Body mass index and dietary intervention: Implications for prognosis of amyotrophic lateral sclerosis

Journal of the Neurological Sciences

Volumn 340, Issue 1-2, 2014, Pages 5-12

  Ngo, S T ^a,b   Steyn, F J ^a   McCombe, P A ^b,c  

^a UNIVERSITY OF QUEENSLAND   (Australia)

^b UNIVERSITY OF QUEENSLAND   (Australia)

^c ROYAL BRISBANE AND WOMEN'S HOSPITAL   (Australia)

Author keywords

Amyotrophic lateral sclerosis; Body mass index; Diet; Dietary intervention; Fat mass

Indexed keywords

COPPER ZINC SUPEROXIDE DISMUTASE; FUSED IN SARCOMA PROTEIN; GLUCOSE; LIPID; MEDIUM CHAIN TRIACYLGLYCEROL; MILK PROTEIN; NUTRACEUTICAL; OPTINEURIN; REACTIVE OXYGEN METABOLITE; TAR DNA BINDING PROTEIN; UBIQUITIN;

AMYOTROPHIC LATERAL SCLEROSIS; BODY COMPOSITION; BODY MASS; CALORIC RESTRICTION; CARBOHYDRATE DIET; DIET SUPPLEMENTATION; DIET THERAPY; DISEASE COURSE; DISEASE DURATION; DISORDERS OF CARBOHYDRATE METABOLISM; ENERGY BALANCE; ENERGY RESOURCE; ENVIRONMENTAL FACTOR; GLUCOSE BLOOD LEVEL; HEREDITY; HUMAN; HYPERMETABOLISM; KETOGENIC DIET; LIPID DIET; MOTOR PERFORMANCE; NONHUMAN; NUTRITIONAL STATUS; OBESITY; PERCUTANEOUS ENDOSCOPIC GASTROSTOMY; PRIORITY JOURNAL; PROGNOSIS; PROTEIN DIET; REVIEW; SYSTEMATIC REVIEW; WEIGHT REDUCTION; CALORIC INTAKE;

AMYOTROPHIC LATERAL SCLEROSIS; BODY MASS INDEX; DIETARY SUPPLEMENTS; ENERGY INTAKE; HUMANS; PROGNOSIS;

EID: 84899928471     PISSN: 0022510X     EISSN: 18785883     Source Type: Journal    
DOI: 10.1016/j.jns.2014.02.035     Document Type: Review

References (148)

1. D.W. Cleveland, and J.D. Rothstein From Charcot to Lou Gehrig: deciphering selective motor neuron death in ALS Nat Rev Neurosci 2 11 2001 806 819
2. J.D. Mitchell, and G.D. Borasio Amyotrophic lateral sclerosis Lancet 369 9578 2007 2031 2041
3. D.R. Borchelt, M.K. Lee, H.S. Slunt, M. Guarnieri, Z.S. Xu, and P.C. Wong et al. Superoxide dismutase 1 with mutations linked to familial amyotrophic lateral sclerosis possesses significant activity Proc Natl Acad Sci U S A 91 17 1994 8292 8296
4. M.E. Gurney, H. Pu, A.Y. Chiu, M.C. Dal Canto, C.Y. Polchow, and D.D. Alexander et al. Motor neuron degeneration in mice that express a human Cu, Zn superoxide dismutase mutation Science 264 5166 1994 1772 1775
5. P.N. Leigh, B.H. Anderton, A. Dodson, J.M. Gallo, M. Swash, and D.M. Power Ubiquitin deposits in anterior horn cells in motor neurone disease Neurosci Lett 93 2-3 1988 197 203
6. H. Maruyama, H. Morino, H. Ito, Y. Izumi, H. Kato, and Y. Watanabe et al. Mutations of optineurin in amyotrophic lateral sclerosis Nature 465 7295 2010 223 226
7. M. van Blitterswijk, P.W. van Vught, M.A. van Es, H.J. Schelhaas, A.J. van der Kooi, and M. de Visser et al. Novel optineurin mutations in sporadic amyotrophic lateral sclerosis patients Neurobiol Aging 33 5 2012 1016 e1-7
8. I.P. Blair, K.L. Williams, S.T. Warraich, J.C. Durnall, A.D. Thoeng, and J. Manavis et al. FUS mutations in amyotrophic lateral sclerosis: clinical, pathological, neurophysiological and genetic analysis J Neurol Neurosurg Psychiatry 81 6 2009 639 645
9. H.X. Deng, H. Zhai, E.H. Bigio, J. Yan, F. Fecto, and K. Ajroud et al. FUS-immunoreactive inclusions are a common feature in sporadic and non-SOD1 familial amyotrophic lateral sclerosis Ann Neurol 67 6 2010 739 748
10. E.J. Groen, M.A. van Es, P.W. van Vught, W.G. Spliet, J. van Engelen-Lee, and M. de Visser et al. FUS mutations in familial amyotrophic lateral sclerosis in the Netherlands Arch Neurol 67 2 2010 224 230
11. C. Hewitt, J. Kirby, J.R. Highley, J.A. Hartley, R. Hibberd, and H.C. Hollinger et al. Novel FUS/TLS mutations and pathology in familial and sporadic amyotrophic lateral sclerosis Arch Neurol 67 4 2010 455 461
12. B.A. Keller, K. Volkening, C.A. Droppelmann, L.C. Ang, R. Rademakers, and M.J. Strong Co-aggregation of RNA binding proteins in ALS spinal motor neurons: evidence of a common pathogenic mechanism Acta Neuropathol 124 5 2012 733 747
13. T. Arai, M. Hasegawa, H. Akiyama, K. Ikeda, T. Nonaka, and H. Mori et al. TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis Biochem Biophys Res Commun 351 3 2006 602 611
14. E. Kabashi, P.N. Valdmanis, P. Dion, D. Spiegelman, B.J. McConkey, and C. Vande Velde et al. TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis Nat Genet 40 5 2008 572 574
15. M. Neumann, D.M. Sampathu, L.K. Kwong, A.C. Truax, M.C. Micsenyi, and T.T. Chou et al. Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis Science 314 5796 2006 130 133
16. M. DeJesus-Hernandez, I.R. Mackenzie, B.F. Boeve, A.L. Boxer, M. Baker, and N.J. Rutherford et al. Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS Neuron 72 2 2011 245 256
17. A.E. Renton, E. Majounie, A. Waite, J. Simon-Sanchez, S. Rollinson, and J.R. Gibbs et al. A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD Neuron 72 2 2011 257 268
18. D.R. Rosen, T. Siddique, D. Patterson, D.A. Figlewicz, P. Sapp, and A. Hentati et al. Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis Nature 362 6415 1993 59 62
19. L. Van Den Bosch, and W. Robberecht Different receptors mediate motor neuron death induced by short and long exposures to excitotoxicity Brain Res Bull 53 4 2000 383 388
20. L. Van Den Bosch, W. Vandenberghe, H. Klaassen, E. Van Houtte, and W. Robberecht Ca(2 +)-permeable AMPA receptors and selective vulnerability of motor neurons J Neurol Sci 180 1-2 2000 29 34
21. W. Vandenberghe, E.C. Ihle, D.K. Patneau, W. Robberecht, and J.R. Brorson AMPA receptor current density, not desensitization, predicts selective motoneuron vulnerability J Neurosci 20 19 2000 7158 7166
22. W. Vandenberghe, W. Robberecht, and J.R. Brorson AMPA receptor calcium permeability, GluR2 expression, and selective motoneuron vulnerability J Neurosci 20 1 2000 123 132
23. C. Bendotti, N. Calvaresi, L. Chiveri, A. Prelle, M. Moggio, and M. Braga et al. Early vacuolization and mitochondrial damage in motor neurons of FALS mice are not associated with apoptosis or with changes in cytochrome oxidase histochemical reactivity J Neurol Sci 191 1-2 2001 25 33
24. V. Crugnola, C. Lamperti, V. Lucchini, D. Ronchi, L. Peverelli, and A. Prelle et al. Mitochondrial respiratory chain dysfunction in muscle from patients with amyotrophic lateral sclerosis Arch Neurol 67 7 2010 849 854
25. A.M. Haidet-Phillips, M.E. Hester, C.J. Miranda, K. Meyer, L. Braun, and A. Frakes et al. Astrocytes from familial and sporadic ALS patients are toxic to motor neurons Nat Biotechnol 29 9 2011 824 828
26. Y. Lee, B.M. Morrison, Y. Li, S. Lengacher, M.H. Farah, and P.N. Hoffman et al. Oligodendroglia metabolically support axons and contribute to neurodegeneration Nature 487 7408 2012 443 448
27. K. Meyer, L. Ferraiuolo, C.J. Miranda, S. Likhite, S. McElroy, and S. Renusch et al. Direct conversion of patient fibroblasts demonstrates non-cell autonomous toxicity of astrocytes to motor neurons in familial and sporadic ALS Proc Natl Acad Sci U S A 11 2 2013 829 832
28. H.P. Phatnani, P. Guarnieri, B.A. Friedman, M.A. Carrasco, M. Muratet, and S. O'Keeffe et al. Intricate interplay between astrocytes and motor neurons in ALS Proc Natl Acad Sci U S A 110 8 2013 E756 E765
29. J. Tong, C. Huang, F. Bi, Q. Wu, B. Huang, and X. Liu et al. Expression of ALS-linked TDP-43 mutant in astrocytes causes non-cell-autonomous motor neuron death in rats EMBO J 32 13 2013 1917 1926
30. C. Bouteloup, J.C. Desport, P. Clavelou, N. Guy, H. Derumeaux-Burel, and A. Ferrier et al. Hypermetabolism in ALS patients: an early and persistent phenomenon J Neurol 256 8 2009 1236 1242
31. A. Cistaro, M.C. Valentini, A. Chio, F. Nobili, A. Calvo, and C. Moglia et al. Brain hypermetabolism in amyotrophic lateral sclerosis: a FDG PET study in ALS of spinal and bulbar onset Eur J Nucl Med Mol Imaging 39 2 2012 251 259
32. J.C. Desport, P.M. Preux, L. Magy, Y. Boirie, J.M. Vallat, and B. Beaufrere et al. Factors correlated with hypermetabolism in patients with amyotrophic lateral sclerosis Am J Clin Nutr 74 3 2001 328 334
33. J.C. Desport, F. Torny, M. Lacoste, P.M. Preux, and P. Couratier Hypermetabolism in ALS: correlations with clinical and paraclinical parameters Neurodegener Dis 2 3-4 2005 202 207
34. B. Funalot, J.C. Desport, F. Sturtz, W. Camu, and P. Couratier High metabolic level in patients with familial amyotrophic lateral sclerosis Amyotroph Lateral Scler 10 2 2009 113 117
35. E.J. Kasarskis, S. Berryman, J.G. Vanderleest, A.R. Schneider, and C.J. McClain Nutritional status of patients with amyotrophic lateral sclerosis: relation to the proximity of death Am J Clin Nutr 63 1 1996 130 137
36. D.S. Gray, and K. Fujioka Use of relative weight and Body Mass Index for the determination of adiposity J Clin Epidemiol 44 6 1991 545 550
37. S. Paganoni, J. Deng, M. Jaffa, M.E. Cudkowicz, and A.M. Wills Body mass index, not dyslipidemia, is an independent predictor of survival in amyotrophic lateral sclerosis Muscle Nerve 44 1 2011 20 24
38. T. Shimizu, M. Honda, T. Ohashi, M. Tsujino, U. Nagaoka, and A. Kawata et al. Hyperosmolar hyperglycemic state in advanced amyotrophic lateral sclerosis Amyotroph Lateral Scler 12 5 2011 379 381
39. R. Reich-Slotky, J. Andrews, B. Cheng, R. Buchsbaum, D. Levy, and P. Kaufmann et al. Body mass index (BMI) as predictor of ALSFRS-R score decline in ALS patients Amyotroph Lateral Scler Frontotemporal Degener 14 3 2013 212 216
40. E.J. O'Reilly, H. Wang, M.G. Weisskopf, K.C. Fitzgerald, G. Falcone, and M.L. McCullough et al. Premorbid body mass index and risk of amyotrophic lateral sclerosis Amyotroph Lateral Scler Frontotemporal Degener 14 3 2013 205 211
41. V. Gallo, P.A. Wark, M. Jenab, N. Pearce, C. Brayne, and R. Vermeulen et al. Prediagnostic body fat and risk of death from amyotrophic lateral sclerosis: the EPIC cohort Neurology 80 9 2013 829 838
42. D.W. Haslam, and W.P. James Obesity Lancet 366 9492 2005 1197 1209
43. A. Chio, A. Calvo, A. Ilardi, E. Cavallo, C. Moglia, and R. Mutani et al. Lower serum lipid levels are related to respiratory impairment in patients with ALS Neurology 73 20 2009 1681 1685
44. A. Jawaid, A.R. Salamone, A.M. Strutt, S.B. Murthy, M. Wheaton, and E.J. McDowell et al. ALS disease onset may occur later in patients with pre-morbid diabetes mellitus Eur J Neurol 17 5 2010 733 739
45. R.A. Maselli, R.L. Wollman, C. Leung, B. Distad, S. Palombi, and D.P. Richman et al. Neuromuscular transmission in amyotrophic lateral sclerosis Muscle Nerve 16 11 1993 1193 1203
46. P. Kuhnlein, H.J. Gdynia, A.D. Sperfeld, B. Lindner-Pfleghar, A.C. Ludolph, and M. Prosiegel et al. Diagnosis and treatment of bulbar symptoms in amyotrophic lateral sclerosis Nat Clin Pract Neurol 4 7 2008 366 374
47. NHLBI Obesity Education Initiative Expert Panel on the Identification E, and Treatment of Obesity in Adults (US) Executive summary of the clinical guidelines on the identification, evaluation, and treatment of overweight and obesity in adults Arch Intern Med 158 17 1998 1855 1867
48. J. Vague The degree of masculine differentiation of obesities: a factor determining predisposition to diabetes, atherosclerosis, gout, and uric calculous disease Am J Clin Nutr 4 1 1956 20 34
49. J. Dorst, P. Kuhnlein, C. Hendrich, J. Kassubek, A.D. Sperfeld, and A.C. Ludolph Patients with elevated triglyceride and cholesterol serum levels have a prolonged survival in amyotrophic lateral sclerosis J Neurol 258 4 2011 613 617
50. L. Dupuis, P. Corcia, A. Fergani, J.L. Gonzalez De Aguilar, D. Bonnefont-Rousselot, and R. Bittar et al. Dyslipidemia is a protective factor in amyotrophic lateral sclerosis Neurology 70 13 2008 1004 1009
51. I.R. Edwards, K. Star, and A. Kiuru Statins, neuromuscular degenerative disease and an amyotrophic lateral sclerosis-like syndrome: an analysis of individual case safety reports from vigibase Drug Saf 30 6 2007 515 525
52. C. Weissman, and M. Kemper Assessing hypermetabolism and hypometabolism in the postoperative critically ill patient Chest 102 5 1992 1566 1571
53. S. Korner, M. Hendricks, K. Kollewe, A. Zapf, R. Dengler, and V. Silani et al. Weight loss, dysphagia and supplement intake in patients with amyotrophic lateral sclerosis (ALS): impact on quality of life and therapeutic options BMC Neurol 13 2013 84
54. L. Mazzini, T. Corra, M. Zaccala, G. Mora, M. Del Piano, and M. Galante Percutaneous endoscopic gastrostomy and enteral nutrition in amyotrophic lateral sclerosis J Neurol 242 10 1995 695 698
55. A.K. Afifi, F.P. Aleu, J. Goodgold, and B. MacKay Ultrastructure of atrophic muscle in amyotrophic lateral sclerosis Neurology 16 5 1966 475 481
56. F.M. Menzies, P.G. Ince, and P.J. Shaw Mitochondrial involvement in amyotrophic lateral sclerosis Neurochem Int 40 6 2002 543 551
57. J.C. Dodge, C.M. Treleaven, J.A. Fidler, T.J. Tamsett, C. Bao, and M. Searles et al. Metabolic signatures of amyotrophic lateral sclerosis reveal insights into disease pathogenesis Proc Natl Acad Sci U S A 110 26 2013 10812 10817
58. L. Dupuis, H. Oudart, F. Rene, J.L. Gonzalez de Aguilar, and J.P. Loeffler Evidence for defective energy homeostasis in amyotrophic lateral sclerosis: benefit of a high-energy diet in a transgenic mouse model Proc Natl Acad Sci U S A 101 30 2004 11159 11164
59. A. Fergani, J. Eschbach, H. Oudart, Y. Larmet, B. Schwalenstocker, and A.C. Ludolph et al. A mutation in the dynein heavy chain gene compensates for energy deficit of mutant SOD1 mice and increases potentially neuroprotective IGF-1 Mol Neurodegener 6 1 2011 26
60. A. Fergani, H. Oudart, J.L. Gonzalez De Aguilar, B. Fricker, F. Rene, and J.F. Hocquette et al. Increased peripheral lipid clearance in an animal model of amyotrophic lateral sclerosis J Lipid Res 48 7 2007 1571 1580
61. G. van den Berghe The role of the liver in metabolic homeostasis: implications for inborn errors of metabolism J Inherit Metab Dis 14 4 1991 407 420
62. R. Dolezalova, Z. Lacinova, M. Dolinkova, P. Kleiblova, D. Haluzikova, and D. Housa et al. Changes of endocrine function of adipose tissue in anorexia nervosa: comparison of circulating levels versus subcutaneous mRNA expression Clin Endocrinol (Oxf) 67 5 2007 674 678
63. A. Tremblay, J.P. Despres, C. Leblanc, C.L. Craig, B. Ferris, and T. Stephens et al. Effect of intensity of physical activity on body fatness and fat distribution Am J Clin Nutr 51 2 1990 153 157
64. H. al-Adsani, L.J. Hoffer, and J.E. Silva Resting energy expenditure is sensitive to small dose changes in patients on chronic thyroid hormone replacement J Clin Endocrinol Metab 82 4 1997 1118 1125
65. Y. Song, X. Yao, and H. Ying Thyroid hormone action in metabolic regulation Protein Cell 2 5 2011 358 368
66. T.J. Visser Pathways of thyroid hormone metabolism Acta Med Austriaca 23 1-2 1996 10 16
67. R. Martinez de Mena, T.S. Scanlan, and M.J. Obregon The T3 receptor beta1 isoform regulates UCP1 and D2 deiodinase in rat brown adipocytes Endocrinology 151 10 2010 5074 5083
68. M.S. Queiroz, Y. Shao, and F. Ismail-Beigi Effect of thyroid hormone on uncoupling protein-3 mRNA expression in rat heart and skeletal muscle Thyroid 14 3 2004 177 185
69. J. Li, J.M. Paulson, F.D. Ye, M. Sung, A.N. Hollenberg, and S.B. Rutkove Reducing systemic hypermetabolism by inducing hypothyroidism does not prolong survival in the SOD1-G93A mouse Amyotroph Lateral Scler 13 4 2012 372 377
70. J. Ilzecka, and Z. Stelmasiak Thyroid function in patients with amyotrophic lateral sclerosis Ann Univ Mariae Curie Sklodowska Med 58 1 2003 343 347
71. J.P. Malin, R. Kodding, H. Fuhrmann, and A. von zur Muhlen T4, T3 and rT3 levels in serum and cerebrospinal fluid of patients with amyotrophic lateral sclerosis J Neurol 236 1 1989 57 59
72. H. Mitsumoto, E.D. Salgado, D. Negroski, M.R. Hanson, V.D. Salanga, and J.F. Wilber et al. Amyotrophic lateral sclerosis: effects of acute intravenous and chronic subcutaneous administration of thyrotropin-releasing hormone in controlled trials Neurology 36 2 1986 152 159
73. F.J. Steyn, K. Lee, M.J. Fogarty, J.D. Veldhuis, P.A. McCombe, and M.C. Bellingham et al. Growth hormone secretion is correlated with neuromuscular innervation rather than motor neuron number in early-symptomatic male amyotrophic lateral sclerosis mice Endocrinology 154 12 2013 4695 4706
74. F.J. Steyn, S.T. Ngo, J.D. Lee, J.W. Leong, A.J. Buckley, and J.D. Veldhuis et al. Impairments to the GH-IGF-I Axis in hSOD1G93A Mice Give Insight into Possible Mechanisms of GH Dysregulation in Patients with Amyotrophic Lateral Sclerosis Endocrinology 153 8 2012 3735 3746
75. A.A. Sakharova, J.F. Horowitz, S. Surya, N. Goldenberg, M.P. Harber, and K. Symons et al. Role of growth hormone in regulating lipolysis, proteolysis, and hepatic glucose production during fasting J Clin Endocrinol Metab 93 7 2008 2755 2759
76. F. Sacca, M. Quarantelli, C. Rinaldi, T. Tucci, R. Piro, and G. Perrotta et al. A randomized controlled clinical trial of growth hormone in amyotrophic lateral sclerosis: clinical, neuroimaging, and hormonal results J Neurol 259 1 2011 132 138
77. R.A. Smith, S. Melmed, B. Sherman, J. Frane, T.L. Munsat, and B.W. Festoff Recombinant growth hormone treatment of amyotrophic lateral sclerosis Muscle Nerve 16 6 1993 624 633
78. J.F. Bak, N. Moller, and O. Schmitz Effects of growth hormone on fuel utilization and muscle glycogen synthase activity in normal humans Am J Physiol 260 5 Pt 1 1991 E736 E742
79. J.O. Jorgensen, L. Moller, M. Krag, N. Billestrup, and J.S. Christiansen Effects of growth hormone on glucose and fat metabolism in human subjects Endocrinol Metab Clin North Am 36 1 2007 75 87
80. N. Moller, and J.O. Jorgensen Effects of growth hormone on glucose, lipid, and protein metabolism in human subjects Endocr Rev 30 2 2009 152 177
81. E.M. Mercken, B.A. Carboneau, S.M. Krzysik-Walker, and R. de Cabo Of mice and men: the benefits of caloric restriction, exercise, and mimetics Ageing Res Rev 11 3 2012 390 398
82. R. Weindruch, R.L. Walford, S. Fligiel, and D. Guthrie The retardation of aging in mice by dietary restriction: longevity, cancer, immunity and lifetime energy intake J Nutr 116 4 1986 641 654
83. B.J. Willcox, D.C. Willcox, H. Todoriki, A. Fujiyoshi, K. Yano, and Q. He et al. Caloric restriction, the traditional Okinawan diet, and healthy aging: the diet of the world's longest-lived people and its potential impact on morbidity and life span Ann N Y Acad Sci 1114 2007 434 455
84. D.C. Willcox, B.J. Willcox, H. Todoriki, J.D. Curb, and M. Suzuki Caloric restriction and human longevity: what can we learn from the Okinawans? Biogerontology 7 3 2006 173 177
85. J. Wang, B. Laferrere, J.C. Thornton, R.N. Pierson Jr., and F.X. Pi-Sunyer Regional subcutaneous-fat loss induced by caloric restriction in obese women Obes Res 10 9 2002 885 890
86. J. Ye, and J.N. Keller Regulation of energy metabolism by inflammation: a feedback response in obesity and calorie restriction Aging (Albany NY) 2 6 2010 361 368
87. M.J. Hamadeh, M.C. Rodriguez, J.J. Kaczor, and M.A. Tarnopolsky Caloric restriction transiently improves motor performance but hastens clinical onset of disease in the Cu/Zn-superoxide dismutase mutant G93A mouse Muscle Nerve 31 2 2005 214 220
88. M.J. Hamadeh, and M.A. Tarnopolsky Transient caloric restriction in early adulthood hastens disease endpoint in male, but not female, Cu/Zn-SOD mutant G93A mice Muscle Nerve 34 6 2006 709 719
89. B.P. Patel, A. Safdar, S. Raha, M.A. Tarnopolsky, and M.J. Hamadeh Caloric restriction shortens lifespan through an increase in lipid peroxidation, inflammation and apoptosis in the G93A mouse, an animal model of ALS PLoS One 5 2 2010 e9386
90. C. Canto, and J. Auwerx PGC-1alpha, SIRT1 and AMPK, an energy sensing network that controls energy expenditure Curr Opin Lipidol 20 2 2009 98 105
91. E.H. Jeninga, K. Schoonjans, and J. Auwerx Reversible acetylation of PGC-1: connecting energy sensors and effectors to guarantee metabolic flexibility Oncogene 29 33 2010 4617 4624
92. G. Paris, O. Martinaud, A. Petit, A. Cuvelier, D. Hannequin, and P. Roppeneck et al. Oropharyngeal dysphagia in amyotrophic lateral sclerosis alters quality of life J Oral Rehabil 40 3 2013 199 204
93. E. Lindauer, L. Dupuis, H.P. Muller, H. Neumann, A.C. Ludolph, and J. Kassubek Adipose Tissue Distribution Predicts Survival in Amyotrophic Lateral Sclerosis PLoS One 8 6 2013 e67783
94. N. Limousin, H. Blasco, P. Corcia, P.H. Gordon, B. De Toffol, and C. Andres et al. Malnutrition at the time of diagnosis is associated with a shorter disease duration in ALS J Neurol Sci 297 1-2 2010 36 39
95. B. Marin, J.C. Desport, P. Kajeu, P. Jesus, B. Nicolaud, and M. Nicol et al. Alteration of nutritional status at diagnosis is a prognostic factor for survival of amyotrophic lateral sclerosis patients J Neurol Neurosurg Psychiatry 82 6 2011 628 634
96. P.M. Andersen, S. Abrahams, G.D. Borasio, M. de Carvalho, A. Chio, and P. Van Damme et al. EFNS guidelines on the clinical management of amyotrophic lateral sclerosis (MALS) - revised report of an EFNS task force Eur J Neurol 19 3 2012 360 375
97. R.G. Miller, J.A. Rosenberg, D.F. Gelinas, H. Mitsumoto, D. Newman, and R. Sufit et al. Practice parameter: the care of the patient with amyotrophic lateral sclerosis (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology: ALS Practice Parameters Task Force Neurology 52 7 1999 1311 1323
98. J.P. Dworkin, and D.E. Hartman Progressive speech deterioration and dysphagia in amyotrophic lateral sclerosis: case report Arch Phys Med Rehabil 60 9 1979 423 425
99. R. Higo, N. Tayama, and T. Nito Longitudinal analysis of progression of dysphagia in amyotrophic lateral sclerosis Auris Nasus Larynx 31 3 2004 247 254
100. S. Kawai, M. Tsukuda, I. Mochimatsu, H. Enomoto, Y. Kagesato, and H. Hirose et al. A study of the early stage of Dysphagia in amyotrophic lateral sclerosis Dysphagia 18 1 2003 1 8
101. G. Ruoppolo, I. Schettino, V. Frasca, E. Giacomelli, L. Prosperini, and C. Cambieri et al. Dysphagia in amyotrophic lateral sclerosis: prevalence and clinical findings Acta Neurol Scand 128 6 2013 397 401
102. E.A. Strand, R.M. Miller, K.M. Yorkston, and A.D. Hillel Management of oral-pharyngeal dysphagia symptoms in amyotrophic lateral sclerosis Dysphagia 11 2 1996 129 139
103. S. Tamburrini, A. Solazzo, A. Sagnelli, L. Del Vecchio, A. Reginelli, and M. Monsorro et al. Amyotrophic lateral sclerosis: sonographic evaluation of dysphagia Radiol Med 115 5 2010 784 793
104. T. Holm, A. Maier, P. Wicks, D. Lang, P. Linke, and C. Munch et al. Severe loss of appetite in amyotrophic lateral sclerosis patients: online self-assessment study Interact J Med Res 2 1 2013 e8
105. A. Eisen, and S. Kuwabara The split hand syndrome in amyotrophic lateral sclerosis J Neurol Neurosurg Psychiatry 83 4 2012 399 403
106. M.M. Braun, M. Osecheck, and N.C. Joyce Nutrition assessment and management in amyotrophic lateral sclerosis Phys Med Rehabil Clin N Am 23 4 2012 751 771
107. J.A. Allen, R. Chen, S. Ajroud-Driss, R.L. Sufit, S. Heller, and T. Siddique et al. Gastrostomy tube placement by endoscopy versus radiologic methods in patients with ALS: a retrospective study of complications and outcome Amyotroph Lateral Scler Frontotemporal Degener 14 4 2013 308 314
108. A. Blondet, J. Lebigot, G. Nicolas, J. Boursier, B. Person, and L. Laccoureye et al. Radiologic versus endoscopic placement of percutaneous gastrostomy in amyotrophic lateral sclerosis: multivariate analysis of tolerance, efficacy, and survival J Vasc Interv Radiol 21 4 2010 527 533
109. A. Chio, R. Galletti, C. Finocchiaro, D. Righi, M.A. Ruffino, and A. Calvo et al. Percutaneous radiological gastrostomy: a safe and effective method of nutritional tube placement in advanced ALS J Neurol Neurosurg Psychiatry 75 4 2004 645 647
110. A. Chio, E. Finocchiaro, P. Meineri, E. Bottacchi, and D. Schiffer Safety and factors related to survival after percutaneous endoscopic gastrostomy in ALS. ALS Percutaneous Endoscopic Gastrostomy Study Group Neurology 53 5 1999 1123 1125
111. R. Spataro, L. Ficano, F. Piccoli, and V. La Bella Percutaneous endoscopic gastrostomy in amyotrophic lateral sclerosis: effect on survival J Neurol Sci 304 1-2 2011 44 48
112. J. Dorst, J. Cypionka, and A.C. Ludolph High-caloric food supplements in the treatment of amyotrophic lateral sclerosis: A prospective interventional study Amyotroph Lateral Scler Frontotemporal Degener 14 7-8 2013 533 536
113. R.G. Miller, C.E. Jackson, E.J. Kasarskis, J.D. England, D. Forshew, and W. Johnston et al. 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 2009 1218 1226
114. J. Rosenfeld, and A. Ellis Nutrition and dietary supplements in motor neuron disease Phys Med Rehabil Clin N Am 19 3 2008 573 589 [x]
115. A. Rio, M.A. Ampong, M.R. Turner, A.S. Shaw, A. Al-Chalabi, and C.E. Shaw et al. Comparison of two percutaneous radiological gastrostomy tubes in the nutritional management of ALS patients Amyotroph Lateral Scler Other Motor Neuron Disord 6 3 2005 177 181
116. S.E. Langmore, E.J. Kasarskis, M.L. Manca, and R.K. Olney Enteral tube feeding for amyotrophic lateral sclerosis/motor neuron disease Cochrane Database Syst Rev 4 2006 CD004030
117. R.G. Miller, J.A. Rosenberg, D.F. Gelinas, H. Mitsumoto, D. Newman, and R. Sufit et al. Practice parameter: the care of the patient with amyotrophic lateral sclerosis (An evidence-based review) Muscle Nerve 22 8 1999 1104 1118
118. M.A. Heffernan, A.W. Thorburn, B. Fam, R. Summers, B. Conway-Campbell, and M.J. Waters et al. Increase of fat oxidation and weight loss in obese mice caused by chronic treatment with human growth hormone or a modified C-terminal fragment Int J Obes Relat Metab Disord 25 10 2001 1442 1449
119. N. Morozova, M.G. Weisskopf, M.L. McCullough, K.L. Munger, E.E. Calle, and M.J. Thun et al. Diet and amyotrophic lateral sclerosis Epidemiology 19 2 2008 324 337
120. K. Okamoto, T. Kihira, T. Kondo, G. Kobashi, M. Washio, and S. Sasaki et al. Nutritional status and risk of amyotrophic lateral sclerosis in Japan Amyotroph Lateral Scler 8 5 2007 300 304
121. J.H. Veldink, S. Kalmijn, G.J. Groeneveld, W. Wunderink, A. Koster, and J.H. de Vries et al. Intake of polyunsaturated fatty acids and vitamin E reduces the risk of developing amyotrophic lateral sclerosis J Neurol Neurosurg Psychiatry 78 4 2007 367 371
122. P. Stanich, A. Chiapetta, A. Oliveria, and A. Gabbai Nutritional supplementation in patients with amyotrophic lateral sclerosis Amyotroph Lateral Scler Other Motor Neuron Disord 3 S2 2002 119
123. L.B. Silva, L.F. Mourao, A.A. Silva, N.M. Lima, S.R. Almeida, and M.C. Franca Jr. et al. Effect of nutritional supplementation with milk whey proteins in amyotrophic lateral sclerosis patients Arq Neuropsiquiatr 68 2 2010 263 268
124. M.P. Mattson, R.G. Cutler, and S. Camandola Energy intake and amyotrophic lateral sclerosis Neuromolecular Med 9 1 2007 17 20
125. J.L. Gonzalez de Aguilar, L. Dupuis, H. Oudart, and J.P. Loeffler The metabolic hypothesis in amyotrophic lateral sclerosis: insights from mutant Cu/Zn-superoxide dismutase mice Biomed Pharmacother 59 4 2005 190 196
126. J. Zhou, J. Yi, R. Fu, E. Liu, T. Siddique, and E. Rios et al. Hyperactive intracellular calcium signaling associated with localized mitochondrial defects in skeletal muscle of an animal model of amyotrophic lateral sclerosis J Biol Chem 285 1 2010 705 712
127. K.J. Bough, J. Wetherington, B. Hassel, J.F. Pare, J.W. Gawryluk, and J.G. Greene et al. Mitochondrial biogenesis in the anticonvulsant mechanism of the ketogenic diet Ann Neurol 60 2 2006 223 235
128. A.L. Hartman, M. Gasior, E.P. Vining, and M.A. Rogawski The neuropharmacology of the ketogenic diet Pediatr Neurol 36 5 2007 281 292
129. Z. Zhao, D.J. Lange, A. Voustianiouk, D. MacGrogan, L. Ho, and J. Suh et al. A ketogenic diet as a potential novel therapeutic intervention in amyotrophic lateral sclerosis BMC Neurosci 7 2006 29
130. A. Amari, N.C. Grace, and W.W. Fisher Achieving and maintaining compliance with the ketogenic diet J Appl Behav Anal 28 3 1995 341 342
131. A. Mosek, H. Natour, M.Y. Neufeld, Y. Shiff, and N. Vaisman Ketogenic diet treatment in adults with refractory epilepsy: a prospective pilot study Seizure 18 1 2009 30 33
132. P. Klein, J. Janousek, A. Barber, and R. Weissberger Ketogenic diet treatment in adults with refractory epilepsy Epilepsy Behav 19 4 2010 575 579
133. P.R. Huttenlocher, A.J. Wilbourn, and J.M. Signore Medium-chain triglycerides as a therapy for intractable childhood epilepsy Neurology 21 11 1971 1097 1103
134. Y.M. Liu, and H.S. Wang Medium-chain triglyceride ketogenic diet, an effective treatment for drug-resistant epilepsy and a comparison with other ketogenic diets Biomed. J. 36 1 2013 9 15
135. W. Zhao, M. Varghese, P. Vempati, A. Dzhun, A. Cheng, and J. Wang et al. 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 2012 e49191
136. V. Brower Nutraceuticals: poised for a healthy slice of the healthcare market? Nat Biotechnol 16 8 1998 728 731
137. A. Ascherio, M.G. Weisskopf, E.J. O'Reilly, E.J. Jacobs, M.L. McCullough, and E.E. Calle et al. Vitamin E intake and risk of amyotrophic lateral sclerosis Ann Neurol 57 1 2005 104 110
138. H. Wang, E.J. O'Reilly, M.G. Weisskopf, G. Logroscino, M.L. McCullough, and A. Schatzkin et al. Vitamin E intake and risk of amyotrophic lateral sclerosis: a pooled analysis of data from 5 prospective cohort studies Am J Epidemiol 173 6 2011 595 602
139. D. Albani, L. Polito, A. Signorini, and G. Forloni Neuroprotective properties of resveratrol in different neurodegenerative disorders Biofactors 36 5 2010 370 376
140. N.C. Whitlock, and S.J. Baek The anticancer effects of resveratrol: modulation of transcription factors Nutr Cancer 64 4 2012 493 502
141. H. Li, N. Xia, and U. Forstermann Cardiovascular effects and molecular targets of resveratrol Nitric Oxide 26 2 2012 102 110
142. K.T. Howitz, K.J. Bitterman, H.Y. Cohen, D.W. Lamming, S. Lavu, and J.G. Wood et al. Small molecule activators of sirtuins extend Saccharomyces cerevisiae lifespan Nature 425 6954 2003 191 196
143. J. Wang, Y. Zhang, L. Tang, N. Zhang, and D. Fan Protective effects of resveratrol through the up-regulation of SIRT1 expression in the mutant hSOD1-G93A-bearing motor neuron-like cell culture model of amyotrophic lateral sclerosis Neurosci Lett 503 3 2011 250 255
144. M. Yanez, L. Galan, J. Matias-Guiu, A. Vela, A. Guerrero, and A.G. Garcia CSF from amyotrophic lateral sclerosis patients produces glutamate independent death of rat motor brain cortical neurons: protection by resveratrol but not riluzole Brain Res 1423 2011 77 86
145. R. Mancuso, J. Del Valle, L. Modol, A. Martinez, A.B. Granado-Serrano, and O. Ramirez-Nunez et al. Resveratrol Improves Motoneuron Function and Extends Survival in SOD1 ALS Mice Neurotherapeutics 2014 10.1007/s13311-013-0253-y (Published online, ahead of print)
146. T. Walle, F. Hsieh, M.H. DeLegge, J.E. Oatis Jr., and U.K. Walle High absorption but very low bioavailability of oral resveratrol in humans Drug Metab Dispos 32 12 2004 1377 1382
147. N. Vaisman, M. Lusaus, B. Nefussy, E. Niv, D. Comaneshter, and R. Hallack et al. Do patients with amyotrophic lateral sclerosis (ALS) have increased energy needs? J Neurol Sci 279 1-2 2009 26 29
148. A.M. Wills, J. Hubbard, E.A. Macklin, J. Glass, R. Tandan, and E.P. Simpson et al. Hypercaloric enteral nutrition in patients with amyotrophic lateral sclerosis: a randomised, double-blind, placebo-controlled phase 2 trial. Lancet 2014 10.1016/S0140-6736(14)60222-1 [Early Online Publication, 28 February 2014]