Ictogenesis and symptomatogenesis of gelastic seizures in hypothalamic hamartomas: An ictal SPECT study
Ictogenesis and symptomatogenesis of gelastic seizures in hypothalamic hamartomas: An ictal SPECT study
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DOI：
10.1111/j.1528-1167.2010.02739.x
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发表时间：
2010-11-01
期刊：
影响因子：
5.6
通讯作者：
Murakami, Hiroatsu
中科院分区：
文献类型：
--
作者：
Kameyama, Shigeki;Masuda, Hiroshi;Murakami, Hiroatsu
目的：为了在地形学上定位下丘脑错构瘤（HHs）和弹性发作（GS）的致状区，我们分析了间歇期和间歇期单光子发射计算机断层扫描（SPECT）的数据。方法：21例HH患者（年龄2-36岁，平均13.8岁）行立体定向射频热凝（SRT）治疗，GS发病后立即行SPECT。SPECT数据的统计学分析采用减相式SPECT共配核磁共振成像（MRI） （SISCOM）和统计参数映射（SPM）。评估颅底高灌注区域的地形定位。结果：27项研究SISCOM显示13例患者（热HH组）16项研究HH界面区出现急性高灌注。在这些患者中，8例患者的11项研究中HH明显大于无高灌注HH（直径21.4 +/- 10.3 vs. 12.3 +/- 7.3 mm, p < 0.05， t检验）。在所有患者和热HH组中，SPM组间期和初期数据的个体差异分析显示，同侧下丘脑、丘脑和壳核的中背侧核、双侧脑桥被和对侧小脑下半月小叶的高灌注区显著（p < 0.001）。乳丘脑扣带通路未见高灌注。讨论：本研究证实，胚胎形成发生在HH界面区，因此应该是SRT的目标。我们认为丘脑桥小脑回路在GS的典型和自动症状发生中起重要作用，下丘脑和丘脑MD核可能参与癫痫性脑病。
P>Purpose:To topographically localize the ictogenic zone within hypothalamic hamartomas (HHs) and the symptomatogenic zone for gelastic seizure (GS), we analyzed data from both interictal and ictal single photon emission computed tomography (SPECT).Methods:Ictal SPECT was performed immediately after GS onset in 21 patients with HH (aged 2-36 years, mean 13.8 years) who underwent stereotactic radiofrequency thermocoagulation (SRT). SPECT data were statistically analyzed by means of subtraction ictal SPECT coregistered to magnetic resonance imaging (MRI) (SISCOM) and statistical parametric mapping (SPM). Topographic localization of ictal hyperperfusion areas was evaluated.Results:SISCOM obtained in 27 studies demonstrated ictal hyperperfusion in the HH interface zone in 16 studies of 13 patients (hot HH group). In these patients, HHs were significantly larger than those without hyperperfusion of HH in 11 studies of 8 patients (21.4 +/- 10.3 vs. 12.3 +/- 7.3 mm in diameter, p < 0.05, t-test). In all patients and in the hot HH group, SPM group analysis of individual differences between interictal and ictal data revealed significantly (p < 0.001) hyperperfused areas in the ipsilateral hypothalamus, mediodorsal (MD) nucleus of the thalamus and putamen, bilateral pontine tegmentum, and contralateral inferior semilunar lobule of the cerebellum. There was no hyperperfusion in the mammillothalamocingulate pathway.Discussion:The present study confirmed that ictogenesis occurs in the HH interface zone, which should accordingly be the target for SRT. We suggest that a thalamopontocerebellar circuit plays an important role for stereotypical and automatic symptomatogenesis of GS and that the hypothalamus and MD nucleus of the thalamus are potentially involved in epileptic encephalopathy.