- Definition
- Rare neuropsychiatric syndrome resulting from bilateral lesions of the medial temporal lobes (amygdala, hippocampus)
- Etiology
- Clinical Features (Classic Triad + Additional)
- Hyperorality: Compulsive examination of objects by mouth, excessive eating (hyperphagia)
- Hypersexuality: Inappropriate sexual behavior, hypersexual drive
- Visual agnosia: Inability to recognize objects visually (psychic blindness)
- Docility/placidity: Loss of fear, emotional blunting
- Hypermetamorphosis: Compulsive attention to visual stimuli, inability to ignore objects in visual field
- Anterograde amnesia (due to hippocampal damage)
- Pathophysiology
- Bilateral amygdala destruction → loss of emotional processing and fear response
- Hippocampal damage → memory impairment
- Diagnosis
- Clinical diagnosis based on characteristic behavioral changes
- MRI brain: Bilateral temporal lobe lesions
- If HSV encephalitis suspected: CSR PCR for HSV, EEG (temporal lobe seizures)
- Treatment
- Treat underlying cause (e.g., acyclovir for HSV encephalitis)
- Symptomatic management: behavioral therapy, SSRIs for hypersexuality, carbamazepine for aggression
- Prognosis: Often irreversible if extensive bilateral damage
- USMLE Pearl
- HSV-1 encephalitis → hemorrhagic necrosis of temporal lobes → Kluver-Bucy syndrome