Department of Neurology, Columbia University Irving Medical Center, New York, NY, USA.
Department of Rehabilitation and Regenerative Medicine, Columbia University Irving Medical Center, New York, NY, USA.
J Neuromuscul Dis. 2020;7(2):183-192. doi: 10.3233/JND-190462.
Weakness affects motor performance and causes skeletal deformities in spinal muscular atrophy (SMA). Scoliosis surgery decision-making is based on curve progression, pulmonary function, and skeletal maturity. Benefits include quality of life, sitting balance, and endurance. Post-operative functional decline has not been formally assessed.
To assess the impact of scoliosis surgery on motor function in SMA types 2 and 3.
Prospective data were acquired during a multicenter natural history study. Seventeen participants (12 type 2, 5 type 3 with 4 of the 5 having lost the ability to ambulate) had motor function assessed using the Hammersmith Functional Motor Scale Expanded (HFMSE) performed pre-operatively and at least 3 months post-operatively. Independent t-tests determined group differences based on post-operative HFMSE changes, age, and baseline HFMSE scores.
Three participants had minimal HFMSE changes (±2 points) representing stability (mean change = -0.7). Fourteen participants lost >3 points, representing a clinically meaningful progressive change (mean change = -12.1, SD = 8.9). No participant improved >2 points. There were no age differences between stable and progressive groups (p = 0.278), but there were significant differences between baseline HFMSE (p = 0.006) and change scores (p = 0.001). Post-operative changes were permanent over time.
Scoliosis surgery has an immediate impact on function. Baseline HFMSE scores anticipate post-operative loss as higher motor function scores were associated with worse decline. Instrumentation that includes fixation to the pelvis reduces flexibility, limiting the ability for compensatory maneuvers. These observations provide information to alert clinicians regarding surgical risk and to counsel families.
在脊髓性肌萎缩症（SMA）中，肌无力会影响运动表现并导致骨骼畸形。脊柱侧弯手术的决策基于曲线进展、肺功能和骨骼成熟度。手术的益处包括提高生活质量、坐立平衡和耐力。术后功能下降尚未得到正式评估。
评估脊柱侧弯手术对 SMA 2 型和 3 型患者运动功能的影响。
在一项多中心自然史研究中获取前瞻性数据。17 名参与者（12 名 2 型，5 名 3 型，其中 4 名已丧失行走能力）接受了运动功能评估，使用扩大的哈默史密斯功能性运动量表（HFMSE）进行术前和至少 3 个月的术后评估。基于术后 HFMSE 变化、年龄和基线 HFMSE 评分，使用独立 t 检验确定组间差异。
3 名参与者的 HFMSE 变化极小（±2 分），代表稳定（平均变化=-0.7）。14 名参与者的 HFMSE 损失超过 3 分，代表具有临床意义的进行性变化（平均变化=-12.1，标准差=8.9）。没有参与者的 HFMSE 增加超过 2 分。稳定组和进展组之间的年龄没有差异（p=0.278），但基线 HFMSE（p=0.006）和变化分数（p=0.001）之间存在显著差异。术后变化随时间推移而永久。
脊柱侧弯手术对功能有直接影响。基线 HFMSE 评分预测术后损失，因为较高的运动功能评分与更严重的下降相关。包括固定到骨盆的器械会降低灵活性，限制代偿性动作的能力。这些观察结果为临床医生提供了有关手术风险的信息，并为患者家属提供了咨询。
