儿童难治性癫癎致癎灶的病理改变

Pathological changes in the epileptogenic foci of children with intractable epilepsy

摘要:

目的：研究儿童难治性癫癎致癎灶的病理改变及其临床意义。方法：儿童难治性癫癎患者 30 例，手术切除致癎灶，在光镜和电镜下观察其病理学改变。结果：致癎灶病理组织学检查发现皮质发育不良 14 例（47%），海马硬化 11 例（37%），胚胎发育不良性神经上皮瘤1例（3%），节细胞胶质瘤 1 例（3%），脑软化 3 例（10%）。电镜下可见海马及海马外皮层突触数量及结构改变，神经元减少及核固缩，星形胶质细胞肿胀、退变，线粒体结构改变。 结论：海马及海马外皮层病理结构改变，特别是突触重塑，可能是难治性癫癎反复自发性发作的形态学基础，该病理变化与癎样活动的产生及神经系统的兴奋抑制平衡失调有关。

Abstract:

OBJECTIVE: To investigate pathological changes in the epileptogenic foci of children with intractable epilepsy and their clinical significance. METHODS: Thirty children with intractable epilepsy were included in the study. The epileptogenic foci were surgically resected and pathological changes in the obtained specimens were observed under a light microscope (LM) and a transmission electron microscope (TEM). RESULTS: Under the LM, cortical dysplasia was found in 14 cases (47%), hippocampal sclerosis in 11 cases (37%), dysembryoplastic neuroepithelial tumor in 1 case (3%), ganglioglioma in 1 case (3%), and encephalomalacia in 3 cases (10%). The TEM observation revealed pathological changes in the ultrastructure of the hippocampus and extra-hippocampal cortex, such as changes in the number of synapses and synaptic structure, decrease in neurons and karyopyknosis, swelling and degeneration of astrocytes, and changes in mitochondrial structures. CONCLUSIONS: Pathological changes in the hippocampus and extra-hippocampal cortex, especially synaptic remodeling, may be the morphological basis for spontaneous recurrent seizures in children with intractable epilepsy. The pathological changes and epileptiform activity are related to an imbalance between excitatory and inhibitory neurotransmission.