Kluver Bucy Syndrome - Causes, Symptoms, and Management

Introduction:

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 dysfunctioning, memory dysfunction and unknown behaviors. The syndrome was first described by Kluver and Bucy, who described it as a neurobehavioral syndrome in rhesus monkeys.

The syndrome affects males and females equally. 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. It is characterized by increased appetite, hypersexuality, hypermetamorphosis, memory disorders, visual agnosia, stagnancy, aphasia, bulimia, polyuria, and polydipsia. It is also known as bilateral temporal lobe disorder or post-encephalitic 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 of 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 present at the moment for the condition. 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 of the disease depends upon the underlying cause and the severity of the disease. The disorder is not life-threatening, but managing the patients 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:

1. Pick Disease, or Frontotemporal Dementia - It is a very rare progressive neurological disease initially predominantly affecting the temporal and frontal lobes of the brain. It is characterized by deterioration of intellect with alterated 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.

2. Alzheimer’s Disease - It is a progressive disorder of the brain affecting thoughts, memory and language. Bundles of nerve endings degenerate in the cortex of the brains and disrupt the passage of electrochemical signals between the cells. Affected individuals become gradually forgetful. As there is an increased memory losses, altered personality, mood and behavior also seen.

3. Korsakoff’s Syndrome - A deficiency of vitamin B1 leads to disturbances in the central and peripheral nervous system. 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.

4. 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.

5. Kleine Levin Syndrome - It is a rare sleep disorder characterized by intermittent insomnolence, behavioral and cognitive dysfunction, hyperphagia, and hypersexuality.

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 dysfunctioning, 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 largely on the underlying pathology.