Partial Klüver-Bucy syndrome in a Paediatric patient: A post-neurosurgical and neuropsychological cases.

A variety of cognitive, behavioural, and emotional impairments have been reported in the literature that are associated with the resection of the temporal cortex. Klüver-Bucy syndrome is one infrequently reported disorder in the paediatric population. This paper describes the neuropsychological findings of a female paediatric patient at 7 and 10 years of age with a diagnosis of partial Klüver-Bucy syndrome (pKBS) following total resection of the amygdala and right hippocampus to resect a glioma. The patient presented emotional problems, aggressiveness, hypermetamorphosis, social indifference, and behavioural dysexecutive syndrome, which was found at both 7 and 10 years, but with a decrease in the severity of alterations in attention, impulsivity, hyperactivity, and aggressive behaviour in a second evaluation after she had a neuropsychological intervention. These findings describe the neuropsychological profile of paediatric case with resection of the amygdala and right temporal lobe.

Pediatric Klüver-Bucy Syndrome: Report of Two Cases and Review of the Literature.

Klüver-Bucy syndrome (KBS) is a rare behavioral phenotype described in monkeys and humans that appears most often after bilateral temporal damage. The main features of KBS are compulsion to examine objects orally, increased sexual activity, placidity, hypermetamorphosis, visual agnosia, and amnesia. Cases in children are scarce, and the most frequently reported etiology is herpes encephalitis. Hyperorality (90%), hypersexuality (82%), and epilepsy (70%) were the most common features of the 51 cases reported in the literature to date. Carbamazepine, selective serotonin reuptake inhibitors (SSRIs), and neuroleptics have been used for symptomatic treatment with variable control. Corticosteroids or immunosupressive agents, such as rituximab, can be an option to use in some cases, according to etiology suspicion. Cognitive and behavioral disturbances after KBS are often severe, but improvement can occur over a long time and residual disabilities vary from major to fairly mild.We report two new encephalitis-associated pediatric patients and review all of the pediatric KBS cases in the literature to better describe the clinical features of this rare neurobehavioral condition.

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

Klüver-Bucy syndrome (KBS) comprises a set of neurobehavioral symptoms with psychic blindness, hypersexuality, disinhibition, hyperorality, and hypermetamorphosis that were originally observed after bilateral lobectomy in Rhesus monkeys. We investigated two siblings with KBS from a consanguineous family by whole-exome sequencing and autozygosity mapping. We detected a homozygous variant in the heparan-α-glucosaminidase-N-acetyltransferase gene (HGSNAT; c.518G>A, p.(G173D), NCBI ClinVar RCV000239404.1), which segregated with the phenotype. Disease-causing variants in this gene are known to be associated with autosomal recessive Mucopolysaccharidosis type IIIC (MPSIIIC, Sanfilippo C). This lysosomal storage disease is due to deficiency of the acetyl-CoA:α-glucosaminidase-N-acetyltransferase, which was shown to be reduced in patient fibroblasts. Our report extends the phenotype associated with MPSIIIC. Besides MPSIIIA and MPSIIIB, due to variants in SGSH and NAGLU, this is the third subtype of Sanfilippo disease to be associated with KBS. MPSIII should be included in the differential diagnosis of young patients with KBS.

Impaired acquisition of new words after left temporal lobectomy despite normal fast-mapping behavior.

Word learning has been proposed to rely on unique brain regions including the temporal lobes, and the left temporal lobe appears to be especially important. In order to investigate the role of the left temporal lobe in word learning under different conditions, we tested whether patients with left temporal lobectomies (N=6) could learn novel words using two distinct formats. Previous research has shown that word learning in contrastive fast mapping conditions may rely on different neural substrates than explicit encoding conditions (Sharon et al., 2011). In the current investigation, we used a previously reported word learning task that implemented two distinct study formats (Warren and Duff, 2014): a contrastive fast mapping condition in which a picture of a novel item was displayed beside a picture of a familiar item while the novel item's name was presented aurally ("Click on the numbat."); and an explicit encoding (i.e., control) condition in which a picture of a novel item was displayed while its name was presented aurally ("This is a numbat."). After a delay, learning of the novel words was evaluated with memory tests including three-alternative forced-choice recognition, free recall, cued recall, and familiarity ratings. During the fast-mapping study condition both the left temporal lobectomy and healthy comparison groups performed well, but at test only the comparison group showed evidence of novel word learning. Our findings indicate that unilateral resection of the left temporal lobe including the hippocampus and temporal pole can severely impair word learning, and that fast-mapping study conditions do not promote subsequent word learning in temporal lobectomy populations.

Kissing or "osculation" in frontotemporal dementia.

The authors investigated the neuropsychiatry of kissing in frontotemporal dementia. Among 15 patients, two had compulsive social kissing, bitemporal involvement, and Klüver-Bucy symptoms, and four pursued kissing with sexually disinhibited behavior. Future research should clarify the neuropsychiatric significance of kissing behavior.

Successful treatment of severe disruptive disorder featuring symptoms of the Klüver-Bucy Syndrome following a massive right temporal-parietal hemorrhage.

We know little about effective treatment for patients suffering from partial or complete Klüver-Bucy Syndrome (KBS) and other disruptive behaviors following a stroke. Reported cases have shown that certain medication, given alone or combined, can be partially effective. In this specific case study, we will try to demonstrate the effectiveness of a combination of carbamazepine, clonidine, quetiapine and methylphenidate in the alleviating of these symptoms. The wide range of symptoms found in KBS led us to use several kinds of psychotropic medication in spite of the inherent risks associated to polypharmacy.

Kluver-Bucy syndrome in one case with systemic lupus erythematosus.

Kluver-Bucy syndrome (KBS) is a collection of neuropsychiatric symptoms, including visual agnosia (prosopagnosia), hypermetamorphosis, placidity, hypersexuality, and hyperorality. Although neuropsychiatric manifestation is prevalent in cases with systemic lupus erythematosus (SLE), only one literature reported a case with SLE that had KBS previously. In this article, a 37-year-old woman with SLE who developed KBS and other neuropsychiatric symptoms is presented. Brain imaging proved the relevant structural lesion. The possible explanation of pathogenesis of KBS in SLE is discussed.

Long-term neuropsychological follow-up of a child with Klüver-Bucy syndrome.

We describe the case of a 10-year-old girl who developed behavioral changes consistent with Klüver-Bucy Syndrome following Listeria meningoencephalitis at 2½ years of age. MRI at age 4 revealed evidence of diffuse brain atrophy with predominant temporal lobe involvement. Electroencephalograpy at 9½ years of age showed abnormal electrical discharges from the left temporal area. Follow-up MRI with volumetric analysis of the mesial temporal structures at 9 years of age demonstrated decreased hippocampal volume bilaterally. Consistent with the morphological abnormalities, serial neuropsychological evaluations demonstrated expressive and receptive language impairment and an amnestic syndrome that significantly decreased her ability to make new declarative memories and maintain adequate academic progress.

Kluver-Bucy syndrome, hypersexuality, and the law.

A 51-year-old right-handed man developed hypersexuality after a second right temporal lobectomy to treat epilepsy. His hypersexuality started with increased marital intercourse and masturbation but he later downloaded child pornography. Hyperphagia and distractibility, other features of the Kluver-Bucy syndrome, also developed. Resection of the amygdala and/or temporal lobe neocortical areas that inhibit other limbic areas may contribute to the pathogenesis of hypersexuality. Neurological factors mitigate the criminal responsibility for hypersexual activity in patients with Kluver-Bucy syndrome. Most previously reported patients were never charged with a crime despite uninvited physical contact in some instances. Our patient was convicted and imprisoned.

[The so-called hypersexual behaviors in dementia]. / Comportements dits d'hypersexualité et démences.

Real hypersexuality is rare in demented subjects. Most inappropriate sexual behaviors are related to disinhibition or lack of taking into account contextual environment and feelings of others. Four dimensions should be taken into account for their analysis: research of intimacy, libido enhancement, disinhibition, and cognitive factors. An integrative model is proposed to adequately adapt their management, using both psychocomportemental and pharmacological approaches.