General movements in spinal muscular atrophy type 1.

PURPOSE: To investigate the motor repertoire of infants diagnosed with spinal muscular atrophy Type I (SMA Type I) without administration of any disease-modifying agent. METHODS: Motor Optimality Score-Revised (MOS-R) was calculated from videos recorded between post-term weeks 9-17 for 22 infants with SMA Type I. The MOS-R of infants with SMA Type I was compared with those of 22 infants with cerebral palsy (CP) and 22 infants with typical development. RESULTS: Of the infants with SMA Type I, 17 had absent fidgety movements (FMs) and 5 had sporadic FMs. Age adequate movement repertoire was absent, and the variety of movements in infants was very low. Movements were symmetrical but movements of four limbs remained on the surface level. Antigravity movements were very rare. Movement characterization was monotonous, slow speed, and small amplitude. The MOS-R of infants with SMA Type I was lower than those of infants with typical development but similar to those of infants with CP. CONCLUSIONS: Infants with SMA Type I had a motor repertoire similar to infants with CP, while they had a poorer motor repertoire than infants with typical development in the fidgety period as evidenced by MOS-R. Central nervous system involvement in these infants with SMA Type I with absent FMs and reduced MOS-R is unknown. Further studies are needed to determine the role of problems in the afferent and efferent pathways of spinal cord and muscle atrophy in the observation of normal FMs.

Analysis of Spontaneous Movements in Infants With Torticollis.

PURPOSE: This study aimed to investigate whether the movements and postures of 3- to 5-month-old infants with torticollis differ from those of infants without torticollis and to determine the relationship between clinical characteristics and the Motor Optimality Score (MOS) in torticollis. METHODS: Participants were 40 infants with torticollis and 40 infants developing typically without torticollis. The infants were evaluated with detailed general movement assessment. RESULTS: There were significant differences in the MOS and subcategory scores for age-adequate movement repertoire and observed postural patterns between groups. Clinical characteristics were not related to the reduction in the MOS. CONCLUSIONS: Infants with torticollis have differences in movements and postures at 3 to 5 months of age compared with controls. Strategies supporting the movement repertoire of infants with torticollis can be added to rehabilitation programs for infants with the lower MOS to optimize their motor development.

The effect of hyperbilirubinemia on motor repertoire of infants between 3 and 5 months of age.

Jaundice is a common problem and occurs as a result of the increase in bilirubin level in infants. Infants may be adversely affected by this situation. The aim of this study is to determine whether neonatal jaundice affects the movements and postures of 3- to 5-month-old infants with hyperbilirubinemia and the correlation of motor repertoire at the same age with bilirubin levels. The study group included 30 infants (9 girls, 21 boys) with hyperbilirubinemia and a control group of 30 infants (11 girls, 19 boys) with typical development without hyperbilirubinemia. The motor repertoires of the infants were evaluated through detailed general movement (GM) analysis using video recordings made at 9-17 weeks post-term. The infants with hyperbilirubinemia had lower motor optimality score and subcategory scores (including observed movement patterns, age-adequate movement repertoire, observed postural patterns, and movement character) compared with the control group. The fidgety movement scores decreased as bilirubin value increased in infants.Conclusion: Infants with hyperbilirubinemia had poorer motor repertoire when compared to a control group. For this reason, detailed GM analysis of these infants will predict probable neurodevelopmental problems and infants with needs can begin individualized early intervention suitable for movement repertoire and posture at the earliest time. What is Known: • GMs of infants with hyperbilirubinemia were previously described. • However, the motor repertoires of infants with hyperbilirubinemia were not described in detail. What is New: • Infants with hyperbilirubinemia had poorer motor repertoire when compared to peers. • This is important in determining the need for individualized early intervention.

Early Spontaneous Movements of Infants With Hypoxic-Ischemic Encephalopathy.

OBJECTIVE: The aim of this study was to determine motor repertoire at 3 to 5 months of age in infants with hypoxic-ischemic encephalopathy (HIE) and to examine changes according to HIE severity. METHODS: Participants were 38 infants aged 3 to 5 months with HIE and 38 infants in the comparison group. The general movement assessment and the Motor Optimality Score (MOS) were used. RESULTS: Infants in the HIE group had a significantly lower total MOS and scores for fidgety movements, age adequacy of motor repertoire, and quality of movement patterns compared with the comparison group. Infants with grade III HIE compared with grade I had a significantly lower MOS. CONCLUSIONS: Infants with HIE had poorer motor repertoire at age 3 to 5 months when compared with peers. Motor repertoire deteriorated as HIE severity increased. Detecting potential developmental delays as early as possible allows for early intervention and rehabilitation in this population.

Motor repertoire is age-inadequate in infants with cystic fibrosis.

BACKGROUND: General movements (GMs) in infants occur as fidgety movements (FMs) between postterm 9 and 20 weeks. We aimed to evaluate FMs and motor repertoire in infants with cystic fibrosis (CF) and their relation with clinical findings. METHODS: Demographic and clinical characteristics were recorded. FMs and motor repertoire were analyzed from a 5-min video recording of each infant. Videos were rated based on the Prechtl General Movement Assessment and motor optimality score (MOS) was calculated. RESULTS: The analysis included 18 infants with CF and 20 healthy infants at postterm age of 3-5 months. MOS was significantly lower in the infants with CF compared to controls (p < 0.05). Fifty percent of the infants with CF had abnormal or absent/sporadic FMs. MOS was negatively associated with hospitalization duration (r = -0.378, p = 0.036); and positively associated with vitamin A level in CF infants (r = 0.665, p = 0.026). CONCLUSIONS: Infants with genetically anticipated severe CF phenotype tended to have lower MOS. MOS may be used in addition to genetic testing to predict disease severity in infants with CF. Infants with CF, absent/sporadic FMs, and lower MOS could be considered for planning specific age-adequate early intervention programs. IMPACT: Motor repertoire was age-inadequate in infants with cystic fibrosis (CF). 50% of infants with CF had abnormal or absent/sporadic fidgety movements (FMs). Motor optimality score (MOS) was positively associated with vitamin A level and negatively correlated with hospitalization duration in infants with CF. MOS tended to decrease as genetically anticipated disease severity increased; thus, MOS might enable us to predict disease severity in CF. The relationship between motor repertoire and phenotype and genotype is unclear and warrants further study. CF infants with absent/sporadic FMs, and lower MOS could be considered for planning early intervention.

Are general movements at 3-5 months correlated and compatible with the Bayley-III at 1,5-2 years age?

Since early intervention is important in risky infants, it is also important to identify developmental problem as early as possible. There are various assessment methods for this. One of them is the General movements assessment (GMs), and the other one is the Bayley Scales of Infants and Toddler Developmental, third edition (Bayley-III). The present study aimed to compare the neurodevelopmental outcomes and Bayley-III scores at the age of 1.5-2 years with fidgety GMs. One hundred and twenty-six infants (57 females, 69 males) were assessed by the GMs at the corrected 3-5 months and also by the Bayley-III at the age of 1.5-2 years. According to the GMs, 21 infants exhibited the absence of fidgety movements, six infants exhibited abnormal fidgety movements, and 99 infants exhibited normal fidgety movements. According to the Bayley-III, 19 infants` motor scores, 13 infants` language scores, and 18 infants` cognitive scores were low (-2SD). Various neurodevelopmental problems were identified in 25.4% of the infants. As a result, although in the present study the Bayley-III underestimates the rates of motor impairment, it was found to be moderately compatible with the GMs at 3-5 months (r= 0.4, p < 0.001). However, the GMs were better than the Bayley-III in predicting neurodevelopmental outcomes at the age of 1.5-2. Although the Bayley-III and GMs may be valuable tools for estimating the later outcomes of infants, care should be taken while interpreting their results.

Assessment of Motor Repertoire in 3- to 5-Month-Old Infants With Obstetric Brachial Plexus Lesion.

PURPOSE: The aim of this study was to define the movement characteristics and postures of infants with obstetric brachial plexus lesion. METHODS: The study group included 20 infants with obstetric brachial plexus lesion and a control group of 20 infants with normal neurological outcome, aged 9 to 17 weeks postterm. Infants were evaluated by global and detailed general movements assessment. RESULTS: There were no significant differences between the motor optimality scores of the 2 groups. However, there were some differences in terms of concurrent movements and the posture. Infants with obstetric brachial plexus lesion demonstrated more excitement bursts, head rotation, hand-knee contact, rolling, and few finger posture and postural asymmetry, and performed jerky movements when compared with the control group. CONCLUSIONS: Obstetric brachial plexus lesion did not affect the quality of fidgety movements of the infants but leads to compensatory movements in concurrent movements on the unaffected sides.

Do adolescents with cerebral palsy agree with their caregivers on their participation and quality of life?

BACKGROUND: It is important to determine the quality of life (QoL) and level of participation in children with Cerebral Palsy (CP). Previous research has used reports from adolescents or caregivers, but there is no evidence that caregivers' reports accurately reflect the experiences of the adolescents they are interested in. OBJECTIVE/HYPOTHESIS: The aim of this study was to investigate whether a difference was present in the views of the adolescents and their caregivers regarding the participation and the quality of life of adolescents with CP, and to reveal the parameters creating such differences. METHODS: The participation levels and QoL of the adolescents were evaluated separately by the caregiver and the adolescent using the Pediatric Outcomes Data Collection Instrument (PODCI). RESULTS: A statistically significant difference was found in terms of caregivers and adolescents' scores of PODCI upper extremity (Z = -2,560, p = 0,008), transfer&basic mobility (Z = -3,839, p = 0,000), sports/physical functioning (Z = -3,103, p = 0,002), happiness (Z = -2,420, p = 0,016) and global functioning (Z = -3,639, p = 0,001). The children's scores were statistically significantly higher than caregivers'. It was found that there was a poor consistence in terms of caregivers and adolescents' scores of upper extremity (ICC = 0,373, p = 0,012), transfer/basic mobility (ICC = 0,289, p = 0,016), sport/physical functioning (ICC = 0,359, p = 0,009); moderate consistence in terms of those of global functioning (ICC = 0,421, p = 0,003). CONCLUSION: It was determined that caregivers and children's answers were not compatible with one another especially in terms of subjective assessments such as happiness and pain, which suggests that the consideration of caregivers or children in the assessment of subjective situations will change the results.

Should botulinum toxin A injections be repeated in children with cerebral palsy? A systematic review.

AIM: The aim of this study was to determine the effects of repeat botulinum toxin A (BoNT-A) injections in children with spastic cerebral palsy (CP) on the basis of a best evidence synthesis. METHOD: This study included 13 original articles after searching the literature to retrieve information. We used the critical review form produced by McMaster University to determine the methodological quality of the studies, and then confirmed the levels of evidence from Sackett. The studies were also evaluated using the International Classification of Function, Disability and Health - Children and Youth Version (ICF-CY). RESULTS: A total of 893 children with spastic CP who had been administered repeat BoNT-A injections were evaluated. The evidence level was II in four of the thirteen studies, III in four studies, and IV in five studies. The McMaster review form score was 14 in two studies, 13 in four studies, and 12 in seven studies. The results showed that repeat BoNT-A may be a safe and an effective approach. The first two injections/one repeat especially relieve spasticity and improve fine and gross motor activities. INTERPRETATION: Future studies to investigate the effectiveness of repeat BoNT-A in children with spastic CP may be planned within the framework of the ICF-CY to include well-designed randomized controlled trials and those conducted on larger homogenous groups.