SMA的病程評估是依照其年齡以及可執行的功能來選擇合適的運動功能量表及電生理檢測1-3

許多運動功能評估量表已發展,證明可在多種條件下使用,包括3-5

  • 臨床研究中對脊髓性肌肉萎縮症自然病史的評估
  • 建立一基準線,以在臨床試驗中觀察試驗藥物對於運動功能的潛在好處


*
以下非完整的評估量表,僅供參考

影響各運動功能評估準確性的因素

若評估者有感患者變化,卻未反應在分數上,則可再次檢查下列可能的影響因素。此外,評估時的陪同者、病人的配合度、過程中是否給予標準正向回饋、評估時間是否符合生理作息以及是否有適當的休息,都可能對評估結果造成影響。

環境安排
環境中地板的軟硬度及室溫盡量維持一致
地板變軟或溫度太低,都可能降低動作表現。
衣著
穿著寬鬆且厚薄適中、亦可脫去尿布
貼身衣物可能影響肢體彎曲的表現、尿布可能降低坐姿穩定度。
年齡或經驗
年齡或動作經驗皆有可能影響評估表現
撕紙、拿筆等項目需要動作經驗,因此年幼患者的家長可在家先陪同練習。曾有病人在家撕紙被制止,連帶造成評估時不願配合而無法得分。
疫情
疫情使多數患者分數退步
疫情可能改變作息、活動量、生活習慣,進而影響動作表現,照顧者應盡量維持病患活動量,並鼓勵完成自己本來會做的事項。

現有運動功能評估工具的挑戰與展望

SMA 疾病表現隨醫療積極介入後有極大改變,
評估 SMA 患者的方式也需與時俱進27

挑戰 1

運動功能較佳的SMA病童,部分評估工具容易遇到天花板效應

解決方式:

物理治療師分享,可併用其他非專為 SMA 設計的運動功能評估工具。



* 模擬個案



挑戰2

除運動功能指標外,如何評估病患在生活面向的變化?

解決方式:

從病患角度出發,可參考併用其他面向評估工具



生活品質
文獻曾使用的評估工具: FSS、MFI、HRQOL 等。
研究文獻曾利用生活品質量,評估 SMA 患者在接收治療後,可改善短期生活品質與運動表現27
上肢精細動作
文獻曾使用評估工具:MRC sum score 等。
研究文獻針對第3, 4型病患在治療後,觀察到握力與手部精細動作的進步,讓病患順利握筆或抓持物品28
吞嚥功能
文獻曾使用評估工具:PASA 問卷、p-FOIS 等。
在臨床試驗中顯示早期藥物治療可維持SMA病童吞嚥功能,減少咳嗽與噎嗆狀況29
呼吸功能
文獻曾使用評估工具:Apnea-Hypopnea Index、FVC 等。
研究文獻針對第3型病患在接受治療後出現 apnea 或 hypopnea 的次數有顯著降低,並可延緩 FVC 退化的速率30
步態與行走功能
文獻曾使用評估工具:OMNI Exertion Scale (主觀疲勞程度) 等。
步態與行走功能是評估病童改善幅度的適合指標,而執行、判讀、分析等方面,仍需更多研究文獻支持31

治療目標:維持現有功能、預防併發症出現32, 33

研究顯示 SMA 患者最期待的治療效果是能穩定疾病並維持生活品質,而如何運用各式工具,綜合評估治療對 SMA 病人生活各面向所帶來的實質進步,將是未來仍需持續探討的議題。




Abbreviations: SMA = Spinal Muscular Atrophy; CHOP-INTEND = Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders; HFMSE = Hammersmith Functional Motor Scale-Expanded; PDMS-II = Peabody Developmental Motor Scale, 2nd edition; GMFM = Gross Motor Function Measure; FSS = Fatigue Severity Score; MFI = Multidimensional Fatigue Inventory; HRQOL = Health-Related Quality of Life; MRC = Medical Research Council; PASA = Parent Assessment of Swallowing Ability; p-FOIS = Pediatric Functional Oral Intake Scale; FVC = Forced Vital Capacity.

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SMA患者的基本活動能力與病情進展如何決定日常活動與生活方式?

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