New insights into the pathophysiology of fasciculations in amyotrophic lateral sclerosis: An ultrasound study

Vázquez-Costa, Juan Francisco; Campins‐Romeu, Marina; Martínez-Payá, J.J.; Tembl, José Ignacio; Baño-Aledo, María Elena del; Ríos-Díaz, José; Fornés-Ferrer, Victoria; Chumillas, María José; Sevilla, Teresa

doi:10.1016/j.clinph.2018.09.014

Cited by 28 publications

(47 citation statements)

References 34 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The most striking implication from these results was the rise and subsequent fall of FF in amyotrophic lateral sclerosis biceps muscles. This non-linear pattern had been previously suggested after statistically modelling fasciculation counts using muscle ultrasound ( Vazquez-Costa et al , 2018 ) and might explain why a previous surface EMG study of FF did not show a significant linear change over time ( De Carvalho and Swash, 2016 ). To help establish this pattern, we divided the data into strong and weak muscles, a clinically meaningful and unambiguous distinction.…”

Section: Discussionsupporting

confidence: 53%

“…However, it is important to consider other key influences on fasciculation amplitude, including the depth of the MU, the effects of muscle atrophy and the size of the MU ( Barkhaus and Nandedkar, 1994 ). Future studies should attempt to elucidate the relative contributions of these factors by correlating surface EMG and appropriate imaging techniques, such as ultrasound and magnetic resonance imaging ( Vazquez-Costa et al , 2018 ; Whittaker et al , 2019 ).…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

The rise and fall of fasciculations in amyotrophic lateral sclerosis

Bashford

Wickham

Iniesta

et al. 2020

Brain Communications

View full text Add to dashboard Cite

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.

show abstract

Section: Discussionsupporting

confidence: 53%

Section: Discussionmentioning

confidence: 99%

The rise and fall of fasciculations in amyotrophic lateral sclerosis

Bashford

Wickham

Iniesta

et al. 2020

Brain Communications

View full text Add to dashboard Cite

show abstract

“…Vázquez- have also added to our understanding of ALS and the role of fasciculations. 19 Forty-four patients with ALS underwent muscle ultrasound imaging within 90 days of their initial diagnosis. Nineteen muscles were imaged in each patient for 30 seconds.…”

Section: Muscle Ultrasound In Alsmentioning

confidence: 99%

Ultrasound in the Diagnosis and Monitoring of Amyotrophic Lateral Sclerosis: A Review

Hobson‐Webb

Simmons

2019

Muscle and Nerve

View full text Add to dashboard Cite

Neuromuscular ultrasound is complementary to electrodiagnostic (EDx) testing and is useful in enhancing the diagnosis of mononeuropathies, peripheral nerve trauma, and demyelinating polyneuropathies. There is increasing interest in using ultrasound both to aid in the diagnosis of amyotrophic lateral sclerosis (ALS) and to monitor its progression. In this article we review the relevant literature on ultrasound in ALS. Ultrasound is more sensitive than EDx in identifying fasciculations in patients with ALS. It can detect decreased muscle thickness, increased muscle echointensity and echovariance, and reduced peripheral nerve size in these patients. Ultrasound is also a helpful tool in assessment of diaphragm function. Although additional studies are required to define the exact role of ultrasound in the evaluation and monitoring of ALS, it can improve the diagnostic yield in patients when ALS is suspected, but insufficiently supported, by clinical and EDx examinations. Muscle Nerve 60: 114–123, 2019

show abstract

“…Another potentially useful finding in our study was that fasciculations are observed uncommonly in proximal muscles. Consequently, the finding of fasciculations in proximal muscles may prove to be an additional marker for neuromuscular pathology and enhance the sensitivity of US in the clinic, with a special relevance for amyotrophic lateral sclerosis (ALS), which is characterized by frequent fasciculations in proximal muscles . In contrast, we found frequent fasciculations in distal muscles, at the FDI and EDB (13–17%), and a very high frequency of fasciculations in the AHB (39–63%), which suggests low specificity of fasciculations in these locations.…”

Section: Discussionmentioning

confidence: 71%

Quantitative sonographic evaluation of muscle thickness and fasciculation prevalence in healthy subjects

et al. 2019

View full text Add to dashboard Cite

Background In the current study, we aimed to determine normative values for muscle thickness and fasciculation prevalence in healthy subjects. Methods We performed a prospective study from October to December 2018 in 65 healthy subjects. All subjects underwent quantitative sonographic evaluation of muscle thickness and fasciculation prevalence in the following 8 muscles: Biceps brachii, abductor pollicis brevis, first dorsal interosseous, abductor digiti minimi, quadriceps, tibialis anterior, extensor digitorum brevis, and abductor hallucis brevis. Results Subject ages ranged from 21 to 82 years, with 63% women. Normative values for muscle thickness were determined using the fifth percentile. Multivariate regression analysis showed that sex, age, body mass index, and hand dominance affected muscle thickness. Fasciculations were observed frequently only in distal muscles. Conclusions Normal values for muscle thickness were determined, and may enhance neuromuscular ultrasound sensitivity and serve as a basis for future studies. Larger series are needed to confirm these values.

show abstract

New insights into the pathophysiology of fasciculations in amyotrophic lateral sclerosis: An ultrasound study

Cited by 28 publications

References 34 publications

The rise and fall of fasciculations in amyotrophic lateral sclerosis

The rise and fall of fasciculations in amyotrophic lateral sclerosis

Ultrasound in the Diagnosis and Monitoring of Amyotrophic Lateral Sclerosis: A Review

Quantitative sonographic evaluation of muscle thickness and fasciculation prevalence in healthy subjects

Contact Info

Product

Resources

About