Quiske Ansgar, Frings Lars, Wagner Kathrin, Unterrainer Josef, Schulze-Bonhage Andreas
Epilepsy Center, University Clinic of Freiburg, Germany.
Epilepsia. 2006 Jan;47(1):153-8. doi: 10.1111/j.1528-1167.2006.00381.x.
To describe extend and severity of cognitive deficits in juvenile and adult patients with gelastic seizures and hypothalamic hamartoma (HH) and to analyze the impact of epilepsy-related variables on cognitive performance.
Thirteen juvenile and adult patients (mean age, 25 years; seven men) underwent comprehensive neuropsychological testing assessing intellectual performance, attention and executive functions, verbal and visual memory, and visuospatial abilities.
Intellectual abilities ranged from moderate mental retardation to good average performance; 54% of the patients displayed below-average global intellectual abilities. Attentional and executive functions were impaired in 23% to 46% of the patients. Below-average visuospatial capabilities were observed in 39% of the cases. Memory functions were impaired regarding both visual (77%) and verbal learning (62%). Nonparametric correlation analysis revealed a significant relation between monthly partial seizure frequency and reduced cognitive flexibility and reduced performance in mental rotation. In addition, HH volume was significantly negatively correlated with cognitive flexibility, whereas age at onset and duration of epilepsy did not show significant correlation to cognitive performance.
More than half of the adult patients with gelastic seizures and HH displayed deficits in a broad range of cognitive functions, expressed mostly in visual and verbal learning and memory. Some of the deficits could be shown to correlate with disease-related characteristics representing the severity of the epilepsy or the size of the underlying lesion. These findings prompt for a longitudinal investigation of the development of these cognitive deficits to analyze further the relevant factors contributing to this wide spectrum of cognitive impairments.
描述患有痴笑性癫痫和下丘脑错构瘤（HH）的青少年及成年患者认知缺陷的范围和严重程度，并分析癫痫相关变量对认知表现的影响。
13名青少年及成年患者（平均年龄25岁；7名男性）接受了全面的神经心理学测试，评估智力表现、注意力和执行功能、言语和视觉记忆以及空间视觉能力。
智力能力范围从中度智力发育迟缓到良好的平均水平；54%的患者整体智力能力低于平均水平。23%至46%的患者注意力和执行功能受损。39%的病例观察到空间视觉能力低于平均水平。视觉（77%）和言语学习（62%）的记忆功能均受损。非参数相关分析显示，每月部分性癫痫发作频率与认知灵活性降低以及心理旋转表现下降之间存在显著关系。此外，HH体积与认知灵活性显著负相关，而发病年龄和癫痫病程与认知表现无显著相关性。
超过一半的患有痴笑性癫痫和HH的成年患者在广泛的认知功能方面存在缺陷，主要表现在视觉和言语学习及记忆方面。部分缺陷与代表癫痫严重程度或潜在病变大小的疾病相关特征有关。这些发现促使对这些认知缺陷的发展进行纵向研究，以进一步分析导致这种广泛认知障碍的相关因素。
