Unusual complication of Herpes simplex encephalitis: complete Klüver-Bucy syndrome.

Klüver-Bucy syndrome is a rare neurobehavioral disorder caused by a bilateral temporal lobe lesion affecting the hippocampus and amygdala; clinically characterised by hyperorality, hypermetamorphosis, placidity, altered sexual behaviour, eating, disorders and visual impairment, agnosia and amnesia. However, the complete syndrome is rarely seen, and diagnosis does not require all the symptoms to be manifested simultaneously.We describe a patient who developed a complete Klüver-Bucy syndrome secondary to bilateral temporal involvement due to herpetic encephalitis.

Klüver-Bucy syndrome secondary to a nondominant middle cerebral artery ischemic stroke: a case report and review of the literature.

BACKGROUND: Klüver-Bucy syndrome is a rare and complex neurobehavioral cluster that occurs in humans and results from a temporal lobe lesion. It can be associated with a variety of causes. Stroke is a rarely reported cause of this syndrome. CASE PRESENTATION: In this report, we present the case of a 68-year-old Saudi male who developed Klüver-Bucy syndrome subsequent to a nondominant middle cerebral artery ischemic stroke involving right temporal lobe. The patient manifested most of the Klüver-Bucy syndrome clinical features, including hypersexuality, hyperphagia, hyperorality, and visual hypermetamorphosis (excessive tendency to react to every visual stimulation with a tendency to touch every such stimulus). These neurobehavioral manifestations improved after he was started on treatment. CONCLUSIONS: The clinical course, anatomical association relying on pathophysiology, and potential treatment have all been deliberated in regard to the rare occurrence of Klüver-Bucy syndrome resulting from temporal lobe pathology.

Neuropsiquiatría del síndrome de Susac: a propósito de un caso / Neuropsychiatry of Susac Syndrome: a Case Report

RESUMEN El síndrome de Susac es una entidad clínica poco frecuente, posiblemente mediada por un proceso autoinmune; la tríada clásica se compone de retinopatía, disminución en la agudeza auditiva y síntomas neuropsiquiátricos (encefalopatía). Hay pocos casos descritos con sintomatología neuropsiquiátrica como la sintomatología principal. Presentamos un caso de síndrome de Susac, que corresponde a una mujer de 34 arios, con predominio de sintomatologia neuropsiquiátrica, caracterizada por un síndrome de Klüver-Bucy parcial, un síndrome apático, risa y llanto patológico y alteraciones cognitivas de predominio atencional; dichos síntomas mejoraron cualitativamente con el uso de terapia inmunológica. Este caso revela la importancia de las manifestaciones neuropsiquiátricas como presentación clínica en pacientes con entidades neurológicas.

ABSTRACT Susac syndrome is a rare clinical condition, possibly mediated by an autoimmune process; the classic triad is composed of retinopathy, decreased hearing acuity and neuropsychiatric symptoms (encephalopathy). There are few cases reported with neuropsychiatric symptoms as the main manifestation. We present a case of Susac syndrome in a 34-year-old female with a predominance of neuropsychiatric symptoms, characterised by partial Klüver-Bucy syndrome, apathy syndrome, pathological laughter and crying, and cognitive dysfunction predominantly affecting attention, which showed a qualitative improvement with the use of immunological therapy. This case report highlights the importance of neuropsychiatric manifestations as clinical presentation in patients with neurological conditions.

Sexual Behavioral Disinhibition Associated with Nucleus Lentiformis Lesion: A Forensic Neuroscience Perspective Through a Case.

Organic brain disturbances particularly related to frontal cortex structures and subcortical areas including the basal ganglia may play a role in behavioral disinhibition disorders. Kluver-Bucy syndrome (KBS), which is one of the better knowns of these syndromes, includes hyperorality, visual agnosia, and hypersexuality, has been reported to occur after temporal lobe and amygdala lesions; however, several patients who had focal lesions in areas other than the temporal cortex and amygdala have been reported to present partial KBS symptoms. Nucleus lentiformis refers to a large portion of the basal ganglia including the putamen and globus pallidus, and specific structures within this broad area are known to be important for reward and value-based decision making. To date, KBS symptoms including hypersexual behavior associated with nucleus lentiformis lesions have never been reported. Here, we present a 38-year-old male patient who developed increased sexual interest and hyperorality after infarctions in the right lenticular nucleus and right occipitotemporal region and committed a first-degree sexual assault. He was sent to our institution for the assessment of criminal responsibility to the index sexual crime. According to a comprehensive and thorough forensic psychiatric evaluation, he was diagnosed as having an organic personality disorder with partial KBS symptoms. To the best of our knowledge, this is the first reported case of deviant sexual behavior and hyperorality developing after nucleus lentiformis infarction. We aimed to discuss possible neurobiologic explanations of late-onset deviant sexual behavior, which resulted in sexual criminal behavior following a cerebrovascular infarction.

Visuospatial learning and memory in children pre- and posttemporal lobe resection: Patterns of localization and lateralization.

In children with epilepsy, the impact of surgery including or sparing the mesial temporal lobes (TLs) on visuospatial memory has not been thoroughly investigated, and a clear pattern of hemispheric lateralization has not been observed. The primary aim of this study was to examine visuospatial learning and memory outcomes in children with epilepsy prior to and one year after surgical excision, to determine whether outcomes differed as a function of the localization and lateralization of surgical excisions. Forty-six children who underwent unilateral TL surgery with sparing of the mesial structures (TL group, N = 21, 16 left) or including mesial structures (TL + M group, N = 25 children, 12 left) were retrospectively recruited. Outcomes on the Children's Memory Scale (CMS) Dot Locations subtest (learning, immediate, and delayed recall scores) were examined prior to and following epilepsy surgery. Results revealed significantly reduced visuospatial memory (delayed recall) in the TL + M compared with the TL group after surgery. Despite this significant postoperative difference, there was no significant change in learning, immediate, or delayed recall scores in either group. However, inspection of individual change scores showed that fewer children in the TL + M group improved in delayed recall after surgery (7.2%) compared with children in the TL group (30%) whereas a similar proportion of children in the TL + M (30.4%) and TL (23.3%) groups showed a decline. There were no significant differences in learning or memory scores as a function of seizure laterality before or after surgery and no differences in change over time. Seizure outcome, age at surgery, age at seizure onset, and percentage life with epilepsy were not related to visuospatial learning or memory outcomes; however, greater number of antiepileptic drugs (AEDs) following surgery was related to poorer visuospatial memory (delayed recall) in the TL + M group. In summary, the results show that visuospatial learning and memory performance do not seem to show a significant decline following TL resections in childhood, regardless of whether or not surgery includes the mesial TL and involves the left or right hemisphere. However, although mesial TL excisions might not result in a deficit in visuospatial memory, they may hinder progressions made after surgery. Further research is needed to examine how resection of the mesial TL (alone or in combination with lateral TL structures) affects visuospatial memory outcomes in children, as well as to investigate the degree to which other treatment factors, such as medication, may affect visual memory outcomes.

Klüver-Bucy Syndrome Following Traumatic Brain Injury: A Systematic Synthesis and Review of Pharmacological Treatment From Cases in Adolescents and Adults.

Klüver-Bucy syndrome (KBS) is a rare clinical presentation following traumatic brain injury (TBI). Symptoms include visual agnosia, placidity, hyperorality, sexual hyperactivity, changes in dietary behavior, and hypermetamorphosis. The purpose of this article was to identify and synthesize the available evidence from case reports and case series on the treatment profile of KBS among adolescents and adults after TBI. Four bibliographic databases (MEDLINE OVID, EMBASE, PsycINFO, and SCOPUS) were searched for relevant literature. No date or language restrictions were applied. All case reports containing original data on KBS following TBI among adolescents and adults were included. Articles were evaluated, and data were extracted according to predefined criteria. The literature search identified 24 case reports of KBS post-TBI published between 1968 and 2017. Most case subjects were male (70.1%), and the mean age at injury was 25.1 years (range, 13-67 years). Injury to one or both temporal lobes occurred in most cases. Inappropriate sexual hyperactivity was the most common KBS symptom, followed by a change in dietary behavior and hyperorality. Visual agnosia was the least reported. In 50% of cases, the patient fully recovered from KBS. One-half of all participants described pharmacological management; the most common medication prescribed was carbamazepine. Overall, there was a lack of data available on pharmacotherapy initiation and duration. The complex presentation of KBS presents challenges in terms of treatment options. Although overall individuals who were prescribed carbamazepine had positive outcomes, given the reliance on case reports, it is difficult to make a definitive recommendation to guide clinical practice.

Klüver & Bucy syndrome: an investigation of social and affective cognition.

Klüver-Bucy syndrome (KBS) leads to important behavioral symptoms and social maladaptation. Rarely described, no previous study has investigated its social and affective cognitive profile. We report the case of ASP, a patient who developed a complete KBS at 9 years that evolved into an incomplete KBS. Orbitofrontal and temporal damages were evidenced. While a classic neuropsychological assessment showed a preserved global functioning, an extensive evaluation of her social and affective cognition (reversal learning, decision-making, emotion recognition, theory of mind, creative thinking) showed remarkable deficits. The relevancy of such findings for the characterization KBS and the field of neuropsychology are discussed.

The Klüver-Bucy Syndrome.

In 1937, Heinrich Klüver and Paul Bucy described a dramatic behavioral syndrome in monkeys after bilateral temporal lobectomy. The full Klüver-Bucy syndrome (KBS) - hyperorality, placidity, hypermetamorphosis, dietary changes, altered sexual behavior, and visual agnosia - is evident within 3 weeks following operation. Some KBS features (i.e., hyperorality, placidity, hypermetamorphosis) persist indefinitely, whereas others gradually resolve over several years. Klüver and Bucy were initially unaware of an earlier report of KBS by Sanger Brown and Edward Schäfer in 1888. Human cases were recognized in the 1950s, as surgeons employed bilateral temporal lobectomies to treat seizures. Various attempts were made to localize the component features to specific areas of the temporal lobe, with mixed success. Bilateral ventral temporal ablations and bilateral temporal lobectomies produced marked impairment in visual discrimination, whereas lateral resections or unilateral lesions did not. Discrete bilateral lesions of the lateral amygdaloid nucleus produced a permanent "hypersexed state." By the 1970s, it was clear that the major symptoms of KBS are produced by destroying either the temporal neocortex or the amygdala bilaterally. KBS is now thought to be caused by disturbances of temporal portions of limbic networks that interface with multiple cortical and subcortical circuits to modulate emotional behavior and affect. The clinical features of KBS in man are similar to those in monkeys, but the full syndrome is rarely seen, probably because the anterior temporal lobe dysfunction is usually less severe than that following total temporal lobe ablation in monkeys. Human KBS does not occur in isolation, but is typically part of a complex behavioral syndrome that almost always includes amnesia and aphasia, and that may also include dementia and seizures. The treatment of KBS is difficult and often unsatisfactory.

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.

Sanger Brown and Edward Schäfer before Heinrich Klüver and Paul Bucy: their observations on bilateral temporal lobe ablations.

Fifty years before a report on the complete bitemporal lobectomy syndrome in primates, known as the Klüver-Bucy syndrome, was published, 2 talented investigators working at the University College in London, England-neurologist Sanger Brown and physiologist Edward Schäfer-also made this discovery. The title of their work was "An investigation into the functions of the occipital and temporal lobes of the monkey's brain," and it involved excisional brain surgery in 12 monkeys. They were particularly interested in the then-disputed primary cortical locations relating to vision and hearing. However, following extensive bilateral temporal lobe excisions in 2 monkeys, they noted peculiar behavior including apparent loss of memory and intelligence resembling "idiocy." These investigators recognized most of the behavioral findings that later came to be known as the Klüver-Bucy syndrome. However, they were working within the late-19th-century framework of cerebral cortical localizations of basic motor and sensory functions. Details of the Brown and Schäfer study and a glimpse of the neurological thinking of that period is presented. In the decades following the pivotal work of Klüver and Bucy in the late 1930s, in which they used a more advanced neurosurgical technique, tools of behavioral observations, and analysis of brain sections after euthanasia, investigators have elaborated the full components of the clinical syndrome and the extent of their resections. Other neuroscientists sought to isolate and determine the specific temporal neocortical, medial temporal, and deep limbic structures responsible for various visual and complex behavioral deficits. No doubt, Klüver and Bucy's contribution led to a great expansion in attention given to the limbic system's role in action, perception, emotion, and affect-a tide that continues to the present time.

Clinical manifestations and morphological changes in one case with post-stroke Klüver-Bucy syndrome.

We present the case of a 71-year-old right-handed male, admitted to the Department of Neurology, Emergency County Hospital of Arad, Romania, on November 2015, with a rare case of Klüver-Bucy syndrome (KBS), following an ischemic stroke of the right temporal lobe, which was previously diagnosed in December 2014 and was treated accordingly. At the moment of second hospital admission, the patient was found somnolent and confused at home, with traumatic signs of biting of the tongue and urine emission. A couple days after admission, our patient became alert and presented hypersexuality, hypermetamorphosis, increased oral tendency, behavior changes including apathy with loss of anger and fear, and a very increased appetite, transient visual agnosia and right-left disorientation. In the initial phase, the patient could not recognize any members of his family, but he had a tendency to touch everything within his reach and place it into his mouth. The KBS presented in this case, following an ischemic stroke of the right temporal lobe provides distinct and intriguing insights into the possible pathophysiology of this syndrome. Often disruption of consciousness during recovery period may hide the clinical manifestation of the syndrome.

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.