Klüver-Bucy syndrome (KBS) is a very rare neurological condition associated with damage to the brain’s temporal lobes. It can cause a number of behavioral symptoms, such as hyperorality and hypersexuality.
KBS may occur after a person experiences a traumatic brain injury. It may also occur secondary to a few other conditions, including:
- Alzheimer’s disease
- Parkinson’s disease
- stroke
- epilepsy
KBS is not life threatening but may greatly affect a person’s quality of life. It may also make day-to-day activities difficult.
In this article, we will discuss KBS in more detail, including the causes, symptoms, diagnosis, and treatment.
Definition
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KBS is a very rare condition that causes a range of behavioral symptoms. The condition affects males and females equally. Medical professionals associate KBS with damage to the temporal lobes in the brain. Several conditions can lead to temporal lobe damage, which may result in KBS.
It most commonly occurs after temporal lobe damage resulting from head injuries or stroke in adults and herpes simplex encephalitis in children.
A person with KBS may experience many behavioral symptoms. The most common symptoms are:
- memory problems
- difficulties with social and sexual functioning
- idiosyncratic behaviors
Causes and risk factors
The anterior temporal lobes sit on either side of the brain, just behind the temples. The anterior temporal lobes are part of the single temporal lobe that stretches the width of the brain. The temporal lobe houses a number of critical brain structures, including the hippocampus and the amygdala. Lesions that affect the temporal lobes, especially the hippocampus and amygdala, are the primary cause of KBS.
This damage may result from trauma to the brain itself or from degenerative brain diseases. It may also be due to tumors or brain infections. Many events and conditions can damage the temporal lobes, causing a person to develop KBS. These include:
- herpes simplex encephalitis
- stroke
- traumatic brain injuries
- central nervous system tuberculosis
- primary cerebral Whipple disease
- Alzheimer’s disease
- Pick’s disease
- hypoglycemia
- acute sporadic porphyria
- Huntington’s disease
- juvenile neuronal lipofuscinosis
- epilepsy
- Parkinson’s disease
Medical professionals still do not fully understand how KBS occurs within these conditions.
Symptoms
The symptoms of KBS, as well as their severity, can vary from person to person. The clinical symptoms of KBS are:
- Hyperorality: This is the tendency or compulsion to place objects in the mouth.
- Hypermetamorphosis: This is an excessive attentiveness to visual stimuli. A person may have a tendency to touch every visual stimulus, regardless of its history or reward value.
- Hypersexuality: This is extreme sexuality, which can cause a person to engage in inappropriate sexual behavior and lack social restraint in terms of sexuality.
- Bulimia: This is a serious mental health condition that causes a person to indulge in binge eating followed by inappropriate compensatory behavior to prevent weight gain. Inappropriate compensatory behavior can include:
- self-induced vomiting
- laxative abuse
- diuretic use
- extreme physical activity
- fasting
- Placidity: This means that a person has a flat affect and a reduced response to emotional stimuli.
- Visual agnosia: This is an impairment in recognizing visually presented objects, despite otherwise normal vision.
- Amnesia: This is a profound loss of memory. People often use the term “amnesia” to refer to several memory problems relating to a variety of medical conditions.
Medical professionals describe a person who has three or more of these clinical symptoms as having partial KBS.
Diagnosis
Diagnosis for KBS is mostly clinical. A doctor will evaluate a person’s symptoms and diagnose KBS if they are showing symptoms of the condition.
Once a doctor has diagnosed KBS, they may carry out a proper evaluation to find the underlying cause of the syndrome. This will help them decide how to effectively manage the person’s symptoms.
A doctor may use an MRI scan of the brain to identify the extent of the person’s temporal lobe damage. They may also order blood tests and cerebrospinal fluid analysis to check for a brain infection, as well as an EEG to evaluate for seizures.
Treatment options
There is currently no cure and no standard treatment for KBS. Therefore, it can be difficult to treat.
Treatment for KBS varies from person to person and often focuses on managing symptoms. A person with KBS may take certain medications to manage some symptoms of the syndrome. These can include:
- mood stabilizers
- antidepressants, particularly selective serotonin reuptake inhibitors
- antipsychotic drugs
- carbamazepine
- leuprolide
Carbamazepine and leuprolide can help reduce sexual behavioral problems.
Prognosis
KBS is not life threatening but can profoundly affect a person’s quality of life. People with KBS may have difficulty navigating daily activities.
The following symptoms of KBS can persist indefinitely:
- hyperorality
- placidity
- hypermetamorphosis
Other symptoms can gradually resolve over several years, particularly with treatment.
KBS that occurs secondary to the following conditions may have a better prognosis:
- epileptic seizures
- infections
- traumatic brain injury
This is because medical professionals may recognize the damage early and begin managing it.
Summary
KBS is a rare neurological condition that results from damage to the temporal lobes. Temporal lobe damage has many possible causes, including head injuries and stroke.
KBS causes a range of symptoms, including memory issues, problems with social and sexual functioning, and hyperorality. At present, there is no cure for KBS, but a person can treat their symptoms with certain medications. KBS is not life threatening, and some symptoms may decrease over time.
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