Inter- and intra-observer reliability of the "Assessment of Motor Repertoire- 3 to 5 Months" based on video recordings of infants with Prader-Willi syndrome.

BACKGROUND: The "Assessment of Motor Repertoire-3 to 5 Months", which is a part of Prechtl's General Movements Assessment (GMA), has been gradually applied to infants with genetic metabolic disorders. However, there have been no studies on the application of the GMA for infants with Prader-Willi syndrome (PWS). AIMS: The purpose of this study was to determine the inter- and intra-observer reliability of the assessment tool in a population of infants with PWS. STUDY DESIGN: This was a reliability and agreement study. SUBJECTS: This was a cross-sectional study with15 infants with PWS born at an average gestational age of 38 weeks. OUTCOME MEASURES: Standardized video recordings of 15 infants with PWS (corrected ages of 3 to 5 months) were independently assessed by three observers. Kappa and ICC statistics were applied in inter- and intra- observer reliability analyses. RESULTS: The overall reliability ICC values of the "Motor Optimality Score" (MOS) ranged from 0.84 to 0.98, and the pairwise agreement ranged between 0.86 and 0.95 for inter- observe reliability. In addition, ICC values for the MOS ranged between 0.95 and 0.98 for tester agreement in intra-observer reliability. Complete agreement reliability (100%) was achieved in the subcategories of "Fidgety Movements" and "Movement Character" for the inter- and intra-observer reliability. Moderate to high inter- and intra-observer reliability were found in the subcategories of "Repertoire of Co-Existent Other Movements", "Quality of Other Movements" and "Posture", with kappa values ranging between 0.63 and 1.00. CONCLUSION: There were high levels of inter-and intra-observer agreement in the "Assessment of Motor Repertoire-3 to 5 Months" for infants with PWS. It is possible to carry out standardized quantitative assessments of the motor performance of infants with PWS.

Early markers of neurodevelopmental disorders based on general movements for very preterm infants: study protocol for a multicentre prospective cohort study in a clinical setting in China.

INTRODUCTION: Very preterm (VPT) infants may experience varying degrees of neurodevelopmental challenges. Lack of early markers for neurodevelopmental disorders may delay referral to early interventions. The detailed General Movements Assessment (GMA) could help us to identify early markers for VPT infants at risk of atypical neurodevelopmental clinical phenotype in the very early stage of life as soon as possible. Preterm infants with high risk of atypical neurodevelopmental outcomes will have the best possible start to life if early precise intervention in critical developmental windows is allowed. METHODS AND ANALYSIS: This is a nationwide, multicentric prospective cohort study that will recruit 577 infants born <32 weeks of age. This study will determine the diagnostic value of the developmental trajectory of general movements (GMs) at writhing and fidgety age with qualitative assessment for different atypical developmental outcomes at 2 years evaluated by the Griffiths Development Scales-Chinese. The difference in the General Movement Optimality Score (GMOS) will be used to distinguish normal (N), poor repertoire (PR) and cramped sychronised (CS) GMs. We plan to build the percentile rank of GMOS (median, 10th, 25th, 75th and 90th percentile rank) in N, PR and CS of each global GM category and analyse the relationship between GMOS in writhing movements and Motor Optimality Score (MOS) in fidgety movements based on the detailed GMA. We explore the subcategories of the GMOS list, and MOS list that may identify specific early markers that help us to identify and predict different clinical phenotypes and functional outcomes in VPT infants. ETHICS AND DISSEMINATION: The central ethical approval has been confirmed from the Research Ethical Board of Children's Hospital of Fudan University (ref approval no. 2022(029)) and the local ethical approval has been also obtained by the corresponding ethics committees of the recruitment sites. Critical analysis of the study results will contribute to providing a basis for hierarchical management and precise intervention for preterm infants in very early life. TRIAL REGISTRATION NUMBER: ChiCTR2200064521.

Early detection relationship of cerebral palsy markers using brain structure and general movements in infants born <32 weeks gestational age.

AIM: To detect early brain structural and clinical functional markers of brain injury and development based on a magnetic resonance imaging (MRI) scoring system and a general movement assessment (GMA) for preterm infants later diagnosed with cerebral palsy (CP). STUDY DESIGN: Retrospective cohort study. General movements (GMs) were scored according to a semiquantitative scoring system: the GMs optimality score (GMOS) at preterm and term ages and the Motor Optimality Score (MOS) at the corrected age of 3 months after birth. Brain magnetic resonance imaging (MRI) at term-equivalent age was scored using an MRI scoring system. We analyzed the relationship between the early degree of cerebral white matter (WM) abnormality and the GMOS and the MOS for infants born <32 weeks gestational age later diagnosed with CP in a comparison group of neurotypical controls. SUBJECTS: Sixteen preterm infants were included in this study who underwent MRI and GMs assessment. 8 out of the 16 preterm infants were later diagnosed with CP, while the other 8 infants with normal motor development (N) were placed into the control group. Their median gestational age was 30w6d and 27w6d for each group respectively. RESULTS: The cerebral WM MRI scores were significantly higher in the CP group than in the control group (p < 0.01). The GMOS and MOS were significantly higher in the control group than in the CP group (p < 0.05). The MOS showed a strong correlation to the cerebral WM MRI score (r = -0.88) and the subscale of cerebral WM items (the cystic degeneration and the focal signal abnormalities) of the MRI score (r = -0.94) in the CP group. The MOS also showed a correlation with corrected biparietal diameter (cBPD) in the preterm infant group with CP (r = 0.75). Results of linear regression analyses between term MRI and GMs measures in preterm infants with CP are presented. Cerebral WM scores were associated with the MOS (ß = -0.63; 95%CI = -0.97, -0.29; p < 0.01). Cerebral WM injury, including the subscale of cystic degeneration and focal signal abnormalities was closely associated with the MOS (ß = -0.83; 95%CI = -1.13, -0.54; p < 0.001). CONCLUSION: Cerebral WM scores show a strong association with a decreased motor performance on the MOS in preterm infants later diagnosed with CP. Severe white matter injury and significantly decreased MOS scores may provide useful early markers and strong evidence to early predict the risk of later development of cerebral palsy in preterm infants.

Safety of Curcuma aromatica oil gelatin microspheres administered via hepatic artery.

AIM: To evaluate the safety of Curcuma aromatica oil gelatin microspheres (CAO-GMS) infused via hepatic artery against primary liver cancer. METHODS: The safety of CAO-GMS was evaluated in view of its acute toxicity in rats, long-term toxicity in Beagle dogs and general pharmacology in rats and mongrel dogs. RESULTS: The 50% lethal dose (LD50) of CAO-GMS infused via the hepatic artery was 17.19 mg/kg, and the serum biochemical indices of dying rats after the administration changed markedly while those of survived rats did not. Subsequent pathological examination of the tissues from the dead rats indicated improper embolism. Similar edema and small necrotic foci in the hepatic lobule were found in the hepatic tissue of rats receiving 10 and 5 mg/kg CAO-GMS and GMS 60 d after the last administration, while not in the rats of the blank control group, indicating that microspheres infused via the hepatic artery may induce irreversible liver damage dose-dependently. General pharmacological study showed that the activities (posture and gait), respiration frequency, blood pressure or heart rate of the dogs were not affected by CAO-GMS, nor were salivation, tremor or pupil changes of the rats observed or their balancing ability compromised, suggesting CAO-GMS infused via the hepatic artery did not significantly affect the nervous, respiratory and cardiovascular systems. CONCLUSION: CAO-GMS embolization administered via the hepatic artery is safe but undesired embolization induced by vascular variation should be given due attention in its clinical application. Individualized embolization dosage and super-selective catheterization technique are recommended to avoid undesired embolism and reduce complications.