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.

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 after unilateral frontotemporal resection in a child with tuberous sclerosis.

Klüver-Bucy syndrome (KBS) is a behavioral phenotype that appears most often after bilateral temporal damage. The main features of KBS are compulsion to examine objects orally, increased sexual activity, placidity, hypermetamorphosis (irresistible impulse to notice and react to everything within sight), visual agnosia, and problems with memory. It is more rarely reported in children than in adults. We present a case of KBS in a 2-year-old boy with tuberous sclerosis complex (TSC) after left frontotemporal resection for refractory epilepsy. This is the first KBS after unilateral temporal resection in a child, although it has already been reported in two adult cases. It also is the first case reported in a TSC patient.

Relapsing herpes simplex encephalitis resulting in Kluver-Bucy syndrome.

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 select patients.

Klüver Bucy syndrome following hypoglycaemic coma in a patient with glycogen storage disease type Ib.

Patients with type I glycogen storage disease (GSD) have poor tolerance to fasting, sometimes less than 3 hours during infancy. Even though most patients are able, as they get older, to tolerate a longer fasting period, they are at permanent risk for fast-induced hypoglycaemia, even in adulthood. Klüver Bucy syndrome, is characterized by psychic blindness (inability to recognize familiar objects), hypermetamorphosis (strong tendency to react to visual stimulus), increased oral exploration, placidity, indiscriminate hyper-sexuality and change in dietary habits. In this case report, we describe the development of Klüver Bucy syndrome in a 28-year-old man with type Ib GSD, following prolonged and severe hypoglycaemia triggered by a common respiratory infection.

The effect of surgery on the postictal state.

The effect of surgery on the postictal state has not been studied in a systematic fashion. There is limited literature looking at the effect on postictal psychosis. The effect of surgery on this phenomenon has been varied with post ictal psychosis occurring post surgery when it has not occurred prior to surgery as well as resolution after surgery. Case reports of post ictal Kluver Bucy Syndrome and Capgas Syndrome have been reported after resective epilepsy surgery as well.

[Postraumatic klüver-Bucy syndrome--description and comparison of two clinical cases]. / Pourazowy zespól klüvera-Bucy'ego--opis i porównanie dwóch przypadków klinicznych.

AIM: Klüver-Bucy syndrome was described in the fifties of the 20th century as a group of neuropsychological symptoms, such as visual agnosia, "oral" tendency, hypermetamorphosis, changes in behaviour, hyper-sexuality (homo-, hetero-, autosexuality) and changes in dietary habits (anorexia, bulimia) that may develop in humans after bilateral damage or dysfunction of the medial temporal lobes. The cause of Klüver-Bucy syndrome may be an injury, central nervous system infection, especially herpetic, Pick disease, temporal epilepsy and paraneoplastic encephalopathy. The condition is very rare in children; its occurrence in childhood was described in a few cases only. Symptom intensity and their manifestation depend on numerous diversified factors. METHODS: A comparative presentation of two clinical cases of Klüver-Bucy syndrome following severe head injuries accompanied by description of computed tomography scans. RESULTS: The diagnosis of the syndrome does not require the presence of all the axial symptoms. Both patients were treated with carbamazepine. Regression of neuropsychological symptoms took a different course in each child, despite their similar brain damage. CONCLUSIONS: Fully symptomatic Klüver-Bucy syndrome is very rare. The psychological status of patients with this disorder depends not only on the extent of the lesion, but also on pre-injury emotional and intellectual development and post-injury social stimulation.

Partial Klüver-Bucy syndrome in a patient with acute disseminated encephalomyelitis.

The symptoms of Klüver-Bucy syndrome (KBS) include hyperorality, hypersexuality, visual agnosia, hypermetamorphosis and decreased motor or vocal reaction to fear- or anger-provoking stimuli. This syndrome has been associated with a wide variety of neurodegenerative disorders, as well as traumatic, non-traumatic and infectious brain injuries. We report an 11-year-old boy who developed a fairly classical presentation of KBS, presumably in the setting of post-infectious acute disseminated encephalomyelitis (ADEM). This patient's presentation is a reminder of this rare syndrome and extends the clinical manifestations of ADEM, which is a relatively more common condition.

[Acute encephalitis/encephalopathy associated with transient involuntary movement and limbic dysfunction in the recovery phase].

We report 3 patients aged 3 - 4 years who presented with impaired consciousness and status epilepticus or a cluster of seizures, followed by transient involuntary movements and limbic dysfunction in the recovery phase. In all the patients, the involuntary movements were observed mainly on the left side and consisted of dystonia, athetosis, chorea, facial myoclonus, and oral dyskinesia. The patients also showed stereotypic movement, oral tendency, visual agnosia, and emotional disturbance, which suggested limbic dysfunction resembling Klüver-Bucy syndrome. Single-photon emission computed tomography (SPECT) revealed hypoperfusion of the unilateral basal ganglia and the adjacent frontal and temporal lobes. No obvious lesions were observed on brain MRI in the acute phase in 2 patients, who recovered completely during the follow-up period. However, the last patient, who had abnormalities with regard to the limbic system and subcortical white matter on diffusion-weighted image in the acute phase, exhibited mental retardation, epilepsy, and persistent oral tendency during the follow-up period.

Alzheimer abnormalities of the amygdala with Klüver-Bucy syndrome symptoms: an amygdaloid variant of Alzheimer disease.

BACKGROUND: Neurofibrillary tangles and beta-amyloid plaques have been observed in the amygdala in Alzheimer disease. A disproportionate abundance of this abnormality in the amygdala may cause behavioral symptoms similar to Klüver-Bucy syndrome. OBJECTIVES: To describe an atypical behavioral presentation of Alzheimer disease and to review the literature on the subject. DESIGN: Case study. SETTING: Outpatient specialty clinic. PATIENT: A 70-year-old man with progressive behavioral symptoms of hyperorality, hypersexuality, hypermetamorphosis, visual agnosia, hyperphagia, and apathy who died at age 77 of asphyxiation on a foreign object. MAIN OUTCOME MEASURES: Clinical symptomatology, brain imaging, and neuropathology. RESULTS: The pathologic diagnosis was Alzheimer disease with abundant tangles and plaques in the lateral amygdala. CONCLUSIONS: This case represents a variant of Alzheimer disease with prominent amygdala abnormalities and a Klüver-Bucy phenotype that was misdiagnosed as frontotemporal dementia. Clinical and imaging findings that may aid in accurate diagnosis are reviewed.

To lose the frame of action: a selective deficit in avoiding unpleasant objects following a unilateral temporal lobe lesion.

Studies on emotion and its neurobiology have been far more focused on the recognition of emotion than on actions that are caused by emotional states. We investigate the performance of a patient, HS, with a unilateral lesion to the left temporal pole and orbito-frontal cortex (OFC) (including left amygdala), on a well-established approach/avoid task that taps into emotion-driven action. The striking finding of the present study is a remarkable, and selective, slowing of HS's avoidance of unpleasant items in her (impaired) contralesional field. This finding suggests that the left temporal lobe and OFC structures, including the amygdala, appear to be involved in the action component of emotion, specifically in avoiding negative items.

Persistent Klüver-Bucy syndrome after bilateral temporal lobe infarction.

Klüver-Bucy (KBS) syndrome is a rare and complicated neurobehavioral syndrome in humans resulting from damage of bilateral anterior temporal portion, especially the amygdala. It can be seen in association with a variety of etiologies. Stroke is a rarely reported. Here we present a 50-year-old right handed man who developed persistent KBS after cardioembolic stroke involving bilateral lateral temporal lobes. He exhibited all clinical features of KBS including visual agnosia, hypersexuality, placidity, hyperorality and hypermetamorphosis. The anatomical basis of pathophysiolgy, clinical course and possible treatment are discussed.