ISSN:
1432-1920
Keywords:
Key words Adrenoleukodystrophy æ Localised proton NMR spectroscopy
;
Demyelination
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Abstract We have performed localised proton magnetic resonance spectroscopy (MRS) of the brain on four patients with X-linked adrenoleukodystrophy (X-ALD). The spectrum is characterised at the beginning of the disease by a decrease in N-acetylaspartate and phosphocreatine-creatine content. Choline is strongly increased, and lactate can be detected in some cases. A proton signal from the CH2 groups borne by free intracellular very long chain fatty acids can also be observed. Later in the disease, the levels of all metabolites, in particular NAA, decrease significantly. The progression of neurometabolism documented by MRS correlates well with MRI and clinical progression on follow-up study. In one case, the metabolic profile recorded by proton MRS was abnormal before any change occurred on MRI. Proton MRS of the brain might be the method of choice for monitoring patients with X-ALD, to screen presumed cases and to study the effects of treatment.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00593724
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