Klein B, Levin B E, Duchowny M S, Llabre M M
Miami Children's Hospital, Department of Neurology, University of Miami, FL, USA.
Neurology. 2000 Jul 25;55(2):230-5. doi: 10.1212/wnl.55.2.230.
To assess intellectual functioning (IQ) in 54 children and adolescents with intractable epilepsy who later underwent cortical resection due to unilateral malformations of cortical development acquired in utero.
Lesion type was classified into circumscribed mass lesions and diffuse cortical dysplasia based on histopathologic analysis of surgical tissue. Cortical dysplastic lesions were further graded as mild, moderate, or severe according to specific microscopic features. Laterality of lesion was determined through neurologic examination and electrophysiologic and neuroradiologic procedures. Classification of lesion type was corroborated by its significant relationship with other disease-related variables known to be related to clinical severity (age at seizure onset, age at resection, and extent of lesion).
Analyses of covariance revealed that circumscribed lesions had a less deleterious effect on nonverbal IQ than did diffuse cortical dysplasia, after controlling for age at seizure onset and extent of lesion. This effect was also found on verbal IQ measures, but only in subjects with right-sided lesions. Subjects with left-sided lesions performed significantly more poorly on verbal IQ measures than those with right-sided lesions. Additionally, younger age at onset and greater extent of lesion were associated with poorer cognitive outcome.
Cortical dysplasia and early left hemisphere lesions have a significantly worse impact on cognitive functioning than circumscribed lesions or right hemisphere developmental lesions in children with epilepsy.
评估54例难治性癫痫患儿及青少年的智力功能（智商），这些患儿及青少年后来因先天性单侧皮质发育畸形接受了皮质切除术。
根据手术组织的组织病理学分析，将病变类型分为局限性肿块病变和弥漫性皮质发育异常。根据特定的微观特征，将皮质发育异常病变进一步分为轻度、中度或重度。通过神经学检查、电生理和神经放射学检查确定病变的部位。病变类型的分类通过其与已知与临床严重程度相关的其他疾病相关变量（癫痫发作起始年龄、切除年龄和病变范围）的显著关系得到证实。
协方差分析显示，在控制癫痫发作起始年龄和病变范围后，局限性病变对非言语智商的有害影响小于弥漫性皮质发育异常。在言语智商测量中也发现了这种影响，但仅在右侧病变的受试者中。左侧病变的受试者在言语智商测量中的表现明显比右侧病变的受试者差。此外，发病年龄较小和病变范围较大与较差的认知结果相关。
在癫痫患儿中，皮质发育异常和早期左半球病变对认知功能的影响明显比局限性病变或右半球发育性病变更差。
