A perfusion-metabolic mismatch in Sturge-Weber syndrome: a multimodality imaging study.
A perfusion-metabolic mismatch in Sturge-Weber syndrome: a multimodality imaging study.
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Sturge-Weber 综合征的灌注代谢不匹配：多模态成像研究
DOI：
10.1016/j.braindev.2011.10.004
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发表时间：
2012-08
影响因子：
1.7
通讯作者：
Juhász C
中科院分区：
文献类型：
--
作者：
Alkonyi B;Miao Y;Wu J;Cai Z;Hu J;Chugani HT;Juhász C
我们将灌注加权成像（PWI）与2-脱氧-2 [18 F]氟-D-葡萄糖（FDG）正电子发射断层扫描（PET）相结合，以研究Sturge-Weber综合征（SWS）儿童局部代谢和灌注异常与其临床相关性之间的关系。15名单侧SWS儿童（年龄：0.9-10岁）前瞻性接受了高分辨率PWI和FDG PET。皮质下白色物质（WM）脑血流（CBF）的区域（脑叶）不对称指数（AI）与相应的皮质FDG摄取不对称性、软脑膜血管畸形的程度和临床癫痫发作变量相关。在6例患者中，所有受血管畸形影响的叶均观察到皮质葡萄糖代谢和/或皮质下WM CBF异常，并延伸至未受异常软脑膜血管影响的叶。较低的CBF与颞叶、顶叶和枕叶皮质葡萄糖代谢较低相关（p≤0.02）。虽然在大多数情况下灌注减少与代谢低下相关，但局部CBF增加（见于6例患者）通常与相对轻度或无代谢低下相关。24个大脑半球葡萄糖代谢正常的10个大脑半球出现异常灌注。癫痫发作频率高与严重的顶枕叶灌注不足相关（p≤0.03），而癫痫持续时间长与额叶代谢低下相关（p=0.015）。区域灌注和皮质代谢异常可以延伸到受软脑膜血管畸形影响的脑叶之外，并与SWS的癫痫相关。尽管灌注和代谢之间存在一般相关性，但在上覆皮质中保留皮质代谢的WM灌注增加是灌注/代谢不匹配的常见模式。这可能代表了一个疾病阶段，其中皮质功能得到保留，而增加的WM灌注通过深静脉系统提供了皮质的侧支引流。
We combined perfusion weighted imaging (PWI) with 2-deoxy-2[18F]fluoro-D-glucose (FDG) positron emission tomography (PET) to study the relationship between regional metabolic and perfusion abnormalities and their clinical correlates in children with Sturge-Weber syndrome (SWS). Fifteen children (age: 0.9-10 years) with unilateral SWS underwent high-resolution PWI and FDG PET prospectively. Regional (lobar) asymmetry indices (AIs) of subcortical white matter (WM) cerebral blood flow (CBF) were correlated with corresponding cortical FDG uptake asymmetries, extent of leptomeningeal vascular malformation and clinical seizure variables. Abnormal cortical glucose metabolism and/or subcortical WM CBF were seen in all lobes affected by vascular malformation and extended to lobes not affected by abnormal pial vessels in 6 patients. Lower CBF was associated with lower cortical glucose metabolism in the temporal, parietal and occipital lobes (p≤0.02). While decreased perfusion was associated with hypometabolism in most cases, increased regional CBF (found in 6 patients) was commonly associated with relatively mild or no hypometabolism. Ten of 24 cerebral lobes with normal glucose metabolism in the affected hemisphere showed abnormal perfusion. High seizure frequency was associated with severe parieto-occipital hypoperfusion (p≤0.03), while long duration of epilepsy was related to frontal lobe hypometabolism (p=0.015). Regional perfusion and cortical metabolic abnormalities can extend beyond lobes affected by leptomeningeal vascular malformations and are related to epilepsy in SWS. Despite a general correlation between perfusion and metabolism, increased WM perfusion with preserved cortical metabolism in overlying cortex is a common pattern of a perfusion/metabolic mismatch. This may represent a disease stage where cortical function is preserved while increased WM perfusion provides collateral drainage of cortex via the deep vein system.