Neuroimaging studies in Rett syndrome
Neuroimaging studies in Rett syndrome
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DOI：
10.1016/s0387-7604(01)00381-3
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发表时间：
2001-12-01
影响因子：
1.7
通讯作者：
Johnston, MV
中科院分区：
文献类型：
--
作者：
Naidu, S;Kaufmann, WE;Johnston, MV
神经影像学是一种无创确定大脑结构和体内功能状态的关键工具。现在多种方法能够确定大脑解剖和神经化学变化的各个方面，并且已在瑞特综合征患者中得到有效应用，以了解这种神经发育障碍的生物学基础。我们研究所进行的研究包括磁共振成像（MRI）的体积分析、磁共振波谱（MRS）、弥散张量成像（DTI）、利用MRI进行的脑血流测量以及正电子发射断层扫描（PET）。这些研究对这种疾病临床特征的潜在机制提供了相当多的见解。体积分析表明，瑞特综合征患者大脑体积减小是由于大脑灰质和白质的全面减少所致。额叶的选择性易损性通过血流优先减少、MRS显示胆碱增加和N - 乙酰天冬氨酸（NAA）减少以及（F - 18）-氟脱氧葡萄糖（FDG）PET扫描显示这些相同区域葡萄糖摄取增加得到证明。我们假设葡萄糖摄取增加与突触中谷氨酸循环增加有关。由此对发育中的大脑造成的神经兴奋性毒性损伤导致了这种疾病I期和II期常见的癫痫发作、行为障碍和呼吸不规则。（C）2001爱思唯尔科学出版社。保留所有权利。
Neuroimaging is a key instrument for determining structural and in vivo functional status of the brain, non-invasively. Multiple approaches can now determine aspects of anatomic and neurochemical changes in brain, and have been utilized effectively in Rett Syndrome patients to understand the biological basis of this neurodevelopmental disorder. Studies performed at our institute include volumetric analyses or MRI, magnetic resonance spectroscopy (MRS), diffusion tensor imaging (DTI), cerebral blood flow measurements with MRI. and positron emission tomography scans (PET). These studies have provided considerable insight into mechanisms underlying the clinical features of this disease. Volumetric analyses suggest that decreased brain volume in RS results from global reductions in both gray and white matter of the brain. A selective vulnerability of the frontal lobes is evidenced by the preferential reduction of blood flow. increased choline and reduced n-acetyl aspartate (NAA) by MRS, and increased glucose uptake in these same regions its shown by (F-18)-fluorodeoxyglucose (FDG) PET scans. We hypothesize that the increased glucose uptake relates to increased glutamate cycling in synapses, The resulting neuroexcitotoxic injury to the developing brain contributes to the seizures, behavioral disturbance and respiratory irregularities commonly wen in phases I and 2 of this disorder. (C) 2001 Elsevier Science B.V. All rights reserved.