Clinical proton MR spectroscopy of neurodegenerative disease in childhood

PURPOSE: 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, neurotransm itters (glutamate) , intracellular mediators (inosi-tols) , 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 neurotransm itter neurotoxicity (increased glutamate, glutamine, and inositols). CONCLUSION: Proton MR spectros-copy may complement MR imaging in diagnostic assessment and therapeutic monitoring of neurodegenerative disorders