Kluver Bucy syndrome in young children

Pradhan, Sunil; Singh, Mantabya; Pandey, Nirmal

doi:10.1016/s0303-8467(98)00055-9

Cited by 42 publications

(26 citation statements)

References 8 publications

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“…The abnormal oral tendencies of these children were quantified in the SBRS parent report questionnaire. Inappropriate sexual behavior is also part of K-Bs; masturbatory activity has been reported in child clinical cases (Jha et al, 2005; Pradhan et al,1998) and was documented among the older children by our questionnaires.…”

Section: Discussionsupporting

confidence: 62%

“…Urbach-Wiethe disease, an autosomal recessive disorder affecting the amygdala, presents with some aspects of K-Bs (Hurlemann et al, 2007; Tranel et al, 2006) While there are case reports of K-Bs in children involving various etiologies (e.g., Jha et al, 2005; Pradhan et al, 1998), no childhood equivalent of Urbach-Wiethe has been identified. The pattern of behavioral abnormalities and associated accentuated rate of amygdala atrophy found in children with MPS IIIA suggests that it may be the first pediatric disease whose behavioral phenotype is identified as a K-Bs variant.…”

Section: Discussionmentioning

confidence: 99%

“…Reported cases of K-Bs in human adults result from diverse etiologies but show similar behavioral features (e.g., Aygun, Guven, Altintop, & Doganay, 2003; Janszky, Fogarasi, Magalova, Tuxhorn, & Ebner, 2005). K-Bs secondary to herpes encephalitis, anoxic-ischemic encephalopathy and other conditions is reported in children (e.g., Jha, Kumar, Kumar, & Kumar, 2005; Pradhan, Singh, & Pandey, 1998). These individuals show marked indifference and lack of emotional attachment to caretakers; they also show hyperorality and increased masturbation.…”

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confidence: 99%

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Mucopolysaccharidosis Type IIIA presents as a variant of Klüver–Bucy syndrome

Potegal¹,

Yund

Rudser

et al. 2013

Journal of Clinical and Experimental Neuropsychology

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Mucopolysaccharidosis type IIIA (MPS IIIA) is a neurodegenerative disease with behavioral symptoms unique among the mucopolysaccharidoses. Children with MPS IIIA reportedly mouth things, explore novel environments almost continuously, disregard danger, and empathize/socialize and comply less with parents. These characteristics resemble Klüver–Bucy syndrome (K-Bs). To test the K-Bs hypothesis, 30 children with MPS IIIA were compared to 8 ‘post-transplant’ Mucopolysaccharidosis type IH patients in an experimental “Risk Room”. The room contained attractive and mildly frightening objects, exposure to a 92 dB startle noise triggered by contact with an attractive toy, mother’s return after a brief absence, and compliance with her clean-up directive. Children with MPS IIIA: 1) left mother sooner, 2) wandered more, 3) were more likely to approach frightening objects, 4) were less likely to respond to loud noise with whole body startle, 5) were less likely to avoid the toy associated with the startle noise, 6) interacted less with mother upon her return, and 7) complied less with her clean-up command. K-Bs is associated with loss of amygdala function. Brain MRIs of a subset of the children with MPS IIIA showed volume loss that was greater in the amygdala than the hippocampus; only amygdala loss correlated with reduced fearfulness. MPS IIIA may be the first identified pediatric disease presenting systematically as a K-Bs variant. If validated by further studies, the K-Bs hypothesis of MPS IIIA would provide important clinical and theoretical information for the guidance of families as well as markers for natural disease progression and treatment effects.

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Section: Discussionsupporting

confidence: 62%

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

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Mucopolysaccharidosis Type IIIA presents as a variant of Klüver–Bucy syndrome

Potegal¹,

Yund

Rudser

et al. 2013

Journal of Clinical and Experimental Neuropsychology

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“…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. 18 We report two siblings with MPSIIIC and severely altered emotional behavior who meet the diagnostic criteria for KBS with onset at MPSIII stage 2.…”

Section: Discussionmentioning

confidence: 99%

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

Hübner

Lukács

et al. 2016

Eur J Hum Genet

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“…Klüver-Bucy syndrome is a neurobehavioral symptom complex. It is caused by bilateral anterior temporal lobe lesions and characterized by hyperorality, irritability, and blunted emotional responses (4). Here, we report an HSE patient who had received 14 days of acyclovir treatment but relapsed, which resulted in Klüver-Bucy syndrome.…”

Section: Introductionmentioning

confidence: 92%

Retraction：Relapsing Herpes Simplex Encephalitis Resulting in Kluver-Bucy Syndrome

Yoon

2011

Intern. Med.

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Relapsing herpes simplex encephalitis (HSE) rarely occurs after acyclovir treatment. We treated a patient with relapsing HSE of the contralateral temporal lobe, resulting in Klüver-Bucy syndrome, after a full-dose acyclovir treatment. This case suggests that physicians should consider sudden behavioral and emotional changes after HSE treatment as a possible indication of relapsing HSE, as well as possible temporal lobe epilepsy, and the need to administer longer acyclovir treatment for selectpatients.

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Kluver Bucy syndrome in young children

Cited by 42 publications

References 8 publications

Mucopolysaccharidosis Type IIIA presents as a variant of Klüver–Bucy syndrome

Mucopolysaccharidosis Type IIIA presents as a variant of Klüver–Bucy syndrome

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

Retraction：Relapsing Herpes Simplex Encephalitis Resulting in Kluver-Bucy Syndrome

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