ABSTRACT
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.
Subject(s)
Brain/pathology , Epilepsy/etiology , Kluver-Bucy Syndrome/pathology , Adolescent , Animals , Brain/diagnostic imaging , Child, Preschool , Female , Fluorodeoxyglucose F18 , Humans , Kluver-Bucy Syndrome/complications , Kluver-Bucy Syndrome/therapy , Magnetic Resonance Imaging , Male , Positron-Emission TomographySubject(s)
Catatonia/etiology , Catatonia/therapy , Electroconvulsive Therapy/methods , Epilepsy, Complex Partial/surgery , Kluver-Bucy Syndrome/therapy , Neurosurgical Procedures/adverse effects , Postoperative Complications/therapy , Anterior Temporal Lobectomy/adverse effects , Epilepsy, Complex Partial/complications , Female , Humans , Kluver-Bucy Syndrome/psychology , Middle AgedABSTRACT
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.
Subject(s)
Kluver-Bucy Syndrome/pathology , Kluver-Bucy Syndrome/physiopathology , Humans , Kluver-Bucy Syndrome/therapyABSTRACT
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.
Subject(s)
Attention Deficit and Disruptive Behavior Disorders/etiology , Attention Deficit and Disruptive Behavior Disorders/therapy , Intracranial Hemorrhages/therapy , Kluver-Bucy Syndrome/therapy , Adrenergic alpha-Agonists/therapeutic use , Attention Deficit Disorder with Hyperactivity/drug therapy , Attention Deficit Disorder with Hyperactivity/etiology , Attention Deficit Disorder with Hyperactivity/psychology , Attention Deficit and Disruptive Behavior Disorders/psychology , Central Nervous System Stimulants/therapeutic use , Cerebral Angiography , Clonidine/therapeutic use , Coma/etiology , Electroencephalography , Female , Humans , Intracranial Hemorrhages/complications , Intracranial Hemorrhages/psychology , Kluver-Bucy Syndrome/complications , Kluver-Bucy Syndrome/psychology , Methylphenidate/therapeutic use , Neuropsychological Tests , Parietal Lobe/pathology , Sexual Behavior , Stroke/complications , Stroke/psychology , Stroke/therapy , Temporal Lobe/pathology , Tomography, Emission-Computed, Single-Photon , Tomography, X-Ray Computed , Young AdultSubject(s)
Brain Edema/complications , Brain Edema/surgery , Kluver-Bucy Syndrome/etiology , Kluver-Bucy Syndrome/surgery , Subarachnoid Hemorrhage/complications , Brain Edema/etiology , Cerebral Angiography , Female , Glasgow Coma Scale , Humans , Image Processing, Computer-Assisted , Kluver-Bucy Syndrome/therapy , Magnetic Resonance Imaging , Middle Aged , Neurosurgical ProceduresABSTRACT
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.
Subject(s)
Coma/etiology , Glycogen Storage Disease Type I/complications , Hypoglycemia/etiology , Kluver-Bucy Syndrome/etiology , Respiratory Tract Infections/complications , Adult , Biomarkers/blood , Blood Glucose/metabolism , Coma/diagnosis , Fasting/blood , Glycogen Storage Disease Type I/diagnosis , Glycogen Storage Disease Type I/therapy , Humans , Hypoglycemia/blood , Hypoglycemia/diagnosis , Hypoglycemia/therapy , Kluver-Bucy Syndrome/diagnosis , Kluver-Bucy Syndrome/psychology , Kluver-Bucy Syndrome/therapy , Magnetic Resonance Imaging , Male , Respiratory Tract Infections/diagnosis , Respiratory Tract Infections/therapy , Severity of Illness Index , Treatment OutcomeABSTRACT
The Klüver-Bucy syndrome (KBS) was first described in 1937 as an experimental neurobehavorial syndrome in monkeys with bitemporal brain lesions. The syndrome in man was subsequently observed to be transient or permanent in a variety of neurodegenerative disorders and after traumatic, nontraumatic, and infectious brain injury. Its most common manifestations are hyperorality with changes in dietary habits, hypersexuality, and visual agnosia. Seizures are another frequent symptom. Here we describe KBS in a female inpatient aged 30 in whom KBS and psychotic symptoms occurred together.
