Nodular heterotopia: A neuropathological study of 24 patients undergoing surgery for drug-resistant epilepsy

Nodular heterotopia: A neuropathological study of 24 patients undergoing surgery for drug-resistant epilepsy
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DOI:
10.1111/j.1528-1167.2008.01717.x
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发表时间:
2009-01-01
期刊:
影响因子:
5.6
通讯作者:
Spreafico, Roberto
Spreafico, Roberto
中科院分区:
医学1区
文献类型:
--
作者:
Meroni, Alessandra;Galli, Carlo;Spreafico, Roberto

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尽管有详细的电临床和影像学资料,但只有少数关于结节性异位的神经病理学研究被发表。为探讨难治性癫痫患者手术切除的不同类型异位结节的神经病理学特征,对24例异位结节进行了常规和免疫细胞化学神经病理学检查,并与磁共振成像(MRI)、临床电图、神经病理学数据区分两组。第1组(14例患者,78%为Engel 1级)具有相似的特征,无论结节的大小、数量或位置如何,结节中的投射神经元和局部回路神经元与胶质细胞混合。13例患者有局灶性皮质发育不良。所有病例均经MRI证实。在第2组(10例患者,90%为Engel 1级)中,所有结节均位于颞叶内,并与海马硬化或神经节细胞胶质瘤相关。它们非常小(MRI未检测到),主要由投射神经元形成,没有神经胶质细胞的证据。两组患者均存在皮质发育不良,其神经病理学特征不同,提示其发病机制不同。在第2组中,与皮质发育不良和海马硬化或神经节细胞胶质瘤相关的结节形成的存在提出了一个关于颞叶所谓的双重病理学的问题。
Despite the availability of detailed electroclinical and imaging data, only a few neuropathological studies of nodular heterotopia have been published. The aim of this study was to describe the neuropathological features of various types of nodular heterotopia obtained from patients undergoing surgery for intractable epilepsy.Specimens of heterotopic nodules taken from 24 patients were neuropathologically investigated using routine and immunocytochemical procedures, and the data were compared with magnetic resonance imaging (MRI), electroclinical findings, and surgical outcomes.The neuropathological data distinguished two groups. Group 1 (14 patients, 78% in Engel class 1) had similar characteristics regardless of the size, number, or location of the nodules, with both projecting and local circuit neurons in the nodules intermingled with glial cells. Thirteen patients had focal cortical dysplasia. The nodules were identified by MRI in all cases. In group 2 (10 patients, 90% in Engel class 1), all of the nodules were within the temporal lobe and associated with hippocampal sclerosis or gangliogliomas. They were very small (undetected by MRI) and mainly formed by projecting neurons with no evidence of glial cells. All of the patients had cortical dysplasia.The distinctive neuropathological features of the nodules in the two groups suggest different etiopathogenetic mechanisms. In group 2, the presence of nodular formations in association with cortical dysplasia and either hippocampal sclerosis or ganglioglioma raises a question concerning so-called dual pathology in the temporal lobe.