Pathologic features of dysplasia and accompanying alterations observed in surgical specimens from patients with intractable epilepsy.
Pathologic features of dysplasia and accompanying alterations observed in surgical specimens from patients with intractable epilepsy.
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DOI：
10.1177/08830738040190031301
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发表时间：
2004-03
影响因子：
1.9
通讯作者：
A. Kakita;S. Kameyama;S. Hayashi;H. Masuda;H. Takahashi
中科院分区：
文献类型：
--
作者：
A. Kakita;S. Kameyama;S. Hayashi;H. Masuda;H. Takahashi
对108例难治性癫痫患者的手术标本进行了皮质发育异常或皮质发育不良所致畸形的检查，以确定组织病理学改变的范围。临床结果的相关性也被评估。在不同的组合中观察到不同类型和程度的发育异常特征，包括结构异常，分子层和/或皮质II层神经元数量增加，神经元聚集，卫星少突胶质细胞数量增加，异常回转，白质内异位神经元的单个和/或聚集，以及细胞学异常细胞的出现，如巨型或变形神经元和气球细胞。在颞叶标本中，微发育不良(对应于皮质发育异常引起的轻度畸形和IA/B型局灶性皮质发育不良)比Taylor型局灶性皮质发育不良(IIA/B型)更常见，而在额叶标本中，两者的发生率相当。后一种类型患者的发病年龄和手术年龄明显低于前者。另一方面，所有病例的皮质和白质均可见明显的星形细胞增多症，部分病例可见大量淀粉样体和神经原纤维缠绕状包涵体。超微结构研究显示，突触后皮质中的树突和树突扩张，白质中出现脱髓鞘和重新髓鞘形成的特征。因此，对于致痫灶，尽管发育不良改变是致病基础，但涉及突触、树突和轴突的重叠的后续退变过程可能有助于致痫过程的发展。星形胶质细胞也可能积极参与癫痫的发病机制。
Malformations caused by abnormalities of cortical development, or cortical dysplasias, were examined in surgical specimens from 108 patients with medically intractable epilepsy to determine the scope of histopathologic changes. The relevance of the clinical findings was also evaluated. Various types and degrees of dysplastic features were observed in various combinations, including architectural abnormalities, an increased number of neurons in the molecular layer and/or cortical layer II, neuronal clustering, an increased number of satellite oligodendrocytes, abnormal gyration, single and/or aggregates of heterotopic neurons in the white matter, and the appearance of cytologically abnormal cells, such as giant or dysmorphic neurons and balloon cells. In the temporal lobe specimens, microdysgenesis (corresponding to mild malformations caused by abnormalities of cortical development and type IA/B focal cortical dysplasias) was more frequently observed than Taylor-type focal cortical dysplasia (type IIA/B), whereas in the frontal lobe specimens, the frequency of occurrence of both types was even. The ages at seizure onset and surgery of patients with the latter type were significantly lower than those of patients with the former. On the other hand, prominent astrocytosis in the cortex and white matter was evident in all cases, and many corpora amylacea and neurofibrillary tangle-like inclusions were observed in a subset of cases. An ultrastructural investigation revealed dilatation of the postsynaptic dendritic spines and shafts in the cortex and features indicating the occurrence in the white matter of demyelination followed by remyelination. Thus, with regard to the epileptogenic lesions, although dysplastic changes constitute the pathogenetic basis, the overlapping subsequent degenerative processes involving synapses, dendrites, and axons might contribute to the development of epileptogenic processes. Astrocytes might also actively participate in the development of the pathogenesis of epilepsy.