Surgical treatment of West syndrome
Surgical treatment of West syndrome
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DOI：
10.1016/s0387-7604(01)00305-9
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发表时间：
2001-11-01
影响因子：
1.7
通讯作者：
Chugani, HT
中科院分区：
文献类型：
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作者：
Asano, E;Chugani, DC;Chugani, HT
在大多数患有West综合征的婴儿中，通过核磁共振成像(MRI)和正电子发射断层扫描(PET)发现局灶性或多灶性皮质病变，为治疗一些顽固性婴儿痉挛患者提供了一种手术方法。在进行皮质切除前，这些病变的位置应与局灶性发作和/或发作间期脑电异常的定位一致。当MRI或PET显示单个病变，且与脑电定位有很好的相关性时，手术治疗通常在癫痫控制和认知发展方面都是相当有利的。患有难治性隐源性婴儿痉挛的儿童的间质葡萄糖代谢PET扫描显示，约20%的病例出现单灶性皮质代谢低下。然而，在大多数情况下，多灶性非对称性低代谢提示多灶性潜在病变，可能是多灶性皮质发育不良。当葡萄糖代谢低下的模式是对称的，皮损病因的可能性较小，因此应考虑神经代谢或神经遗传障碍。因此，患有难治性隐源性痉挛的婴儿在PET上的葡萄糖低代谢模式是决定是否应该采取内科或外科方法的有用指南。为了通过手术获得最佳的认知结果，重要的是切除整个“伤害区”，而不仅仅是癫痫灶。我们用新的PET成像探针进行的研究试图提供对致痫区域的全面评估，包括“伤害性”皮质。我们使用了[C-11]氟马西尼(FMZ)，它标记了γ-氨基丁酸(A)(GABA(A))受体，并发现这在显示：(I)与内侧颞叶受累的受体结合减少，从而表明内侧颞叶结构被切除；(Ii)MRI病变周围的癫痫致痫区；(Iii)MRI阴性病例的癫痫起始区，以及(Iv)潜在的继发性癫痫灶特别有用。另一种新近发展的正电子发射计算机断层扫描探针，α[C-11]甲基-L-色氨酸(AMT)是5-羟色胺和犬尿氨酸代谢途径的前体，能够区分结节性硬化症患者和顽固性癫痫(包括婴儿痉挛)患者的致痫和非致痫结节。随后，我们在多灶性皮质发育不良患者中应用AMT PET来确定主要的癫痫灶，结果在癫痫控制方面是有希望的，但在认知发展方面没有。因此，用于癫痫的更新的、更具特异性的PET探头的引入导致了癫痫灶的改进和更准确的定位，这最终应该会改善West综合征癫痫手术的结果。(C)2001 Elsevier Science B.V.保留所有权利。
The discovery of focal or multifocal cortical lesions using magnetic resonance imaging (MRI) and Positron emission tomography (PET) scanning in the majority of infants with West syndrome has led to a surgical approach in the treatment of some patients with intractable infantile spasms. The locations of these lesions should be concordant with localization of focal ictal and/or interictal electroencephalographic (EEG) abnormalities prior to proceeding with cortical resection. When a single lesion is present on the MRI or PET, and there is good correlation with EEG localization, surgical treatment is generally quite favorable in terms of both seizure control and cognitive development. Interietal glucose metabolism PET scans in children with intractable cryptogenic infantile spasms show unifocal cortical hypometabolism in about 20% of cases. In the majority, however, multifocal asymmetric hypometabolism is suggestive of multifocal underlying lesions, possibly multifocal cortical dysplasia. When the pattern of glucose hypometabolism is symmetric, a lesional etiology is less likely, thus neurometabolic or neurogenetic disorders should be considered. Therefore, the pattern of glucose hypometabolism on PET in infants with intractable cryptogenic spasms is a useful guide to decide whether a medical or surgical approach should be undertaken, In order to achieve the best cognitive outcome with surgery, it is important to resect the entire 'nociferous' area rather than just the seizure focus. Our research with new PET imaging probes has attempted to provide a comprehensive evaluation of the epileptogenic zone including the 'nociferous' cortex. We have used [C-11]flumazenil (FMZ), which labels gamma aminobutyric acid(A) (GABA(A)) receptors, and have found this to be particularly useful in showing: (i) decreased receptor binding with medial temporal involvement thus indicating resection of medial temporal structures, (ii) the peri-lesional epileptogenic zone surrounding MRI lesions, (iii) the seizure onset zone in MRI-negative cases, and (iv) potential secondary epileptic foci. Another recently developed PET probe, alpha[C-11]methyl-L-tryptophan (AMT) which is a precursor for the serotonin and the kynurenine metabolism pathways, is capable of differentiating between epileptogenic and non-epileptogenic tubers in patients with tuberous sclerosis complex and intractable epilepsy (including infantile spasms). Subsequently, we have applied AMT PET in patients with multifocal cortical dysplasia to determine the predominant seizure focus, and the results have been promising with regard to seizure control but not cognitive development. Thus, the introduction of newer more specific PET probes for epilepsy has led to improved and more accurate localization of seizure foci that should ultimately improve outcome of epilepsy surgery in West syndrome. (C) 2001 Elsevier Science B.V. All rights reserved.