Localized 1H NMR spectroscopy in Canavan's disease: a report of two cases.
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.
Austin
S J
SJ
Sub-Department of Academic Radiology, Institute of Child Health, London, England.
Connelly
A
A
Gadian
D G
DG
Benton
J S
JS
Brett
E M
EM
D001224:Aspartic Acid / Q000031:analogs & derivatives / Q000378:metabolism; D001479:Basal Ganglia / Q000378:metabolism; D001921:Brain / Q000473:pathology; D002675:Child, Preschool; D002794:Choline / Q000378:metabolism; D003401:Creatine / Q000378:metabolism; D002549:Diffuse Cerebral Sclerosis of Schilder / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology; D005260:Female; D006801:Humans; D006859:Hydrogen; D007223:Infant; D009682:Magnetic Resonance Spectroscopy / Q000379:methods; D008297:Male; D009778:Occipital Lobe / Q000378:metabolism
D001224:Aspartic Acid; D006859:Hydrogen; C000179:N-acetylaspartate; D003401:Creatine; D002794:Choline