Jun Tsugawa1, Thanuja Dharmadasa1, Yan Ma1, William Huynh1, Steve Vucic1, Matthew C Kiernan2. 1. Brain and Mind Centre, Sydney Medical School, University of Sydney, Sydney, Australia; Institute of Clinical Neurosciences, Royal Prince Alfred Hospital, Sydney, Australia. 2. Brain and Mind Centre, Sydney Medical School, University of Sydney, Sydney, Australia; Institute of Clinical Neurosciences, Royal Prince Alfred Hospital, Sydney, Australia. Electronic address: matthew.kiernan@sydney.edu.au.
Abstract
OBJECTIVE: To investigate the association between the frequency and intensity of fasciculations with clinical measures of disease progression in amyotrophic lateral sclerosis (ALS). METHODS: Twenty-four consecutive patients with ALS underwent clinical review and neuromuscular ultrasound assessment to detect intensity of fasciculations. Results were correlated with clinical markers of disease severity, as measured by the ALS Functional Rating Scale-revised (ALSFRS-R) and rate of disease progression (ΔFS), in addition to assessment of cortical motor function. RESULTS: Disease duration negatively correlated (R = -0.530, p < 0.01) with fasciculation intensity, while the ΔFS positively correlated with the fasciculation number (R = 0.626, p < 0.01). In terms of potential central contributions to ectopic impulse generation, patients were classified into cohorts based on their fasciculation intensity and short interval intracortical inhibition (SICI). ΔFS was significantly higher in patients with established hyperexcitability (low SICI) with high fasciculation intensity compared to those patients with minimal SICI change. CONCLUSIONS: Fasciculation intensity appears linked to disease progression and separately to markers of cortical dysfunction, specifically the advent of cortical hyperexcitability. SIGNIFICANCE: Assessment of the intensity of patient fasciculations is a noninvasive approach that may provide further insight disease pathophysiology in ALS.
OBJECTIVE: To investigate the association between the frequency and intensity of fasciculations with clinical measures of disease progression in amyotrophic lateral sclerosis (ALS). METHODS: Twenty-four consecutive patients with ALS underwent clinical review and neuromuscular ultrasound assessment to detect intensity of fasciculations. Results were correlated with clinical markers of disease severity, as measured by the ALS Functional Rating Scale-revised (ALSFRS-R) and rate of disease progression (ΔFS), in addition to assessment of cortical motor function. RESULTS: Disease duration negatively correlated (R = -0.530, p < 0.01) with fasciculation intensity, while the ΔFS positively correlated with the fasciculation number (R = 0.626, p < 0.01). In terms of potential central contributions to ectopic impulse generation, patients were classified into cohorts based on their fasciculation intensity and short interval intracortical inhibition (SICI). ΔFS was significantly higher in patients with established hyperexcitability (low SICI) with high fasciculation intensity compared to those patients with minimal SICI change. CONCLUSIONS:Fasciculation intensity appears linked to disease progression and separately to markers of cortical dysfunction, specifically the advent of cortical hyperexcitability. SIGNIFICANCE: Assessment of the intensity of patientfasciculations is a noninvasive approach that may provide further insight disease pathophysiology in ALS.
Authors: Kate Bibbings; Peter J Harding; Ian D Loram; Nicholas Combes; Emma F Hodson-Tole Journal: Ultrasound Med Biol Date: 2019-03-08 Impact factor: 2.998
Authors: Martin R Turner; Richard J Barohn; Philippe Corcia; John K Fink; Matthew B Harms; Matthew C Kiernan; John Ravits; Vincenzo Silani; Zachary Simmons; Jeffrey Statland; Leonard H van den Berg; Hiroshi Mitsumoto Journal: J Neurol Neurosurg Psychiatry Date: 2020-02-06 Impact factor: 10.154
Authors: Paulo Victor Sgobbi de Souza; Wladimir Bocca Vieira de Rezende Pinto; Igor Braga Farias; Bruno de Mattos Lombardi Badia; Icaro França Navarro Pinto; Gustavo Carvalho Costa; Carolina Maria Marin; Ana Carolina Dos Santos Jorge; Emília Correia Souto; Paulo de Lima Serrano; Roberta Ismael Lacerda Machado; Marco Antônio Troccoli Chieia; Enrico Bertini; Acary Souza Bulle Oliveira Journal: Orphanet J Rare Dis Date: 2021-08-11 Impact factor: 4.123