Ectopic impulse generation in 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.

Noto Y, Simon NG, Selby A, Garg N, Kazumoto S, Shahrizaila N, Huynh W, Matamala JM, Dharmadasa T, Park SB, Vucic S, Kiernan MC.

Clinical Neurophysioloy (Clin Neurophysiol.)

mayo 02, 2018

DOI: 10.1016/j.clinph.2018.01.061

Investigador BNI: Dr. José Manuel Matamala (MD, PhD)