Neuroradiological assessment of brain structure and function and its implication in the pathogenesis of West syndrome
Neuroradiological assessment of brain structure and function and its implication in the pathogenesis of West syndrome
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DOI：
10.1016/s0387-7604(01)00295-9
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发表时间：
2001-11-01
影响因子：
1.7
通讯作者：
Chugani, DC
中科院分区：
文献类型：
--
作者：
Juhász, C;Chugani, HT;Chugani, DC
磁共振成像(MRI)和正电子发射断层扫描(PET)的神经成像研究对我们理解West综合征有很大的贡献。皮质发育不良损害是痉挛婴儿最常见的MRI异常，但偶尔也会发现其他结构性损害。潜在的皮质发育不良可能在脑内髓鞘形成进展和灰白质分化不良变得明显之前不明显。许多皮质发育不良病变只能用葡萄糖代谢的PET扫描或α-氨基丁酸(A)(GABA(A))受体结合来检测。核磁共振和正电子发射计算机断层扫描的结果以及神经生理学观察有力地表明，婴儿痉挛是以皮层癫痫放电的形式开始的，在“关键的”发育期，可能会经历二次泛化，以年龄相关的机制出现痉挛。痉挛的发生往往与大脑皮层的功能成熟相吻合。根据婴儿痉挛患者的葡萄糖代谢PET扫描数据以及电生理和神经化学结果，我们推测，这种损害是局灶性或弥漫性皮质异常，在成熟的关键阶段，会导致与脑干中缝核的异常功能相互作用，这些核团广泛投射到大脑各处。中缝皮质投射可以调节EEG上所见的节律变化。突出的5-羟色胺能中缝-纹状体通路和下行脊髓通路可能与皮质放电的继发性泛化有关，从而导致相对对称的痉挛。其他因素(如遗传)很可能在West综合征特定年龄的电临床特征的表现中起作用。新近发展的正电子发射计算机断层扫描示踪剂可用于检测致痫脑区，也可用于研究5-羟色胺能(使用示踪剂α[C-11]甲基L色氨酸)和GABA能(使用[C-11]氟马西尼)神经递质系统的发育异常。这些系统与癫痫的发生有关，它们在West综合征的病理生理学中的作用可以通过未来的功能神经成像研究来进一步解决。(C)2001年爱思唯尔科学公司。版权所有。
Neuroimaging studies with magnetic resonance imaging (MRI) and positron emission tomography (PET) scanning a have contributed significantly to our understanding of West syndrome. Cortical dysplastic lesions are the most common abnormalities seen with MRI in infants with spasms, but other structural lesions are also detected occasionally. An underlying cortical dysplasia may not be apparent until myelination has advanced in the brain and poor gray-white matter differentiation becomes observable. Many cortical dysplastic lesions can only be detected using PET scanning of glucose metabolism or alpha -aminobutyric acid(A) (GABA(A)) receptor binding. The MRI and PET findings, together,vith neurophysiological observations, strongly suggest that infantile spasms are initiated as cortical epileptic discharges that, during a 'critical' developmental period, may undergo secondary generalization in an age-dependent mechanism to emerge as spasms. The onset of spasms often coincides with the functional maturation of cerebral cortex. Based on data from glucose metabolism PET scanning as well as electrophysiological and neurochemical findings on infants with spasms, we have postulated that the offending lesion is a focal or diffuse cortical abnormality which, at a critical stage of maturation, causes abnormal functional interactions with brainstem raphe nuclei which project widely throughout the brain. Raphe-cortical projections could mediate the hypsarrhythmic changes seen on EEG. The prominent serotonergic raphe-striatal pathway and descending spinal pathways may be responsible for secondary generalization of the cortical discharges to result in the relatively symmetric spasms. It is likely that additional factors (e.g. genetic) play a role in the manifestation of the age-specific electroclinical features of West syndrome. Recently developed PET tracers can be used to detect epileptogenic brain regions and also to investigate developmental abnormalities of serotonergic (using the tracer alpha[C-11]methyl-L-tryptophan) and GABAergic (using [C-11]flumazenil) neurotransmitter systems. These systems are implicated in epileptogenesis, and their involvement in the pathophysiology of West syndrome can be further addressed by future functional neuroimaging studies. (C) 2001 Elsevier Science BN. All rights reserved.