Shefner JM, Al-Chalabi A, Baker MR, et al. A proposal for new diagnostic criteria for ALS. Clin Neurophysiol. 2020;131(8):1975–8.

PubMed  Article  Google Scholar 

de Carvalho M, Dengler R, Eisen A, et al. Electrodiagnostic criteria for diagnosis of ALS. Clin Neurophysiol. 2008;119(3):497–503.

PubMed  Article  Google Scholar 

Brooks BR, Miller RG, Swash M, et al. El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord. 2000;1(5):293–9.

CAS  PubMed  Article  Google Scholar 

Brooks BR. El Escorial World Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis. Subcommittee on Motor Neuron Diseases/Amyotrophic Lateral Sclerosis of the World Federation of Neurology Research Group on Neuromuscular Diseases and the El Escorial “Clinical limits of amyotrophic lateral sclerosis” workshop contributors. J Neurol Sci. 1994;124:96–107.

PubMed  Article  Google Scholar 

Shatunov A, Al-Chalabi A. The genetic architecture of ALS. Neurobiol Dis. 2021;147: 105156.

CAS  PubMed  Article  Google Scholar 

Peters OM, Ghasemi M, Brown RH Jr. Emerging mechanisms of molecular pathology in ALS. J Clin Invest. 2015;125(5):1767–79.

PubMed  PubMed Central  Article  Google Scholar 

Taylor JP, Brown RH Jr, Cleveland DW, Decoding ALS. from genes to mechanism. Nature. 2016;539:197.

PubMed  PubMed Central  Article  Google Scholar 

Mejzini R, Flynn LL, Pitout IL, et al. ALS genetics, mechanisms, and therapeutics: where are we now? Front Neurosci. 2019;13:1310.

PubMed  PubMed Central  Article  Google Scholar 

Alonso A, Logroscino G, Hernán MA. Smoking and the risk of amyotrophic lateral sclerosis: a systematic review and meta-analysis. J Neurol Neurosurg Psychiatry. 2010;81(11):1249–52.

PubMed  Article  Google Scholar 

Oskarsson B, Horton DK, Mitsumoto H. Potential environmental factors in amyotrophic lateral sclerosis. Neurol Clin. 2015;33(4):877–88.

PubMed  PubMed Central  Article  Google Scholar 

Vucic S, Rothstein JD, Kiernan MC. Advances in treating amyotrophic lateral sclerosis: insights from pathophysiological studies. Trends Neurosci. 2014;37(8):433–42.

CAS  PubMed  Article  Google Scholar 

Goutman SA, Hardiman O, Al-Chalabi A, et al. Emerging insights into the complex genetics and pathophysiology of amyotrophic lateral sclerosis. Lancet Neurol. 2022;21(5):465–79.

CAS  PubMed  Article  Google Scholar 

Al-Chalabi A, Calvo A, Chio A, et al. Analysis of amyotrophic lateral sclerosis as a multistep process: a population-based modelling study. Lancet Neurol. 2014;13(11):1108–13.

PubMed  PubMed Central  Article  Google Scholar 

Dadon-Nachum M, Melamed E, Offen D. The “dying-back” phenomenon of motor neurons in ALS. J Mol Neurosci. 2011;43(3):470–7.

CAS  PubMed  Article  Google Scholar 

Fischer LR, Culver DG, Tennant P, et al. Amyotrophic lateral sclerosis is a distal axonopathy: evidence in mice and man. Exp Neurol. 2004;185(2):232–40.

PubMed  Article  Google Scholar 

IQVIA. Understanding neuromuscular disease care: current state and future prospects. Current State and Future Prospects [online] 2018 2018 October 30 [cited 2022 August 5]. Available from: https://www.iqvia.com/insights/the-iqvia-institute/reports/understanding-neuromuscular-disease-care.

Kiernan MC, Vucic S, Talbot K, et al. Improving clinical trial outcomes in amyotrophic lateral sclerosis. Nat Rev Neurol. 2021;17(2):104–18.

PubMed  Article  Google Scholar 

Paganoni S, Berry JD, Quintana M, et al. Adaptive platform trials to transform amyotrophic lateral sclerosis therapy development. Ann Neurol. 2022;91(2):165–75.

PubMed  Article  Google Scholar 

Bensimon G, Lacomblez L, Meininger V. A controlled trial of riluzole in amyotrophic lateral sclerosis. ALS/Riluzole Study Group. N Engl J Med. 1994;330(9):585–91.

CAS  PubMed  Article  Google Scholar 

Lacomblez L, Bensimon G, Leigh PN, et al. Dose-ranging study of riluzole in amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis/Riluzole Study Group II. Lancet. 1996;347(9013):1425–31.

CAS  PubMed  Article  Google Scholar 

Van Den Bosch L, Van Damme P, Bogaert E, et al. The role of excitotoxicity in the pathogenesis of amyotrophic lateral sclerosis. Biochimica et Biophysica Acta (BBA) Mol Basis Dis. 2006;1762(11):1068–82.

Article  CAS  Google Scholar 

Zoing MC, Burke D, Pamphlett R, et al. Riluzole therapy for motor neurone disease: an early Australian experience (1996–2002). J Clin Neurosci. 2006;13(1):78–83.

CAS  PubMed  Article  Google Scholar 

Riviere M, Meininger V, Zeisser P, et al. An analysis of extended survival in patients with amyotrophic lateral sclerosis treated with riluzole. Arch Neurol. 1998;55(4):526–8.

CAS  PubMed  Article  Google Scholar 

Zoccolella S, Beghi E, Palagano G, et al. Analysis of survival and prognostic factors in amyotrophic lateral sclerosis: a population based study. J Neurol Neurosurg Psychiatry. 2008;79(1):33–7.

CAS  PubMed  Article  Google Scholar 

Traynor BJ, Alexander M, Corr B, et al. An outcome study of riluzole in amyotrophic lateral sclerosis—a population-based study in Ireland, 1996–2000. J Neurol. 2003;250(4):473–9.

CAS  PubMed  Article  Google Scholar 

Geevasinga N, Menon P, Ng K, et al. Riluzole exerts transient modulating effects on cortical and axonal hyperexcitability in ALS. Amyotroph Lateral Scler Frontotemp Degener. 2016;17(7–8):580–8.

CAS  Article  Google Scholar 

Andrews JA, Jackson CE, Heiman-Patterson TD, et al. Real-world evidence of riluzole effectiveness in treating amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemp Degener. 2020;21(7–8):509–18.

CAS  Article  Google Scholar 

Fang T, Al Khleifat A, Meurgey JH, et al. Stage at which riluzole treatment prolongs survival in patients with amyotrophic lateral sclerosis: a retrospective analysis of data from a dose-ranging study. Lancet Neurol. 2018;17(5):416–22.

CAS  PubMed  PubMed Central  Article  Google Scholar 

Seibold H, Zeileis A, Hothorn T. Individual treatment effect prediction for amyotrophic lateral sclerosis patients. Stat Methods Med Res. 2018;27(10):3104–125.

Hodgkinson VL, Lounsberry J, Mirian A, et al. Provincial differences in the diagnosis and care of amyotrophic lateral sclerosis. Can J Neurol Sci. 2018;45(6):652–9.

PubMed  Article  Google Scholar 

Qureshi I, Lovegren M, Wirtz V, et al. A pharmacokinetic bioequivalence study comparing sublingual riluzole (BHV-0223) and oral tablet formulation of riluzole in healthy volunteers. Clin Pharmacol Drug Dev. 2020;9(4):476–85.

CAS  PubMed  Article  Google Scholar 

Brooks BR, Bettica P, Cazzaniga S. Riluzole oral suspension: bioavailability following percutaneous gastrostomy tube-modeled administration versus direct oral administration. Clin Ther. 2019;41(12):2490–9.

PubMed  Article  Google Scholar 

Dyer AM, Smith A. Riluzole 5 mg/mL oral suspension: for optimized drug delivery in amyotrophic lateral sclerosis. Drug Des Dev Ther. 2017;11:59–64.

CAS  Article  Google Scholar 

Watanabe K, Tanaka M, Yuki S, et al. How is edaravone effective against acute ischemic stroke and amyotrophic lateral sclerosis? J Clin Biochem Nutr. 2018;62(1):20–38.

CAS  PubMed  Article  Google Scholar 

Cruz MP. Edaravone (Radicava): a novel neuroprotective agent for the treatment of amyotrophic lateral sclerosis. P T. 2018;43(1):25–8.

PubMed  PubMed Central  Google Scholar 

Yoshino H, Kimura A. Investigation of the therapeutic effects of edaravone, a free radical scavenger, on amyotrophic lateral sclerosis (Phase II study). Amyotroph Lateral Scler. 2006;7(4):241–5.

CAS  PubMed  Article  Google Scholar 

Aoki M, Warita H, Mizuno H, et al. Feasibility study for functional test battery of SOD transgenic rat (H46R) and evaluation of edaravone, a free radical scavenger. Brain Res. 2011;1382:321–5.

CAS  PubMed  Article  Google Scholar 

Ito H, Wate R, Zhang J, et al. Treatment with edaravone, initiated at symptom onset, slows motor decline and decreases SOD1 deposition in ALS mice. Exp Neurol. 2008;213(2):448–55.

CAS  PubMed  Article  Google Scholar 

Ikeda K, Iwasaki Y. Edaravone, a free radical scavenger, delayed symptomatic and pathological progression of motor neuron disease in the wobbler mouse. PLoS ONE. 2015;10(10): e0140316.

PubMed  PubMed Central  Article  CAS  Google Scholar 

Abe K, Itoyama Y, Sobue G, et al. Confirmatory double-blind, parallel-group, placebo-controlled study of efficacy and safety of edaravone (MCI-186) in amyotrophic lateral sclerosis patients. Amyotroph Lateral Scler Frontotemp Degener. 2014;15(7–8):610–7.

CAS  Article  Google Scholar 

Edaravone Als 16 Study G. A post-hoc subgroup analysis of outcomes in the first phase III clinical study of edaravone (MCI-186) in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemp Degener. 2017;18(sup 1):11–9.

Article  CAS  Google Scholar 

Group W. Safety and efficacy of edaravone in well defined patients with amyotrophic lateral sclerosis: a randomised, double-blind, placebo-controlled trial. Lancet Neurol. 2017;16(7):505–12.