Dysphagia in Amyotrophic Lateral Sclerosis: Impact on Patient Behavior, Diet Adaptation, and Riluzole Management.
Dysphagia in Amyotrophic Lateral Sclerosis: Impact on Patient Behavior, Diet Adaptation, and Riluzole Management.
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DOI：
10.3389/fneur.2017.00094
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发表时间：
2017
影响因子：
3.4
通讯作者：
Inghilleri M
中科院分区：
文献类型：
--
作者：
Onesti E;Schettino I;Gori MC;Frasca V;Ceccanti M;Cambieri C;Ruoppolo G;Inghilleri M
这项回顾性研究旨在调查脊柱和延髓发病的肌萎缩侧索硬化症 (ALS) 患者吞咽功能恶化相关的临床特征，描述饮食和液体摄入的改变，并评估吞咽困难对利鲁唑使用的影响。每 3-6 个月对 145 名患者进行定期观察。他们定期接受光纤内窥镜吞咽评估（FEES）和肺活量测定；吞咽困难的严重程度根据穿透吸入量表和汇集评分（P 评分）与其他参数（如感觉、协作和年龄）相结合（P-SCA 评分）进行分类。在平均约 2 年的随访期间，患有吞咽困难的 ALS 患者比例增加到 85 例（脊柱发病的患者从 35% 上升到 73%，延髓发病的患者从 95% 上升到 98%）。此外，8% 的 FEES 吞咽困难患者并未察觉到这种疾病。随着时间的推移，正常饮食和半固体饮食的频率减少，而泥状饮食和经皮内镜胃造口术（PEG）处方的频率增加。 44% 的吞咽困难患者拒绝使用增稠剂或 PEG。未接受利鲁唑治疗的患者与接受利鲁唑口服混悬液治疗的患者之间的死亡率存在显着差异（p＜＜0.05）。疾病持续时间主要影响脊柱发病患者吞咽困难的频率，在延髓发病患者中很早就出现。利鲁唑口服混悬液将允许吞咽困难的 ALS 患者安全给药，避免片剂压碎并随后分散在食物中，这是与药物的安全和有效使用不一致的常见做法。
This retrospective study aimed to investigate the clinical features associated with deteriorated swallow in amyotrophic lateral sclerosis (ALS) patients with spinal and bulbar onset, describe the modification of diet and liquid intake, and assess the impact of dysphagia on the use of riluzole. One hundred forty-five patients were observed periodically every 3–6 months. They underwent routinely fiberoptic endoscopic evaluation of swallowing (FEES) and spirometry; dysphagia severity was classified according to the Penetration Aspiration Scale and the Pooling score (P-score) integrated with other parameters such as sensation, collaboration, and age (P-SCA score). During a mean follow-up period of about 2 years, the percentage of ALS patients suffering from dysphagia increased to 85 (rising from 35 to 73% in patients with spinal onset and from 95 to 98% in those with bulbar onset). Also, 8% of patients with dysphagia by FEES did not perceive the disorder. The frequency of normal and semi-solid diets decreased over time, while that of pureed diets and percutaneous endoscopic gastrostomy (PEG) prescription increased. Forty-four percent of dysphagic patients refused thickeners or PEG. A significant difference was observed in the mortality rate between patients untreated with riluzole and patients treated with riluzole oral suspension (p < 0.05). Disease duration mainly impacted on the frequency of dysphagia in spinal onset patients, appearing very early in those with bulbar onset. Riluzole oral suspension would allow the safe administration in dysphagic ALS patients to avoid tablet crushing and consequent dispersion in food, common practices that are inconsistent with the safe and effective use of the drug.