Abstract
Two children with Canavan's Disease, an autosomal recessive leukodystrophy, were studied by localized 1H spectroscopy. The N-acetylaspartate (NAA) signal intensity was high relative to other metabolite signals, and the signal intensity from choline-containing compounds was low. These findings are discussed in relation to a possible role for NAA in normal myelination.
MeSH terms
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Aspartic Acid / analogs & derivatives
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Aspartic Acid / metabolism
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Basal Ganglia / metabolism
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Brain / pathology
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Child, Preschool
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Choline / metabolism
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Creatine / metabolism
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Diffuse Cerebral Sclerosis of Schilder / diagnosis*
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Diffuse Cerebral Sclerosis of Schilder / genetics
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Diffuse Cerebral Sclerosis of Schilder / pathology
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Female
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Humans
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Hydrogen
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Infant
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Magnetic Resonance Spectroscopy* / methods
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Male
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Occipital Lobe / metabolism
Substances
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Aspartic Acid
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Hydrogen
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N-acetylaspartate
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Creatine
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Choline