Abstract
The Kluver-Bucy Syndrome, first described in monkeys following bilateral temporal lobectomy, has rarely been described in adults following head trauma, infections, and tumors. No cases have been reported in children. An 8 year old developed diabetic ketoacidosis and stupor. Although medical management of ketoacidosis was uncomplicated, she developed hyperphagia, hyper-sexuality, hypermetamorphosis, visual agnosia, dull affect, rage alternating with apathy, and a mild right hemiparesis. Cerebrospinal fluid and serum analysis suggested an acute adenovirus infection (titer rise 1:16 to 1:64). CT initially showed striking enhancement of both mesial temporal lobes, the olfactory and orbital-frontal regions, and the thai amic-hypothalamic regions. Repeat CT at three years revealed atrophy and cavitation in these regions. Psychometric testing at three years revealed above average intelligence, severe visual attentive difficulties, a marked deterioration of cognitiive control of emotions, and thought processes suggestive of psychosis. Numerous trials of psychopharmacology have failed to alter the girl's behavior. This case resembles adults with Kluver-Bucy Syndrome, and is the first reported case in childhood of acquired limbic dementia with neuroradiographic and psychometric correlation.
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Woody, R., Blaw, M. & Talbot, A. 1718 THE KLUVER-BUCY SYNDROME IN A CHILD: NEURORADIOLOGIC AND PSYCHOMETRIC CORRELATES AND LONGTERM FOLLOMUP. Pediatr Res 19, 397 (1985). https://doi.org/10.1203/00006450-198504000-01736
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DOI: https://doi.org/10.1203/00006450-198504000-01736