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
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
相关论文
共 28 条
  • [1] BRAND A, 1992, 11TH SOC MAGN RES ME, P649
  • [2] MULTIPLE-SCLEROSIS IN CHILDREN - CEREBRAL METABOLIC ALTERATIONS MONITORED BY LOCALIZED PROTON MAGNETIC-RESONANCE SPECTROSCOPY INVIVO
    BRUHN, H
    FRAHM, J
    MERBOLDT, KD
    HANICKE, W
    HANEFELD, F
    CHRISTEN, HJ
    KRUSE, B
    BAUER, HJ
    [J]. ANNALS OF NEUROLOGY, 1992, 32 (02) : 140 - 150
  • [3] CEREBRAL METABOLISM IN MAN AFTER ACUTE STROKE - NEW OBSERVATIONS USING LOCALIZED PROTON NMR-SPECTROSCOPY
    BRUHN, H
    FRAHM, J
    GYNGELL, ML
    MERBOLDT, KD
    HANICKE, W
    SAUTER, R
    [J]. MAGNETIC RESONANCE IN MEDICINE, 1989, 9 (01) : 126 - 131
  • [4] PROTON NMR-SPECTROSCOPY OF CEREBRAL METABOLIC ALTERATIONS IN INFANTILE PEROXISOMAL DISORDERS
    BRUHN, H
    KRUSE, B
    KORENKE, GC
    HANEFELD, F
    HANICKE, W
    MERBOLDT, KD
    FRAHM, J
    [J]. JOURNAL OF COMPUTER ASSISTED TOMOGRAPHY, 1992, 16 (03) : 335 - 344
  • [5] BRUHN H, 1992, 11TH SOC MAGN RES ME, P235
  • [6] BRUHN H, 1991, 10TH SOC MAGN RES ME, P400
  • [7] CLEMENTS RS, 1973, LANCET, V1, P1137
  • [8] LONGITUDINAL NEUROPHYSIOLOGIC STUDIES IN A PATIENT WITH METACHROMATIC LEUKODYSTROPHY FOLLOWING BONE-MARROW TRANSPLANTATION
    DHUNA, A
    TORO, C
    TORRES, F
    KENNEDY, WR
    KRIVIT, W
    [J]. ARCHIVES OF NEUROLOGY, 1992, 49 (10) : 1088 - 1092
  • [9] IMPROVEMENTS IN LOCALIZED PROTON NMR-SPECTROSCOPY OF HUMAN BRAIN - WATER SUPPRESSION, SHORT ECHO TIMES, AND 1-ML RESOLUTION
    FRAHM, J
    MICHAELIS, T
    MERBOLDT, KD
    BRUHN, H
    GYNGELL, ML
    HANICKE, W
    [J]. JOURNAL OF MAGNETIC RESONANCE, 1990, 90 (03): : 464 - 473
  • [10] FRAHM J, 1991, MAGNETIC RESONANCE I, P165