Department of Neurology, University of California, Irvine, Irvine, CA.
Ann Neurol. 2014 Nov;76(5):660-8. doi: 10.1002/ana.24240. Epub 2014 Aug 18.
Adults with juvenile myoclonic epilepsy (JME) have subtle brain structural abnormalities in the frontothalamocortical network, poorer cognitive function, and worse long-term social outcomes, even when their seizures are controlled and/or remitted. The natural history of JME and development of abnormalities in brain structure and cognition from epilepsy onset has not been studied.
The maturational trajectories of cognitive and brain development were prospectively compared between 19 children with new-onset JME in the first 2 years after diagnosis and 57 healthy controls.
Cognitive abilities of children with JME were similar to or worse than healthy controls at baseline but failed to reach the competence level of healthy controls at follow-up across most of the tested cognitive abilities. Abnormal patterns of brain development, as assessed by magnetic resonance imaging studies, were evident in children with JME and included attenuation of age-related decline in cortical volume, thickness, and surface area compared to typically developing children. The altered brain developmental trajectory in the JME group was evident in higher-association frontoparietotemporal brain regions (p < 0.05, corrected for multiple comparisons).
Children with JME have abnormal structural brain development and impaired cognitive development early in the course of their epilepsy.
患有青少年肌阵挛性癫痫(JME)的成年人在前脑-丘脑-皮质网络中存在细微的大脑结构异常,认知功能较差,长期社会结局较差,即使他们的癫痫发作得到控制和/或缓解。JME 的自然病史以及癫痫发作后大脑结构和认知异常的发展尚未得到研究。
前瞻性比较 19 名新诊断的 JME 患儿在诊断后 2 年内和 57 名健康对照者的认知和大脑发育的成熟轨迹。
JME 患儿的认知能力在基线时与健康对照组相似或更差,但在大多数测试的认知能力中,未能在随访时达到健康对照组的能力水平。磁共振成像研究显示,JME 患儿存在异常的大脑发育模式,包括与正常发育儿童相比,皮质体积、厚度和表面积的年龄相关下降减弱。在 JME 组中,这种改变的大脑发育轨迹在更高关联的额顶颞叶大脑区域中明显(p < 0.05,经多重比较校正)。
在癫痫发作的早期,JME 患儿就存在异常的结构性脑发育和认知发育受损。