Researchers at Medical College of Georgia, Augusta, GA, report a 10-year-old girl with a history of Listeria monocytogenes meningoencephalitis, contracted at 2½ years of age, who developed behavioral changes consistent with Kluver-Bucy syndrome at 2 months following discharge. Following a week of fever, vomiting, and diarrhea with dehydration, she developed status epilepticus. A postinfectious communicating hydrocephalus was treated with ventriculoperitoneal shunt. Two months after discharge, she gradually exhibited a behavioral change characterized by aggression toward family members and pets, uncontrolled screaming and crying, inability to sustain attention span, hyperactivity, and decreased language output. Persistent behavior problems were treated with clonidine and Concerta, and were complicated at 4 and 1/2 years of age by hypersexuality (frequent masturbation), hyperorality, and significant weight gain. MRI at age 4 showed diffuse brain atrophy with maximal temporal lobe involvement and at 9 years, decreased hippocampal volume bilaterally. Complex partial seizures at 6 years of age were partially controlled with carbamazepine and lamotrigine. Except for tactile exploratory behavior and clinging to the examiner, the neurological exam was normal. EEG showed focal slowing bilaterally with epileptiform discharges over right and left midtemporal regions. Serial neuropsychological evaluations at 5, 7, and 10 years of age demonstrated expressive and receptive language impairment and an amnestic syndrome. She had poor working memory and verbal learning performance and was unable to make new declarative memories. Her reading, spelling, and arithmetic performance decreased from low average/average to borderline at age 10 years. Oral reading fluency, comprehension, and written expression were in deficient range. [1]

COMMENT. Kluver and Bucy at the University of Illinois, Chicago, in 1937 and 1938, described a “psychic blindness” and other symptoms following bilateral temporal lobectomy in rhesus monkeys. Terzian et al reported the first case of KB syndrome in man in 1955, following bilateral removal of temporal lobes for intractable epilepsy. In adults, the characteristic symptoms of KB syndrome include 1) visual agnosia, inability to recognize objects without loss of visual discrimination; 2) hyperorality, urge to examine objects by mouth; 3) hypermetamorphosis, excessive attentiveness/touching of visual stimuli; 4) placidity, loss of normal fear or anger; 5) hypersexuality; and 6) changes in dietary habits. Also, aphasia, memory loss, and seizures occur in human cases. The syndrome is rare, only 27 pediatric cases reported, and is usually incomplete. Hyperorality and hypersexuality are most frequent features, followed by bulimia. Causes in children are various, including herpes simplex encephalitis, HIE, traumatic brain injury, neurocysticercosis, mycoplasma infection, bilateral arachnoid cysts, and encephalopathy.