Kluver Bucy Syndrome - Causes, Symptoms, and Management

What Is Meant by Kluver Bucy Syndrome?

Kluver bucy syndrome is a very rare neurological disorder, characterized by memory loss and behavioral disturbances. Sufferers from such conditions may attempt to eat non-food items, while others become intimately interested in a person of either sex. In severe cases, symptoms might include seizures and dementia. An injury to the temporal lobes of the brain causes KBS.

Damage to these areas prevents some of the most important brain functions, such as:

• Making new memories.
• Regulating behaviors related to food and sex.
• Integration of sensory input and emotion.

KBS might be associated with secondary traumatic brain injury, herpes simplex encephalitis, or other neurologic pathology. It may present at any age. There is no known cure for Kluver Bucy syndrome; however, management or mitigation of symptoms can be achieved by treatment. Early diagnosis usually provides a much better prognosis.

What Are the Alternative Names for Kluver Bucy Syndrome?

• Bilateral temporal lobe disorder.
• Post-encephalitic Kluver Bucy syndrome.
• Post-traumatic Kluver Bucy syndrome.

What Causes Kluver-Bucy Syndrome?

Kluver-Bucy syndrome, in its complete adaptation, seldom occurs in humans. However, partial infliction or presentation of this syndrome is caused by massive bilateral temporal damage attributable to the following:

• Herpes simplex encephalitis.
• Head trauma.
• Subarachnoid hemorrhage, epilepsy.
• Bilateral thalamic infarct.
• Bilaterally temporal lobe resection.
• Glioblastoma.
• Shigellosis.
• Pick disease.
• Adrenoleukodystrophy.
• Hypoglycemia.
• Reye syndrome.
• Alzheimer's disease.
• Porphyrinuria.
• Anoxic-ischemic encephalopathy.
• Neurocysticercosis.
• Tuberculosis meningitis.
• Arachnoid cyst.
• Huntington chorea.
• Parkinson's disease.
• Systemic lupus erythematosus.
• Carbon monoxide intoxication.
• Leukoencephalopathy is associated with methotrexate, radiation, and stroke.

The symptoms are mainly due to

• Destruction of the temporal neocortex or the amygdala bilaterally.
• Disturbances in the temporal portions of limbic networks that connect with multiple cortical and subcortical circuits to modulate emotional behavior and affect.
• Injuries include the amygdala, uncus, hippocampus, orbitofrontal and cingulate gyri, and insular cortex.

What Are the Risk Factors for Kluver Bucy Syndrome?

The risk factors of Kluver Bucy syndrome include

• Epilepsy.
• Cerebrovascular Disease.
• Alzheimer's Disease.
• Pick's a disease or frontotemporal dementia.
• Stroke.
• Head injury or trauma.
• Herpes simplex encephalitis.

What Are the Signs and Symptoms of Kluver Bucy Syndrome?

Individuals with Kluver-Bucy syndrome display the following behavioral manifestations:

• Visual Agnosia - Inability to identify familial persons or objects.
• Hypermetamorphosis - It is excessive attention to visual stimuli with a tendency to touch every perceived stimulus.
• Oral Tendency or Hyperorality - It is a strong urge to bring objects to the mouth.
• Placidity - It is a diminution or loss of anger and fear responses, blunted affect, and obedience.
• Acts of licking and touching are unacceptable for public display.
• Demonstrating an excessive desire to engage in human sexual practices or hypersexuality.
• Engagement in binge-eating excessive, uncontrollable overeating with feelings of guilt and depression afterward or bulimia.
• Memory disorders include short-term memory loss and other serious presentations of memory-related ailments or amnesia.
• Inability to recognize people.
• Lack of fear reaction.
• Difficulty in distinguishing and identifying some objects.

What Are the Diagnostic Tests to Be Carried Out?

The diagnosis is mainly based on clinical presentations.

• Magnetic Resonance Imaging (MRI) of the Brain - It is beneficial in analyzing the extent of temporal lobe damage.
• Electroencephalogram - It is also used to identify seizures originating from the temporal lobe.

What Are the Management of Kluver Bucy Syndrome?

There is no specific treatment protocol for the condition at the moment. Most treatment focuses on providing symptomatic relief and supportive care to the patient.

The main drugs used in the management are

• Mood stabilizers.
• Antidepressants (selective serotonin reuptake inhibitors).
• Antipsychotic drugs.
• Anticonvulsant drugs.
• Carbamazepine.
• Leuprolide.

Drugs such as Carbamazepine and Leuprolide are typically administered to reduce sexual behavioral disorders. Drugs such as haloperidol and anticholinergics are useful in treating behavioral abnormalities. Antidepressants, mood stabilizers, and antipsychotic drugs are usually given to treat behavioral disorders.

The prognosis depends upon the underlying cause and the severity of the disease. The disorder is not life-threatening, but managing the patient may be difficult in certain situations. With treatment, symptoms may slowly decline.

What Is the Differential Diagnosis of Kluver Bucy Syndrome?

Symptoms of the following disorders can be similar to those of Kluver-Bucy syndrome:

• Pick Disease or Frontotemporal Dementia - It is a sporadic progressive neurological disease that initially predominantly affects the temporal and frontal lobes of the brain. It is characterized by deterioration of intellect and altered behavior and personality. However, memory is usually intact in the early stages of the disease, and there is less disorientation than in Alzheimer’s disease.
• Alzheimer’s Disease - It is a progressive brain disorder that affects thoughts, memory, and language. Bundles of nerve endings degenerate in the cortex of the brain and disrupt the passage of electrochemical signals between the cells. Affected individuals gradually become forgetful. As memory loss increases, altered personality, mood, and behavior are also seen.
• Korsakoff’s Syndrome - A vitamin B1 deficiency leads to disturbances in the central and peripheral nervous systems. The most common symptoms of Korsakoff’s syndrome include fatigue, irritation, difficulty sleeping, poor memory, chest pain, abdominal discomfort, anorexia, and constipation. Later, the symptoms are mainly neurological and cardiovascular.
• Prader-Willi Syndrome - It is a rare, autosomal dominant disorder. It usually affects all races and both sexes. It is characterized by poor muscle tone, short stature with small hands and feet, poor growth, and delayed development.
• Kleine Levin Syndrome - It is a rare sleep disorder characterized by intermittent insomnolence, behavioral and cognitive dysfunction, hyperphagia, and hypersexuality.

What Are the Complications of Kluver Bucy Syndrome?

Hyperorality and hypermetamorphosis can make the patient put everything in his/her mouth, which gives way to huge risks. Hypersexuality leads to sexual activities by the patient towards strangers as well. Without proper knowledge of the condition, such activities can lead to legal consequences. Bulimia can also cause weight gain, electrolyte imbalances, and bad oral hygiene.

What Is the Prognosis for Kluver Bucy Syndrome?

At least some features of KBS, including hyperorality, placidity, and hypermetamorphosis, may continue to exist until death; many, however, gradually improve over several years. The clinical course of KBS varies widely between cases. In cases where KBS starts as a result of epileptic spasms-induced seizures, infection, or traumatic head injuries, the prospects for recovery are more favorable because most of the associated destructions can be reversed if they are diagnosed and treated in time.

Conclusion:

Kluver-Bucy syndrome is a rare neuropsychiatric disorder associated with damage to both anterior temporal lobes of the brain, especially the hippocampus, and amygdala, resulting in social and sexual dysfunction, memory loss, and unknown behaviors. The disease is usually associated with herpes simplex encephalitis, head trauma, degenerative brain disorders like Pick’s disease and Alzheimer’s disease or cerebral vascular diseases, and brain tumors. Patients with epilepsy should be closely followed for Kluver Bucy syndrome, especially those with hypersexuality, hallucinations, hypermetamorphosis, increased appetite, memory disturbance, speech disturbance, hyperactivity, agitation, and aggressiveness. Overall, the prognosis of the syndrome depends mainly on the underlying pathology.