EARLY PREDICTION OF CP

这个子项目是许多研究子项目中利用 资源由NIH/NCRR资助的中心拨款提供。子项目和 调查员(PI)可能从NIH的另一个来源获得了主要资金， 并因此可以在其他清晰的条目中表示。列出的机构是 该中心不一定是调查人员的机构。 脑性瘫痪(CP)被认为是一种临床综合征，由大脑发育早期的先天脑损伤引起，导致静态神经功能障碍。这些缺陷通常涉及躯体运动系统，表现为偏瘫、双瘫或四肢瘫痪。脑性瘫痪还可表现为高张力和痉挛、感觉障碍、听力和视觉障碍、进食问题和全球发育迟缓以及其他慢性医学问题，如肺部疾病。 脑性瘫痪最常见的原因是出生第一年时发生的缺氧缺血性脑损伤、脑室周围白质软化或脑室和/或脑实质出血。不太常见的原因是遗传疾病、感染和产中损伤。目前脑性瘫痪的治疗以维持功能、解除痉挛、改善营养和提供发育支持护理为目标，但到目前为止还没有治愈或预防指南。此外，由于脑瘫只能在18-24个月大的时候被诊断出来，支持措施和家庭咨询被推迟了。如果发现了可以预防脑瘫发展的干预或治愈措施，由于在生命的前六个月缺乏预测性诊断评估，这种干预将被推迟。 直到大约十年前，仍然没有临床、实验室或成像研究可以准确预测脑瘫在生命的前六个月的发展。在真正诊断脑性瘫痪之前，对头部超声、EEG和功能MRI的预测价值进行了测试，但这些研究的敏感性较低，表明它们不能作为筛查工具。 奥地利神经学家Heinz F.R.Prechtl开发了一种临床评估方法来研究早产儿和足月儿的自发活动。监测痉挛、同步的全身运动和烦躁的运动对预测脑性瘫痪的灵敏度和特异度分别为100%和95%。另外两个小组重复了这些研究，得到了类似的结果。一项脑性瘫痪预测工具的荟萃分析表明，Prechtl的方法是最优越的评估方法，其阳性预测值(PPV)高于头部超声或MRI。这些研究在美国没有重复过。 我们在这项建议中的目的是评估Prechtl的方法在早产儿和足月儿中的预测价值，这些早产儿和足月儿有或没有肺部疾病，他们有发生脑性瘫痪的风险。我们将比较肺部疾病和脑瘫的发生率，并观察肺部疾病的发展与脑损伤之间的任何关系。我们将根据Prechtl的评估识别高危婴儿并观察他们的全身运动。我们将对我们的患者进行为期两年的纵向随访，并根据脑性瘫痪的诊断解释我们的评估的预测价值。我们将比较头部超声和全身运动评估的敏感性和PPV。 这项评估将使我们能够在生命的早期阶段就开始采取支持措施，从而改善脑瘫儿童的预后。他们肌肉骨骼状况和发育的改善使脑瘫儿童的肺部状况更好，并将减少主要由于慢性肺部疾病并发症而住院的次数和时间。

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Cerebral palsy (CP) is considered a clinical syndrome caused by a preceding brain injury early in brain development that results in static neurological deficits. These deficits usually involve the somatomotor system manifesting as hemi-, di- or quadriplegia. Cerebral palsy can also manifest as hypertonicity and contractures, sensory deficits, hearing and visual difficulties, feeding problems and global developmental delay and other chronic medical problems such as lung disease. The most common cause of cerebral palsy is a hypoxic-ischemic brain injury, periventricular leukomalacia or intraventricular and/or parenchymal hemorrhage that occurs in the first year of life. Less common etiologies are genetic disorders, infections and intrapartum injuries. The current treatments of cerebral palsy are targeted to maintaining function, relieving contractures, improving nutrition and providing developmental supportive care, but to date there is no cure or preventive guideline. Moreover, supportive measures and family counseling is delayed since cerebral palsy can be diagnosed only at the age of 18-24 months. If an intervention or cure was discovered that could prevent the development of cerebral palsy, this intervention would be delayed due to the lack of a predictive diagnostic assessment within the first six months of life. Until about ten years ago, there was still no clinical, laboratory or imaging study that could accurately predict the development of cerebral palsy in the first six months of life. Head ultrasound, EEG and functional MRI were tested for their predictive value before the actual diagnosis of cerebral palsy, however, the low sensitivity of these studies showed that they are not useful as screening tools. Heinz F.R. Prechtl, an Austrian neurologist, developed a clinical assessment method to study the spontaneous movements of preterm and term infants. Monitoring of cramped synchronized generalized movements and fidgety movements resulted in 100% sensitivity and 95% specificity in predicting cerebral palsy. These studies were repeated by two other groups with similar results. A meta-analysis of predictive tools for cerebral palsy identified Prechtl's method as the most superior assessment that showed higher positive predictive value (PPV) than head ultrasound or MRI. These studies have not been repeated in the USA. Our aim in this proposal is to assess the predictive value of Prechtl's method in preterm and term infants with and without lung disease, who are at risk for development of cerebral palsy in Hawaii. We will compare the incidence of pulmonary diseases and cerebral palsy and observe any relationship between the development of lung disease and brain injury. We will identify at risk infants and observe their generalized movements according to Prechtl's assessment. We will conduct a 2-year longitudinal follow up of our patients and interpret the predictive value of our assessment based on the diagnosis of cerebral palsy. We will compare the sensitivity and PPV of head ultrasound and the assessment of generalized movements. This assessment will allow us to start supportive measures at an earlier stage of life, thus improving the outcome of children with cerebral palsy. The improvement of their musculoskeletal status and development allow children with cerebral palsy to have a better pulmonary status and will decrease the number and length of hospitalizations mainly due to complications of chronic lung disease.