Motor function measure scale, steroid therapy and patients with Duchenne muscular dystrophy

Motor function measure scale, steroid therapy and patients with Duchenne muscular dystrophy
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DOI:
10.1590/s0004-282x2012000300007
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发表时间:
2012-03-01
影响因子:
1.4
通讯作者:
Reed, Umbertina C.
Reed, Umbertina C.
中科院分区:
医学4区
文献类型:
--
作者:
Silva, Elaine C. da;Machado, Darlene L.;Reed, Umbertina C.

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目的:通过运动功能量表(MFM)评价Duchenne型肌营养不良症(DMD)患者运动功能的三个维度(D1、D2、D3),评价激素(强的松龙或呋喃西林)对DMD患者运动功能的影响。方法:对33例DMD患者(22例可行走,6例非可行走,5例在研究期间丧失行走能力)进行为期18个月的6次MFM量表评定。结果:除步行功能丧失者外,其余患者术后14个月运动功能均保持稳定。在非卧床患者中,D2(轴向和近端运动能力)运动功能在6个月内得到改善;D3(远端运动能力)在整个随访期间得到改善。D_1(站立姿势和移位)和总分对预测行走能力的丧失是有用的。结论:MFM在DMD患者中的应用证实了类固醇治疗在延缓疾病进展方面的益处。
Objective: To assess the evolution of motor function in patients with Duchenne muscular dystrophy (DMD) treated with steroids (prednisolone or deflazacort) through the Motor Function Measure (MFM), which evaluates three dimensions of motor performance (D1, D2, D3). Methods: Thirty-three patients with DMD (22 ambulant, 6 non-ambulant and 5 who lost the capacity to walk during the period of the study) were assessed using the MFM scale six times over a period of 18 months. Results: All the motor functions remained stable for 14 months in all patients, except D1 for those who lost their walking ability. In ambulant patients, D2 (axial and proximal motor capacities) motor functions improved during six months; an improvement in D3 (distal motor capacity) was noted during the total follow-up. D1 (standing posture and transfers) and total score were useful to predict the loss of the ability to walk. Conclusions: The use of the MFM in DMD patients confirms the benefits of the steroid treatment for slowing the progression of the disease.