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.

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.

Mesiotemporal Disconnection and Hypoactivity in Klüver-Bucy Syndrome: Case Series and Literature Review.

OBJECTIVE: Klüver-Bucy syndrome (KBS) is often perceived as rare and limited to cases with bilateral amygdala destruction. In fact, various alternate mechanisms may be involved, warranting exploration of the syndrome's presentation, pathophysiology, prognosis, and management. DATA SOURCES: Clinical management and the electronic medical records were examined for 2 patients diagnosed with partial KBS (ICD-10 F07.0) after experiencing ≥ 3 of the following: placidity, indiscriminate dietary behavior, hyperorality, hypersexuality, visual agnosia, and hypermetamorphosis. A literature search was performed in April 2015 by using the keyword Kluver-Bucy in PubMed and Ovid databases for English language publications since inception. Additionally, the authors reviewed the reference list of these publications in order to identify additional reports. STUDY SELECTION: Studies were included if they had information about presentation, pathophysiology, syndrome treatment or management, and course of KBS. DATA EXTRACTION: Information about our KBS cases was obtained by reviewing electronic medical records and by direct observation of the patients. A total of 186 (PubMed) and 137 (Ovid) publications were identified in each database. We ultimately reviewed 109 articles containing information about KBS, finding 51 publications addressing relevant aspects of this syndrome. RESULTS: The first case demonstrates KBS secondary to mesiotemporal structural atrophy, and the second illustrates transient KBS due to functional, postictal, hypoactivity within such structures. Literature review and discussion regarding both prognosis and treatment of KBS follows. CONCLUSIONS: Klüver-Bucy syndrome may be underreported due to a limited understanding of the syndrome as one necessitating bilateral amygdaloid destruction. The syndrome can be seen with damage/hypofunction of the hippocampal-amygdaloid complex and its projections. The prognosis of KBS is variable, and its treatment is based on a combination of environmental and pharmacologic measures.

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.

Síndrome de Klüver-Bucy por lesión frontal / No disponible

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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.

[Klüver-Bucy syndrome. Seven year follow-up of one patient]. / Síndrome de Klüver-Bucy. Seguimiento de un paciente durante 7 años.

OBJECTIVE: To present a left-handed patient who had an acute encephalopathy, possibly of viral etiology, followed by remote and recent memory loss, several types of apraxia and emotional disturbance, without any motor abnormalities. He had cerebral lesions that involved both temporal lobes and other brain regions. All features corresponded to the Klüver-Bucy syndrome. After a seven year follow-up, no improvement of the neurological and neuroradiological, mainly by magnetic resonance imaging (MRI) features was observed. CASE REPORT: A 14 year-old left-handed boy suffered sudden onset of fever (40.4 degrees C), headache, vomiting, focal and generalized seizures and coma. After the acute illness, the patient had severe neurological sequels consisting in total loss of memory without any capacity to recognize persons (including family members) and remote events and he was not capability of remember hardly anything that he was taught after his disease, these alterations continued almost completely during the seven years (from 14 to 21 years) that we followed him up. The only abilities that he conserved in similar conditions to those prior to his acute disease were his capacity to swim (including the style of jumping into the water), bike riding, playing football, dominoes, cards, etc., which he had learned during his childhood, to say the numbers and the alphabet letters rapidly by heart (without knowing them) and to avoid cars on the street. MRI showed post-inflammatory lesions in the temporal and the parieto-temporo-occipital regions bilaterally (cortical and subcortical regions) and in the left occipital region. He presented almost all types of apraxia. CONCLUSION: Klüver-Bucy syndrome, which can be secondary to more than 50 different causes, not only presents remote memory loss but also recent memory loss as in our patient, who appeared to be isolated from the surrounding world. Motor function and automated activities learned before the acute brain illness were not affected and could be recovered. Etiology, location and extent of the anatomic brain lesion appear to be the most important prognostic conditions.

Síndrome de Klüver-Bucy. Seguimiento de un paciente durante 7 años / Klüver-Bucy syndrome. Seven year follow-up of one patient

Objetivo. Presentar a un paciente zurdo que sufrió un proceso agudo cerebral, posiblemente una encefalitis vírica, que le dejó alteraciones de la memoria para los hechos remotos y recientes, diferentes tipos de apraxia y falta de reacción afectiva, sin alteraciones motrices. Existía lesión cerebral en ambos lóbulos temporales y en otras zonas, todo ello compatible con síndrome de Klüver-Bucy. Su seguimiento durante 7 años apenas mostró mejoría de las alteraciones neurológicas y neurorradiológicas, principalmente resonancia magnética (RM). Caso clínico. Hombre que a los 14 años de edad presentó un cuadro brusco de fiebre alta (40,4 oC), cefalea, vómitos, convulsiones y coma. Tras pasar el proceso agudo, el paciente presentó secuelas neurológicas graves consistentes en absoluta falta de memoria sin capacidad alguna de reconocimiento de las personas (incluidos los miembros de la familia) y de hechos remotos, e incapacidad de recordar apenas nada de lo que se le iba enseñando tras su enfermedad, continuando con estas alteraciones, casi en su totalidad, a lo largo de los 7 años (desde los 14 hasta los 21 años) que seguimos su evolución. Sólo conservó, en condiciones similares a como lo realizaba antes de su enfermedad aguda, la capacidad para nadar (incluida la forma perfecta de tirarse al agua), para correr en bicicleta, jugar al fútbol, al dominó, a las cartas, etc., que él aprendió durante su infancia, decir los números y las letras del abecedario «de carrerilla» (sin reconocerlos cuando se le ponían delante) y para sortear los coches en la calle. La RM cerebral mostraba lesiones postinflamatorias en ambos lóbulos temporales, en ambas regiones parietotemporooccipitales (zonas corticales y subcorticales) y en la región occipital izquierda. Presentaba casi todos los tipos de apraxia. Conclusión. El síndrome de Klüver-Bucy, que puede ser secundario a más de 50 causas, no se limita a la pérdida de la memoria para los hechos remotos, sino que puede presentar también incapacidad para rememorar los conocimientos recientes, quedando los pacientes aislados mentalmente del mundo exterior. Su motricidad y la actividad automatizada, aprendida en épocas anteriores al proceso agudo que causó su encefalopatía, no fueron afectados y pudieron recuperarse. La etiología junto con la localización y extensión de las lesiones cerebrales parecen los factores pronósticos más importantes (AU)

Objective: To present a left-handed patient who had an acute encephalopathy, possibly of viral etiology, followed by remote and recent memory loss, several types of apraxia and emotional disturbance, without any motor abnormalities. He had cerebral lesions that involved both temporal lobes and other brain regions. All features corresponded to the Klüver-Bucy syndrome. After a seven year follow-up, no improvement of the neurological and neuroradiological, mainly by magnetic resonance imaging (MRI) features was observed. Case Report: A 14 year-old left-handed boy suffered sudden onset of fever (40.4 degrees C), headache, vomiting, focal and generalized seizures and coma. After the acute illness, the patient had severe neurological sequels consisting in total loss of memory without any capacity to recognize persons (including family members) and remote events and he was not capability of remember hardly anything that he was taught after his disease, these alterations continued almost completely during the seven years (from 14 to 21 years) that we followed him up. The only abilities that he conserved in similar conditions to those prior to his acute disease were his capacity to swim (including the style of jumping into the water), bike riding, playing football, dominoes, cards, etc., which he had learned during his childhood, to say the numbers and the alphabet letters rapidly by heart (without knowing them) and to avoid cars on the street. MRI showed post-inflammatory lesions in the temporal and the parieto-temporo-occipital regions bilaterally (cortical and subcortical regions) and in the left occipital region. He presented almost all types of apraxia. Conclusion: Klüver-Bucy syndrome, which can be secondary to more than 50 different causes, not only presents remote memory loss but also recent memory loss as in our patient, who appeared to be isolated from the surrounding world. Motor function and automated activities learned before the acute brain illness were not affected and could be recovered. Etiology, location and extent of the anatomic brain lesion appear to be the most important prognostic conditions (AU)

Klüver-Bucy syndrome following status epilepticus associated with hepatic encephalopathy.

Described here is the case of a patient with liver cirrhosis who developed bilateral temporo-occipital lobe lesions on MRI and Klüver-Bucy syndrome following status epilepticus. Herpes encephalitis, paraneoplastic syndrome, Hashimoto's encephalopathy, reversible posterior leukoencephalopathy syndrome, mitochondrial encephalomyopathy, lactic acidosis, and strokelike episode syndrome were judged not to be involved on the basis of laboratory results. The possible cause of the temporo-occipital lesions on MRI in this patient was cortical damage related mainly to status epilepticus and partially to coexisting hepatic encephalopathy.

[Klüver-Bucy-like syndrome and frontal symptoms following cerebrovascular disease]. / Beyin Damar Hastaligi Sonrasi Gelisen Frontal Belirtiler ve Klüver-Bucy Benzeri Sendrom.

We present a case with frontal lobe symptoms and Klüver-Bucy-like syndrome following subarachnoid hemorrhage and hydrocephaly. Klüver-Bucy syndrome is a rare neurobehavioral condition characterized by placidity, visual agnosia, hypersexuality, hyperorality, and hypermetamorphosis (the tendency to react to or to touch every visual stimulus). The syndrome is usually associated with lesions of the amygdala or its pathways, and it occurs after head trauma, anoxia-ischemic encephalopathy, herpes simplex encephalitis, and Reye 's syndrome. A 45-year-old right-handed female patient, who developed hydrocephaly after meningitis due to bilateral middle cerebral artery aneurysm surgery presented to our psychiatry clinic with various behavioral and emotional changes. In her psychiatric examination, increased and disinhibited speech, perseveration, placidity, impaired go/no go task performance, and hyperphagia were observed. The patient was treated with risperidone 0.5 mg/day. Magnetic resonance imaging (MRI) of the brain showed encephalomalacic-gliotic changes in the anterior superior medial temporal lobe (including bilateral amygdala), hydrocephalus, bilateral abnormal signal intensity in the white matter of the frontal region, and bilateral infarction in the centrum semiovale. Symptoms, such as placidity (loss of anger and fear) and altered dietary habits are some of the clinical features of Klüver-Bucy syndrome, whereas disinhibition and perseveration are associated with prefrontal cortex dysfunction.

[The amygdaloid complex and its implication in psychiatric disorders]. / El complejo amigdalino humano y su implicación en los trastornos psiquiátricos.

The amygdaloid complex is a group of nuclei located deep in the temporal lobe and closely involved in the limbic system. Its alteration has been associated with some psychiatric processes. In this article, an overall review was made of the published data concerning the amygdaloid complex in the most common psychiatric diseases. A damaged amygdaloid complex is commonly observed, that in the Klüver-Bucy syndrome presents the fullest expression. A decrease in the amygdaloid complex of schizophrenic patients has been observed. This finding was found bilaterally in men whereas in women it was only located in one hemisphere. This finding suggests that morphometric alterations in the amygdaloid complex are more diffuse and more severe in men with schizophrenia. This subcortical complex is larger in children with autism, but not in adolescents, in whom the amygdaloid complex volume matches the normal volume of an adolescent or an adult without this pathology. However, neuroanatomical studies have shown microscopic alterations. In patients with mood disorders, it has been reported that the left amygdaloid complex presents a lesser volume. Moreover, in frontotemporal dementia and Alzheimer disease a slight amygdaloid atrophia was found related to the healthy controls. It can be concluded that the amygdaloid complex is involved in several psychiatric processes, due to structural or functional damage. However, more studies are still needed in order to delimitate the real influence of the amygdaloid complex in these disorders.

Postinfectious immune-mediated encephalitis after pediatric herpes simplex encephalitis.

We report a 3-year-old patient who presented a secondary acute neurological deterioration clinically characterized by a partial Kluver-Bucy syndrome, 1 month after the onset of herpes simplex encephalitis. This episode is unlikely due to continuation or resumption of cerebral viral replication but might be related to an immune-inflammatory process. In children, postinfectious immune-mediated encephalitis occurring after HSE are usually clinically characterized by choreoathetoid movements. This type of movement disorder was, however, not observed in this patient. On the basis of this case and a review of the literature, we hypothesize the existence of a spectrum of secondary immune-mediated process triggered by herpes simplex virus cerebral infection ranging from asymptomatic cases with diffuse white matter involvement to secondary acute neurological deteriorations with or without extrapyramidal features.

[Klüver-Bucy syndrome as the initial symptom of adult-type ceroid lipofuscinosis (Kufs' disease)]. / Síndrome de Klüver-Bucy como manifestación inicial de ceroidolipofuscinosis del adulto (enfermedad de Kufs).

AIMS: The purpose of this paper is to report the case of a patient with Kluver-Bucy syndrome caused by adult-type ceroid lipofuscinosis (Kufs' disease) and to review the literature dealing with the causes of this syndrome. CASE REPORT: A 38-year-old male examined because of behavioural changes and cognitive impairment. Brain biopsy findings were characteristic of adult-type ceroid lipofuscinosis. This patient fulfilled the criteria of Kufs' disease, since he had mixed clinical features belonging to both type A (neuropsychiatric disorders) and B (aphasia-apraxia-agnosia syndrome) of the disease. The initial symptoms included several clinical features of Klüver-Bucy syndrome (probable visual agnosia, apathy, increased sexual activity, lack of sexual inhibition, hypermetamorphopsia, increased oral behaviour and changes in dietary habits). CONCLUSIONS: Adult-type ceroid lipofuscinosis is an infrequent clinical entity that is difficult to diagnose owing to the absence of peripheral biological markers and the need to confirm such a diagnosis by means of a histopathological study.

Kluver-Bucy syndrome -- an experience with six cases.

The Kluver-Bucy syndrome (KBS) is a neurobehavioral syndrome and can be seen in association with a variety of neurological disorders. Case records of 6 patients with KBS seen during a period of 5 years in a university hospital were reviewed. During the study period 6 patients with KBS, aged between 4 and 14 years, were seen. Hyperorality, hypersexuality, and abnormal behavior were the most common manifestations. Of the 6 patients, 5 had recurrent unprovoked seizures. The associated neurological disorders included anoxia-ischemic encephalopthy (2), herpes simplex encephalitis (1), neurocysticercosis (NCC) (1), traumatic brain injury with gliosis (1 case) and tuberculous meningitis (1 case). Prognosis was poor in all the patients except in the patient with NCC.

Herpes simplex encephalitis: some interesting presentations.

Herpes Simplex Encephalitis (HSE) is the most common cause of fatal viral encephalitis. A high index of suspicion is mandatory for early diagnosis and successful therapy to restrict morbidity and mortality. We report 4 patients of HSE, with interesting presentations, viz. brainstem involvement in an immunosuppressed patient, Kluver-Bucy Syndrome-a consequence of untreated HSE, HSE in the postpartum period mistaken as cortical venous thrombosis, and response to inadequate treatment. They demonstrate the wide spectrum of clinical features, pitfalls in diagnosis, and a variable response to therapy in HSE.