Don Carlo Gnocchi Foundation, Rome, Italy.
Eur J Phys Rehabil Med. 2013 Apr;49(2):169-78. Epub 2012 Nov 9.
Autosomal dominant facioscapulohumeral dystrophy (FSHD), the third most common muscular dystrophy, is characterised by asymmetric and highly variable muscle weakness. In FSHD patients, the coupling of the ankle muscles impairment with the knee, hip and abdominal muscles impairment, causes complex alterations of balance and walking with deterioration of quality of life (QoL).
The aim of this pilot study is to evaluate the effects of custom orthoses (foot orthosis-FO and ankle foot orthosis-AFO) on balance, walking and QoL of FSHD patients through a multidimensional approach.
Pilot study.
Outpatient Rehabilitation Department of Don Gnocchi Foundation.
Fifteen patients with facioscapulohumeral muscular dystrophy were studied.
On 15 FSHD patients clinical evaluation (Manual Muscle Test-MMT, Clinical Severity Score), performance tests (10 meter Walking test-10mWT and 2 minute Walking Test-2minWT), instrumental assessment (stabilometric evaluation), disability (Rivermead Mobility Index- RMI, Berg Balance Scale-BBS) and patient-oriented (Medical Outcome Study 36-item Short Form-SF-36, North American Spine Society-NASS and Visual Analogue Scale-VAS) measures were performed. Patients were evaluated first, wearing their shoes and then wearing their shoes plus orthoses. This evaluation was performed 1 month after wearing the orthoses.
The shoes plus orthoses evaluation, performed after one month in which the patients daily wore the custom lower limb orthoses, showed a significant improvement of walking performance (10-mWT p<0.01), balance (Romberg Index p<0.05; Medio-Lateral Velocity p<0.05) and QoL (PCS p<0.01).
This pilot study shows that in FSHD patients' custom lower limb orthoses (foot-orthoses and ankle-foot-orthoses); evaluated by using a multidimensional approach, improve walking, balance and QoL.
These preliminary results suggest that custom lower limb orthoses could reduce the risk of falling with a positive effect on our patients' safety. Our results should encourage the scientific community to do efficacy study on this hot topic.
常染色体显性面肩肱型肌营养不良症（FSHD）是第三大常见的肌肉疾病，其特征是肌肉无力的不对称和高度可变。在 FSHD 患者中，踝关节肌肉损伤与膝关节、髋关节和腹肌损伤的结合导致平衡和行走的复杂变化，生活质量（QoL）恶化。
本初步研究旨在通过多维方法评估定制矫形器（足矫形器-FO 和踝足矫形器-AFO）对 FSHD 患者平衡、行走和 QoL 的影响。
初步研究。
唐·诺基基金会门诊康复科。
研究了 15 例面肩肱型肌营养不良症患者。
对 15 例 FSHD 患者进行临床评估（徒手肌力测试-MMT、临床严重程度评分）、运动测试（10 米步行测试-10mWT 和 2 分钟步行测试-2minWT）、仪器评估（稳定度评估）、残疾（Rivermead 移动指数-RMI、伯格平衡量表-BBS）和以患者为导向的（医疗结果研究 36 项简短形式-SF-36、北美脊柱协会-NASS 和视觉模拟量表-VAS）措施。患者首先穿着鞋子进行评估，然后穿着鞋子加矫形器。在佩戴矫形器一个月后进行了这项评估。
在患者每天穿着定制下肢矫形器的一个月后进行的鞋子加矫形器评估显示，行走性能（10-mWT p<0.01）、平衡（Romberg 指数 p<0.05；中侧向速度 p<0.05）和 QoL（PCS p<0.01）显著改善。
本初步研究表明，在 FSHD 患者中，定制下肢矫形器（足矫形器和踝足矫形器）；通过多维方法评估，可改善行走、平衡和 QoL。
这些初步结果表明，定制下肢矫形器可以降低跌倒风险，并对我们患者的安全产生积极影响。我们的结果应鼓励科学界在这个热门话题上进行疗效研究。
