Juliá-Palacios N, Boronat S, Delgado I, Felipe A, Macaya A
Pediatric Neurology, Vall d'Hebron Hospital, UAB, Barcelona, Spain.
Clinical Genetics, Vall d'Hebron Hospital, UAB, Barcelona, Spain.
Neuropediatrics. 2018 Apr;49(2):104-111. doi: 10.1055/s-0037-1609036. Epub 2017 Dec 13.
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.
克吕弗-布西综合征（KBS）是一种在猴子和人类中描述的罕见行为表型，最常出现在双侧颞叶损伤后。KBS的主要特征包括强迫性口部检查物体、性活动增加、平静、反应过度、视觉失认和失忆。儿童病例稀少，最常报告的病因是疱疹性脑炎。口欲亢进（90%）、性欲亢进（82%）和癫痫（70%）是迄今为止文献报道的51例病例中最常见的特征。卡马西平、选择性5-羟色胺再摄取抑制剂（SSRI）和抗精神病药物已用于对症治疗，但控制效果不一。根据病因怀疑，皮质类固醇或免疫抑制剂，如利妥昔单抗，在某些情况下可以作为一种选择。KBS后的认知和行为障碍通常很严重，但随着时间的推移可能会有所改善，残留残疾程度从严重到相当轻微不等。我们报告了两例新的与脑炎相关的儿科患者，并回顾了文献中所有儿科KBS病例，以更好地描述这种罕见神经行为疾病的临床特征。
