Lidström J, Stokland E, Hagberg B
Department of Orthopaedics, Kungsbacka Hospital, Sweden.
Spine (Phila Pa 1976). 1994 Jul 15;19(14):1632-5. doi: 10.1097/00007632-199407001-00013.
The natural history of progression of scoliosis was studied. The authors included a wide range of ages and correlated progression and progression rate to both age of the patient and the neurobiologic staging of the disease.
The authors studied information compiled by Hagberg and coworkers regarding Rett syndrome. The total number of patients was 78, with age ranging from 1 to 34 years. Standing or sitting anteroposterior roentgenograms were collected and measured.
The material was studied initially separately regarding orthopedic and radiologic analysis on one hand and neurobiologic staging on the other. Radiographic films were measured both retrospectively and prospectively, and scoliosis angle and progression and progression rate was calculated. Staging of patients with Rett syndrome was done according to Hagberg.
When correlating curve magnitude and progression, the authors found that patients progressing > 15 degrees/year were classified as IV-A or IV-B stages. In the 0-5 year group, of the patients already having a curve of 15 degrees or more, all but one rapidly progressed to stage IV. The ten worst cases were characterized by early hypotonia, weakness, and gross motor disturbance.
The scoliosis in Rett syndrome is of a neurogenic type, and it develops earlier than idiopathic scoliosis. The development of scoliosis is dependent more on stage of disease than on age. Curve progression is usually more rapid than in idiopathic scoliosis and in most other types of neurogenic scoliosis in childhood and occurs in a broader age span. Early hypotonia, weakness, and muscular insufficiency, and an early clinical referral to disease stages IV-A or IV-B are ominous factors. Clinical follow-up should begin early and be repeated regularly and frequently.
对脊柱侧弯进展的自然史进行了研究。作者纳入了广泛的年龄范围，并将进展情况及进展速率与患者年龄和疾病的神经生物学分期相关联。
作者研究了哈格伯格及其同事收集的关于雷特综合征的信息。患者总数为78例，年龄范围为1至34岁。收集并测量了站立位或坐位前后位X线片。
材料一方面先分别进行骨科和放射学分析，另一方面进行神经生物学分期。对X线片进行回顾性和前瞻性测量，并计算脊柱侧弯角度、进展情况及进展速率。根据哈格伯格方法对雷特综合征患者进行分期。
在关联曲线严重程度和进展情况时，作者发现进展速度>15度/年的患者被归类为IV - A或IV - B期。在0至5岁组中，已有15度或更大侧弯的患者中，除1例之外均迅速进展至IV期。病情最严重的10例患者的特征为早期肌张力减退、肌无力和严重运动障碍。
雷特综合征中的脊柱侧弯属于神经源性类型，其发展早于特发性脊柱侧弯。脊柱侧弯的发展更多地取决于疾病分期而非年龄。与特发性脊柱侧弯以及儿童期大多数其他类型的神经源性脊柱侧弯相比，曲线进展通常更快，且发生在更广泛的年龄范围内。早期肌张力减退、肌无力和肌肉功能不全，以及早期临床诊断为IV - A或IV - B期疾病是不祥因素。临床随访应尽早开始，并定期频繁进行。
