脊髓性肌肉萎縮症吞嚥障礙的處理

Management of Dysphagia in Spinal Muscular Atrophy

10.6315/TJPMR.202106_49(1).0002

魏國展(Kuo-Chang Wei)；翁妏謹(Wen-Chin Weng)；王亭貴(Tyng-Guey Wang)

脊髓性肌肉萎縮症（spinal muscular atrophy） ； 吞嚥障礙（dysphagia） ； 螢光錄影吞嚥檢查（video fluoroscopic swallowing study） ； 吞嚥內視鏡檢查（fiber-optic endoscopic evaluation of swallowing） ； spinal muscular atrophy ； dysphagia ； video fluoroscopic swallowing study ； fiber-optic endoscopic evaluation of swallowing

台灣復健醫學雜誌

49卷1期（2021 / 06 / 01）

11 - 16

繁體中文

脊髓性肌肉萎縮症（spinal muscular atrophy）為一自體隱性遺傳的運動神經元病變，依據發病時間點的不同以及動作發展的最好表現可以分為不同型態，患者近端肌肉無力的嚴重程度會大於遠端肌肉，且肌肉無力的狀況不只侷限於肢體，頸部控制以及攝食相關的吞嚥咀嚼肌群也可能受到影響進而產生進食困難及吞嚥障礙。脊髓性肌肉萎縮症吞嚥障礙的臨床表現與脊髓性肌肉萎縮症疾病的分型有很大的關聯，吞嚥障礙絕大部分是發生在第一型以及第二型脊髓性肌肉萎縮症患者，在吞嚥功能的評估上除了仰賴患者與家屬提供的資訊，也應結合較客觀的評估工具如：螢光錄影吞嚥檢查（video fluoroscopic swallowing study, VFSS）或吞嚥內視鏡檢查（fiberoptic endoscopic evaluation of swallowing, FEES）；在臨床處置上最重要的目標有兩點，第一為確保吞嚥攝食的安全性，第二為吞嚥攝食的效率是否足夠，為了達成上述目標往往需要照護團隊進行全面性的評估以及依據評估結果給予食物質地調整建議、復健處方以及管灌餵食等處置。此篇文章之目的為統合性的介紹脊髓性肌肉萎縮症患者吞嚥障礙的臨床表現、評估方式以及現有的可能處理方式。

Spinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disease. SMA phenotypes can be determined by the timing of disease onset and the highest level of motor development reachable by the patient. Generally speaking, proximal muscle involvement is more severe than in the distal extremities. In addition, cervical and the masticatory and pharyngeal muscles were likely affected by the disease, which can cause feeding problems and dysphagia. Clinically, dysphagia is more common among patients with type I and type II SMA. To thoroughly evaluate swallowing-related issues, both subjective information from patients and their caregivers and findings from objective tools including video fluoroscopic swallowing studies and fiber-optic endoscopic swallowing evaluations are required. Based on the results of the comprehensive swallowing assessment, further management including food content adjustment, swallowing training and tube feeding can be implemented. The aim of this review is to present the current available information and evidence regarding the clinical presentation, assessment, and management of dysphagia in SMA patients.

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