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

Abstract

Objective: To describe the fasciculation pattern in ALS and to analyse its clinical and pathophysiological significance. Methods: Ultrasound of 19 muscles was performed in 44 patients with a recent diagnosis (<90 days) of ALS. The numberof fasciculationswas recorded in each muscle and the muscle thickness and strengthwere additionally measured in limb muscles. A subgroup of patients were electromyographically assessed. Results: US was performed in 835 muscles and EMG was available in 263 muscles. US detected fasciculations more frequently than EMG. Fasciculations were widespread, especially in upper limbs onset patients and in the cervical region. Fasciculations’ number inversely associated with ALSFR-R and body mass index (BMI) and directly with BMI loss and upper motor neuron (UMN) impairment. Our statistical model suggest that fasciculations increasewith the initial lower motor neuron (LMN) degeneration, reach their peak when the muscle became mildly to moderately weak, decreasing afterwards with increasing muscle weakness and atrophy.