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肌萎缩侧索硬化症的限时生酮饮食:一项病例研究。

Time-restricted ketogenic diet in amyotrophic lateral sclerosis: a case study.

作者信息

Phillips Matthew C L, Johnston Samuel E, Simpson Pat, Chang David K, Mather Danielle, Dick Rognvald J

机构信息

Department of Neurology, Waikato Hospital, Hamilton, New Zealand.

Older Persons and Rehabilitation Service, Waikato Hospital, Hamilton, New Zealand.

出版信息

Front Neurol. 2024 Jan 18;14:1329541. doi: 10.3389/fneur.2023.1329541. eCollection 2023.

Abstract

Amyotrophic lateral sclerosis (ALS) is an incurable neurodegenerative disorder. The most devastating variant is bulbar-onset ALS, which portends a median survival of 24 months from the onset of symptoms. Abundant evidence indicates that neuron metabolism and mitochondrial function are impaired in ALS. Metabolic strategies, particularly fasting and ketogenic diet protocols, alter neuron metabolism and mitochondria function in a manner that may mitigate the symptoms of this disorder. We report the case of a 64-year-old man with a 21-month history of progressive, deteriorating bulbar-onset ALS, with an associated pseudobulbar affect, who implemented a time-restricted ketogenic diet (TRKD) for 18 months. During this time, he improved in ALS-related function (7% improvement from baseline), forced expiratory volume (17% improvement), forced vital capacity (13% improvement), depression (normalized), stress levels (normalized), and quality of life (19% improvement), particularly fatigue (23% improvement). His swallowing impairment and neurocognitive status remained stable. Declines were measured in physical function, maximal inspiratory pressure, and maximal expiratory pressure. Weight loss was attenuated and no significant adverse effects occurred. This case study represents the first documented occurrence of a patient with ALS managed with either a fasting or ketogenic diet protocol, co-administered as a TRKD. We measured improved or stabilized ALS-related function, forced expiratory volume, forced vital capacity, swallowing, neurocognitive status, mood, and quality of life. Measurable declines were restricted to physical function, maximal inspiratory pressure, and maximal expiratory pressure. Now over 45 months since symptom onset, our patient remains functionally independent and dedicated to his TRKD.

摘要

肌萎缩侧索硬化症(ALS)是一种无法治愈的神经退行性疾病。最具毁灭性的变异型是延髓起病的ALS,从症状出现起,其预估中位生存期为24个月。大量证据表明,ALS患者的神经元代谢和线粒体功能受损。代谢策略,尤其是禁食和生酮饮食方案,以一种可能减轻该疾病症状的方式改变神经元代谢和线粒体功能。我们报告了一例64岁男性患者,他患有进行性、恶化的延髓起病ALS达21个月,伴有假性延髓情绪,实施限时生酮饮食(TRKD)18个月。在此期间,他在ALS相关功能(较基线改善7%)、用力呼气量(改善17%)、用力肺活量(改善13%)、抑郁(恢复正常)、压力水平(恢复正常)和生活质量(改善19%)方面均有改善,尤其是疲劳(改善23%)。他的吞咽障碍和神经认知状态保持稳定。身体功能、最大吸气压力和最大呼气压力有所下降。体重减轻得到缓解,且未出现明显不良反应。本病例研究是首例记录在案的采用禁食或生酮饮食方案(作为TRKD联合使用)治疗的ALS患者。我们测量到ALS相关功能、用力呼气量、用力肺活量、吞咽、神经认知状态、情绪和生活质量得到改善或稳定。可测量的下降仅限于身体功能、最大吸气压力和最大呼气压力。自症状出现至今已超过45个月,我们的患者仍保持功能独立,并坚持其TRKD。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f39e/10830838/027b52c91d1a/fneur-14-1329541-g0001.jpg

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