Abstract
Frontotemporal lobar degeneration is responsible for the cognitive abnormalities seen in patients with ALS. We sought to evaluate the in vivo neurochemical changes associated with this pathology indicative of neuronal loss and gliosis. Twenty-four patients with ALS (2 with ALS-FTD) and 15 healthy controls were studied. High-field proton MR spectroscopy of the mesial prefrontal cortex was used to determine concentrations of NAA and mIns, markers of neuronal integrity and gliosis, respectively. Metabolite concentrations were correlated with cognitive tests (verbal fluency, ACE). NAA/mIns was decreased 17% (P =.002). Abnormalities were present to a lesser degree in the individual metabolites NAA (decreased 9%; P =.08) and mIns (increased 11%; P =.06) than the ratio of the 2 metabolites. These measures did not correlate significantly with verbal fluency or the ACE. Prefrontal lobe degeneration exists in patients with ALS as indicated by an abnormal mesial prefrontal cortex neurochemical profile. Further study is necessary to determine the potential utility of the NAA/mIns ratio as a biomarker for frontal lobe degeneration in ALS.
Highlights
AND PURPOSE: Frontotemporal lobar degeneration is responsible for the cognitive abnormalities seen in patients with ALS
Neuronal inclusions in FTLD associated with motor neuron disease are ubiquitinimmunoreactive and - and ␣-synuclein negative
TDP-43 is present within ubiquinated inclusions in cases of FTLD without motor neuron disease as well as in sporadic ALS
Summary
The gain in number of metabolites accessible with other methods, namely, those incorporating short echo times, must be weighed with the inaccuracies secondary to spectral overlap and the emergence of peaks from macromolecules and lipids.
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