ALTERATIONS OF BRAIN-METABOLITES IN METACHROMATIC LEUKODYSTROPHY AS DETECTED BY LOCALIZED PROTON MAGNETIC-RESONANCE SPECTROSCOPY IN-VIVO

被引：93
|
作者：
KRUSE, B [1 ]
HANEFELD, F [1 ]
CHRISTEN, HJ [1 ]
BRUHN, H [1 ]
MICHAELIS, T [1 ]
HANICKE, W [1 ]
FRAHM, J [1 ]
机构：
[1] MAX PLANCK INST BIOPHYS CHEM,D-37085 GOTTINGEN,GERMANY
来源：
JOURNAL OF NEUROLOGY | 1993年 / 241卷 / 02期
关键词：
METACHROMATIC LEUKODYSTROPHY; MYOINOSITOL BRAIN METABOLISM; MAGNETIC RESONANCE IMAGING; PROTON MAGNETIC RESONANCE SPECTROSCOPY;
D O I：
10.1007/BF00869766
中图分类号：
R74 [神经病学与精神病学];
学科分类号：
摘要：
The brain morphology and chemistry of seven children with late infantile (4/7) and juvenile (3/7) forms of metachromatic leukodystrophy (MLD) were investigated by magnetic resonance imaging (MRI) and localized proton magnetic resonance spectroscopy (MRS). Patients who were examined at least 6 months after the onset of symptoms (6/7) had severe leukodystrophic changes on MRI. Proton MRS revealed a marked reduction of the neuronal marker N-acetylaspartate in white and grey matter and elevated lactate in demyelinated areas. In contrast to other leukodystrophies MLD patients showed a generalized increase of brain myo-inositol (2- to 3-fold in white matter), indicating a specific role in the pathophysiology of demyelination in MLD.
引用
收藏
页码：68 / 74
页数：7
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