筋萎縮性側索硬化症の代謝異常と栄養療法
「I. 体重減少とエネルギー代謝亢進」筋萎縮性側索硬化症(amyotrophic lateral sclerosis:ALS)は, 上位・下位運動ニューロンが選択的に変性することにより, 進行性の骨格筋萎縮を呈するが, その病初期もしくは病前から筋萎縮や嚥下障害では説明できない体重減少を呈することが知られている. 体重減少の原因は多要因であると考えられており, 骨格筋量の減少, 摂食嚥下障害によるエネルギー摂取量の低下, 進行期における呼吸障害による呼吸筋エネルギー消費の増大のほか, 疾患特異的なエネルギー代謝の亢進状態(hypermetabolism)が存在すると言われている. 体重減少を呈...
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| Published in | 神経治療学 Vol. 39; no. 1; pp. 22 - 26 |
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| Main Author | |
| Format | Journal Article |
| Language | Japanese |
| Published |
日本神経治療学会
2022
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| Subjects | |
| Online Access | Get full text |
| ISSN | 0916-8443 2189-7824 |
| DOI | 10.15082/jsnt.39.1_22 |
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| Abstract | 「I. 体重減少とエネルギー代謝亢進」筋萎縮性側索硬化症(amyotrophic lateral sclerosis:ALS)は, 上位・下位運動ニューロンが選択的に変性することにより, 進行性の骨格筋萎縮を呈するが, その病初期もしくは病前から筋萎縮や嚥下障害では説明できない体重減少を呈することが知られている. 体重減少の原因は多要因であると考えられており, 骨格筋量の減少, 摂食嚥下障害によるエネルギー摂取量の低下, 進行期における呼吸障害による呼吸筋エネルギー消費の増大のほか, 疾患特異的なエネルギー代謝の亢進状態(hypermetabolism)が存在すると言われている. 体重減少を呈する神経変性疾患は多数あるが, ALSが他の疾患と異なるのは, 病初期の体重減少が独立した生命予後予測因子であることが確立されていることである. 1999年に初診時の肥満度指数(body mass index:BMI)が18.5kg/m2未満の患者では生命予後が極めて悪いことが最初に報告された後, 同じような報告が現在まで数多く発表されている. 本邦でも発症から診断時までのBMI減少率が2～2.5kg/m2/年以上の患者は生命予後が悪いと報告された. |
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| AbstractList | 「I. 体重減少とエネルギー代謝亢進」筋萎縮性側索硬化症(amyotrophic lateral sclerosis:ALS)は, 上位・下位運動ニューロンが選択的に変性することにより, 進行性の骨格筋萎縮を呈するが, その病初期もしくは病前から筋萎縮や嚥下障害では説明できない体重減少を呈することが知られている. 体重減少の原因は多要因であると考えられており, 骨格筋量の減少, 摂食嚥下障害によるエネルギー摂取量の低下, 進行期における呼吸障害による呼吸筋エネルギー消費の増大のほか, 疾患特異的なエネルギー代謝の亢進状態(hypermetabolism)が存在すると言われている. 体重減少を呈する神経変性疾患は多数あるが, ALSが他の疾患と異なるのは, 病初期の体重減少が独立した生命予後予測因子であることが確立されていることである. 1999年に初診時の肥満度指数(body mass index:BMI)が18.5kg/m2未満の患者では生命予後が極めて悪いことが最初に報告された後, 同じような報告が現在まで数多く発表されている. 本邦でも発症から診断時までのBMI減少率が2～2.5kg/m2/年以上の患者は生命予後が悪いと報告された. |
| Author | 清水, 俊夫 |
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| References | 50) Shimizu T, Honda M, Ohashi T et al : Hyperosmolar hyperglycemic state in advanced amyotrophic lateral sclerosis. Amyotroph Lateral Scler 12 : 379–381, 2011 27) Ingre C, Chen L, Zhan Y et al : Lipids, apolipoproteins, and prognosis of amyotrophic lateral sclerosis. Neurology 94 : e1835–e1844, 2020 33) Dupuis L, Oudart H, Rene F et al : 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 : 11159–11164, 2004 21) Gabery S, Ahmed RM, Caga J et al : Loss of the metabolism and sleep regulating neuronal populations expressing orexin and oxytocin in the hypothalamus in amyotrophic lateral sclerosis. Neuropathol Appl Neurobiol, early online, 2021 49) Nishiyama Y, Abe K, Tokunaga J et al : Metabolic syndrome in advanced amyotrophic lateral sclerosis patients with tracheostomy–invasive ventilation. Neurol Clin Neurosci 7 : 174–179, 2019 32) Schumacher J, Peter RS, Nagel G et al : Statins, diabetes mellitus and prognosis of amyotrophic lateral sclerosis : data from 501 patients of a population–based registry in southwest Germany. Eur J Neurol 27 : 1405–1414, 2020 23) Vercruysse P, Vieau D, Blum D et al : Hypothalamic alterations in neurodegenerative diseases and their relation to abnormal energy metabolism. Front Mol Neurosci 11 : 2, 2018 19) Gorges M, Vercruysse P, Muller HP et al : Hypothalamic atrophy is related to body mass index and age at onset in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry 88 : 1033–1041, 2017 43) McDonnell E, Schoenfeld D, Paganoni S et al : Causal inference methods to study gastric tube use in amyotrophic lateral sclerosis. Neurology 89 : 1483–1489, 2017 15) Shimizu T, Ishikawa–Takata K, Sakata A et al : The measurement and estimation of total energy expenditure in Japanese patients with ALS : a doubly labelled water method study. Amyotroph Lateral Scler Frontotemporal Degener 18 : 37–45, 2017 31) Zinman L, Sadeghi R, Gawel M et al : Are statin medications safe in patients with ALS? Amyotroph Lateral Scler 9 : 223–228, 2008 30) Tefera TW, Tan KN, McDonald TS et al : Alternative fuels in epilepsy and amyotrophic lateral sclerosis. Neurochem Res 42 : 1610–1620, 2017 35) Ludolph AC, Dorst J, Dreyhaupt J et al : Effect of high–caloric nutrition on survival in amyotrophic lateral sclerosis. Ann Neurol 87 : 206–216, 2020 45) Amano H, Imamura K, Hatano F et al : A newly developed mask facilitates management during percutaneous endoscopic gastrostomy introduction in patients with respiratory compromise. Am Surg 82 : 298–300, 2016 37) Jesus P, Marin B, Fayemendy P et al : Resting energy expenditure equations in amyotrophic lateral sclerosis, creation of an ALS–specific equation. Clin Nutr 38 : 1657–1665, 2019 14) Kasarskis EJ, Mendiondo MS, Matthews DE et al : Estimating daily energy expenditure in individuals with amyotrophic lateral sclerosis. Am J Clin Nutr 99 : 792–803, 2014 29) Ferri A, Coccurello R : What is “hyper” in the ALS hypermetabolism? Mediators Inflamm 2017 : 7821672, 2017 40) Ichihara N, Namba K, Ishikawa–Takata K et al : Energy requirement assessed by doubly–labeled water method in patients with advanced amyotrophic lateral sclerosis managed by tracheotomy positive pressure ventilation. Amyotroph Lateral Scler 13 : 544–549, 2012 22) Ahmed RM, Irish M, Piguet O et al : Amyotrophic lateral sclerosis and frontotemporal dementia : distinct and overlapping changes in eating behaviour and metabolism. Lancet Neurol 15 : 332–342, 2016 7) Lee I, Kazamel M, McPherson T et al : Fat mass loss correlates with faster disease progression in amyotrophic lateral sclerosis patients : Exploring the utility of dual–energy x–ray absorptiometry in a prospective study. PLoS One 16 : e0251087, 2021 16) Hayashi K, Mochizuki Y, Takeuchi R et al : Clinicopathological characteristics of patients with amyotrophic lateral sclerosis resulting in a totally locked–in state (communication Stage V). Acta Neuropathol Commun 4 : 107, 2016 2) Bouteloup C, Desport JC, Clavelou P et al : Hypermetabolism in ALS patients : an early and persistent phenomenon. J Neurol 256 : 1236–1242, 2009 47) Stavroulakis T, Walsh T, Shaw PJ et al : Gastrostomy use in motor neurone disease (MND) : a review, meta–analysis and survey of current practice. Amyotroph Lateral Scler Frontotemporal Degener 14 : 96–104, 2013 55) Ngo ST, Wang H, Henderson RD et al : Ghrelin as a treatment for amyotrophic lateral sclerosis. J Neuroendocrinol 33 : e12938, 2021 11) Shimizu T, Nakayama Y, Matsuda C et al : Prognostic significance of body weight variation after diagnosis in ALS : a single–centre prospective cohort study. J Neurol 266 : 1412–1420, 2019 24) Dupuis L, Corcia P, Fergani A et al : Dyslipidemia is a protective factor in amyotrophic lateral sclerosis. Neurology 70 : 1004–1009, 2008 1) Dupuis L, Pradat PF, Ludolph AC et al : Energy metabolism in amyotrophic lateral sclerosis. Lancet Neurol 10 : 75–82, 2011 10) Shimizu T, Nagaoka U, Nakayama Y et al : Reduction rate of body mass index predicts prognosis for survival in amyotrophic lateral sclerosis : a multicenter study in Japan. Amyotroph Lateral Scler 13 : 363–366, 2012 34) Wills AM, Hubbard J, Macklin EA et al : Hypercaloric enteral nutrition in patients with amyotrophic lateral sclerosis : a randomised, double–blind, placebo–controlled phase 2 trial. Lancet 383 : 2065–2072, 2014 39) Georges M, Morelot–Panzini C, Similowski T et al : Noninvasive ventilation reduces energy expenditure in amyotrophic lateral sclerosis. BMC Pulm Med 14 : 17, 2014 18) Cykowski MD, Takei H, Schulz PE et al : TDP–43 pathology in the basal forebrain and hypothalamus of patients with amyotrophic lateral sclerosis. Acta Neuropathol Commun 2 : 171, 2014 51) Nakayama Y, Shimizu T, Matsuda C et al : Non–motor manifestations in ALS patients with tracheostomy and invasive ventilation. Muscle Nerve 57 : 735–741, 2018 48) 清水俊夫：NPPVと摂食・嚥下および栄養管理．筋萎縮性側索硬化症の包括的呼吸ケア指針—呼吸理学療法と非侵襲陽圧換気療法（NPPV），http://www.nanbyou.or.jp/pdf/2008als.pdf, 2008 13) Nakamura R, Kurihara M, Ogawa N et al : Prognostic prediction by hypermetabolism varies depending on the nutritional status in early amyotrophic lateral sclerosis. Sci Rep 11 : 17943, 2021 53) McKee HR, Escott E, Damm D et al : Macroglossia in amyotrophic lateral sclerosis. JAMA Neurol 70 : 1432–1435, 2013 36) Dorst J, Schuster J, Dreyhaupt J et al : Effect of high–caloric nutrition on serum neurofilament light chain levels in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry 91 : 1007–1009, 2020 54) Shimizu T, Hayashi H, Kato S et al : Circulatory collapse and sudden death in respirator–dependent amyotrophic lateral sclerosis. J Neurol Sci 124 : 45–55, 1994 17) Geser F, Brandmeir NJ, Kwong LK et al : Evidence of multisystem disorder in whole–brain map of pathological TDP–43 in amyotrophic lateral sclerosis. Arch Neurol 65 : 636–641, 2008 42) Miller RG, Jackson CE, Kasarskis EJ 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 : 1218–1226, 2009 6) Janse van Mantgem MR, van Eijk RPA, van der Burgh HK et al : Prognostic value of weight loss in patients with amyotrophic lateral sclerosis : a population–based study. J Neurol Neurosurg Psychiatry 91 : 867–875, 2020 12) Nakayama Y, Shimizu T, Matsuda C et al : Body weight variation predicts disease progression after invasive ventilation in amyotrophic lateral sclerosis. Sci Rep 9 : 12262, 2019 5) Desport JC, Preux PM, Truong TC et al : Nutritional status is a prognostic factor for survival in ALS patients. Neurology 53 : 1059–1063, 1999 46) Banfi P, Volpato E, Valota C et al : Use of Noninvasive ventilation during feeding tube placement. Respir Care 62 : 1474–1484, 2017 9) Moglia C, Calvo A, Grassano M et al : Early weight loss in amyotrophic lateral sclerosis : outcome relevance and clinical correlates in a population–based cohort. J Neurol Neurosurg Psychiatry 90 : 666–673, 2019 4) Steyn FJ, Ioannides ZA, van Eijk RPA et al : Hypermetabolism in ALS is associated with greater functional decline and shorter survival. J Neurol Neurosurg Psychiatry 89 : 1016–1023, 2018 20) Vercruysse P, Sinniger J, El Oussini H et al : Alterations in the hypothalamic melanocortin pathway in amyotrophic lateral sclerosis. Brain 139 : 1106–1122, 2016 3) Jesus P, Fayemendy P, Nicol M et al : Hypermetabolism is a deleterious prognostic factor in patients with amyotrophic lateral sclerosis. Eur J Neurol 25 : 97–104, 2018 38) Kurihara M, Bamba S, Yasuhara S et al : Factors affecting energy metabolism and prognosis in patients with amyotrophic lateral sclerosis. Ann Nutr Metab 77 : 236–243, 2021 52) Matsuda C, Shimizu T, Nakayama Y et al : Macroglossia in advanced amyotrophic lateral sclerosis. Muscle Nerve 54 : 386–390, 2016 41) 清水俊夫，林 秀明，田邊 等：呼吸器補助・経管栄養下のALS患者の必要エネルギー量の検討．臨神経 31 : 255–259, 1991 44) Bokuda K, Shimizu T, Imamura K et al : Predictive factors for prognosis following unsedated percutaneous endoscopic gastrostomy in ALS patients. Muscle Nerve 54 : 277–283, 2016 25) Chelstowska B, Baranczyk–Kuzma A, Kuzma–Kozakiewicz M : Dyslipidemia in patients with amyotrophic lateral sclerosis – a case control retrospective study. Amyotroph Lateral Scler Frontotemporal Degener 22 : 195–205, 2021 26) Chio A, Calvo A, Ilardi A et al : Lower serum lipid levels are related to respiratory impairment in patients with ALS. Neurology 73 : 1681–1685, 2009 28) Tefera TW, Steyn FJ, Ngo ST et al : CNS glucose metabolism in Amyotrophic Lateral Sclerosis : a therapeutic target? Cell Biosci 11 : 14, 2021 8) Marin B, Desport JC, Kajeu P et al : Alteration of nutritional status at diagnosis is a prognostic factor for survival of amyotrophic lateral sclerosis patients. J Neurol Neurosurg Psychiatry 82 : 628–634, 2011 |
| References_xml | – reference: 27) Ingre C, Chen L, Zhan Y et al : Lipids, apolipoproteins, and prognosis of amyotrophic lateral sclerosis. Neurology 94 : e1835–e1844, 2020 – reference: 24) Dupuis L, Corcia P, Fergani A et al : Dyslipidemia is a protective factor in amyotrophic lateral sclerosis. Neurology 70 : 1004–1009, 2008 – reference: 38) Kurihara M, Bamba S, Yasuhara S et al : Factors affecting energy metabolism and prognosis in patients with amyotrophic lateral sclerosis. Ann Nutr Metab 77 : 236–243, 2021 – reference: 8) Marin B, Desport JC, Kajeu P et al : Alteration of nutritional status at diagnosis is a prognostic factor for survival of amyotrophic lateral sclerosis patients. J Neurol Neurosurg Psychiatry 82 : 628–634, 2011 – reference: 18) Cykowski MD, Takei H, Schulz PE et al : TDP–43 pathology in the basal forebrain and hypothalamus of patients with amyotrophic lateral sclerosis. Acta Neuropathol Commun 2 : 171, 2014 – reference: 32) Schumacher J, Peter RS, Nagel G et al : Statins, diabetes mellitus and prognosis of amyotrophic lateral sclerosis : data from 501 patients of a population–based registry in southwest Germany. Eur J Neurol 27 : 1405–1414, 2020 – reference: 30) Tefera TW, Tan KN, McDonald TS et al : Alternative fuels in epilepsy and amyotrophic lateral sclerosis. Neurochem Res 42 : 1610–1620, 2017 – reference: 44) Bokuda K, Shimizu T, Imamura K et al : Predictive factors for prognosis following unsedated percutaneous endoscopic gastrostomy in ALS patients. Muscle Nerve 54 : 277–283, 2016 – reference: 47) Stavroulakis T, Walsh T, Shaw PJ et al : Gastrostomy use in motor neurone disease (MND) : a review, meta–analysis and survey of current practice. Amyotroph Lateral Scler Frontotemporal Degener 14 : 96–104, 2013 – reference: 34) Wills AM, Hubbard J, Macklin EA et al : Hypercaloric enteral nutrition in patients with amyotrophic lateral sclerosis : a randomised, double–blind, placebo–controlled phase 2 trial. Lancet 383 : 2065–2072, 2014 – reference: 15) Shimizu T, Ishikawa–Takata K, Sakata A et al : The measurement and estimation of total energy expenditure in Japanese patients with ALS : a doubly labelled water method study. Amyotroph Lateral Scler Frontotemporal Degener 18 : 37–45, 2017 – reference: 41) 清水俊夫，林 秀明，田邊 等：呼吸器補助・経管栄養下のALS患者の必要エネルギー量の検討．臨神経 31 : 255–259, 1991 – reference: 39) Georges M, Morelot–Panzini C, Similowski T et al : Noninvasive ventilation reduces energy expenditure in amyotrophic lateral sclerosis. BMC Pulm Med 14 : 17, 2014 – reference: 19) Gorges M, Vercruysse P, Muller HP et al : Hypothalamic atrophy is related to body mass index and age at onset in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry 88 : 1033–1041, 2017 – reference: 31) Zinman L, Sadeghi R, Gawel M et al : Are statin medications safe in patients with ALS? Amyotroph Lateral Scler 9 : 223–228, 2008 – reference: 37) Jesus P, Marin B, Fayemendy P et al : Resting energy expenditure equations in amyotrophic lateral sclerosis, creation of an ALS–specific equation. Clin Nutr 38 : 1657–1665, 2019 – reference: 17) Geser F, Brandmeir NJ, Kwong LK et al : Evidence of multisystem disorder in whole–brain map of pathological TDP–43 in amyotrophic lateral sclerosis. Arch Neurol 65 : 636–641, 2008 – reference: 48) 清水俊夫：NPPVと摂食・嚥下および栄養管理．筋萎縮性側索硬化症の包括的呼吸ケア指針—呼吸理学療法と非侵襲陽圧換気療法（NPPV），http://www.nanbyou.or.jp/pdf/2008als.pdf, 2008 – reference: 52) Matsuda C, Shimizu T, Nakayama Y et al : Macroglossia in advanced amyotrophic lateral sclerosis. Muscle Nerve 54 : 386–390, 2016 – reference: 11) Shimizu T, Nakayama Y, Matsuda C et al : Prognostic significance of body weight variation after diagnosis in ALS : a single–centre prospective cohort study. J Neurol 266 : 1412–1420, 2019 – reference: 54) Shimizu T, Hayashi H, Kato S et al : Circulatory collapse and sudden death in respirator–dependent amyotrophic lateral sclerosis. J Neurol Sci 124 : 45–55, 1994 – reference: 35) Ludolph AC, Dorst J, Dreyhaupt J et al : Effect of high–caloric nutrition on survival in amyotrophic lateral sclerosis. Ann Neurol 87 : 206–216, 2020 – reference: 43) McDonnell E, Schoenfeld D, Paganoni S et al : Causal inference methods to study gastric tube use in amyotrophic lateral sclerosis. Neurology 89 : 1483–1489, 2017 – reference: 20) Vercruysse P, Sinniger J, El Oussini H et al : Alterations in the hypothalamic melanocortin pathway in amyotrophic lateral sclerosis. Brain 139 : 1106–1122, 2016 – reference: 45) Amano H, Imamura K, Hatano F et al : A newly developed mask facilitates management during percutaneous endoscopic gastrostomy introduction in patients with respiratory compromise. Am Surg 82 : 298–300, 2016 – reference: 4) Steyn FJ, Ioannides ZA, van Eijk RPA et al : Hypermetabolism in ALS is associated with greater functional decline and shorter survival. J Neurol Neurosurg Psychiatry 89 : 1016–1023, 2018 – reference: 22) Ahmed RM, Irish M, Piguet O et al : Amyotrophic lateral sclerosis and frontotemporal dementia : distinct and overlapping changes in eating behaviour and metabolism. Lancet Neurol 15 : 332–342, 2016 – reference: 16) Hayashi K, Mochizuki Y, Takeuchi R et al : Clinicopathological characteristics of patients with amyotrophic lateral sclerosis resulting in a totally locked–in state (communication Stage V). Acta Neuropathol Commun 4 : 107, 2016 – reference: 29) Ferri A, Coccurello R : What is “hyper” in the ALS hypermetabolism? Mediators Inflamm 2017 : 7821672, 2017 – reference: 25) Chelstowska B, Baranczyk–Kuzma A, Kuzma–Kozakiewicz M : Dyslipidemia in patients with amyotrophic lateral sclerosis – a case control retrospective study. Amyotroph Lateral Scler Frontotemporal Degener 22 : 195–205, 2021 – reference: 13) Nakamura R, Kurihara M, Ogawa N et al : Prognostic prediction by hypermetabolism varies depending on the nutritional status in early amyotrophic lateral sclerosis. Sci Rep 11 : 17943, 2021 – reference: 23) Vercruysse P, Vieau D, Blum D et al : Hypothalamic alterations in neurodegenerative diseases and their relation to abnormal energy metabolism. Front Mol Neurosci 11 : 2, 2018 – reference: 7) Lee I, Kazamel M, McPherson T et al : Fat mass loss correlates with faster disease progression in amyotrophic lateral sclerosis patients : Exploring the utility of dual–energy x–ray absorptiometry in a prospective study. PLoS One 16 : e0251087, 2021 – reference: 40) Ichihara N, Namba K, Ishikawa–Takata K et al : Energy requirement assessed by doubly–labeled water method in patients with advanced amyotrophic lateral sclerosis managed by tracheotomy positive pressure ventilation. Amyotroph Lateral Scler 13 : 544–549, 2012 – reference: 42) Miller RG, Jackson CE, Kasarskis EJ 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 : 1218–1226, 2009 – reference: 21) Gabery S, Ahmed RM, Caga J et al : Loss of the metabolism and sleep regulating neuronal populations expressing orexin and oxytocin in the hypothalamus in amyotrophic lateral sclerosis. Neuropathol Appl Neurobiol, early online, 2021 – reference: 26) Chio A, Calvo A, Ilardi A et al : Lower serum lipid levels are related to respiratory impairment in patients with ALS. Neurology 73 : 1681–1685, 2009 – reference: 28) Tefera TW, Steyn FJ, Ngo ST et al : CNS glucose metabolism in Amyotrophic Lateral Sclerosis : a therapeutic target? Cell Biosci 11 : 14, 2021 – reference: 55) Ngo ST, Wang H, Henderson RD et al : Ghrelin as a treatment for amyotrophic lateral sclerosis. J Neuroendocrinol 33 : e12938, 2021 – reference: 2) Bouteloup C, Desport JC, Clavelou P et al : Hypermetabolism in ALS patients : an early and persistent phenomenon. J Neurol 256 : 1236–1242, 2009 – reference: 1) Dupuis L, Pradat PF, Ludolph AC et al : Energy metabolism in amyotrophic lateral sclerosis. Lancet Neurol 10 : 75–82, 2011 – reference: 3) Jesus P, Fayemendy P, Nicol M et al : Hypermetabolism is a deleterious prognostic factor in patients with amyotrophic lateral sclerosis. 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| Snippet | 「I. 体重減少とエネルギー代謝亢進」筋萎縮性側索硬化症(amyotrophic lateral sclerosis:ALS)は, 上位・下位運動ニューロンが選択的に変性することにより, 進行性の骨格筋萎縮を呈するが, その病初期もしくは病前から筋萎縮や嚥下障害では説明できない体重減少を呈することが知られている.... |
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| SubjectTerms | amyotrophic lateral sclerosis hypermetabolism nutritional intervention survival weight loss |
| Title | 筋萎縮性側索硬化症の代謝異常と栄養療法 |
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