Keret D, Bassett G S, Bunnell W P, Marks H G
Department of Medical Education, Alfred I. duPont Institute, Wilmington, DE 19899.
J Pediatr Orthop. 1988 Mar-Apr;8(2):138-42.
Rett syndrome is a progressive encephalopathy observed only in girls, who are apparently normal until 6 to 12 months of age. It is characterized by autism, dementia, ataxia, stereotypic hand movements, hyperreflexia, spasticity, and seizures. Eight of 10 females with Rett syndrome evaluated at the Alfred I. duPont Institute have C-shaped neuromuscular curves averaging 29 degrees (range 22-48 degrees). Curve progression was seen in all eight patients and occurred despite bracing in four, averaging 21 degrees (range 12-31 degrees). Five patients, two of whom were braced, have undergone posterior spinal fusion with segmental instrumentation for curves ranging in size from 49 to 105 degrees (average 67 degrees).
雷特综合征是一种仅在女孩中出现的进行性脑病，这些女孩在6至12个月大之前看起来都是正常的。其特征包括自闭症、痴呆、共济失调、刻板手部动作、反射亢进、痉挛和癫痫发作。在阿尔弗雷德·I·杜邦研究所接受评估的10名雷特综合征女性患者中，有8名有C形神经肌肉曲线，平均为29度（范围为22 - 48度）。所有8名患者均出现了曲线进展，其中4名患者尽管使用了支具，曲线仍平均进展了21度（范围为12 - 31度）。5名患者（其中2名使用了支具）因曲线大小在49至105度（平均67度）之间，接受了后路脊柱融合术并采用了节段性内固定。
