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Corticomotorneuronal hyper-excitability in amyotrophic lateral sclerosis
1Department of Neurology, University of British Columbia, Vancouver General Hospital, Canada.
Journal of the Neurological Sciences
|December 16, 1998
Summary
Motor unit behavior in Amyotrophic Lateral Sclerosis (ALS) shows altered corticomotoneuronal excitability. Findings suggest potential therapeutic targets involving glutamate and GABA mechanisms for ALS treatment.
Area of Science:
- Neuroscience
- Motor Control
- Neuromuscular Disorders
Background:
- Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disease affecting motor neurons.
- Understanding corticomotoneuronal integrity is crucial for ALS pathogenesis and treatment development.
Purpose of the Study:
- To investigate changes in motor unit behavior under cortical stimulation in ALS patients.
- To assess the integrity of the corticomotoneuronal pathways in ALS using peristimulus time histograms (PSTHs).
Main Methods:
- Analysis of voluntarily activated motor units subjected to threshold cortical stimuli.
- Construction of peristimulus time histograms (PSTHs) with 1-ms and 0.2-ms bins.
- Comparison of PSTH characteristics between 29 ALS patients and 8 age-matched controls.
Main Results:
- In ALS patients, the primary peak of the PSTH showed delayed onset and prolonged duration.
- Finer bin analysis revealed increased sub-components in the ALS PSTH primary peak.
- These findings suggest hyper-excitability of corticomotoneurons in ALS.
Conclusions:
- Hyper-excitability in ALS may be linked to glutamate or impaired GABA inhibition.
- Glutamate antagonists with anticonvulsant or GABergic properties may be therapeutic.
- GABA(B) agonists could be considered for future combination therapies in ALS.