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

Highlights • Fasciculations are more frequent in proximal and cervical muscles and in upper limb onset patients. • Fasciculations associate directly with BMI loss and upper motor neuron impairment and inversely with disability. • Fasciculations increase with the initial lower motor neuron impairment but they decrease when it progresses. 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 number of fasciculations was recorded in each muscle and the muscle thickness and strength were 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 increase with 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. Conclusions Our study suggests that both UMN and LMN degeneration trigger fasciculations causing BMI loss. The degree of LMN impairment could account for differences in fasciculations' rates within and between muscles. Significance In ALS, fasciculations could explain the link between hyperexcitability and BMI loss. [ABSTRACT FROM AUTHOR]