Boronat Susana, Newberry Peter, Mehan William, Thiele Elizabeth Anne, Duhaime Ann-Christine
Department of Neurology, Massachusetts General Hospital, Boston, USA.
Childs Nerv Syst. 2013 Aug;29(8):1391-4. doi: 10.1007/s00381-013-2127-3. Epub 2013 May 1.
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.
克吕弗-布西综合征（KBS）是一种行为表型，最常出现在双侧颞叶受损后。KBS的主要特征包括口部检查物体的强迫行为、性活动增加、平静、视物显多症（对视野内一切事物注意并做出反应的不可抗拒冲动）、视觉失认以及记忆问题。儿童中报道的KBS病例比成人中更为罕见。我们报告一例2岁患有结节性硬化症（TSC）的男孩，在因难治性癫痫接受左额颞叶切除术后出现KBS。这是儿童单侧颞叶切除术后首例KBS病例，尽管此前已有两例成人病例报道。这也是TSC患者中报道的首例病例。
