Please wait a minute...
 Home  About the Journal Editorial Board Aims & Scope Peer Review Policy Subscription Contact us
 
Early Edition  //  Current Issue  //  Open Special Issues  //  Archives  //  Most Read  //  Most Downloaded  //  Most Cited
Aging and Disease    2013, Vol. 4 Issue (5) : 295-310     DOI: 10.14336/AD.2013.0400295
|
Amyotrophic Lateral Sclerosis: An update for 2013 Clinical Features, Pathophysiology, Management and Therapeutic Trials
Paul H. Gordon1, 2, *
1AP-HP, Hôpital de la Pitié-Salpêtrière, Département des Maladies du Système Nerveux, Paris, France
2Northern Navajo Medical Center, Shiprock, NM 87420, USA
Download: PDF(541 KB)   HTML
Export: BibTeX | EndNote | Reference Manager | ProCite | RefWorks    
Abstract  

Amyotrophic lateral sclerosis (ALS), first described by Jean-Martin Charcot in the 1870s, is an age-related disorder that leads to degeneration of motor neurons. The disease begins focally in the central nervous system and then spreads relentlessly. The clinical diagnosis, defined by progressive signs and symptoms of upper and lower motor neuron dysfunction, is confirmed by electromyography. Additional testing excludes other conditions. The disease is heterogeneous, but most patients die of respiratory muscle weakness less than 3 years from symptom-onset. Like other age-related neurodegenerative diseases, ALS has genetic and environmental triggers. Of the five to 10% of cases that are inherited, mutations have been discovered for a high proportion. In addition to genetic factors, age, tobacco use, and athleticism may contribute to sporadic ALS, but important etiologies are unidentified for most patients. Complex pathophysiological processes, including mitochondrial dysfunction, aggregation of misfolded protein, oxidative stress, excitotoxicity, inflammation and apoptosis, involve both motor neurons and surrounding glial cells. There is clinical and pathological overlap with other neurodegenerative diseases, particularly frontotemporal dementia. The mechanisms leading to disease propagation in the brain are a current focus of research. To date, one medication, riluzole, licensed in 1996, has been proved to prolong survival in ALS. Numerous clinical trials have so far been unable to identify another neuroprotective agent. Researchers now aim to slow disease progression by targeting known pathophysiological pathways or genetic defects. Current approaches are directed at muscle proteins such as Nogo, energetic balance, cell replacement, and abnormal gene products resulting from mutations. Until better understanding of the causes and mechanisms underlying progression lead to more robust neuroprotective agents, symptomatic therapies can extend life and improve quality of life. Palliative care programs such as hospice give emotional and physical support to patients and families throughout much of the disease course.

Keywords amyotrophic lateral sclerosis      neurodegeneration      epidemiology      pathophysiology      diagnosis      treatment     
Corresponding Authors: Paul H. Gordon   
Issue Date: 01 May 2013
Service
E-mail this article
E-mail Alert
RSS
Articles by authors
Paul H. Gordon
Cite this article:   
Paul H. Gordon. Amyotrophic Lateral Sclerosis: An update for 2013 Clinical Features, Pathophysiology, Management and Therapeutic Trials[J]. Aging and Disease, 2013, 4(5): 295-310.
URL:  
http://www.aginganddisease.org/EN/10.14336/AD.2013.0400295     OR     http://www.aginganddisease.org/EN/Y2013/V4/I5/295
[1] Rowland LP, Shneider NA(2001). Amyotrophic lateral sclerosis. N Engl J Med, 344:1688-1700
[2] Gordon PH, Cheng B, Katz IB, Pinto M, Hays AP, Mitsumoto H, Rowland LP(2006). The natural history of primary lateral sclerosis. Neurology, 66:647-653
[3] 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, 81:385-390
[4] 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
[5] Ragonese P, Filippini G, Salemi G, Beghi E, Citterio A, D'Alessandro R, Marini C, Monsurro MR, Aiello I, Morgante L(2004). Accuracy of death certificates for amyotrophic lateral sclerosis varies significantly from north to south of Italy: implications for mortality studies. Neuroepidemiology, 23:73-77
[6] Sorenson EJ, Stalker AP, Kurland LT, Windebank AJ(2002). Amyotrophic lateral sclerosis in Olmsted County, Minnesota, 1925 to 1998. Neurology, 59:280-282
[7] Gordon PH, Mehal JM, Holman RC, Rowland LP, Rowland AS, Cheek JE(2013). Incidence of Amyotrophic Lateral Sclerosis Among American Indians and Alaska Natives. JAMA Neurol, 1-5
[8] Kurland LT, Hirano A, Malamud N, LESSELL S(1961). Parkinsonism-dementia complex, en endemic disease on the island of Guam. Clinical, pathological, genetic and epidemiological features. Trans Am Neurol Assoc, 86:115-120
[9] Alonso A, Logroscino G, Jick SS, Hernan MA(2009). Incidence and lifetime risk of motor neuron disease in the United Kingdom: a population-based study. Eur J Neurol, 16:745-751
[10] Fang F, Valdimarsdottir U, Bellocco R, Ronnevi LO, Sparen P, Fall K, Ye W(2009). Amyotrophic lateral sclerosis in Sweden, 1991–2005. Arch Neurol, 66:515-519
[11] Logroscino G, Traynor BJ, Hardiman O, Chio' A, Couratier P, Mitchell JD, Swingler RJ, Beghi E(2008). Descriptive epidemiology of amyotrophic lateral sclerosis: new evidence and unsolved issues. J Neurol Neurosurg Psychiatry, 79:6-11
[12] Gordon P History of ALS. Mitsumoto H, Przedborski S, Gordon P Amyotrophic Lateral SclerosisNew YorkTaylor & Francis Group2006). 1-16
[13] Kasarskis EJ, Winslow M(1989). When did Lou Gehrig's personal illness begin?. Neurology, 39:1243-1245
[14] Lewis M, Gordon PH(2007). Lou Gehrig, rawhide, and 1938. Neurology, 68:615-618
[15] Wechsler I(1940). The treatment of amyotrophic lateral sclerosis with vitamin E (tocopherol)American Journal of the Medical Sciences, 200:765-778
[16] Gordon PH, Meininger V(2011). How can we improve clinical trials in amyotrophic lateral sclerosis?. Nat Rev Neurol, 7:650-654
[17] Kanouchi T, Ohkubo T, Yokota T(2012). Can regional spreading of amyotrophic lateral sclerosis motor symptoms be explained by prion-like propagation?. J Neurol Neurosurg Psychiatry, 83:739-745
[18] Ravits J, Appel S, Baloh RH, Barohn R, Brooks BR, Elman L, Floeter MK, Henderson C, Lomen-Hoerth C, Macklis JD(2013). Deciphering amyotrophic lateral sclerosis: what phenotype, neuropathology and genetics are telling us about pathogenesis. Amyotroph Lateral Scler Frontotemporal Degener, 14(Suppl 1):5-18
[19] Traynor BJ, Alexander M, Corr B, Frost E, Hardiman O(2003). Effect of a multidisciplinary amyotrophic lateral sclerosis (ALS) clinic on ALS survival: a population based study, 1996–2000. J Neurol Neurosurg Psychiatry, 74:1258-1261
[20] Gordon PH, Mitsumoto H(2007). Chapter 20 Symptomatic therapy and palliative aspects of clinical care. Handb Clin Neurol, 82:389-424
[21] Lacomblez L, Bensimon G, Leigh PN, Guillet P, Meininger V(1996). Dose-ranging study of riluzole in amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis/Riluzole Study Group II. Lancet, 347:1425-1431
[22] Gordon PH, Salachas F, Lacomblez L, Le FN, Pradat PF, Bruneteau G, Elbaz A, Meininger V(2012). Predicting Survival of Patients with Amyotrophic Lateral Sclerosis at Presentation: A 15-Year Experience. Neurodegener Dis
[23] Chio A, Logroscino G, Hardiman O, Swingler R, Mitchell D, Beghi E, Traynor BG(2008). Prognostic factors in ALS: A critical review. Amyotroph Lateral Scler, 1-14
[24] Marie P Leçons sur les maladies de la moelleParisMasson1892
[25] Lomen-Hoerth C, Murphy J, Langmore S, Kramer JH, Olney RK, Miller B(2003). Are amyotrophic lateral sclerosis patients cognitively normal?. Neurology, 60:1094-1097
[26] Gordon PH, Delgadillo D, Piquard A, Bruneteau G, Pradat PF, Salachas F, Payan C, Meininger V, Lacomblez L(2011). The range and clinical impact of cognitive impairment in French patients with ALS: A cross-sectional study of neuropsychological test performance. Amyotroph Lateral Scler
[27] Olney RK, Murphy J, Forshew D, Garwood E, Miller BL, Langmore S, Kohn MA, Lomen-Hoerth C(2005). The effects of executive and behavioral dysfunction on the course of ALS. Neurology, 65:1774-1777
[28] Rabkin JG, Albert SM, Rowland LP, Mitsumoto H(2009). How common is depression among ALS caregivers? A longitudinal study. Amyotroph Lateral Scler, 10:448-455
[29] Lou JS, Reeves A, Benice T, Sexton G(2003). Fatigue and depression are associated with poor quality of life in ALS. Neurology, 60:122-123
[30] Rowland LP, Mitsumoto H, Przedborski S Amyotrophic lateral sclerosis, progressive muscular atrophy, and primary lateral sclerosis. Rowland LP, Pedley TA Merritt's Neurology12 edPhiladelphiaLippincott, Williams & Wilkins2010). 802-808
[31] Brooks BR, Miller RG, Swash M, Munsat TL(2000). El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord, 1:293-299
[32] Carvalho MD, Swash M(2009). Awaji diagnostic algorithm increases sensitivity of El Escorial criteria for ALS diagnosis. Amyotroph Lateral Scler, 10:53-57
[33] Millecamps S, Salachas F, Cazeneuve C, Gordon P, Bricka B, Camuzat A, Guillot-Noel L, Russaouen O, Bruneteau G, Pradat PF(2010). SOD1, ANG, VAPB, TARDBP, and FUS mutations in familial amyotrophic lateral sclerosis: genotype-phenotype correlations. J Med Genet, 47:554-560
[34] Mandrioli J, Faglioni P, Merelli E, Sola P(2003). The epidemiology of ALS in Modena, Italy. Neurology, 60:683-689
[35] Rosen DR, Siddique T, Patterson D, Figlewicz DA, Sapp P, Hentati A, Donaldson D, Goto J, O'Regan JP, Deng HX(1993). Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis. Nature, 362:59-62
[36] Mackenzie IR, Bigio EH, Ince PG, Geser F, Neumann M, Cairns NJ, Kwong LK, Forman MS, Ravits J, Stewart H(2007). Pathological TDP-43 distinguishes sporadic amyotrophic lateral sclerosis from amyotrophic lateral sclerosis with SOD1 mutations. Ann Neurol, 61:427-434
[37] Rothstein JD, Martin LJ, Kuncl RW(1992). Decreased glutamate transport by the brain and spinal cord in amyotrophic lateral sclerosis. N Engl J Med, 326:1464-1468
[38] Camu W, Khoris J, Moulard B, Salachas F, Briolotti V, Rouleau GA, Meininger V(1999). Genetics of familial ALS and consequences for diagnosis. French ALS Research Group. J Neurol Sci, 165(Suppl 1):S21-S26
[39] Valdmanis PN, Daoud H, Dion PA, Rouleau GA(2009). Recent advances in the genetics of amyotrophic lateral sclerosis. Curr Neurol Neurosci Rep, 9:198-205
[40] Corcia P, Valdmanis P, Millecamps S, Lionnet C, Blasco H, Mouzat K, Daoud H, Belzil V, Morales R, Pageot N(2012). Phenotype and genotype analysis in amyotrophic lateral sclerosis with TARDBP gene mutations. Neurology, 78:1519-1526
[41] Andersen PM, Al-Chalabi A(2011). Clinical genetics of amyotrophic lateral sclerosis: what do we really know?. Nat Rev Neurol, 7:603-615
[42] Kwiatkowski TJJr, Bosco DA, Leclerc AL, Tamrazian E, Vanderburg CR, Russ C, Davis A, Gilchrist J, Kasarskis EJ, Munsat T(2009). Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis. Science, 323:1205-1208
[43] Renton AE, Majounie E, Waite A, Simon-Sanchez J, Rollinson S, Gibbs JR, Schymick JC, Laaksovirta H, van Swieten JC, Myllykangas L(2011). A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD. Neuron, 72:257-268
[44] Byrne S, Elamin M, Bede P, Shatunov A, Walsh C, Corr B, Heverin M, Jordan N, Kenna K, Lynch C(2012). Cognitive and clinical characteristics of patients with amyotrophic lateral sclerosis carrying a C9orf72 repeat expansion: a population-based cohort study. Lancet Neurol, 11:232-240
[45] Lindquist SG, Duno M, Batbayli M, Puschmann A, Braendgaard H, Mardosiene S, Svenstrup K, Pinborg LH, Vestergaard K, Hjermind LE(2013). Corticobasal and ataxia syndromes widen the spectrum of C9ORF72 hexanucleotide expansion disease. Clin Genet, 83:279-283
[46] Al-Chalabi A, Fang F, Hanby MF, Leigh PN, Shaw CE, Ye W, Rijsdijk F(2010). An estimate of amyotrophic lateral sclerosis heritability using twin data. J Neurol Neurosurg Psychiatry, 81:1324-1326
[47] Chio A, Logroscino G, Hardiman O, Swingler R, Mitchell D, Beghi E, Traynor BG(2009). Prognostic factors in ALS: A critical review. Amyotroph Lateral Scler, 10:310-323
[48] Majoor-Krakauer D, Ottman R, Johnson WG, Rowland LP(1994). Familial aggregation of amyotrophic lateral sclerosis, dementia, and Parkinson's disease: evidence of shared genetic susceptibility. Neurology, 44:1872-1877
[49] Fallis BA, Hardiman O(2009). Aggregation of neurodegenerative disease in ALS kindreds. Amyotroph Lateral Scler, 10:95-98
[50] Corcia P, Mayeux-Portas V, Khoris J, De TB, Autret A, Muh JP, Camu W, Andres C(2002). Abnormal SMN1 gene copy number is a susceptibility factor for amyotrophic lateral sclerosis. Ann Neurol, 51:243-246
[51] Diekstra FP, Saris CG, van RW, Franke L, Jansen RC, van Es MA, van Vught PW, Blauw HM, Groen EJ, Horvath S(2012). Mapping of gene expression reveals CYP27A1 as a susceptibility gene for sporadic ALS. PLoS One, 7:e35333
[52] Dunckley T, Huentelman MJ, Craig DW, Pearson JV, Szelinger S, Joshipura K, Halperin RF, Stamper C, Jensen KR, Letizia D(2007). Whole-genome analysis of sporadic amyotrophic lateral sclerosis. N Engl J Med, 357:775-788
[53] van Es MA, van Vught PW, Blauw HM, Franke L, Saris CG, Andersen PM, Van den BL, de Jong SW, van 't SR, Birve A(2007). ITPR2 as a susceptibility gene in sporadic amyotrophic lateral sclerosis: a genome-wide association study. Lancet Neurol, 6:869-877
[54] van Es MA, van Vught PW, Blauw HM, Franke L, Saris CG, Van den BL, de Jong SW, de J V, Baas F, van't SR(2008). Genetic variation in DPP6 is associated with susceptibility to amyotrophic lateral sclerosis. Nat Genet, 40:29-31
[55] Simpson CL, Lemmens R, Miskiewicz K, Broom WJ, Hansen VK, van Vught PW, Landers JE, Sapp P, Van den BL, Knight J(2009). Variants of the elongator protein 3 (ELP3) gene are associated with motor neuron degeneration. Hum Mol Genet, 18:472-481
[56] Nelson LM, McGuire V, Longstreth WTJr, Matkin C(2000). Population-based case-control study of amyotrophic lateral sclerosis in western Washington State. I. Cigarette smoking and alcohol consumption. Am J Epidemiol, 151:156-163
[57] Horner RD, Kamins KG, Feussner JR, Grambow SC, Hoff-Lindquist J, Harati Y, Mitsumoto H, Pascuzzi R, Spencer PS, Tim R(2003). Occurrence of amyotrophic lateral sclerosis among Gulf War veterans. Neurology, 61:742-749
[58] Gallo V, Bueno-De-Mesquita HB, Vermeulen R, Andersen PM, Kyrozis A, Linseisen J, Kaaks R, Allen NE, Roddam AW, Boshuizen HC(2009). Smoking and risk for amyotrophic lateral sclerosis: analysis of the EPIC cohort. Ann Neurol, 65:378-385
[59] Weisskopf MG, Ascherio A(2009). Cigarettes and amyotrophic lateral sclerosis: only smoke or also fire?. Ann Neurol, 65:361-362
[60] Sutedja NA, Veldink JH, Fischer K, Kromhout H, Wokke JH, Huisman MH, Heederik DJ, van den Berg LH(2007). Lifetime occupation, education, smoking, and risk of ALS. Neurology, 69:1508-1514
[61] Wang H, O'Reilly EJ, Weisskopf MG, Logroscino G, McCullough ML, Thun MJ, Schatzkin A, Kolonel LN, Ascherio A(2011). Smoking and risk of amyotrophic lateral sclerosis: a pooled analysis of 5 prospective cohorts. Arch Neurol, 68:207-213
[62] Scarmeas N, Shih T, Stern Y, Ottman R, Rowland LP(2002). Premorbid weight, body mass, and varsity athletics in ALS. Neurology, 59:773-775
[63] Sutedja NA, Veldink JH, Fischer K, Kromhout H, Heederik D, Huisman MH, Wokke JH, van den Berg LH(2009). Exposure to chemicals and metals and risk of amyotrophic lateral sclerosis: a systematic review. Amyotroph Lateral Scler, 10:302-309
[64] McGuire V, Longstreth WTJr, Nelson LM, Koepsell TD, Checkoway H, Morgan MS, van BG(1997). Occupational exposures and amyotrophic lateral sclerosis. A population-based case-control study. Am J Epidemiol, 145:1076-1088
[65] Chio A, Benzi G, Dossena M, Mutani R, Mora G(2005). Severely increased risk of amyotrophic lateral sclerosis among Italian professional football players. Brain, 128:472-476
[66] Beghi E, Logroscino G, Chio A, Hardiman O, Millul A, Mitchell D, Swingler R, Traynor BJ(2010). Amyotrophic lateral sclerosis, physical exercise, trauma and sports: results of a population-based pilot case-control study. Amyotroph Lateral Scler, 11:289-292
[67] Salazar-Grueso EF, Roos RP(1995). Amyotrophic lateral sclerosis and viruses. Clin Neurosci, 3:360-367
[68] Armon C(2003). An evidence-based medicine approach to the evaluation of the role of exogenous risk factors in sporadic amyotrophic lateral sclerosis. Neuroepidemiology, 22:217-228
[69] Rothstein JD(2009). Current hypotheses for the underlying biology of amyotrophic lateral sclerosis. Ann Neurol, 65(Suppl 1):S3-S9
[70] Dupuis L, Gonzalez de Aguilar JL, di SF, Rene F, de TM, Pradat PF, Lacomblez L, Seihlan D, Prinjha R, Walsh FS(2002). Nogo provides a molecular marker for diagnosis of amyotrophic lateral sclerosis. Neurobiol Dis, 10:358-365
[71] Pradat PF, Bruneteau G, Gonzalez de Aguilar JL, Dupuis L, Jokic N, Salachas F, Le FN, Echaniz-Laguna A, Dubourg O, Hauw JJ(2007). Muscle Nogo-A expression is a prognostic marker in lower motor neuron syndromes. Ann Neurol, 62:15-20
[72] Clement AM, Nguyen MD, Roberts EA, Garcia ML, Boillee S, Rule M, McMahon AP, Doucette W, Siwek D, Ferrante RJ(2003). Wild-type nonneuronal cells extend survival of SOD1 mutant motor neurons in ALS mice. Science, 302:113-117
[73] Douaud G, Filippini N, Knight S, Talbot K, Turner MR(2011). Integration of structural and functional magnetic resonance imaging in amyotrophic lateral sclerosis. Brain, 134:3470-3479
[74] Mulligan VK, Chakrabartty A(2013). Protein misfolding in the late-onset neurodegenerative diseases: Common themes and the unique case of amyotrophic lateral sclerosis. Proteins
[75] Bensimon G, Lacomblez L, Meininger V(1994). A controlled trial of riluzole in amyotrophic lateral sclerosis. ALS/Riluzole Study Group. N Engl J Med, 330:585-591
[76] Miller RG, Mitchell JD, Moore DH(2012). Riluzole for amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND)Cochrane Database Syst Rev, 3:CD001447
[77] Walley T(2004). Neuropsychotherapeutics in the UK: what has been the impact of NICE on prescribing?. CNS Drugs, 18:1-12
[78] Lee JR, Annegers JF, Appel SH(1995). Prognosis of amyotrophic lateral sclerosis and the effect of referral selection. J Neurol Sci, 132:207-215
[79] Haute Autorité de Santé. Prise en charge de personnes atteintes de sclérose latéral amyotrophique; conférence de consensus. 2006.
[80] Miller RG, Jackson CE, Kasarskis EJ, England JD, Forshew D, Johnston W, Kalra S, Katz JS, Mitsumoto H, Rosenfeld J(2009). Practice parameter update: The care of the patient with amyotrophic lateral sclerosis: multidisciplinary care, symptom management, and cognitive/behavioral impairment (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology, 73:1227-1233
[81] Kasarskis EJ, Berryman S, Vanderleest JG, Schneider AR, McClain CJ(1996). Nutritional status of patients with amyotrophic lateral sclerosis: relation to the proximity of death. Am J Clin Nutr, 63:130-137
[82] Marin B, Desport JC, Kajeu P, Jesus P, Nicolaud B, Nicol M, Preux PM, Couratier P(2011). Alteration of nutritional status at diagnosis is a prognostic factor for survival of amyotrophic lateral sclerosis patients. J Neurol Neurosurg Psychiatry, 82:628-634
[83] Limousin N, Blasco H, Corcia P, Gordon PH, De TB, Andres C, Praline J(2010). Malnutrition at the time of diagnosis is associated with a shorter disease duration in ALS. J Neurol Sci, 297:36-39
[84] Chio A, Mora G, Leone M, Mazzini L, Cocito D, Giordana MT, Bottacchi E, Mutani R(2002). Early symptom progression rate is related to ALS outcome: a prospective population-based study. Neurology, 59:99-103
[85] Mitsumoto H, Davidson M, Moore D, Gad N, Brandis M, Ringel S, Rosenfeld J, Shefner JM, Strong MJ, Sufit R(2003). Percutaneous endoscopic gastrostomy (PEG) in patients with ALS and bulbar dysfunction. Amyotroph Lateral Scler Other Motor Neuron Disord, 4:177-185
[86] Miller RG, Jackson CE, Kasarskis EJ, England JD, Forshew D, Johnston W, Kalra S, Katz JS, Mitsumoto H, Rosenfeld J(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, 73:1218-1226
[87] bdelnour-Mallet M, Verschueren A, Guy N, Soriani MH, Chalbi M, Gordon P, Salachas F, Bruneteau G, Le FN, Lenglet T(2011). Safety of home parenteral nutrition in patients with amyotrophic lateral sclerosis: a French national survey. Amyotroph Lateral Scler, 12:178-184
[88] Morgan RK, McNally S, Alexander M, Conroy R, Hardiman O, Costello RW(2005). Use of Sniff nasal-inspiratory force to predict survival in amyotrophic lateral sclerosis. Am J Respir Crit Care Med, 171:269-274
[89] Gordon PH, Corcia P, Lacomblez L, Pochigaeva K, Abitbol JL, Cudkowicz M, Leigh PN, Meininger V(2009). Defining survival as an outcome measure in amyotrophic lateral sclerosis. Arch Neurol, 66:758-761
[90] Heckmatt JZ, Loh L, Dubowitz V(1990). Night-time nasal ventilation in neuromuscular disease. Lancet, 335:579-582
[91] Bourke SC, Tomlinson M, Williams TL, Bullock RE, Shaw PJ, Gibson GJ(2006). Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial. Lancet Neurol, 5:140-147
[92] Newsom-Davis IC, Lyall RA, Leigh PN, Moxham J, Goldstein LH(2001). The effect of non-invasive positive pressure ventilation (NIPPV) on cognitive function in amyotrophic lateral sclerosis (ALS): a prospective study. J Neurol Neurosurg Psychiatry, 71:482-487
[93] Heiman-Patterson TD, Miller RG(2006). NIPPV: a treatment for ALS whose time has come. Neurology, 67:736-737
[94] Lechtzin N, Wiener CM, Clawson L, Davidson MC, Anderson F, Gowda N, Diette GB(2004). Use of noninvasive ventilation in patients with amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord, 5:9-15
[95] Amirjani N, Kiernan MC, McKenzie DK, Butler JE, Gandevia SC(2012). Is there a case for diaphragm pacing for amyotrophic lateral sclerosis patients?. Amyotroph Lateral Scler, 13:521-527
[96] Gater DRJr, Dolbow D, Tsui B, Gorgey AS(2011). Functional electrical stimulation therapies after spinal cord injury. Neuro Rehabilitation, 28:231-248
[97] Scherer K, Bedlack RS(2012). Diaphragm pacing in amyotrophic lateral sclerosis: a literature review. Muscle Nerve, 46:1-8
[98] Gordon PH(2011). Amyotrophic lateral sclerosis: pathophysiology, diagnosis and management. CNS Drugs, 25:1-15
[99] Oliver D(1998). Opioid medication in the palliative care of motor neurone disease. Palliat Med, 12:113-115
[100] Aggarwal S, Cudkowicz M(2008). ALS drug development: reflections from the past and a way forward. Neurotherapeutics, 5:516-527
[101] Ludolph AC, Bendotti C, Blaugrund E, Chio A, Greensmith L, Loeffler JP, Mead R, Niessen HG, Petri S, Pradat PF(2010). Guidelines for preclinical animal research in ALS/MND: A consensus meeting. Amyotroph Lateral Scler, 11:38-45
[102] Mitsumoto H, Gordon P, Kaufmann P, Gooch CL, Przedborski S, Rowland LP(2004). Randomized control trials in ALS: lessons learned. Amyotroph Lateral Scler Other Motor Neuron Disord, 5(Suppl 1):8-13
[103] Cudkowicz ME, Katz J, Moore DH, O'Neill G, Glass JD, Mitsumoto H, Appel S, Ravina B, Kieburtz K, Shoulson I(2010). Toward more efficient clinical trials for amyotrophic lateral sclerosis. Amyotroph Lateral Scler, 11:259-265
[104] Otto M, Bowser R, Turner M, Berry J, Brettschneider J, Connor J, Costa J, Cudkowicz M, Glass J, Jahn O(2012). Roadmap and standard operating procedures for biobanking and discovery of neurochemical markers in ALS. Amyotroph Lateral Scler, 13:1-10
[105] Walmsley AR, Mir AK(2007). Targeting the Nogo-A signalling pathway to promote recovery following acute CNS injury. Curr Pharm Des, 13:2470-2484
[106] bello-Haas V, Florence JM, Krivickas LS(2008). Therapeutic exercise for people with amyotrophic lateral sclerosis or motor neuron disease. Cochrane Database Syst Rev, CD005229
[107] Maragakis NJ(2010). Stem cells and the ALS neurologist. Amyotroph Lateral Scler, 11:417-423
[108] Traub R, Mitsumoto H, Rowland LP(2011). Research advances in amyotrophic lateral sclerosis, 2009 to 2010. Curr Neurol Neurosci Rep, 11:67-77
[109] Xu L, Ryugo DK, Pongstaporn T, Johe K, Koliatsos VE(2009). Human neural stem cell grafts in the spinal cord of SOD1 transgenic rats: differentiation and structural integration into the segmental motor circuitry. J Comp Neurol, 514:297-309
[110] Chew S, Khandji AG, Montes J, Mitsumoto H, Gordon PH(2007). Olfactory ensheathing glia injections in Beijing: misleading patients with ALS. Amyotroph Lateral Scler, 8:314-316
[111] Abukhalil F, Lam BL, Guy J(2012). Visual observations of an American patient with Leber hereditary optic neuropathy after purported injections of stem cells in China. Arch Ophthalmol, 130:532-534
[112] Winer L, Srinivasan D, Chun S, Lacomis D, Jaffa M, Fagan A, Holtzman DM, Wancewicz E, Bennett CF, Bowser R(2013). SOD1 in cerebral spinal fluid as a pharmacodynamic marker for antisense oligonucleotide therapy. JAMA Neurol, 70:201-207
[113] Miller TM, Pestronk A, David W, Rothstein J, Simpson E, Appel SH, Andres PL, Mahoney K, Allred P, Alexander K(2013). An antisense oligonucleotide against SOD1 delivered intrathecally for patients with SOD1 familial amyotrophic lateral sclerosis: a phase 1, randomised, first-in-man study. Lancet Neurol, 12:435-442
[114] Brooks BR, Thisted RA, Appel SH, Bradley WG, Olney RK, Berg JE, Pope LE, Smith RA(2004). Treatment of pseudobulbar affect in ALS with dextromethorphan/quinidine: a randomized trial. Neurology, 63:1364-1370
[115] Ludolph AC, Bendotti C, Blaugrund E, Hengerer B, Loffler JP, Martin J, Meininger V, Meyer T, Moussaoui S, Robberecht W(2007). Guidelines for the preclinical in vivo evaluation of pharmacological active drugs for ALS/MND: report on the 142nd ENMC international workshop. Amyotroph Lateral Scler, 8:217-223
[116] Ravina BM, Fagan SC, Hart RG, Hovinga CA, Murphy DD, Dawson TM, Marler JR(2003). Neuroprotective agents for clinical trials in Parkinson's disease: a systematic assessment. Neurology, 60:1234-1240
[117] raynor 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, 67:20-27
[118] Leigh PN, Swash M, Iwasaki Y, Ludolph A, Meininger V, Miller RG, Mitsumoto H, Shaw P, Tashiro K, van den BL(2004). Amyotrophic lateral sclerosis: a consensus viewpoint on designing and implementing a clinical trial. Amyotroph Lateral Scler Other Motor Neuron Disord, 5:84-98
[119] Shefner JM(2008). Designing clinical trials in amyotrophic lateral sclerosis. Phys Med Rehabil Clin N Am, 19:495-508 ix
[120] Gordon PH, Cheung YK, Levin B, Andrews H, Doorish C, MacArthur RB, Montes J, Bednarz K, Florence J, Rowin J(2008). A novel, efficient, randomized selection trial comparing combinations of drug therapy for ALS. Amyotroph Lateral Scler, 9:212-222
[121] Dibernardo AB, Cudkowicz ME(2006). Translating preclinical insights into effective human trials in ALS. Biochim Biophys Acta, 1762:1139-1149
[122] Cudkowicz M, Qureshi M, Shefner J(2004). Measures and markers in amyotrophic lateral sclerosis. NeuroRx, 1:273-283
[123] Gordon PH, Miller RG, Moore DH(2004). ALSFRS-R. Amyotroph Lateral Scler Other Motor Neuron Disord, 5(Suppl 1):90-93
[124] Groeneveld GJ, Veldink JH, van dT I, Kalmijn S, Beijer C, de VM, Wokke JH, Franssen H, van den Berg LH(2003). A randomized sequential trial of creatine in amyotrophic lateral sclerosis. Ann Neurol, 53:437-445
[125] Cheung YK, Gordon PH, Levin B(2006). Selecting promising ALS therapies in clinical trials. Neurology, 67:1748-1751
[126] Levy G, Kaufmann P, Buchsbaum R, Montes J, Barsdorf A, Arbing R, Battista V, Zhou X, Mitsumoto H, Levin B(2006). A two-stage design for a phase II clinical trial of coenzyme Q10 in ALS. Neurology, 66:660-663
[127] Riccio A, Mattia D, Simione L, Olivetti M, Cincotti F(2012). Eye-gaze independent EEG-based brain-computer interfaces for communication. J Neural Eng, 9:045001
[128] Silvoni S, Cavinato M, Volpato C, Ruf CA, Birbaumer N, Piccione F(2013). Amyotrophic lateral sclerosis progression and stability of brain-computer interface communication. Amyotroph Lateral Scler Frontotemporal Degenerin press.
[1] Jue Wang,Bin Cao,Haiping Zhao,Juan Feng. Emerging Roles of Ganoderma Lucidum in Anti-Aging[J]. A&D, 2017, 8(6): 691-707.
[2] Ilia Stambler. Recognizing Degenerative Aging as a Treatable Medical Condition: Methodology and Policy[J]. A&D, 2017, 8(5): 583-589.
[3] Anindita Banerjee,Vineet Kumar Khemka,Debashree Roy,Aparajita Dhar,Tapan Kumar Sinha Roy,Atanu Biswas,Barun Mukhopadhyay,Sasanka Chakrabarti. Role of Pro-Inflammatory Cytokines and Vitamin D in Probable Alzheimer's Disease with Depression[J]. A&D, 2017, 8(3): 267-276.
[4] Hongxia Zhang,Fen Sun,Jixian Wang,Luokun Xie,Chenqi Yang,Mengxiong Pan,Bei Shao,Guo-Yuan Yang,Shao-Hua Yang,Qichuan ZhuGe,Kunlin Jin. Combining Injectable Plasma Scaffold with Mesenchymal Stem/Stromal Cells for Repairing Infarct Cavity after Ischemic Stroke[J]. A&D, 2017, 8(2): 203-214.
[5] Xiaoting Niu,Xun Wang,Huanjie Huang,Peiqi Ni,Yuanshao Lin,Bei Shao. Bulbocavernosus Reflex Test for Diagnosis of Pudendal Nerve Injury in Female Patients with Diabetic Neurogenic Bladder[J]. A&D, 2016, 7(6): 715-720.
[6] Maureen E. Czick,Christine L. Shapter,David I. Silverman. Atrial Fibrillation: The Science behind Its Defiance[J]. A&D, 2016, 7(5): 635-656.
[7] Qian Li, Yuanshao Lin, Wensi Huang, Yulei Zhou, Xiaoli Chen, Brian Wang, Wanli Zhang, Zhengyi Cai, Jie Xue, Wenhui Zhang, Tieer Yu, Hong Wang, Jincai He, Kunlin Jin, Bei Shao. Serum IL-33 Is a Novel Diagnostic and Prognostic Biomarker in Acute Ischemic Stroke[J]. A&D, 2016, 7(5): 614-622.
[8] Murat Serdar Gurses,Mustafa Numan Ural,Mehmet Akif Gulec,Omer Akyol,Sumeyya Akyol. Pathophysiological Function of ADAMTS Enzymes on Molecular Mechanism of Alzheimer’s Disease[J]. A&D, 2016, 7(4): 479-490.
[9] Daniel O. Claassen,David G. Dobolyi,David A. Isaacs,Olivia C. Roman,Joshua Herb,Scott A. Wylie,Joseph S. Neimat,Manus J. Donahue,Peter Hedera,David H. Zald,Bennett A. Landman,Aaron B. Bowman,Benoit M. Dawant,Swati Rane. Linear and Curvilinear Trajectories of Cortical Loss with Advancing Age and Disease Duration in Parkinson’s Disease[J]. A&D, 2016, 7(3): 220-229.
[10] Kevin H.J. Park. Mechanisms of Muscle Denervation in Aging: Insights from a Mouse Model of Amyotrophic Lateral Sclerosis[J]. A&D, 2015, 6(5): 380-389.
[11] Claudia Rangel-Barajas,Israel Coronel,Benjamín Florán. Dopamine Receptors and Neurodegeneration[J]. A&D, 2015, 6(5): 349-368.
[12] João M. N. Duarte. Metabolic Alterations Associated to Brain Dysfunction in Diabetes[J]. A&D, 2015, 6(5): 304-321.
[13] Sasanka Chakrabarti,Vineet Kumar Khemka,Anindita Banerjee,Gargi Chatterjee,Anirban Ganguly,Atanu Biswas. Metabolic Risk Factors of Sporadic Alzheimer's Disease: Implications in the Pathology, Pathogenesis and Treatment[J]. A&D, 2015, 6(4): 282-299.
[14] Kosaku Komiya,Hiroshi Ishii,Jun-ichi Kadota. Healthcare-associated Pneumonia and Aspiration Pneumonia[J]. Aging and Disease, 2015, 6(1): 27-37.
[15] Tetsuro Hida,Atsushi Harada,Shiro Imagama,Naoki Ishiguro. Managing Sarcopenia and Its Related-Fractures to Improve Quality of Life in Geriatric Populations[J]. Aging and Disease, 2014, 5(4): 226-237.
Viewed
Full text


Abstract

Cited

  Shared   
  Discussed   
Copyright © 2014 Aging and Disease, All Rights Reserved.
Address: Aging and Disease Editorial Office 3400 Camp Bowie Boulevard Fort Worth, TX76106 USA
Fax: (817) 735-0408 E-mail: editorial@aginganddisease.org
Powered by Beijing Magtech Co. Ltd