. These include therapies for managing hypersexuality, dietary changes and improving social and emotional skills[15].
However, research in this area is need to better understand this rare syndrome.
Disclaimer
This article does not offer health advice. Always consult a medical professional regarding your condition.