White matter lesions in Fabry disease occur in 'prior' selectively hypometabolic and hyperperfused brain regions
White matter lesions in Fabry disease occur in 'prior' selectively hypometabolic and hyperperfused brain regions
复制标题
DOI：
10.1016/j.brainresbull.2003.09.021
复制
发表时间：
2003-12-30
影响因子：
3.8
通讯作者：
Schiffmann, R
中科院分区：
文献类型：
--
作者：
Moore, DF;Altarescu, G;Schiffmann, R
法布里病是一种与早发性卒中相关的X连锁疾病。我们先前发现法布里病患者的脑血流量（CBF）显著升高。本研究旨在确定法布里病患者静息CBF升高主要是脑血管异常还是继发于神经元代谢增强。并探讨了脑代谢和脑血流与Fabry白质脑病的关系。我们使用18-氟脱氧葡萄糖（FDG）和PET测量了16例Fabry病患者（7例脑白质疏松性病变，9例无脑白质疏松性病变）和7例对照组的整体和局部脑葡萄糖代谢率。MRI液体衰减反转恢复（FLAIR）研究也进行了患者和对照组。所有对照受试者的MRI FLAIR研究均正常，无高信号深部白色病变（WML）。将患者分为FLAIR病变组和非FLAIR病变组。我们没有发现Fabry病患者存在脑葡萄糖高代谢的证据。相反，在Fabry病非病变组中，特别是在深层白色物质中发现显著降低的局部脑葡萄糖代谢（rCMRGlu），并且在病变组中加重。与对照组相比，非病变患者的病变易感区域相对高灌注。我们的结论是rCBF升高和白色物质rCMRGlu降低表明代谢和血流之间的分离，提示慢性深部白色物质代谢不足。爱思唯尔公司出版
Fabry disease is an X-linked disorder associated with early onset stroke. We previously found a significantly elevated cerebral blood flow (CBF) in patients with Fabry disease. We set to determine whether elevated resting CBF in Fabry disease is primarily a cerebrovascular abnormality or is secondary to enhanced neuronal metabolism. The relationship of cerebral metabolism and blood flow to Fabry leukoencephalopathy was also investigated. We measured the global and regional cerebral metabolic rate of glucose using 18-fluoro-deoxyglucose (FDG) and PET in 16 patients with Fabry disease (7 patients with leukoaraiotic lesions and 9 without) and in 7 control subjects. MRI fluid attenuated inversion recovery (FLAIR) studies were also performed in the patient and control groups. All control subjects had normal MRI FLAIR studies with no high-signal deep white matter lesions (WML). Patients were partitioned into FLAIR lesion and non-FLAIR lesion groups. We found no evidence of cerebral glucose hypermetabolism in Fabry disease. On the contrary, significantly decreased regional cerebral glucose metabolism (rCMRGlu) was found particularly in the deep white matter in the Fabry non-lesion group and exacerbated in the lesion group. Lesion-susceptible regions were relatively hyperperfused in non-lesion patients compared to the control group. We conclude that the elevated rCBF and decreased white matter rCMRGlu indicates a dissociation between metabolism and blood flow suggesting chronic deep white matter metabolic insufficiency. Published by Elsevier Inc.