Booth C M, Cortina-Borja M J, Theologis T N
Department of Zoology, University of Oxford, UK.
Dev Med Child Neurol. 2001 May;43(5):314-20. doi: 10.1017/s0012162201000597.
Muscle function often becomes progressively more compromised in children with spastic cerebral palsy, leading to reduced mobility. This study aimed to examine the role that muscle connective tissue plays in this process. Severity of spasticity as determined by a range of clinical measures was assessed in 26 children (14 males 12 females; age range 4 to 17 years) with either diplegic or quadriplegic cerebral palsy (CP). Muscle biopsies from the vastus laeralis muscle were obtained for biomedical and histological analysis during orthopaedic surgery as part of the child's ongoing care. Total collagen was quantified by hydroxyproline determination. Two clinical measures of severity, Modified Ashworth Scale and Balance, were shown to have a highly significant correlation with collagen content, and Ambulatory Status, Clonus, and Selective Muscle Control all showed positive trends. Collagen I accumulated in spastic muscle's endomysium which appeared to be thickened, and fibrotic regions with sparse muscle fibres were evident in more severe cases. This suggests that collagen may be involved in increases in muscle stiffness observed in spasticity. Once developed, these changes are essentially irreversible and we suggest that future treatments should consider including prevention of muscle fibrosis.
在痉挛型脑瘫患儿中，肌肉功能通常会逐渐受到更严重的损害，导致活动能力下降。本研究旨在探讨肌肉结缔组织在此过程中所起的作用。对26例双侧瘫或四肢瘫脑瘫（CP）患儿（14例男性，12例女性；年龄范围4至17岁）进行评估，通过一系列临床测量方法确定痉挛的严重程度。在骨科手术期间，从股外侧肌获取肌肉活检样本，用于生物医学和组织学分析，这是患儿持续治疗的一部分。通过羟脯氨酸测定对总胶原蛋白进行定量。痉挛严重程度的两项临床测量指标，改良Ashworth量表和平衡能力，与胶原蛋白含量高度相关，而步行状态、阵挛和选择性肌肉控制均呈现出正相关趋势。I型胶原蛋白在痉挛肌肉的肌内膜中积聚，肌内膜似乎增厚，在更严重的病例中可见肌肉纤维稀疏的纤维化区域。这表明胶原蛋白可能与痉挛时观察到的肌肉僵硬增加有关。一旦出现这些变化，基本上是不可逆的，我们建议未来的治疗应考虑包括预防肌肉纤维化。
