Dexpramipexole effects on functional decline and survival in subjects with amyotrophic lateral sclerosis in a Phase II study: Subgroup analysis of demographic and clinical characteristics

References

• Rowland LP. Riluzole for the treatment of amyotrophic lateral sclerosis: too soon to tell? N Engl J Med. 1994;330: 636–7.
   PubMed Web of Science ®Google Scholar
• Sejvar JJ, Holman RC, Bresee JS, Kochanek KD, Schonberger LB. Amyotrophic lateral sclerosis mortality in the United States, 1979–2001. Neuroepidemiology 2005;25:144–52.
   PubMed Web of Science ®Google Scholar
• Chio A, Logroscino G, Hardiman O, Swingler R, Mitchell D, Beghi E, . Prognostic factors in ALS: a critical review. Amyotroph Lateral Scler. 2009;10:310–23.
   PubMed Web of Science ®Google Scholar
• del Aguila MA, Longstreth WT Jr, McGuire V, Koepsell TD, van Belle G. Prognosis in amyotrophic lateral sclerosis: a population based study. Neurology. 2003;60:813–9.
   PubMed Web of Science ®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;77:390–2.
   PubMed Web of Science ®Google Scholar
• Kaufmann P, Levy G, Thompson JL, Delbene ML, Battista V, Gordon PH, . The ALSFRS-R predicts survival time in an ALS clinic population. Neurology. 2005;64:38–43.
   PubMed Web of Science ®Google Scholar
• Kimura F, Fujimura C, Ishida S, Nakajima H, Furutama D, Uehara H, . Progression rate of ALSFRS-R at time of diagnosis predicts survival time in ALS. Neurology. 2006;66:265–7.
   PubMed Web of Science ®Google Scholar
• Kollewe K, Mauss U, Krampfl K, Petri S, Dengler R, Mohammadi B. ALSFRS-R score and its ratio: a useful predictor for ALS progression. J Neurol Sci. 2008;275: 69–73.
   PubMed Web of Science ®Google Scholar
• Bruijn LI, Cudkowicz M. Therapeutic targets for amyotrophic lateral sclerosis: current treatments and prospects for more effective therapies. Expert Rev Neurother. 2006;6: 417–28.
   PubMedGoogle Scholar
• Cozzolino M, Ferri A, Carri MT. Amyotrophic lateral sclerosis: from current developments in the laboratory to clinical implications. Antioxid Redox Signal. 2008;10:405–43.
   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;330:585–91.
   Google Scholar
• Lacomblez L, Bensimon G, Leigh PN, Guillet P, Meininger V. Dose-ranging study of riluzole in amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis/Riluzole Study Group II. Lancet. 1996;347:1425–31.
   Google Scholar
• Duffy LM, Chapman AL, Shaw PJ, Grierson AJ. Review: the role of mitochondria in the pathogenesis of amyotrophic lateral sclerosis. Neuropathol Appl Neurobiol. 2011;37: 336–52.
   PubMed Web of Science ®Google Scholar
• Dupuis L, Gonzalez de Aguilar JL, Echaniz-Laguna A, Loeffler JP. Mitochondrial dysfunction in amyotrophic lateral sclerosis also affects skeletal muscle. Muscle Nerve. 2006;34:253–4.
   Google Scholar
• Cudkowicz M, Bozik ME, Ingersoll EW, Miller R, Mitsumoto H, Shefner J, . The effects of dexpramipexole (KNS-760704) in individuals with amyotrophic lateral sclerosis. Nat Med. 2011;17:1652–6.
   PubMed Web of Science ®Google Scholar
• ALS CNTF Treatment Study Phase I-II Group, Brooks B, Sanjak M, Ringel S, England J, Brinkmann J, . The ALS functional rating scale: assessment of activities of daily living in patients with amyotrophic lateral sclerosis. Arch Neurol. 1996;53:141–7.
   PubMedGoogle Scholar
• Cedarbaum JM, Stambler N, Malta E, Fuller C, Hilt D, Thurmond B, . The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. BDNF ALS Study Group (Phase III). J Neurol Sci. 1999;169:13–21.
   PubMed Web of Science ®Google Scholar
• Gordon PH, Moore DH, Miller RG, Florence JM, Verheijde JL, Doorish C, . Efficacy of minocycline in patients with amyotrophic lateral sclerosis: a phase III randomized trial. Lancet Neurol. 2007;6:1045–53.
   PubMed Web of Science ®Google Scholar
• Miller R, Bradley W, Cudkowicz M, Hubble J, Meininger V, Mitsumoto H, . Phase II/III randomized trial of TCH346 in patients with ALS. Neurology. 2007;69: 776–84.
   PubMed Web of Science ®Google Scholar
• Rosenfeld J, King RM, Jackson CE, Bedlack RS, Barohn RJ, Dick A, . Creatine monohydrate in ALS: effects on strength, fatigue, respiratory status and ALSFRS. Amyotroph Lateral Scler. 2008;9:266–72.
   PubMed Web of Science ®Google Scholar
• Gordon PH, Cheng B, Salachas F, Pradat PF, Bruneteau G, Corcia P, . Progression in ALS is not linear but is curvilinear. J Neurol. 2010;257:1713–7.
   Google Scholar
• Finkelstein DM, Schoenfeld DA. Combining mortality and longitudinal measures in clinical trials. Stat Med. 1999;18: 1341–54.
   PubMed Web of Science ®Google Scholar
• Castrillo-Viguera C, Grasso DL, Simpson E, Shefner J, Cudkowicz ME. Clinical significance in the change of decline in ALSFRS-R. Amyotroph Lateral Scler. 2010;11:178–80.
   PubMed Web of Science ®Google Scholar