van Es MA, Hardiman O, Chio A, Al-Chalabi A, Pasterkamp RJ, Veldink JH, et al. Amyotrophic lateral sclerosis. Lancet. 2017;390(10107):2084–98.

Article  PubMed  Google Scholar 

Brown RH, Al-Chalabi A. Amyotrophic Lateral Sclerosis. N Engl J Med. 2017;377(2):162–72.

Article  PubMed  CAS  Google Scholar 

Van Eijk RPA, Westeneng HJ, Nikolakopoulos S, Verhagen IE, Van Es MA, Eijkemans MJC, et al. Refining eligibility criteria for amyotrophic lateral sclerosis clinical trials. Neurology. 2019;92(5):E451–60.

Article  PubMed  PubMed Central  Google Scholar 

Johnston CA, Stanton BR, Turner MR, Gray R, Blunt AHM, Butt D, et al. Amyotrophic lateral sclerosis in an urban setting: A population based study of inner city London. J Neurol. 2006;253(12):1642–3.

Article  PubMed  Google Scholar 

Lacomblez L, Bensimon G, Leigh PN, Guillet P, Meininger V. Dose-ranging study of riluzole in amyotrophic lateral sclerosis. Lancet. 1996;347(9013):1425–31.

Article  PubMed  CAS  Google Scholar 

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

Article  PubMed  Google Scholar 

van Eijk RPA, Kliest T, McDermott CJ, Roes KCB, Van Damme P, Chio A, et al. TRICALS: creating a highway toward a cure. Amyotroph Lateral Scler Front Degener. 2020;21(7–8):496–501.

Article  Google Scholar 

Van Eijk RPA, Jones AR, Sproviero W, Shatunov A, Shaw PJ, Leigh PN, et al. Meta-analysis of pharmacogenetic interactions in amyotrophic lateral sclerosis clinical trials. Neurology. 2017;89(18):1915–22.

Article  PubMed  PubMed Central  Google Scholar 

Cade JFJ. Lithium salts in the treatment of psychotic excitement. Med J Aust. 1949;2(10):349–52.

Article  PubMed  CAS  Google Scholar 

Schou M, Juel-Nielsen N, Stromgren E, Voldby H. The treatment of manic psychoses by the administration of lithium salts. J Neurol Neurosurg Psychiatry. 1954 Nov;17(4):250–60.

Article  PubMed  PubMed Central  CAS  Google Scholar 

Lees AJ. Lithium: A slow burner. J Neurol Neurosurg Psychiatry. 2020;91(4):335–7.

Article  PubMed  Google Scholar 

Forlenza OV, De-Paula VJR, Diniz BSO. Neuroprotective effects of lithium: Implications for the treatment of Alzheimer’s disease and related neurodegenerative disorders. ACS Chem Neurosci. 2014;5(6):443–50.

Article  PubMed  PubMed Central  CAS  Google Scholar 

Broeke JHP, Roelandse M, Luteijn MJ, Boiko T, Matus A, Toonen RF, et al. Munc18 and Munc13 regulate early neurite outgrowth. Biol Cell. 2010;102(8):479–88.

Article  PubMed  PubMed Central  CAS  Google Scholar 

Al-Chalabi A, Allen C, Counsell C, Farrin A, Dickie B, Kelly J, et al. Lithium in patients with amyotrophic lateral sclerosis (LiCALS): A phase 3 multicentre, randomised, double-blind, placebo-controlled trial. Lancet Neurol. 2013;12(4):339–45.

Article  PubMed  Google Scholar 

Verstraete E, Veldink JH, Huisman MHB, Draak T, Uijtendaal EV, Van Der Kooi AJ, et al. Lithium lacks effect on survival in amyotrophic lateral sclerosis: A phase IIb randomised sequential trial. J Neurol Neurosurg Psychiatry. 2012;83(5):557–64.

Article  PubMed  Google Scholar 

Brooks BR, Miller RG, Swash M, Munsat TL. World Federation of Neurology Research Group on Motor Neuron Diseases. El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord. 2000 Dec;1(5):293–9.

Article  PubMed  CAS  Google Scholar 

TRICALS. Risk Profile Calculator. https://tricals.shinyapps.io/risk-profile/. Accessed 21 Sep 2022.

van Eijk RPA, Nikolakopoulos S, Roes KCB, Kendall L, Han SS, Lavrov A, et al. Innovating Clinical Trials for Amyotrophic Lateral Sclerosis: Challenging the Established Order. Neurology. 2021;97(11):528–36.

Article  PubMed  PubMed Central  Google Scholar 

Westeneng HJ, Debray TPA, Visser AE, van Eijk RPA, Rooney JPK, Calvo A, et al. Prognosis for patients with amyotrophic lateral sclerosis: development and validation of a personalised prediction model. Lancet Neurol. 2018;17(5):423–33.

Article  PubMed  Google Scholar 

Chiò A, Borghero G, Calvo A, Capasso M, Caponnetto C, Corbo M, et al. Lithium carbonate in amyotrophic lateral sclerosis: Lack of efficacy in a dose-finding trial. Neurology. 2010;75(7):619–25.

Article  PubMed  Google Scholar 

Haddad P, Wieck A, Yarrow M, Denham P. The Lithium Side Effects Rating Scale (LISERS); development of a self-rating instrument. Eur Neuropsychopharmacol. 1999 Sep;9:231–2.

Google Scholar 

Cedarbaum JM, Stambler N, Malta E, Fuller C, Hilt D, Thurmond B, et al. The ALSFRS-R: A revised ALS functional rating scale that incorporates assessments of respiratory function. J Neurol Sci. 1999;169(1–2):13–21.

Article  PubMed  CAS  Google Scholar 

Kaufmann P, Levy G, Thompson JLP, DelBene ML, Battista V, Gordon PH, et al. The ALSFRSr predicts survival time in an ALS clinic population. Neurology. 2005;64(1):38–43.

Article  PubMed  CAS  Google Scholar 

Czaplinski A, Yen AA, Appel SH. Forced vital capacity (FVC) as an indicator of survival and disease progression in an ALS clinic population. J Neurol Neurosurg Psychiatry. 2006;77(3):390–2.

Article  PubMed  PubMed Central  CAS  Google Scholar 

Quanjer PH, Stanojevic S. Do the global lung function initiative 2012 equations fit my population? Eur Respir J. 2016;48(6):1782–5.

Article  PubMed  Google Scholar 

Van Eijk RPA, Eijkemans MJC, Ferguson TA, Nikolakopoulos S, Veldink JH, Van Den Berg LH. Monitoring disease progression with plasma creatinine in amyotrophic lateral sclerosis clinical trials. J Neurol Neurosurg Psychiatry. 2018;89(2):156–61.

Article  PubMed  Google Scholar 

Tetsuka S, Morita M, Ikeguchi K, Nakano I. Utility of cystatin C for renal function in amyotrophic lateral sclerosis. Acta Neurol Scand. 2013;128(6):386–90.

Article  PubMed  CAS  Google Scholar 

Chiò A, Hammond ER, Mora G, Bonito V, Filippini G. Development and evaluation of a clinical staging system for amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry. 2015;86(1):38–44.

Article  PubMed  Google Scholar 

Balendra R, Jones A, Jivraj N, Steen IN, Young CA, Shaw PJ, et al. Use of clinical staging in amyotrophic lateral sclerosis for phase 3 clinical trials. J Neurol Neurosurg Psychiatry. 2015;86(1):45–9.

Article  PubMed  Google Scholar 

Chen X, Wei QQ, Chen Y, Cao B, Ou RW, Hou Y, et al. Clinical staging of amyotrophic lateral sclerosis in Chinese patients. Front Neurol. 2018;9:1–9.

Article  Google Scholar 

Shepheard SR, Wuu J, Cardoso M, Wiklendt L, Dinning PG, Chataway T, et al. Urinary p75ECD: A prognostic, disease progression, and pharmacodynamic biomarker in ALS. Neurology. 2017;88(12):1137–43.

Article  PubMed  PubMed Central  CAS  Google Scholar 

Lu CH, Macdonald-Wallis C, Gray E, Pearce N, Petzold A, Norgren N, et al. Neurofilament light chain: A prognostic biomarker in amyotrophic lateral sclerosis. Neurology. 2015;84(22):2247–57.

Article  PubMed  PubMed Central  CAS  Google Scholar 

Crockford C, Newton J, Lonergan K, Chiwera T, Booth T, Chandran S, et al. ALS-specific cognitive and behavior changes associated with advancing disease stage in ALS. Neurology. 2018;91(15):E1370–80.

Article  PubMed  PubMed Central  Google Scholar 

Abrahams S, Newton J, Niven E, Foley J, Bak TH. Screening for cognition and behaviour changes in ALS. Amyotroph Lateral Scler Front Degener. 2014;15(1–2):9–14.

Article  Google Scholar 

Raaphorst J, Beeldman E, Schmand B, Berkhout J, Linssen WHJP, Van Den Berg LH, et al. The ALS-FTD-Q: A new screening tool for behavioral disturbances in ALS. Neurology. 2012;79(13):1377–83.

Article  PubMed  Google Scholar 

De Boer AGEM, Van Lanschot JJB, Stalmeier PFM, Van Sandick JW, Hulscher JBF, De Haes JCJM, et al. Is a single-item visual analogue scale as valid, reliable and responsive as multi-item scales in measuring quality of life? Qual Life Res. 2004;13(2):311–20.

Article  PubMed  Google Scholar 

Jones AR, Jivraj N, Balendra R, Murphy C, Kelly J, Thornhill M, et al. Health utility decreases with increasing clinical stage in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Front Degener. 2014;15(3–4):285–91.

Article  Google Scholar 

Maathuis EM, Drenthen J, Van Doorn PA, Visser GH, Blok JH. The CMAP scan as a tool to monitor disease progression in ALS and PMA. Amyotroph Lateral Scler Front Degener. 2013;14(3):217–23.

Article  Google Scholar 

Henderson RD, Ridall GR, Pettitt AN, McCombe PA, Daube JR. The stimulus-response curve and motor unit variability in normal subjects and subjects with amyotrophic lateral sclerosis. Muscle Nerve. 2006;34(1):34–43.

Article  PubMed  CAS