Istanbul University, Istanbul Faculty of Medicine, Department of Neurology, Istanbul, Turkey.
Istanbul University, Istanbul Faculty of Medicine, Department of Neurology, Istanbul, Turkey.
Epilepsy Behav. 2019 Oct;99:106447. doi: 10.1016/j.yebeh.2019.106447. Epub 2019 Aug 6.
Although the courses of self-limited focal epilepsies of childhood are considered as benign, a handful of studies suggested that these children may suffer from cognitive problems. Implementing tailor-made educational strategies would aid these children to reach their full potentials. Therefore, it is crucial to understand and differentiate the complete neuropsychological and behavioral profiles of these rather common syndromes. We aimed to examine the distinct cognitive and behavioral profiles of the Panayiotopoulos syndrome (PS) and the Gastaut syndrome (GS), comparatively.
Twenty patients with PS, 20 patients with GS, and 20 healthy controls have been recruited. The testing protocol included Wechsler Intelligence Scale for Children-Revised, Conner's Continuous Performance Test, Verbal Fluency Test, Stroop Color and Word Test, Color Trails Test, Tower of London Test, Symbol Digit Modalities Test, California Verbal Learning Test-Children's Version, Rey Complex Figure Test, Benton Face Recognition Test, Benton Judgment of Line Orientation, Peabody Picture Vocabulary Test, Reading and Writing Test, Child Behavior Checklist, Conner's Parent Rating Scale-48, and Behavior Rating Inventory of Executive Function. Demographical, clinical, electrophysiological data, and imaging findings have also been evaluated.
With regard to intelligence, the patients with PS scored less in all scales compared to the healthy controls. However, only the performance IQ (intelligence quotient) scores differed significantly between the patient groups, with the patients with PS scoring lower than the patients with GS. Verbal memory problems were eminent in both of the patient groups; whereas, visual memory was impaired only in the group with PS. Psychomotor speed was affected in both groups. Reading problems were prominent only in the patients with PS. Writing and arithmetic skills were defective in both patient groups. There were no noteworthy behavioral problems in comparison to healthy subjects.
Using neuropsychological profiles, this study demonstrated that the GS and the PS are two distinct entities. Cognitive dysfunction is a more prominent and widespread feature of the patients with PS; whereas, the patients with GS suffer only from milder and isolated cognitive problems.
尽管儿童自限性局灶性癫痫的病程被认为是良性的，但少数研究表明，这些儿童可能存在认知问题。实施定制的教育策略将有助于这些儿童充分发挥潜力。因此，了解和区分这些常见综合征的完整神经心理学和行为特征至关重要。我们旨在比较研究 Panayiotopoulos 综合征 (PS) 和 Gastaut 综合征 (GS) 的认知和行为特征。
招募了 20 例 PS 患者、20 例 GS 患者和 20 名健康对照者。测试方案包括韦氏儿童智力量表修订版、康纳连续执行测试、词语流畅性测试、Stroop 颜色和文字测试、颜色追踪测试、伦敦塔测试、符号数字模式测试、加利福尼亚词语学习测试-儿童版、雷氏复杂图形测试、本顿面部识别测试、本顿判断线定向、Peabody 图片词汇测试、阅读和写作测试、儿童行为检查表、康纳父母评定量表-48 版和行为评定量表-执行功能。还评估了人口统计学、临床、电生理数据和影像学发现。
在智力方面，PS 组患者在所有量表上的得分均低于健康对照组。然而，只有 PS 组患者的表现智商（智商）得分存在显著差异，PS 组患者的得分低于 GS 组患者。两组患者均存在明显的言语记忆问题；而 PS 组患者仅存在视觉记忆障碍。两组患者的运动速度均受影响。阅读问题仅在 PS 组患者中突出。PS 组和 GS 组患者的书写和算术技能均存在缺陷。与健康受试者相比，两组患者均无明显行为问题。
通过神经心理学特征，本研究表明 GS 和 PS 是两种不同的实体。认知功能障碍是 PS 患者更为突出和广泛的特征；而 GS 患者仅存在轻度孤立的认知问题。
