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Kluver-Bucy Syndrome


Kluver-Bucy Syndrome is a rare neurobehavioral disorder associated with bilateral destruction of the anterior part of the temporal lobes of the brain. This removes not only, portions of temporal cortex but also of the amygdalas that lie inside these parts of the temporal lobe.

Anatomic Basis:
Amygdala lies deep within medial temporal lobes of the brain. The centromedial amygdala projects through the stria terminalis primarily to the hypothalamus and through the ventral amygdalofugal tract to the brain stem, where it can influence hormonal and somatomotor aspects of behavior & emotional states (eating, drinking & sex).

Monkey Experiment:

kluver bucy syndrome 300x284 Kluver Bucy SyndromeHeinrich Kluver and Paul Bucy found that the bilateral removal of the temporal lobe in rhesus monkeys caused a dramatic effect on the animal’s behavior. The set of behavioral changes were:

  1. Visual agnosia (psychic blindness): Inability to recognize or interpret objects in the visual field
  2. Increased oral tendency (hyperorality): Tendency to place everything in mouth and sometimes even tries to eat solid objects
  3. Hypermetamorphosis: Extreme curiosity about everything
  4. Decreased emotional reactions: Dulled emotions and Less expressive facial movements and vocalisations. They lost fear where it would normally occur. Even after being attacked by a snake, they would casually approach it again. This was called “placidity”.
  5. Increased sexual behavior: Sex drive so strong that it attempts to copulate with immature animals, animals of the wrong sex or even animals of different species
  6. Forgets rapidly
  7. Reduced maternal behavior: Monkey mothers showed a reduction in maternal behaviors towards their infants, often physically abusing or neglecting them

Signs and Symptoms:

  1. Inability to recognize people
  2. Lack of fear reaction and rage reaction
  3. Emotional flatenning (placidity)
  4. Short term memory loss
  5. Hypersexuality (lack social sexual restraint)
  6. Hyperphagia
  7. Bulimia
  8. Weight gain (Read about complications of obesity)
  9. Socially inappropriate licking or touching
  10. Seizures

Causes:

  1. Facial or cerebral trauma
  2. Temporal lobectomy
  3. Herpes Simplex encephalitis
  4. Meningoencephalitis
  5. Niemann Pick disease of the brain
  6. Alzheimer’s disease
  7. Progressive subcortical gliosis
  8. Rett syndrome
  9. Porphyria (Read about Porphyria: A true story of Vampire)
  10. Carbon monoxide poisoning
  11. Cerebrovascular disease
  12. Hypoglycemia

Diagnosis:

  1. Neuroimaging
  2. Frontotemporal dementia
  3. MRI (Magnetic Resonance Imaging)
  4. SPECT (Single Photon Emission Computed Tomography)

Treatment:
The disease is incurable but symptomatic treatment may include drug therapy.

Case Report:

  1. Case of a 43 year old man who developed features of Kluver-Bucy Syndrome
  2. Case of a 24 year old Korean women who met with motor vehicle accident

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Last updated: June 5, 2013



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This entry was posted by on March 6, 2011 at 5:41 pm and filed under Neurology, Physiology category.

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