Quantitative measurement with localized H-1 MR spectroscopy in children with Canavan's disease

被引：53

作者：

Wittsack, HJ ^{[1
]}

Kugel, H ^{[1
]}

Roth, B ^{[1
]}

Heindel, W ^{[1
]}

机构：

[1] UNIV COLOGNE, DEPT DIAGNOST RADIOL, D-50924 COLOGNE, GERMANY

来源：

JOURNAL OF MAGNETIC RESONANCE IMAGING | 1996年 / 6卷 / 06期

关键词：

MR spectroscopy; brain; metabolism; children; central nervous system; Canavan's disease; N-acetylaspartic acid; proton spectroscopy; quantitation;

D O I：

10.1002/jmri.1880060609

中图分类号：

R8 [特种医学]; R445 [影像诊断学];

学科分类号：

1002 ; 100207 ; 1009 ;

摘要：

Canavan's disease is an autosomal recessive hereditary leukodystrophy resulting from deficiency of the enzyme aspartoacylase, Two children suffering from this metabolic brain disease were examined using image-guided localized proton spectroscopy, The absolute concentrations of metabolites were determined, These data demonstrate, for the first time, that the well known increase of the N-Acetylaspartic acid (NAA)/Cho ratio in this disease may be not only due to a reduction of choline-containing compounds in brain tissue but, at least in specific cases, also due to an increase of the NAA concentration, which is a result of the enzyme defect.

引用

页码：889 / 893

页数：5

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