PT  - JOURNAL ARTICLE
AU  - M.M. van der Graaff
AU  - C. Lavini
AU  - E.M. Akkerman
AU  - Ch.B. Majoie
AU  - A.J. Nederveen
AU  - A.H. Zwinderman
AU  - F. Brugman
AU  - L.H. van den Berg
AU  - J.M.B.V. de Jong
AU  - M. de Visser
TI  - MR Spectroscopy Findings in Early Stages of Motor Neuron Disease
AID  - 10.3174/ajnr.A2217
DP  - 2010 Nov 01
TA  - American Journal of Neuroradiology
PG  - 1799--1806
VI  - 31
IP  - 10
4099  - http://www.ajnr.org/content/31/10/1799.short
4100  - http://www.ajnr.org/content/31/10/1799.full
SO  - Am. J. Neuroradiol.2010 Nov 01; 31
AB  - BACKGROUND AND PURPOSE: Upper motor neuron degeneration varies in different phenotypes of MND. We used single-voxel MR spectroscopy of the primary motor cortex to detect corticomotoneuron degeneration and glial hyperactivity in different phenotypes of MND with a relatively short disease duration, contributing to further delineation of the phenotypes. MATERIALS AND METHODS: We prospectively included patients with ALS-B, ALS-L, and PMA and compared their data with those of patients with PLS and healthy controls. Each cohort consisted of 12 individuals. Disease duration was <1 year in ALS and PMA, but longer in PLS by definition. Follow-up examination was at 6 months. We measured ALSFRS-R, finger- and foot-tapping speed, and levels of the following: 1) NAAx, 2) mIns, and 3) Glx in the primary motor cortex. RESULTS: At baseline, we found significantly decreased NAAx levels and increased mIns levels in PLS. Levels of NAAx and mIns in patients with ALS-L and ALS-B were not significantly different from those in controls, but NAAx levels were significantly lower compared with those in PMA. At follow-up, only in PMA was a decrease of NAAx demonstrated. Glx levels varied widely in all groups. Levels of NAAx and mIns correlated well with clinical variables. CONCLUSIONS: Metabolite changes suggest neuronal dysfunction and active glial involvement in PLS. The corticomotoneuron is affected in early ALS-B and ALS-L, but at a later stage also in PMA. MR spectroscopy data are useful to obtain insight into the disease process at the level of the upper motor neuron in various phenotypes of MND. ALSamyotrophic lateral sclerosisALS-Bbulbar onset ALSALSFRS-RRevised ALS Functional Rating ScaleALS-Llimb-onset ALSCIconfidence intervalGlxglutamate + glutamineLleftmInsmyo-inositolMNDmotor neuron diseaseMRSMR spectroscopyNAnot applicableNAAN-acetylaspartateNAAxN-acetylaspartate + N-acetyl aspartylglutamateNSnot significantPLSprimary lateral sclerosisPMAprogressive muscular atrophyRrightROCreceiver operating characteristic analysisVCvital capacity