MR spectroscopy findings in early stages of motor neuron disease.
OBJECTIVE
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.


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.
van der Graaff
M M
MM
Departments of Neurology, Academic Medical Center, Amsterdam, the Netherlands. m.m.vandergraaff@amc.uva.nl
Lavini
C
C
Akkerman
E M
EM
Majoie
Ch B
ChB
Nederveen
A J
AJ
Zwinderman
A H
AH
Brugman
F
F
van den Berg
L H
LH
de Jong
J M B V
JM
de Visser
M
M
D000368:Aged; D000369:Aged, 80 and over; D015331:Cohort Studies; D042241:Early Diagnosis; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D009682:Magnetic Resonance Spectroscopy / Q000379:methods* / Q000592:standards; D008297:Male; D008875:Middle Aged; D009044:Motor Cortex / Q000378:metabolism; D016472:Motor Neuron Disease / Q000175:diagnosis* / Q000378:metabolism*; D009046:Motor Neurons / Q000166:cytology / Q000378:metabolism*; D009457:Neuroglia / Q000166:cytology / Q000378:metabolism; D010641:Phenotype; D011446:Prospective Studies; D012372:ROC Curve; D015203:Reproducibility of Results
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