Hypothalamic hamartoma: basic mechanisms of intrinsic epileptogenesis.
Hypothalamic hamartoma: basic mechanisms of intrinsic epileptogenesis.
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DOI：
10.1016/j.spen.2007.03.002
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发表时间：
2007-06-01
影响因子：
2.7
通讯作者：
Kerrigan, John F
中科院分区：
文献类型：
--
作者：
Fenoglio, Kristina A;Wu, Jie;Kerrigan, John F
下丘脑错构瘤（HH）是一种罕见的发育畸形，通常与药物治疗难治性的痉挛性癫痫发作相关。最近的证据支持 HH 固有的癫痫发作倾向。尽管临床上对这种情况的认识不断增加，但 HH 组织中癫痫发作的发生机制仍不清楚。本综述总结了 HH 神经元的组织化学和电生理学特性，并将这些发现与其他类型癫痫组织中发现的特征相关联。初步研究揭示了手术切除的 HH 组织中存在两个不同的神经元群。一组由表达γ-氨基丁酸（GABA）的小神经元组成，这些神经元主要成簇出现并表现出自发的节律性放电。第二组由大的、静止的、锥体样神经元组成，具有更广泛的树突和轴突分枝。我们提出，自发放电的小型 GABA 能神经元向大输出神经元发送抑制性投射并驱动其同步。这些观察结果构成了未来研究的基础，旨在阐明皮质下癫痫发生的机制。
The hypothalamic hamartoma (HH) is a rare developmental malformation commonly associated with gelastic seizures that are notoriously refractory to medical therapy. Recent evidence supports the intrinsic seizure propensity of HH. Despite increasing clinical recognition of this condition, the mechanisms of seizure genesis in HH tissue remain unclear. This review summarizes the histochemical and electrophysiological properties of HH neurons, and relates these findings to those characteristics identified in other types of epileptic tissue. Initial studies have revealed two distinct populations of neurons in surgically resected HH tissue. One group consisted of small gamma-aminobutyric acid (GABA)-expressing neurons that occurred principally in clusters and displayed spontaneous rhythmic firing. The second group was composed of large, quiescent, pyramidal-like neurons with more extensive dendritic and axonal arborization. We propose that the small, spontaneously firing GABAergic neurons send inhibitory projections to and drive the synchrony of large output neurons. These observations constitute the basis for future investigations aimed at elucidating the mechanisms of subcortical epileptogenesis.