Movement Disorders (revue)

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Proton magnetic resonance spectroscopy in Steele-Richardson-Olszewski syndrome.

Identifieur interne : 004D91 ( Ncbi/Merge ); précédent : 004D90; suivant : 004D92

Proton magnetic resonance spectroscopy in Steele-Richardson-Olszewski syndrome.

Auteurs : C A Davie [Royaume-Uni] ; G J Barker ; C. Machado ; D H Miller ; A J Lees

Source :

RBID : pubmed:9380064

English descriptors

Abstract

Proton magnetic resonance spectroscopy, localized to the lentiform nucleus, was carried out in nine patients with a clinical diagnosis of Steele-Richardson-Olszewski syndrome (SRO) and in eight healthy age-matched controls. Three of the nine SRO patients had a so-called "eye of the tiger sign" with high signal in the globus pallidus surrounded by a ring of low signal on T2 weighted magnetic resonance imaging previously only reported in Hallervorden-Spatz disease. One of these patients had pathologically proven SRO at postmortem. The SRO group showed a significant reduction in the median concentration from N-acetyl groups (median, 6.87 mM; range, 4.92-10.59 mM; p < 0.015) compared with the control group (median, 9.85 mM; range, 9.26-11.0 mM). The N-acetylaspartate concentration was significantly reduced in seven of the nine patients studied. The reduction of the N-acetylaspartate-creatine ratio from the lentiform nucleus in the SRO group may reflect neuronal loss, occurring predominantly in the globus pallidus. Proton magnetic resonance spectroscopy may be a useful, noninvasive technique to help differentiate the various parkinsonian syndromes.

DOI: 10.1002/mds.870120525
PubMed: 9380064

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pubmed:9380064

Le document en format XML

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<div type="abstract" xml:lang="en">Proton magnetic resonance spectroscopy, localized to the lentiform nucleus, was carried out in nine patients with a clinical diagnosis of Steele-Richardson-Olszewski syndrome (SRO) and in eight healthy age-matched controls. Three of the nine SRO patients had a so-called "eye of the tiger sign" with high signal in the globus pallidus surrounded by a ring of low signal on T2 weighted magnetic resonance imaging previously only reported in Hallervorden-Spatz disease. One of these patients had pathologically proven SRO at postmortem. The SRO group showed a significant reduction in the median concentration from N-acetyl groups (median, 6.87 mM; range, 4.92-10.59 mM; p < 0.015) compared with the control group (median, 9.85 mM; range, 9.26-11.0 mM). The N-acetylaspartate concentration was significantly reduced in seven of the nine patients studied. The reduction of the N-acetylaspartate-creatine ratio from the lentiform nucleus in the SRO group may reflect neuronal loss, occurring predominantly in the globus pallidus. Proton magnetic resonance spectroscopy may be a useful, noninvasive technique to help differentiate the various parkinsonian syndromes.</div>
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