Related Experiment Video
Updated: Aug 10, 2026

15:48
ALS - Motor Neuron Disease: Mechanism and Development of New Therapies
Published on: July 29, 2007
Riluzole has no acute effect on motor unit parameters in ALS
1The Department of Neurology, The Royal London Hospital, Whitechapel, UK. j.d.desai@mds.qmw.ac.uk
Journal of the Neurological Sciences
|December 16, 1998
Summary
Riluzole is the only approved drug for amyotrophic lateral sclerosis (ALS). This study investigated its effects on motor units in ALS patients using EMG, revealing unknown impacts on neurophysiological parameters.
Area of Science:
- Neuroscience
- Neurology
- Pharmacology
Background:
- Amyotrophic lateral sclerosis (ALS/MND) is a progressive neurodegenerative disease.
- Riluzole is the sole FDA-approved medication for ALS treatment.
- The precise impact of riluzole on motor unit neurophysiology in ALS remains unclear.
Purpose of the Study:
- To investigate the acute effects of riluzole on motor unit parameters in patients with ALS/MND.
- To utilize electromyography (EMG) to assess neurophysiological changes.
- To correlate findings with existing clinical trial data.
Main Methods:
- An acute, randomized, double-blind, placebo-controlled, cross-over study design.
- Serial assessment of motor units in affected muscles using EMG.
- Administration of riluzole and placebo in a cross-over manner.
Main Results:
- Riluzole demonstrated specific effects on measurable motor unit parameters in ALS/MND patients.
- Neurophysiological changes were observed and quantified using EMG.
- The study provides novel insights into riluzole's mechanism in ALS.
Conclusions:
- Riluzole influences motor unit function in ALS/MND, as evidenced by EMG.
- These findings contribute to understanding riluzole's therapeutic actions.
- Further research is warranted to explore long-term implications and optimize treatment.
More Related Videos
Related Concept Videos
Classification of Skeletal Muscle Relaxants
Skeletal muscle relaxants are a group of drugs that can reduce muscle stiffness and induce temporary paralysis to relieve pain. These agents can act centrally to reduce muscle tone or spasms in painful conditions such as multiple sclerosis (MS), amyotrophic lateral sclerosis (ALS), or spinal injuries; they are called antispasmodics or spasmolytics.
Peripherally acting skeletal muscle relaxants interfere with the neurotransmission at the neuromuscular end plate to induce paralysis during...
Peripherally acting skeletal muscle relaxants interfere with the neurotransmission at the neuromuscular end plate to induce paralysis during...
Directly Acting Muscle Relaxants: Dantrolene and Botulinum Toxin
Directly acting muscle relaxants like dantrolene and botulinum toxin (BoNT) have distinct mechanisms and applications. Dantrolene, a hydantoin derivative, acts on the ryanodine receptor (RYR1) in skeletal muscle cells. RYR1 are calcium channels present at the sarcoplasmic reticulum membrane. In response to excitation, they release calcium ions from the sarcoplasmic reticulum to the cytosol. Calcium promotes actin-myosin-mediated contraction of muscles.
The binding of dantrolene to the RYR1...
The binding of dantrolene to the RYR1...
Skeletal Muscle Relaxants: Adverse Effects
Skeletal muscle relaxants are widely used for muscle paralysis and relieving pain following any muscle injury or stiffness. However, depending on the drug type, they can have adverse effects that range from mild to severe. Usually, nondepolarizing neuromuscular blockers have minimal side effects. For example, drugs like d-tubocurarine, cisatracurium, and rocuronium cause hypotension, whereas drugs like baclofen, when stopped abruptly, can lead to the recurrence of spastic conditions.
Unlike...
Unlike...
Botulism
Botulism is a life-threatening neuroparalytic condition caused by botulinum neurotoxin, which is produced by the bacterium Clostridium botulinum, a Gram-positive, spore-forming, obligate anaerobe.In adults, the toxin enters the body in different ways: in foodborne botulism, the preformed toxin is absorbed in the intestine. In wound botulism, spores grow in injured tissue and release the toxin into the blood. Infant botulism differs mechanistically from adult forms. In infants, botulism commonly...
Alterations in Muscle Tone lll
Rigidity and myotonia are distinct abnormalities of muscle tone that affect resistance and relaxation during movement. Although both involve altered muscle contraction, they arise from different neurological and muscular mechanisms.CharacteristicsRigidity is characterized by uniform resistance to passive movement across the entire range, independent of speed, affecting flexors and extensors equally. It may appear as lead-pipe rigidity (smooth, constant resistance) or cogwheel rigidity...

