Kluver bucy syndrome is a rare behavioral impairment characterized by inappropriate sexual behaviors and mouthing of objects. The syndrome is characterized by complex behaviors, including. Essa doenca foi determinada pela primeira vez em 1939 pelo estadunidense paul clancy bucy e pelo alemao heinrich kluver. We report three additional pediatric patients who developed kluverbucy syndrome following hypoxic insults. Kluver bucy syndrome an experience with six cases s. Several symptoms responded dramatically to carbamazepine. The syndrome has so far been noted only in adults after bilateral temporal lobe affection. Only four children have been reported previously with this syndrome. In clinical neurology for psychiatrists sixth edition, 2007. Kluverbucy syndrome kbs is a rare neuropsychiatric disorder that can occur following traumatic brain injury tbi. Kluver bucy syndrome an overview sciencedirect topics. Pdf kluverbucy syndrome kbs leads to important behavioral symptoms and social maladaptation. The first case of kluver bucy syndrome kbs was reported in a 22yearold male patient with bilateral temporal damage due to herpes simplex meningoencephalitis.
They developed kluverbucy syndrome rarity in human pathology combined with three or more of the following symptoms and signs. Kluver bucy syndrome genetic and rare diseases information. Extended clinical neuropsychological evaluation documented all the characteristic features of the syndrome described by kluver and bucy following bilateral. Ignacio pascual castroviejo, samuel ignacio pascual pascual, j. Kluverbucy syndrome may present with compulsive eating, hypersexuality, insertion of inappropriate objects in the mouth hyperorality, visual agnosia, and docility.
Bucy syndrome, a famous and quite dramatic disorder, represents the closest example of limbic system injury that may occur in humans. Office document 307 kb available from felix larocca. Apr 11, 2016 kluver bucy syndrome is a rare behavioral impairment characterized by inappropriate sexual behaviors and mouthing of objects. In two patients, features of the syndrome were transient. Kluverbucy syndrome following traumatic brain injury.
Url consultato l11 dicembre 2007 archiviato dallurl originale il 26 settembre 2009. The kluverbucy syndrome kbs is a neurobehavioral syndrome and can be seen in association with a variety of neurological disorders. For the first time, kluver bucy syndrome kbs is described in young children who. The syndrome was first described in 1937 as an experimental neurobehavioral syndrome in monkeys with bitemporal brain lesions 1. Total score ranges from 0 to 3,600 being 0 the worst and 3,600 the best. Kluverbucy syndrome in children pediatric neurology. The syndrome is characterized by psychic blindness inability to recognize familiar objects, hypermetamorphosis strong tendency to react to visual stimulus, increased oral exploration, placidity, indiscriminate hypersexuality and. Kluverbucy syndrome is an uncommon syndrome of behavioral abnormalities following bilateral temporal lobe injury. Kluverbucy and it was this disease which was dominating the patients behavior. Patel department of neurology, sanjay gandhi post graduate institute of medical sciences, raebareli road, lucknow 226014, india. Kluverbucy syndrome temporal lobectomy behavior syndrome kluver bucy syndrome syndrome, kluverbucy. November, 1904 september 22, 1992 was an american neurosurgeon and neuropathologist who was a native of hubbard, iowa. Other signs and symptoms, include a diminished ability to visually recognize objects, loss of normal fear and anger responses, memory loss, distractibility, seizures, and dementia. Kluverbucy syndrome kbs is a rare behavioral phenotype described in monkeys and humans that appears most often after bilateral.
The full kluverbucy syndrome kbs hyperorality, placidity, hypermetamorphosis, dietary changes, altered sexual behavior, and visual. They developed kluverbucy syndrome rarity in human pathology combined with three or more of the following. He is known mostly for his work with the neurosurgeon paul bucy and the description of the kluverbucy syndrome, which was described in nonhuman primates. Kluver bucy syndrome kbs is a rare neuropsychiatric disorder that can occur following traumatic brain injury tbi. In the laboratory experiment that first produced it, neurosurgeons performed bilateral anterior temporal lobectomies, removing both amygdalae greek, almond. Kluver bucy syndrome an experience with six cases sanjeev. En the national institute of neurological disorders and stroke kluverbucy syndrome, su ninds. Kluverbucy syndrome is a syndrome resulting from bilateral lesions of the medial temporal lobe including amygdaloid nucleus. Spiccate tendenze allesplorazione orale o digitale, ipersessualita sia omo che eterosessuale, agnosia visiva e a volte tattile e uditiva, ipermetamorfosi eccessiva labilita dellattenzione, e alterazioni comportamentali configurano una rarissima sindrome neurologica descritta nel 1973 da kluver e bucy kluverbucy su modelli animali.
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