肌萎缩侧索硬化症(ALS)是一种无法治愈的神经退行性疾病。最具破坏性的变种是延髓性肌萎缩侧索硬化症,这预示着从症状开始的中位生存期为24个月。大量证据表明ALS中神经元代谢和线粒体功能受损。代谢策略,特别是禁食和生酮饮食方案,改变神经元代谢和线粒体功能,以减轻这种疾病的症状。我们报告了一名64岁的男性,有21个月的进步史,球性肌萎缩侧索硬化症恶化,伴随着相关的假球影响,实施限时生酮饮食(TRKD)18个月。在此期间,ALS相关功能改善(从基线改善7%),用力呼气量(改善17%),强制肺活量(改善13%),抑郁症(正常化),应力水平(归一化),和生活质量(提高19%),特别是疲劳(改善23%)。他的吞咽障碍和神经认知状态保持稳定。身体机能的下降是衡量的,最大吸气压力,和最大呼气压力。体重减轻,没有明显的不良反应发生。该案例研究代表了首次记录的ALS患者通过禁食或生酮饮食方案进行管理。作为TRKD共同施用。我们测量了改善或稳定的ALS相关功能,用力呼气容积,强制肺活量,吞咽,神经认知状态,心情,和生活质量。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。现在症状发作已经超过45个月了,我们的患者保持功能独立,并致力于他的TRKD.
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.