Complete Klüver-Bucy Syndrome in Man

Abstract

A 22 year old right-handed man suffered a viral meningoencephalitis, possibly herpetic, resulting in bilateral damage to the temporal lobes as confirmed by appropriate clinical, electrophysiological and neuroradiological studies. Extended clinical neuropsychological evaluation documented all the characteristic features of the syndrome described by Klüver and Bucy following bilateral ablation of the temporal lobes in adult Rhesus monkeys, including "psychic blindness," oral exploration, hypermetamorphic impulse to action," lack of emotional responsiveness, aberrant sexual behavior, and an insatiable appetite. Additionally, a severe Wernicke's aphasia and a profound memory disorder were evident. The significance of these features as regards limbic function in the human is discussed.

Klüver–Bucy syndrome associated with a recessive variant in HGSNAT in two siblings with Mucopolysaccharidosis type IIIC (Sanfilippo C)

“…In adults KBS may result from amyotrophic lateral sclerosis, 11 bilateral temporal lobectomy, 2 adrenoleukodystrophy, 12 Alzheimer`s disease, 13 herpes encephalitis, 14 and rapid correction of hyponatremia. 2 In children KBS has been observed after herpes encephalitis, 15 hypoxic insult, 16 in neuronal ceroid-lipofuscinosis, 17 MPSIIIA, 4 MPSIIIB, 5 and as a result of congenital bilateral anterior temporal malformation of the amygdalar-hippocampal complex.…”

The Neuropsychology and Neurology of Sexual Deviance: A Review and Pilot Study

“…Vascular lesions affecting the temporal lobes (Monga et al 1986) and damage of the anterior temporal lobes inducing the human Klüver-Bucy syndrome might also lead to deviant sexual behavior and hypersexuality (e.g. Marlowe et al 1975;Ghika-Schmid et al 1995). Hypersexuality and paraphilias have also been observed in conjunction with damage to subcortical structures such as the thalamus or hypothalamus (Cummings and Mendez 1984;Miller et al 1986;Absher et al 2000), the septum (Miller et al 1986;Gorman and Cummings 1992), the hippocampus, (Mendez et al 2000;Casanova et al 2002), and the basal ganglia (globus pallidus; Mendez et al 2004; striatum in Huntington disease; e.g.…”

Emotion, olfaction, and the human amygdala: Amygdala activation during aversive olfactory stimulation

“…The observation that electrical stimulation and seizures focused on the human amygdala frequently produce fear or other emotional responses provides strong evidence implicating the amygdala in emotional processing in humans (7,8). However, lesions of the amygdala in humans rarely produce the constellation of emotional abnormalities associated with lesions of the amygdala in nonhuman primates, except when amygdala damage occurs in conjunction with diffuse cerebral disease (9). Recently, several cases of selective amygdala lesions due to Urbach-Wiethe syndrome have been reported.…”