临床与病理杂志2017，Vol.37Issue(3)：522-526,5.DOI:10.3978/j.issn.2095-6959.2017.03.013
儿童结节性硬化致难治性癫痫12例临床病理特点
Clinical and pathological characteristics of 12 cases of epilepsy in children with tuberous sclerosis complex
摘要
Abstract
Objective: To study the clinicopathologic features of tuberous sclerosis complex (TSC) occurring in children with medically intractable epilepsy. Methods: The clinical, radiologic and pathologic features of tuberous sclerosis complex occurring in 12 children with intractable epilepsy encountered during the period from 2011 to 2016 in the Epilepsy Center of Haidian Hospital were retrospectively reviewed. Results: All patients including 8 boys and 4 girls had medically intractable seizures. The mean age of seizure onset and disease duration were 3.28 years and 5.32 years respectively. Multiple lesions were existed in single lobe or bilateral cerebral hemisphere. Giant neuron, dysmorphic neurons and balloon cells were pathological characteristic. Seizure outcome after the epilepsy operation revealed 4 children (33.3%) had an Engel grade Ⅰ, 2 kids (16.7%) had an Engel grade Ⅱ, 5 children (41.7%) had an Engel grade Ⅲ, and 1 patient (8.3%) was lost. Conclusion: The children with refractory epilepsy suffered the onset of the disease early and long duration of the disease. Lesion of brain tissue has pathological characteristics. There was a certain correlation between the effect of surgery and the range of the lesion. The effect of surgery was poor in children with extensive lesions.关键词
癫痫/神经外科手术/结节性硬化Key words
epilepsy/neurosurgery/tuberous sclerosis complex引用本文复制引用
李岩,李云林,刘永玲,付静,邢炜..儿童结节性硬化致难治性癫痫12例临床病理特点[J].临床与病理杂志,2017,37(3):522-526,5.基金项目
首都儿科研究所基金(Fx201704).This work was supported by Capital Institute of Pediatrics Funds, P. R. China (Fx201704). （Fx201704）
