Monitoring disease progression using high-density motor unit number estimation in amyotrophic lateral sclerosis

2010 ◽  
Vol 42 (2) ◽  
pp. 239-244 ◽  
Author(s):  
Johannes P. van Dijk ◽  
Helenius J. Schelhaas ◽  
Ivo N. Van Schaik ◽  
Henny M.H.A. Janssen ◽  
Dick F. Stegeman ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Disease Progression ◽  
Motor Unit ◽  
High Density ◽  
Motor Unit Number Estimation ◽  
Lateral Sclerosis

scholarly journals Motor Unit Number Estimation in Evaluating Disease Progression in Patients with Amyotrophic Lateral Sclerosis

2010 ◽  
Vol 25 (9) ◽  
pp. 1359 ◽  
Author(s):  
Suk-Won Ahn ◽  
Su-Hyun Kim ◽  
Dong-Hoon Oh ◽  
Sung-Min Kim ◽  
Kyung Seok Park ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Disease Progression ◽  
Motor Unit ◽  
Motor Unit Number Estimation ◽  
Lateral Sclerosis

scholarly journals Motor Unit Number Estimation in Neuromuscular Disease

2008 ◽  
Vol 35 (2) ◽  
pp. 153-159 ◽  
Author(s):  
Omid Rashidipour ◽  
K. Ming Chan
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Unit ◽  
Neuromuscular Disease ◽  
Muscular Atrophy ◽  
Neuromuscular Diseases ◽  
Neuronal Loss ◽  
Response To Treatment ◽  
Motor Unit Number Estimation ◽  
Electrophysiological Method ◽  
Lateral Sclerosis

Motor unit number estimation (MUNE) is an electrophysiological method designed to quantify motor unit loss in target muscles of interest. Most of the techniques are noninvasive and are therefore well suited for longitudinal monitoring. In this brief review, we describe the more commonly used techniques and their applications in amyotrophic lateral sclerosis, poliomyelitis, spinal muscular atrophy and hereditary sensorimotor neuropathies. Findings in some of these studies offer important pathophysiological insights. Since conventional electrophysiologic methods are not sensible measures of motor neuronal loss, MUNE could play a potentially important role in the diagnosis, monitoring of disease progression and response to treatment in neuromuscular diseases in which motor unit loss is a major feature.

scholarly journals The rise and fall of fasciculations in amyotrophic lateral sclerosis

2020 ◽  
Vol 2 (1) ◽  
Author(s):  
James A Bashford ◽  
Aidan Wickham ◽  
Raquel Iniesta ◽  
Emmanuel M Drakakis ◽  
Martyn G Boutelle ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Unit ◽  
Surface Electromyography ◽  
Rating Scale ◽  
Muscle Power ◽  
Pooled Analysis ◽  
High Density ◽  
Mixed Effect ◽  
Density Surface ◽  
Lateral Sclerosis

Abstract Amyotrophic lateral sclerosis is a devastating neurodegenerative disease with a median survival of 3 years from symptom onset. Accessible and reliable biomarkers of motor neuron decline are urgently needed to quicken the pace of drug discovery. Fasciculations represent an early pathophysiological hallmark of amyotrophic lateral sclerosis and can be reliably detected by high-density surface electromyography. We set out to quantify fasciculation potentials prospectively over 14 months, seeking comparisons with established markers of disease progression. Twenty patients with amyotrophic lateral sclerosis and five patients with benign fasciculation syndrome underwent up to seven assessments each. At each assessment, we performed the amyotrophic lateral sclerosis-functional rating scale, sum power score, slow vital capacity, 30-min high-density surface electromyography recordings from biceps and gastrocnemius and the motor unit number index. We employed the Surface Potential Quantification Engine, which is an automated analytical tool to detect and characterize fasciculations. Linear mixed-effect models were employed to account for the pseudoreplication of serial measurements. The amyotrophic lateral sclerosis-functional rating scale declined by 0.65 points per month (P < 0.0001), 35% slower than average. A total of 526 recordings were analysed. Compared with benign fasciculation syndrome, biceps fasciculation frequency in amyotrophic lateral sclerosis was 10 times greater in strong muscles and 40 times greater in weak muscles. This was coupled with a decline in fasciculation frequency among weak muscles of –7.6/min per month (P = 0.003), demonstrating the rise and fall of fasciculation frequency in biceps muscles. Gastrocnemius behaved differently, whereby strong muscles in amyotrophic lateral sclerosis had fasciculation frequencies five times greater than patients with benign fasciculation syndrome while weak muscles were increased by only 1.5 times. Gastrocnemius demonstrated a significant decline in fasciculation frequency in strong muscles (−2.4/min per month, P < 0.0001), which levelled off in weak muscles. Fasciculation amplitude, an easily quantifiable surrogate of the reinnervation process, was highest in the biceps muscles that transitioned from strong to weak during the study. Pooled analysis of >900 000 fasciculations revealed inter-fasciculation intervals <100 ms in the biceps of patients with amyotrophic lateral sclerosis, particularly in strong muscles, consistent with the occurrence of doublets. We hereby present the most comprehensive longitudinal quantification of fasciculation parameters in amyotrophic lateral sclerosis, proposing a unifying model of the interactions between motor unit loss, muscle power and fasciculation frequency. The latter showed promise as a disease biomarker with linear rates of decline in strong gastrocnemius and weak biceps muscles, reflecting the motor unit loss that drives clinical progression.

Motor unit number index (MUNIX): A novel neurophysiological technique to follow disease progression in amyotrophic lateral sclerosis

2010 ◽  
Vol 42 (3) ◽  
pp. 379-384 ◽  
Author(s):  
Christoph Neuwirth ◽  
Sanjeev Nandedkar ◽  
Erik StåLberg ◽  
Markus Weber
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Disease Progression ◽  
Motor Unit ◽  
Lateral Sclerosis

P7-10 Asymmetry of motor unit number estimation (MUNE) in patients with amyotrophic lateral sclerosis

2010 ◽  
Vol 121 ◽  
pp. S142-S143
Author(s):  
J.E. Kim ◽  
S.W. Ahn ◽  
S.Y. Park ◽  
S.M. Kim ◽  
K.S. Park ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Unit ◽  
Motor Unit Number Estimation ◽  
Lateral Sclerosis
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