Publications (1)8.31 Total impact
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Article: Hyperexcitability and amyotrophic lateral sclerosis.
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ABSTRACT: Although amyotrophic lateral sclerosis (ALS) was described some 150 years ago, there remain critical questions about the origin and progression of the disease. A recent focus of clinical research has been the potential role of excitotoxicity underlying the pathophysiology of ALS.(1) Several strands of evidence indicate that a net increase in excitatory neurotransmission in ALS results from reduced cortical inhibition. In this issue of Neurology®, Foerster and colleagues(2) add a further piece to this complex pathophysiologic puzzle, by identifying differences in γ-aminobutyric acid (GABA) content in the motor cortex of patients with ALS. Specifically, using a novel magnetic resonance spectroscopy approach, they found reduced cortical GABA in patients with ALS compared to controls. This in vivo characterization of GABA levels in patients with ALS supports the emerging evidence for cortical hyperexcitability in patients with sporadic and familial ALS. The data are also relevant to the urgently needed development of disease-specific biomarkers: while EMG is more sensitive than clinical examination for the detection of lower motor neuronal involvement, a robust biomarker is lacking that may identify subclinical upper motor neuron degeneration and thereby facilitate an early diagnosis of ALS.Neurology 04/2012; 78(20):1544-5. · 8.31 Impact Factor
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- Neurology (1)
Institutions
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2012
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University of New South Wales
- Prince of Wales Clinical School
Kensington, New South Wales, Australia
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