Gibson D A, Wilkins K E
Clin Orthop Relat Res. 1975 May(108):41-51. doi: 10.1097/00003086-197505000-00008.
A clinical study of 62 patients with Duchenne Muscular Dystrophy has suggested 2 pathways in the pathogenesis of the spinal deformities. In the first pathway, the patients develop an initial kyphosis due to the loss of paravertebral muscle thus placing the intrinsic ligamentous spine in an unstable position. The spine then develops a lateral curve accentuated by pelvic obliquity. In the final form, axial rotation of the spine and pelvis occurs to produce a severe deformity. In the second pathway the intrinsic spine assumes the more stable position of hyperextension with a level pelvis. This position remains relatively unchanged during the period of wheelchair confinement even though muscle deterioration progresses. A significant increase in the severity of the deformity occurs with age in those patients with unstable spines. None of the other factors examined affected the severity of the lateral curvature. A spinal brace has been designed to keep the spine extended and the pelvis level while the patients sits in his wheelchair. The purpose is to encourage the inexorable progression of muscular deficiencies to follow the second pathway. Various modifications of wheelchairs also may help to maintain spinal stability.
一项对62例杜氏肌营养不良患者的临床研究表明，脊柱畸形的发病机制有两条途径。在第一条途径中，患者由于椎旁肌丧失而最初出现后凸，从而使脊柱固有韧带处于不稳定位置。然后脊柱出现侧弯，骨盆倾斜加剧了侧弯。在最终形式中，脊柱和骨盆发生轴向旋转，导致严重畸形。在第二条途径中，脊柱固有部分处于脊柱过伸且骨盆水平的更稳定位置。即使肌肉退化进展，在轮椅受限期间这个位置也相对保持不变。脊柱不稳定的患者，其畸形严重程度随年龄显著增加。所检查的其他因素均未影响侧弯的严重程度。已设计出一种脊柱支具，以便患者坐在轮椅上时保持脊柱伸展和骨盆水平。目的是促使肌肉缺陷不可阻挡地发展遵循第二条途径。轮椅的各种改进也可能有助于维持脊柱稳定性。
