Proton magnetic resonance spectroscopy in steele-richardson-olszewski syndrome
✍ Scribed by Charles A. Davie; Gareth J. Barker; Christine Machado; David H. Miller; Dr. Andrew J. Lees
- Publisher
- John Wiley and Sons
- Year
- 1997
- Tongue
- English
- Weight
- 638 KB
- Volume
- 12
- Category
- Article
- ISSN
- 0885-3185
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✦ Synopsis
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 m__M__; range, 4.92–10.59 m__M__; p < 0.015) compared with the control group (median, 9.85 m__M__; range, 9.26–11.0 m__M__). 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.
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A deficit of fatty alcohol:NAD+ oxidoreductase complex (FAO) activity has been detected in patients with the Sjögren-Larsson syndrome (SLS). A moderate decrease in FAO activity has also been reported in heterozygote SLS subjects. Abnormal peaks were detected with proton magnetic resonance spectrosco