Clinical proton MR spectroscopy of neurodegenerative disease in childhood.
OBJECTIVE
To determine the contribution of MR spectroscopy in the assessment of childhood neurodegenerative disease.


METHODS
Fifty-one subjects (7 weeks to 17 years of age), 22 with either hereditary (n = 16) or acquired (n = 6) neurodegenerative disorders and 29 age-matched control subjects, were studied with combined proton MR spectroscopy and MR imaging. Single-voxel (2.0-8.0 cc) MR spectra were acquired at 1.5 T, with either short-echo-stimulated echoes and/or long-echo spin echoes.


RESULTS
MR spectra exhibited signals from n-acetyl-, creatine-, and choline-containing compounds, neurotransmitters (glutamate), intracellular mediators (inositols), and glycolytic products (lactate). Abnormal MR spectra in neurodegenerative disorders reflected: demyelination, neuronal loss, and gliosis (increased mobile lipid presence and reduction of n-acetylaspartate to choline); metabolic acidosis (lactate accumulation); and neurotransmitter neurotoxicity (increased glutamate, glutamine, and inositols).


CONCLUSIONS
Proton MR spectroscopy may complement MR imaging in diagnostic assessment and therapeutic monitoring of neurodegenerative disorders.
Tzika
A A
AA
Department of Radiology, Children's Hospital Medical Center, Cincinnati, OH 45229.
Ball
W S
WS
Vigneron
D B
DB
Dunn
R S
RS
Kirks
D R
DR
D000293:Adolescent; D000326:Adrenoleukodystrophy / Q000175:diagnosis / Q000378:metabolism; D000592:Amino Acid Metabolism, Inborn Errors / Q000175:diagnosis / Q000378:metabolism; D001921:Brain / Q000473:pathology; D001923:Brain Chemistry; D001927:Brain Diseases / Q000175:diagnosis* / Q000378:metabolism; D001928:Brain Diseases, Metabolic / Q000175:diagnosis / Q000378:metabolism; D001932:Brain Neoplasms / Q000175:diagnosis / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D016464:Lysosomal Storage Diseases / Q000175:diagnosis / Q000378:metabolism; D008279:Magnetic Resonance Imaging; D009682:Magnetic Resonance Spectroscopy; D008297:Male; D017237:Mitochondrial Encephalomyopathies / Q000175:diagnosis / Q000378:metabolism; D009410:Nerve Degeneration; D017253:Neurofibromatoses / Q000175:diagnosis / Q000378:metabolism