African Journal of
Pharmacy and Pharmacology

  • Abbreviation: Afr. J. Pharm. Pharmacol.
  • Language: English
  • ISSN: 1996-0816
  • DOI: 10.5897/AJPP
  • Start Year: 2007
  • Published Articles: 2288

Full Length Research Paper

Efficacy and effectiveness of drug treatments in amyotrophic lateral sclerosis: A systematic review with meta-analysis

Mirian Conceicao Moura
  • Mirian Conceicao Moura
  • State Secreariat of Health of Federal District, Brazil.
  • Google Scholar
Maria Rita Carvalho Garbi Novaes
  • Maria Rita Carvalho Garbi Novaes
  • State Secreariat of Health of Federal District, Brazil.
  • Google Scholar
Yuri SSP Zago
  • Yuri SSP Zago
  • School of Health Sciences, Brasilia-DF, Brazil Brazil.
  • Google Scholar
Emanoel Junio Eduardo
  • Emanoel Junio Eduardo
  • School of Health Sciences, Brasilia-DF, Brazil Brazil.
  • Google Scholar
Luiz Augusto Casulari
  • Luiz Augusto Casulari
  • University Hospital of Brasília, Brasilia-DF, Brazil.
  • Google Scholar


  •  Received: 28 December 2015
  •  Accepted: 18 February 2016
  •  Published: 08 April 2016

References

Aggarwal SP, Zinman L, Simpson E, McKinley J, Jackson KE, Pinto H, Kaufman P, Conwit RA, Schoenfeld D, Shefner J, Cudkowicz M (2010). Safety and efficacy of lithium in combination with riluzole for treatment of amyotrophic lateral sclerosis: a randomized, double-blind, placebo-controlled trial. Lancet Neurol. 9(5):481-488.
Crossref

 

Ahn SW, Jeon GS, Kim MJ, Shon JH, Kim JE, Shin JY, Kim SM, Kim SH, Ye, IH, Lee KW Hong YH (2014). Neuroprotective effects of JGK-263 in transgenic SOD1-G93A mice of amyotrophic lateral sclerosis. J. Neurol. Sci. 340(1-2):112-116.
Crossref

 
 

Atassi N, Ratai EM, Greenblatt DJ, Pulley D, Zhao Y, Bombardier J, Wallace S, Eckenrode J, Cudkowicz M, Dibernardo A (2010). A phase I pharmacokinetic dosage escalation study of creatine monohydrate in subjects with amyotrophic lateral sclerosis. Amyotroph. Lateral Scler. 11(6):508-513.
Crossref

 
 

Bame M, Pentiak PA, Needleman R, Brusilow WS (2012). Effect of sex on lifespan, disease progression, and the response to methionine sulfoximine in the SOD1 G93A mouse model for ALS. Gend Med. 9(6):524-535.
Crossref

 
 

Beghi E, Chiò A, Couratier P, Esteban J, Hardiman O, Logroscino G, Millul A, Mitchell D, Preux PM, Pupillo E, Stevic Z (2011). The epidemiology and treatment of ALS: focus on the heterogeneity of the disease and critical appraisal of therapeutic trials. Amyotroph Lateral Scler. 12(1):1-10.
Crossref

 
 

Beghi E, Pupillo E, Bonito V, Buzzi P, Caponnetto C, Chiò A, Corbo M, Giannini F, Inghilleri M, Bella VL, Logroscino G (2013). Randomized double-blind placebo-controlled trial of acetyl-l-carnitine for ALS. Amyotroph Lateral Scler Frontotemporal Degener.14(5-6):397-405.
Crossref

 
 

Berry JD, Shefner JM, Conwit R, Schoenfeld D, Keroack M, Felsenstein D, Krivickas L, David WS, Vriesendorp F, Pestronk A, Caress JB (2013). Design and initial results of a multi-phase randomized trial of ceftriaxone in amyotrophic lateral sclerosis. PLoS One 8(4):e61177.
Crossref

 
 

Boillée S, Yamanaka K, Lobsiger CS, Copeland NG, Jenkins NA, Kassiotis G, Kollias G, Cleveland DW (2006). Onset and progression in inherited ALS determined by motor neurons and microglia. Science 312(5778):1389-1392.
Crossref

 
 

Boll MC, Bayliss L, Vargas-Ca-as S, Burgos J, Montes S, Pe-aloza-Solano G, Rios C, Alcaraz-Zubeldia M (2014). Clinical and biological changes under treatment with lithium carbonate and valproic acid in sporadic amyotrophic lateral sclerosis. J. Neurol. Sci. 340(1-2):103-108.
Crossref

 
 

Calderó J, Brunet N, Tarabal O, Piedrafita L, Hereu M, Ayala V, Esquerda JE (2010). Lithium prevents excitotoxic cell death of motoneurons in organotypic slice cultures of spinal cord. Neuroscience 165(4):1353-1369.
Crossref

 
 

Cappello V, Vezzoli E, Righi M, Fosati M, Mariotti R, Crespi A, Patruno M, Bentivoglio M, Pietrini G, Francolini M (2012). Analysis of neuromuscular junctions and effects of anabolic steroid administration in the SOD1G93A mouse model f ALS. Mol. Cell Neurosci. 51(1):12-21.
Crossref

 
 

Chiò A, Borghero G, Calvo A, Capasso M, Caponnetto C, Corbo M, Giannini F, Logroscino G, Mandrioli J, Marcello N, Mazzini L (2010). Lithium carbonate in amyotrophic lateral sclerosis: lack of efficacy in a dose-finding trial. Neurology 75(7):619-625.
Crossref

 
 

Chiò A, Logroscino G, Traynor BJ, Collins J, Simeone JC, Goldstein LA, White LA (2013). Global epidemiology of amyotrophic lateral sclerosis: a systematic review of the published literature. Neuroepidemiology 41(2):118-130.
Crossref

 
 

Chiò A, Mora G, La Bella V, Caponnetto C, Mancardi G, Sabatelli M, Siciliano G, Silani V, Corbo M, Moglia C Calvo A (2011). Repeated courses of granulocyte colony-stimulating factor in amyotrophic lateral sclerosis: clinical and biological results from a prospective multicenter study. Muscle Nerve 43(2):189-195.
Crossref

 
 

Costa J, Swash M, de Carvalho M (2012). Awaji criteria for the diagnosis of amyotrophic lateral sclerosis: a systematic review. Arch Neurol. 69(11):1410-1416.
Crossref

 
 

Couglan KS, Mitchem MR, Hogg MC, Prehn JH (2015). "Preconditioning" with latrepirdine, an adenosine 5'-monophosphate-activated protein kinase activator, delays amyotrophic lateral sclerosis progression in SOD1(G93A) mice. Neurobiol. Aging 36(2):1140-1150.
Crossref

 
 

Cudkowicz M, Bozik ME, Ingersoll EW, Miller R, Mitsumoto H, Shefner J, Moore DH, Schoenfeld D, Mather JL, Archibald D, Sullivan M (2011). The effects of dexpramipexole (KNS-760704) in individuals with amyotrophic lateral sclerosis. Nature Med. 17(12):1652-1656.
Crossref

 
 

Cudkowicz ME, Titus S, Kearney M, Yu H, Sherman A, Schoenfeld D, Hayden D, Shui A, Brooks B, Conwit R, Felsenstein D (2014). Safety and efficacy of ceftriaxone for amyotrophic lateral sclerosis: a multi-stage, randomised, double-blind, placebo-controlled trial. Lancet Neurol. 13(11):1083-1091.
Crossref

 
 

Cudkowicz ME, van den Berg LH, Shefner JM, Mitsumoto H, Mora JS, Ludolph A, Hardiman O, Bozik ME, Ingersoll EW, Archibald D, Meyers AL (2013). Dexpramipexole versus placebo for patients with amyotrophic lateral sclerosis (EMPOWER): a randomised, double-blind, phase 3 trial. Lancet Neurol. 12(11):1059-1067.
Crossref

 
 

Dodge JC, Treleaven CM, Fidler JA, Hester M, Haidet A, Handy C, Rao M, Eagle A, Matthews JC, Taksir TV Cheng SH (2010). AAV4-mediated expression of IGF-1 and VEGF within cellular components of the ventricular system improves survival outcome in familial ALS mice. Mol. Ther. 18(12):2075-2084.
Crossref

 
 

Dorst J, Cypionka J, Ludolph AC (2013) High-caloric food supplements in the treatment of amyotrophic lateral sclerosis: a prospective interventional study. Amyotroph. Lateral Scler. Frontotemporal Degener. 14(7-8):533-536.
Crossref

 
 

Duning T, Schiffbauer H, Warnecke T, Mohammadi S, Floel A, Kolpatzik K, Kugel H, Schneider A, Knecht S, Deppe M, Schäbitz WR (2011). G-CSF prevents the progression of structural disintegration of white matter tracts in amyotrophic lateral sclerosis: a pilot trial. PLoS One 6(3):e17770.
Crossref

 
 

Dupuis L, Dengler R, Heneka MT, Meyer T, Zierz S, Kassubek J, Fischer W, Steiner F, Lindauer E, Otto M, Dreyhaupt J (2012). A randomized, double blind, placebo-controlled trial of pioglitazone in combination with riluzole in amyotrophic lateral sclerosis. PLoS ONE 7(6):e37885.
Crossref

 
 

Feng XH, Yuan W, Peng Y, Liu MS, Cui LY (2012). Therapeutic effects of dl-3-n-butylphthalide in a transgenic mouse model of amyotrophic lateral sclerosis. Chin Med. J. (Engl). 125(10):1760-1766.

 
 

Ferrucci M, Spalloni A, Bartalucci A (2010). A systematic study of brainstem motor nuclei in a mouse model of ALS, the effects of lithium. Neurobiol Dis. 37(2):370-383.
Crossref

 
 

Fidler J, Treleaven CM, Frakes A (2011). Disease progression in a mouse model of amyotrophic lateral sclerosis: the influence of chronic stress and corticosterone. FASEB J. 25(12):4369-4377.
Crossref

 
 

Fondell E, O'Reilly ÉJ, Fitzgerald KC, Falcone GJ, McCullough ML, Thun MJ, Park Y, Kolonel LN, Ascherio A (2012). Non-steroidal anti-inflammatory drugs and amyotrophic lateral sclerosis: results from five prospective cohort studies. Amyotroph. Lateral Scler. 13(6):573-579
Crossref

 
 

Fornai F, Longone P, Cafaro L, Kastsiuchenka O, Ferrucci M, Manca ML, Lazzeri G, Spalloni A, Bellio N, Lenzi P, Modugno N (2008). Lithium delays progression of amyotrophic lateral sclerosis. Proc. Natl. Acad. Sci. U S A. 105(6):2052-2057.
Crossref

 
 

Gerber YN, Privat A, Perrin FE (2013). Gacyclidine improves the survival and reduces motor deficits in a mouse model of amyotrophic lateral sclerosis. Front Cell Neurosci. 7:280.
Crossref

 
 

Ghoddoussi F, Galloway MP, Jambekar A, Bame M, Needleman R, Brusilow WS (2010). Methionine sulfoximine, an inhibitor of glutamine synthetase, lowers brain glutamine and glutamate in a mouse model of ALS. J. Neurol. Sci. 290(1-2):41-47.
Crossref

 
 

Gianforcaro A, Hamadeh M (2012). Dietary Vitamin D3 Supplementation at 10X the adequate intaque improves functional capacity in the G93A transgenic mouse model of ALS, a pilot study. Neurosci ther.18: 547-557.

 
 

Gianforcaro A, Solomon J, Hamadeh M (2013). Vitamin D3 at 5-X Al Attenuates the decline in paw grip endurance, but not disease outcomes, in te G93A Mouse Modelo of ALS, and is Toxic in Females. PloS ONE 8(2):e30243.
Crossref

 
 

Gordon PH (2013). Amyotrophic Lateral Sclerosis: An update for 2013 Clinical Features, Pathophysiology, Management and Therapeutic Trials. Aging Dis. 4(5):295-310.
Crossref

 
 

Gordon PH, Meininger V (2011). How can we improve clinical trials in amyotrophic lateral sclerosis? Nat. Rev. Neurol. 7:650-654.
Crossref

 
 

Goursaud S, Schäfer S, Dumont AO, Vergouts M, Gallo A, Desmet N, Deumens R, Hermans E (2015). The anti-inflammatory peptide stearyl-norleucine-VIP delays disease onset and extends survival in a rat model of inherited amyotrophic lateral sclerosis. Exp. Neurol. 263:91-101.
Crossref

 
 

Grad LI, Fernando SM, Cashman NR (2015). From molecule to molecule and cell to cell: prion-like mechanisms in amyotrophic lateral sclerosis. Neurobiol Dis. 77:257-265.
Crossref

 
 

Grassinger J, Khomenko A, Hart C, Baldaranov D, Johannesen SW, Mueller G, Schelker R, Schulte-Mattler W, Andreesen R, Bogdahn U (2014). Safety and feasibility of long term administration of recombinant human granulocyte-colony stimulating factor in patients with amyotrophic lateral sclerosis. Cytokine 67(1):21-28.
Crossref

 
 

Gu R, Hou X, Pang R, Li L, Chen F, Geng J, Xu Y, Zhang C (2010). Human adipose-derived stem cells enhance the glutamate uptake function of GLT1 in SOD1G93A-bearing astrocytes. Biochem. Biophys. Res Com. 393(3):481-486.
Crossref

 
 

Guo Y, Zhang K, Wang Q, Li Z, Yin Y, Xu Q, Duan W, Li C (2011). Neuroprotective effects of diallyl trisulfide in SOD1-G93A transgenic mouse model of amyotrophic lateral sclerosis. Brain Res. 1374:110-115.
Crossref

 
 

Guyatt GH, Oxman AD, Vist GE, Kunz R, Falck-Ytter Y, Alonso-Coello P, Schünemann HJ (2008). GRADE: an emerging consensus on rating quality of evidence and strength of recommendations. BMJ 336:924-926.
Crossref

 
 

Hayes-Punzo A, Mulcrone P, Meyer M, McHugh J, Svendsen CN, Suzuki M (2012). Gonadectomy and dehydroepiandrosterone (DHEA) do not modulate disease progression in the G93A mutant SOD1 rat model of amyotrophic lateral sclerosis. Amyotroph Lateral Scler. 13(3):311-314.
Crossref

 
 

Henriques A, Pitzer C, Dittgen T, Klugmann M, Dupuis L, Schneider A (2011). CNS-targeted viral delivery of G-CSF in an animal model for ALS: improved efficacy and preservation of the neuromuscular unit. Mol. Ther. 19(2):284-292.
Crossref

 
 

Jablonka S, Holtmann B, Sendtner M (2011). Therapeutic effects of PEGylated insulin-like growth factor I in the pmn mouse model of motoneuron disease. Exp. neurol. 232:261-269.
Crossref

 
 

Jiang HQ, Ren M, Jiang HZ, Wang J, Zhang J, Yin X, Wang SY, Qi Y, Wang XD, Feng HL (2014). Guanabenz delays the onset of disease symptoms, extends lifespan, improves motor performance and attenuates motor neuron loss in the SOD1 G93A mouse model of amyotrophic lateral sclerosis. Neuroscience 277:132-138.
Crossref

 
 

Kaneb HM, Sharp PS, Rahmani-Kondori N, Wells DJ (2011) Metformin treatment has no beneficial effect in a dose-response survival study in the SOD1 (G93A) mouse model of ALS and is harmful in female mice. PLoS ONE 6(9):e24189.
Crossref

 
 

Katsumata R, Ishigaki S, Katsuno M, Kawai K, Sone J, Huang Z, Adachi H, Tanaka F, Urano F, Sobue G (2012). c-Abl inhibition delays motor neuron degeneration in the G93A mouse, an animal model of amyotrophic lateral sclerosis. PLoS ONE 7(9):e46185.
Crossref

 
 

Kaufmann P, Thompson JL, Levy G, Buchsbaum R, Shefner J, Krivickas LS, Krivickas LS, Katz J, Rollins Y, Barohn RJ, Jackson CE, Tiryaki E (2009). Phase II trial of CoQ10 for ALS finds insufficient evidence to justify phase III. Ann Neurol. 66(2):235-244.
Crossref

 
 

Keller AF, Gravel M, Kriz J.(2011) Treatment with minocycline after disease onset alters astrocyte reactivity and increases microgliosis in SOD1 mutant mice. Exp Neurol. 228(1):69-79.
Crossref

 
 

Kung J, Chiappelli F, Cajulis OO, Avezova R, Kossan G, Chew L, Maida CA (2010). From systematic reviews to clinical recommendations for evidence-based health care: validation of revised assessment of multiple systematic reviews (R-AMSTAR) for grading of clinical relevance. Open Dent. J. 4:84-91.
Crossref

 

Le Pichon CE, Dominguez SL, Solanoy H, Ngu H, Lewin-Koh N, Chen M, Eastham-Anderson J, Watts R, Scearce-Levie K (2013). EGFR inhibitor erlotinib delays disease progression but does not extend survival in the SOD1 mouse model of ALS. PLoS ONE 8(4):e62342.
Crossref

 

Lenglet T, Lacomblez L, Abitbol JL, Ludolph A, Mora JS, Robberecht W, Shaw PJ, Pruss RM, Cuvier V, Meininger V (2014). A phase II-III trial of olesoxime in subjects with amyotrophic lateral sclerosis. Eur. J. Neurol. 21(3):529-536.
Crossref

 
 

Li J, Sung M, Rutkove SB (2013) Electrophysiologic biomarkers for assessing disease progression and the effect of riluzole in SOD1 G93A ALS mice. PLoS ONE 8(6):e65976.
Crossref

 
 

Linares E, Seixas LV, dos Prazeres JN (2013). Tempol moderately extends survival in a hSOD1 (G93A) ALS rat model by inhibiting neuronal cell loss, oxidative damage and levels of non-native hSOD1(G93A) forms. PLoS ONE 8(2):e55868.
Crossref

 
 

Logroscino G, Traynor BJ, Hardiman O, Chio A, Mitchell D, Swingler RJ, Millul A, Benn E, Beghi E (2010). Incidence of amyotrophic lateral sclerosis in Europe. J. Neurol. Neurosurg. Psychiatry 81(4):385-390.
Crossref

 
 

Lougheed R, Turnbull J (2011). Lack of effect of methylene blue in the SOD1 G93A mouse model of amyotrophic lateral sclerosis. PLoS ONE. 6(10):e23141.
Crossref

 
 

Lucchetti J, Marino M, Papa S, Tortarolo M, Guiso G, Pozzi S, Bonetto V, Caccia S, Beghi E, Bendotti C, Gobbi M (2013). A mouse model of familial ALS has increased CNS levels of endogenous ubiquinol9/10 and does not benefit from exogenous administration of ubiquinol10. PLoS ONE 8(7):e69540.
Crossref

 
 

Ludolph AC, Bendotti C, Blaugrund E, Chio A, Greensmith L, Loeffler JP, Mead R, Niessen HG, Petri S, Pradat PF, Robberecht W (2010) Guidelines for preclinical animal research in ALS/MND: A consensus meeting.Amyotroph Lateral Scler.11(1-2):38-45.
Crossref

 
 

Mancuso R, del Valle J, Modol L, Martinez A, Granado-Serrano AB, Ramirez-Nú-ez O, et al. (2014) Resveratrol improves motoneuron function and extends survival in DOD1(G93A) ALS mice. Neurotherapeutics 11(2):419-432.

 
 

Mancuso R, Navarro X. (2015) Amyotrophic lateral sclerosis: Current perspectives from basic research to the clinic. Prog. Neurobiol. 133:1-26
Crossref

 
 

Mancuso R, Oliván S, Rando A, Casas C, Osta R, Navarro X (2012). Sigma-1R agonist improves motor function and motoneuron survival in ALS mice. Neurotherapeutics. 9(4):814-826.
Crossref

 
 

Marin B, Gil J, Preux PM, Funalot B, Couratier P (2009) Incidence of amyotrophic lateral sclerosis in the Limousin region of France, 1997-2007. Amyotroph. Lateral Scler.10:216-220.
Crossref

 
 

Markert CD, Kim E, Gifondorwa DJ, Childers MK, Milligan CE (2010). A single-dose resveratrol treatment in a mouse model of amyotrophic lateral sclerosis. J. Med. Food13(5):1081-1085.
Crossref

 
 

Miller RG, Jackson CE, Kasarskis EJ, England JD, Forshew D, Johnston W, Kalra S, Katz JS, Mitsumoto H, Rosenfeld J, Shoesmith C (2009). Practice parameter update: The care of the patient with amyotrophic lateral sclerosis: drug, nutritional, and respiratory therapies (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 73(15):1218-1226.
Crossref

 
 

Miller RG, Mitchell JD, Moore DH (1997). Riluzole for amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND). Cochrane Database Syst. Rev. 3:CD001447.

 
 

Min JH, Hong YH, Sung JJ, Kim SM, Lee JB, Lee KW (2012). Oral solubilized ursodeoxycholic acid therapy in amyotrophic lateral sclerosis: a randomized cross-over trial. J. Korean Med. Sci. 27(2):200-206.
Crossref

 
 

Nefussy B, Artamonov I, Deutsch V, Naparstek E, Nagler A, Drory VE (2010). Recombinant human granulocyte-colony stimulating factor administration for treating amyotrophic lateral sclerosis: A pilot study. Amyotroph Lateral Scler 11(1-2): 187-193.
Crossref

 
 

Orrell RW (2010). Motor neuron disease: systematic reviews of treatment for ALS and SMA. Br. Med. Bull. 93:145-159
Crossref

 
 

Paganoni S, Macklin EA, Lee A (2014). Diagnostic timelines and delays in diagnosing amyotrophic lateral sclerosis (ALS). Amyotroph Lateral Scler Frontotemporal Degener. 15(5-6):453-456.
Crossref

 
 

Pascuzzi RM, Shefner J, Chappell AS, Bjerke JS, Tamura R, Chaudhry V, Clawson L, Haas L, Rothstein JD (2010). A phase II trial of talampanel in subjects with Amyotrophic Lateral Sclerosis. Amyotroph Later Scler. 11(13):266-271.
Crossref

 
 

Pasquali L, Longone P, Isidoro C, Ruggieri S, Paparelli A, Fornai F (2009). Autophagy, lithium, and amyotrophic lateral sclerosis. Muscle Nerve 40(2):173-194.
Crossref

 
 

Pastula DM, Moore DH, Bedlack RS (2012). Creatine for amyotrophic lateral sclerosis/motor neuron disease. Cochrane Database Syst. Rev. 12:CD005225.
Crossref

 
 

Perrin S (2012). Preclinical research: make mouse studies work. Nature. 507(7493):423-425.
Crossref

 
 

Pizzasegola C, Caron I, Daleno C (2009). Treatment with lithium carbonate does not improve disease progression in two different strains of SOD1 mutant mice. Amyotroph Lateral Scler. 10(4):221-228.
Crossref

 
 

Ravits J, Appel S, Baloh RH, Barohn R, Brooks BR, Elman L, Floeter MK, Henderson C, Lomen-Hoerth C, Macklis JD, McCluskey L (2013). Deciphering amyotrophic lateral sclerosis: what phenotype, neuropathology and genetics are telling us about pathogenesis. Amyotroph. Lateral Scler. Frontotemporal Degener. 14(sup1):5-18.

 
 

Roberts BR, Lim NK, McAllum EJ, Donnelly PS, Hare DJ, Doble PA, Turner BJ, Price KA, Lim SC, Paterson BM, Hickey JL (2014) Oral treatment with Cu(II)(atsm) increases mutant SOD1 in vivo but protects motor neurons and improves the phenotype of a transgenic mouse model of amyotrophic lateral sclerosis. J. Neurosci. 34(23):8021-8031.
Crossref

 
 

Rudnicki SA, Berry JD, Ingersoll E, Archibald D, Cudkowicz ME, Kerr DA, Dong Y (2013). 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. Amyotroph Lateral scler. Frontotemporal Degener. 14(1):44-51.
Crossref

 
 

Saccà F, Quarantelli M, Rinaldi C, Tucci T, Piro R, Perrotta G, Carotenuto B, Marsili A, Palma V, De Michele G, Brunetti A (2012). A randomized controlled trial of growth hormone in amyotrophic lateral sclerosis: clinical, neuroimaging and hormonal results. J Neurol. 259(1):132-138.
Crossref

 
 

Saenger S, Holtmann B, Nilges MR, Schroeder S, Hoeflich A, Kletzl H, Spooren W, Ostrowitzki S, Hanania T, Sendtner M, Metzger F (2012). Functional improvement in mouse models of familial amyotrophic lateral sclerosis by PEGylated insulin-like growth factor I treatment depends on disease severity. Amyotroph Lateral Scler. 13(5):418-429.
Crossref

 
 

Schmitt F, Hussain G, Dupuis L, Loeffler JP, Henriques A (2014) A plural hole for lipids in motor neuron diseases: energy, signaling and structure. Frontiers Cell Neurosci. 8:1-10.
Crossref

 
 

Schuster J, Fu R, Heckman CJ (2010). Fluoxetine treatment has modest effects on disease progression in the mSOD1G93A mouse model of ALS. Amyotroph Lateral Scler. 11(1) 72-73.

 
 

Schuster JE, Fu R, Siddique T, Heckman CJ (2012). Effect of prolonged riluzole on cultured motoneurons in a mouse model of ALS. J. Neurophysiol. 107(1):484-492.
Crossref

 
 

Seo JS, Baek IS, Leem YH, Kim TK, Cho Y, Lee SM, Park YH, Han PL (2011). SK-PC-B70M alleviates neurologic symptoms in G93A-SOD1 amyotrophic lateral sclerosis mice. Brain Res.1368:299-307.
Crossref

 
 

Song L, Chen L, Zhang X, Li J, Le W (2014). Resveratrol ameliorates motor neuron degeneration and improves survival in SOD1(G93A) mouse model of amyotrophc lateral sclerosis. Biomed. Res. Int. 2014:483501.
Crossref

 
 

Soon CPW, Donelly OS, Turner BJ (2011). Diacetylbis (N(4)-methylthiosemicarbazonato) copper(II) (CuII(atsm)) protects against peroxynitrite-induced nitrosative damage and prolongs survival in amyotrophic lateral sclerosis mouse model. J. Biol. Chem. 286(51):44035-44044.
Crossref

 
 

Staats KA, Van Rillaer M, Scheveneels W, Verbesselt R, Van Damme P, Robberecht W, Van Den Bosch L (2012). Dantrolene is neuroprotective in vitro, but does not affect survival in SOD1 (G93A) mice. Neuroscience 220:26-31.
Crossref

 
 

Strong MJ (2010). The evidence for altered RNA metabolism in amyotrophic lateral sclerosis (ALS). J. Neurol. Sci. 288(1-2):1-12.
Crossref

 
 

Sunyach C, Michaud M, Arnoux T, Bernard-marrisal N (2012). Olesoxime delays muscle denervation, astrogliosis, microglial activation and motoneuron death in na als mouse model. Neuropharmacology 62:2346-2353.
Crossref

 
 

Tanaka K, Kanno T, Yanagisawa Y, Yasutake K, Hadano S, Yoshii F, Ikeda JE (2011). Bromocriptine methylate suppresses glial inflammation and moderates disease progression in a mouse model of amyotrophic lateral sclerosis. Exp. Neurol. 232(1):41-52.
Crossref

 
 

Tanaka K, Kanno T, Yanagisawa Y, Yasutake K, Inoue S, Hirayama N, Ikeda JE (2014). A novel acylaminoimidazole derivative, WN1316, alleviates disease progression via suppression of glial inflammation in ALS mouse model. PLoS ONE 9(1):e87728.
Crossref

 
 

The Amyotrophic Lateral Sclerosis Functional Rating Scale (1996). Assessment of activities of daily living in patients with amyotrophic lateral sclerosis The ALS CNTF treatment study (ACTS) phase I-II Study Group. Arch Neurol. 53:141-147.
Crossref

 
 

Tovar-y-Romo LB, Tapia R (2012). Delayed administration of VEGF rescues spinal motor neurons from death with a short effective time frame in excitotoxic experimental models in vivo. ASN NEURO. 4(2):e00081.
Crossref

 
 

Towne C, Setola V, Schneider BL, Aebischer P (2011). Neuroprotection by gene therapy targeting mutant SOD1 in individual pools of motor neurons does not translate into therapeutic benefit in fALS mice. Mol. Ther. 19(2):274-283.
Crossref

 
 

Traynor BJ, Bruijn L, Conwit R, Beal F, O'Neill G, Fagan SC, Cudkowicz ME (2006). Neuroprotective agents for clinical trials in ALS: a systematic assessment. Neurology 67(1):20-27.
Crossref

 
 

UKMND-LiCALS Study Group (2013). Lithium in patients with amyotrophic lateral sclerosis (LiCALS): a phase 3 multicentre, randomised, double-blind, placebo-controlled trial. Lancet Neurol.12(4):339-345.
Crossref

 
 

Verstraete E, Veldink JH, Huisman MH, Draak T, Uijtendaal EV, van der Kooi AJ, Schelhaas HJ, de Visser M, van der Tweel I, van den Berg LH (2012). Lithium lacks effect on survival in amyotrophic lateral sclerosis: a phase IIb randomised sequential trial. J. Neurol. Neurosurg. Psychiatry 83(5):557-564.
Crossref

 
 

Wang L, Popko B, Tixier E, Roos RP (2014). Guanabenz, which enhances the unfolded protein response, ameliorates mutant SOD1-induced amyotrophic lateral sclerosis. Neurobiol. Dis. 71: 317-324.
Crossref

 
 

Yazhou L, Srinivaslu H, Mohamed W (2012). Exendin-4 Ameliorates Motor Neuron Degeneration in Cellular and Animal Models of Amyotrophic Lateral Sclerosis. PLoS ONE 7(2):E 32008.

 
 

Yoo YE, Ko CP (2011). Treatment with trichostatin A initiated after disease onset delays disease progression and increases survival in a mouse model of amyotrophic lateral sclerosis. Exp Neurol. 231(1):147-159.
Crossref

 
 

Yoo YE, Ko CP (2012). Dihydrotestosterone ameliorates degeneration in muscle, axons and motoneurons and improves motor function in amyotrophic lateral sclerosis model mice. PLoS ONE 7(5):e37258.
Crossref

 
 

Zhao W, Varghese M, Vempati P, Dzhun A, Cheng A, Wang J, Lange D, Bilski A, Faravelli I, Pasinetti GM (2012). Caprylic Triglyceride as a novel therapeutic approach to effectively improve the performance and attenuate the symptoms due to the motor neuron loss in ALS disease. PloS ONE 7(11):e49191.
Crossref