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儿童肾上腺脑白质营养不良:质子磁共振波谱评估

Childhood adrenoleukodystrophy: assessment with proton MR spectroscopy.

作者信息

Tzika A A, Ball W S, Vigneron D B, Dunn R S, Nelson S J, Kirks D R

机构信息

Department of Radiology, Children's Hospital Medical Center, Cincinnati, Ohio.

出版信息

Radiology. 1993 Nov;189(2):467-80. doi: 10.1148/radiology.189.2.8210375.

DOI:10.1148/radiology.189.2.8210375
PMID:8210375
Abstract

PURPOSE

Image-guided, single-voxel, localized proton magnetic resonance (MR) spectroscopy was performed to assess white matter in childhood adrenoleukodystrophy (ALD).

MATERIALS AND METHODS

Eleven X-linked ALD subjects, seven with neurologic symptoms or white matter lesions at MR imaging and four asymptomatic patients, were compared with nine aged-matched, healthy volunteers.

RESULTS

Compared with those from normal white matter, MR spectra from white matter lesions (n = 6) showed 65% reduction in the ratio of N-acetyl aspartate (NAA) to total creatine (tCr) (P < .01); 55% increase in the ratio of choline-containing compounds (Cho) to tCr (P < .02); substantial levels of tCr; 94% increase in the ratio of glutamate, glutamine, or inositol to tCr (P < .02); and lactate accumulation in four patients. Patients without brain lesions (n = 4) exhibited a 51% increase in Cho-to-tCr ratio (P < .01) and 11% nonsignificant increase in NAA-to-tCr ratio.

CONCLUSION

Proton MR spectroscopy may prove a valuable technique for noninvasive diagnostic and prognostic assessment of ALD.

摘要

目的

采用影像引导下的单体素局部质子磁共振(MR)波谱分析来评估儿童肾上腺脑白质营养不良(ALD)中的白质情况。

材料与方法

将11名X连锁ALD患者(7名在MR成像时有神经系统症状或白质病变,4名无症状患者)与9名年龄匹配的健康志愿者进行比较。

结果

与正常白质的MR波谱相比,白质病变区域(n = 6)的波谱显示N - 乙酰天门冬氨酸(NAA)与总肌酸(tCr)的比值降低了65%(P <.01);含胆碱化合物(Cho)与tCr的比值增加了55%(P <.02);tCr水平较高;谷氨酸、谷氨酰胺或肌醇与tCr的比值增加了94%(P <.02);4名患者有乳酸蓄积。无脑病变的患者(n = 4)显示Cho与tCr的比值增加了51%(P <.01),NAA与tCr的比值有11%的增加但无统计学意义。

结论

质子MR波谱分析可能是一种用于ALD无创诊断和预后评估的有价值技术。

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