Abstract
Amyotrophic lateral sclerosis (ALS) is a rapidly progressive degenerative neurological disease of unknown etiology. As the first step in evaluating systematically neurotransmitter metabolism in ALS, we measured norepinephrine (NE) in plasma and cerebrospinal fluid (CSF) by radioimmunoassay and γ-aminobutyric acid (GABA) in CSF by radioreceptor assay of 34 patients with ALS, 31 patients with myopathies but no central nervous system disease and 167 patients with various neurological disorders. CSF GABA was significantly decreased in ALS patients compared to myophathy ( p<0.05) and neurological ( p<0.01) patients. However CSF NE was significantly increased ( p<0.01) with respect to either comparison group. This dichotomy in neurotransmitter concentrations could result from a single neurotoxin leading to neuronal degeneration and a decrease in glutamic acid decarboxylase. The resultant decrease in GABA which is inhibitory on noradrenergic neurons could lead to the observed elevation in CSF NE due to activation of tyrosine hydroxylase.
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