Epilepsy Center, Neurological Institute, Cleveland Clinic, Cleveland, Ohio, USA.
Department of Neurology, Neurological Institute, Cleveland Clinic, Cleveland, Ohio, USA.
Epilepsia. 2022 Jul;63(7):1671-1681. doi: 10.1111/epi.17260. Epub 2022 May 3.
Neuropsychological profiles are heterogeneous both across and within epilepsy syndromes, but especially in frontal lobe epilepsy (FLE), which has complex semiology and epileptogenicity. This study aimed to characterize the cognitive heterogeneity within FLE by identifying cognitive phenotypes and determining their demographic and clinical characteristics.
One hundred and six patients (age 16-66; 44% female) with FLE completed comprehensive neuropsychological testing, including measures within five cognitive domains: language, attention, executive function, processing speed, and verbal/visual learning. Patients were categorized into one of four phenotypes based on the number of impaired domains. Patterns of domain impairment and clinical and demographic characteristics were examined across phenotypes.
Twenty-five percent of patients met criteria for the Generalized Phenotype (impairment in at least four domains), 20% met criteria for the Tri-Domain Phenotype (impairment in three domains), 36% met criteria for the Domain-Specific Phenotype (impairment in one or two domains), and 19% met criteria for the Intact Phenotype (no impairment). Language was the most common domain-specific impairment, followed by attention, executive function, and processing speed. In contrast, learning was the least impacted cognitive domain. The Generalized Phenotype had fewer years of education compared to the Intact Phenotype, but otherwise, there was no differentiation between phenotypes in demographic and clinical variables. However, qualitative analysis suggested that the Generalized and Tri-Domain Phenotypes had a more widespread area of epileptogenicity, whereas the Intact Phenotype most frequently had seizures limited to the lateral frontal region.
This study identified four cognitive phenotypes in FLE that were largely indistinguishable in clinical and demographic features, aside from education and extent of epileptogenic zone. These findings enhance our appreciation of the cognitive heterogeneity within FLE and provide additional support for the development and use of cognitive taxonomies in epilepsy.
神经心理学特征在癫痫综合征之间以及在同一综合征内都是异质的，但在额叶癫痫（FLE）中尤其复杂，因为其具有复杂的症状学和致痫性。本研究旨在通过确定认知表型及其人口统计学和临床特征，来描述 FLE 内认知异质性。
106 名 FLE 患者（年龄 16-66 岁；44%为女性）完成了全面的神经心理学测试，包括五个认知领域的测试：语言、注意力、执行功能、加工速度和言语/视觉学习。根据受损域的数量，将患者分为四个表型之一。研究了表型之间的域损伤模式以及临床和人口统计学特征。
25%的患者符合广义表型标准（至少四个域受损），20%的患者符合三域表型标准（三个域受损），36%的患者符合特定域表型标准（一个或两个域受损），19%的患者符合完整表型标准（无损伤）。语言是最常见的特定领域损伤，其次是注意力、执行功能和加工速度。相比之下，学习是受影响最小的认知域。广义表型的受教育年限比完整表型少，但在人口统计学和临床变量方面，各表型之间没有差异。然而，定性分析表明，广义和三域表型的致痫区范围更广，而完整表型的发作最常局限于外侧额叶区。
本研究在 FLE 中确定了四个认知表型，这些表型在临床和人口统计学特征方面除了教育程度和致痫区范围外，大多无法区分。这些发现增强了我们对 FLE 内认知异质性的认识，并为在癫痫中开发和使用认知分类提供了更多支持。
