Kluver Bucy syndrome entities

Clinic

• Kluver-Bucy syndrome (KBS) is a neuropsychiatric behavioral disorder that is most commonly associated with damage to both of the anterior temporal lobes of the brain.
• Most commonly it is due to infection of herpes simplex encephalitis (HSE).
• Although CT, MRI and EEG suggest the injury to the temporal region, the definitive diagnosis is only established by the finding of the viral antigen in brain. Previously viral antigen is obtained by biopsy, currently it is demonstrated by PCR technique.
• It is a very rare disorder and most of the literature relates to animal models rather than human cases.
  

Symptoms

• Docility: Exhibiting diminished fear responses or reacting with unusually low aggression. This has also been termed "placidity" or "tameness".
• Dietary changes and hyperphagia: Eating inappropriate objects (pica), or overeating, or both.
• Hyperorality: An oral tendency, or compulsion to examine objects by mouth
• Hypersexuality
• Visual agnosia: Inability to recognize familiar objects or people.

Inconsistent criteria

• Hypermetamorphosis: An irresistible impulse to notice and react to everything within sight
• Lack of emotional response, diminished emotional affect
• Amnesia, characterised by an inability to recall memories. This only occurs when the damage extends bilaterally into the hippocampus.

Pathology

Controversial:

• Klüver–Bucy syndrome is thought to occur from damage to temporal sections of the limbic networks, which connects to other structures that regulate emotional behavior.
• Norman Geschwind's theory: It is caused by disconnect syndrome (a condition of the brain where the two hemispheres develop separately or at different rates) and that the initial contributor for this is the blockage of visual input to the limbic circuit.
• Muller theory: Disconnection of pathways used for emotional regulation and memory, such as those connecting the dorsomedial thalamus to the prefrontal cortex. The medial temporal sections of the limbic system can be associated with more primitive functions such as reproduction, food, and defence. This can be seen in the symptoms of increased hypersexuality, hyperorality, and general aggression.

In humans

• Temporal lobectomy
• Acute herpes simplex encephalitis
• Stroke

Related disease

• Pick's disease
• Alzheimer's disease
• Ischemia, anoxia, Carbon monoxide poisoning
• Progressive subcortical gliosis
• Rett syndrome
• Porphyria

In children

• Herpes simplex encephalitis

Miasm

HSV