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

Citation
B. Kruse et al., ALTERATIONS OF BRAIN-METABOLITES IN METACHROMATIC LEUKODYSTROPHY AS DETECTED BY LOCALIZED PROTON MAGNETIC-RESONANCE SPECTROSCOPY IN-VIVO, Journal of neurology, 241(2), 1993, pp. 68-74
Citations number
28
Categorie Soggetti
Clinical Neurology
Journal title
ISSN journal
03405354
Volume
241
Issue
2
Year of publication
1993
Pages
68 - 74
Database
ISI
SICI code
0340-5354(1993)241:2<68:AOBIML>2.0.ZU;2-L
Abstract
The brain morphology and chemistry of seven children with late infanti le (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 examin ed at least 6 months after the onset of symptoms (6/7) had severe leuk odystrophic changes on MRI. Proton MRS revealed a marked reduction of the neuronal marker N-acetylaspartate in white and grey matter and ele vated lactate in demyelinated areas. In contrast to other leukodystrop hies MLD patients showed a generalized increase of brain myo-inositol (2- to 3-fold in white matter), indicating a specific role in the path ophysiology of demyelination in MLD.