MAGNETIC RESONANCE SPECTROSCOPY AND NEUROPSYCHOLOGICAL FUNCTION

磁共振波谱与神经心理功能

• 批准号: 7375865
• 负责人: ELSA G SHAPIRO
• 金额: $ 0.14万
• 依托单位: UNIVERSITY OF MINNESOTA
• 依托单位国家: 美国
• 财政年份: 2005
• 资助国家: 美国
• 起止时间: 2005-12-01 至 2006-11-30
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/7375865
• 关键词: MAGNETIC; RESONANCE; SPECTROSCOPY; NEUROPSYCHOLOGICAL; FUNCTION

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Summary: Adrenoleukodystrophy is an X-linked disorder with the gene localized to Xq28. Very-long-chain fatty acids (VLCFA) accumulate in plasma, brain, and adrenal glands. One phenotype is a cerebral form which results in rapid deterioration and death, usually between ages 5 and 14. Although VLCFA levels in plasma serve to identify abnormal genotype, VLCFA levels cannot identify the rapidly progressive and fatal cerebral form (COCALD). Bone marrow transplantation (BMT) has been effective for many children with COCALD. We have found stability after BMT for up to five years for boys transplanted relatively early in the course of the disease. However, if performed too late, the outcomes are very poor. Thus, early identification of cerebral disease in boys with the biochemical abnormality is critical for positive outcome. Initial diagnosis is currently made with MR imaging together with neuropsychological testing, a rapid deterioration of function can have already occurred; sensitive early detection of such changes that herald cerebral disease is needed. Furthermore, after BMT, MR imaging has been sensitive to functional outcomes, a biological measure is needed that reflects functional change and informs about cerebral changes after BMT. MR spectroscopy offers the possibility of meeting both those needs. Preliminary evidence from our group using the 1.5 Tesla system and other groups indicate that MRS is sensitive to abnormalities associated with ALD. Such findings suggest the enormous potential for early identification and sensitive post-treatment monitoring of COCALD MRS. We have the opportunity to measure these brain metabolites using the more powerful 4Tesla scanner in this research that will yield more precise information. This study will examine the effectiveness of the 4Tesla system in detecting changes in neurochemical profile that may herald the onset of cerebral disease in conjunction with MRI and neuropsychological testing. In addition, we will examine changes in cerebral metabolites before and after bone marrow transplantation to determine whether MRS will more sensitively reflect functional changes as reflected on neuropsychological testing than does MRI or 1.5 T MRS> Patients selected in the study will be scanned in the 4 Tesla magnet at the CMRR. Patients under 8 will be sedated and those over 8 will not be sedated unless they are delayed and unable to stay still during the procedure.

该子项目是利用 NIH/NCRR 资助的中心拨款提供的资源的众多研究子项目之一。子项目和研究者 (PI) 可能已从另一个 NIH 来源获得主要资金，因此可以在其他 CRISP 条目中得到体现。列出的机构是中心的机构，不一定是研究者的机构。摘要：肾上腺脑白质营养不良是一种 X 连锁疾病，其基因定位于 Xq28。极长链脂肪酸 (VLCFA) 在血浆、大脑和肾上腺中积聚。一种表型是脑型，通常在 5 岁至 14 岁之间导致迅速恶化和死亡。虽然血浆中的 VLCFA 水平可用于识别异常基因型，但 VLCFA 水平无法识别快速进展和致命的脑型 (COCALD)。 骨髓移植 (BMT) 对许多患有 COCALD 的儿童有效。我们发现，在病程相对早期进行移植的男孩在 BMT 后的稳定性长达五年。但如果实施得太晚，效果会很差。因此，早期识别生化异常男孩的脑部疾病对于获得积极结果至关重要。目前初步诊断是通过磁共振成像和神经心理学测试进行的，功能可能已经迅速恶化；需要敏感地及早发现此类预示脑部疾病的变化。 此外，BMT 后，MR 成像对功能结果很敏感，需要一种生物测量来反映 BMT 后的功能变化并告知大脑变化。 磁共振光谱提供了满足这两种需求的可能性。我们使用 1.5 Tesla 系统的小组和其他小组的初步证据表明，MRS 对与 ALD 相关的异常很敏感。这些发现表明 COCALD MRS 的早期识别和敏感的治疗后监测具有巨大潜力。在这项研究中，我们有机会使用更强大的 4Tesla 扫描仪来测量这些大脑代谢物，这将产生更精确的信息。 这项研究将检验 4Tesla 系统在检测神经化学特征变化方面的有效性，这些变化可能预示着脑部疾病的发作，并结合 MRI 和神经心理学测试。此外，我们将检查骨髓移植前后脑代谢物的变化，以确定 MRS 是否会比 MRI 或 1.5 T MRS 更敏感地反映神经心理学测试所反映的功能变化> 研究中选择的患者将在 CMRR 下用 4 特斯拉磁铁进行扫描。 8 岁以下的患者将被注射镇静剂，而 8 岁以上的患者将不会被注射镇静剂，除非他们在手术过程中被延误或无法保持静止。