The rise and fall of fasciculations in amyotrophic lateral sclerosis

• Published in: Brain communications 2020-01, Vol.2 (1), p.fcaa018
• Main Authors: Bashford, James A, Wickham, Aidan, Iniesta, Raquel, Drakakis, Emmanuel M, Boutelle, Martyn G, Mills, Kerry R, Shaw, Chris E
• Format: Article
• Language: English
• Subjects: Original
• ISSN: 2632-1297; 2632-1297
• DOI: 10.1093/braincomms/fcaa018

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