Ectopic impulse generation in peripheral nerve hyperexcitability syndromes and amyotrophic lateral sclerosis

Abstract

Objective: To elucidate differences in the distribution and firing frequency of fasciculations between peripheral nerve hyperexcitability syndromes and amyotrophic lateral sclerosis (ALS) and to explore the generator site of fasciculations.

Methods: Ultrasound of 14 preselected muscles was performed in patients with peripheral hyperexcitability and ALS. The distribution and firing frequency of fasciculations were calculated. Cortical excitability assessment was also done by threshold tracking transcranial magnetic stimulation.

Results: In total, 518 muscles from 37 peripheral hyperexcitability patients and 756 muscles from 54 ALS patients were examined. Regarding the detection rate, 74% of muscles in ALS patients demonstrated fasciculations, compared with 34% of muscles in peripheral hyperexcitability patients (P < 0.001). The number of unique repeating focal muscle fasciculation movements per muscle and firing frequency of individual fasciculations in ALS were both significantly higher than those in peripheral hyperexcitability (P < 0.001). Furthermore, cortical silent period duration negatively correlated with the number and firing frequency of fasciculations in ALS (P < 0.05). A similar relationship was not evident in peripheral hyperexcitability.

Conclusions: In ALS patients, fasciculations were more widespread, greater in number and higher in firing frequency than in peripheral hyperexcitability patients.

Significance: A significant proportion of fasciculations in ALS may be influenced by changes in central excitability.

Keywords: Amyotrophic lateral sclerosis; Fasciculation; Motor neuron disease; Peripheral nerve hyperexcitability; Transcranial magnetic stimulation; Ultrasound.