Reliability and Validity of the Trunk Control Measurement Scale in Children With Duchenne Muscular Dystrophy.

While trunk control is essential for daily activities and motor function, there is currently sparse information regarding the psychometric properties of the outcome measures used for assessing trunk control in Duchenne muscular dystrophy (DMD), characterized by proximal muscle weakness. The Trunk Control Measurement Scale (TCMS) was developed to assess trunk control in children with cerebral palsy. We aimed to examine the reliability and validity of the Turkish version of the TCMS for children with DMD. We determined the functional level of 52 children with DMD (median age: 8.83 years) with the Vignos Scale, and we further evaluated them with the Motor Function Measure (MFM), a gold standard for determining the validity of the TCMS. Internal consistency of the Turkish TCMS was excellent with a Cronbach's alpha of .95. Intraclass correlation coefficient (ICC) values for intra-rater reliability were between 0.90-0.96 and for inter-rater reliability were between 0.91-0.95. We reported positive moderate to strong correlations between the TCMS and trunk flexors strength (p < .001, r = 0.77), back extensors strength (p < .001, r = 0.68) and MFM (p < .001, r = 0.85). Thus, we found the Turkish version of TCMS to be a reliable and valid instrument for assessing trunk control of children with DMD in clinical and research settings.

Sleep characteristics, early spontaneous movements, and developmental functioning in preterm infants in the early postnatal period.

OBJECTIVE: This study aimed to investigate the following: (i) sleep characteristics in preterm infants at 9-20 weeks of corrected age, and (ii) differences in early spontaneous movements and developmental functioning results between the groups based on some sleep characteristics. METHODS: Seventy-four preterm infants (36 female) were included. Sleep characteristics were assessed according to the Brief Infant Sleep Questionnaire (BISQ). The infants were divided into two groups based on total sleep duration: less than 12 h (38 infants), and 12 h and more (36 infants). Video recordings were made for the General Movements Assessment (GMA) and evaluated using the Motor Optimality Score for 3- to 5-Month-Old-Infants-Revised (MOS). Cognitive, language, and motor development were assessed using the Bayley Scales of Infant and Toddler Development, Third Edition (Bayley-III). RESULTS: The total sleep duration of all preterm infants (mean ± SD) was 11.8 ± 3.3 h. Infants who had absent fidgety movements slept less than 12 h, and fidgety movements differed between the groups (p = 0.012). Infants who slept 12 h or more had significantly higher MOS (p = 0.041), cognitive (p = 0.002), language (p < 0.001), and motor (p = 0.002) development results. Infants who snored had lower MOS (p = 0.001), cognitive (p = 0.004), language (p = 0.002), and motor (p = 0.001) development results. Infants with fewer than three nocturnal awakenings had significantly higher Bayley-III cognitive (p = 0.007), language (p = 0.032), and motor (p = 0.005) domain results. Prone and supine sleeping positions showed higher motor domain results than lateral positions (p = 0.001). CONCLUSIONS: Sleep in preterm infants might be a key factor in early developmental functioning processes and nervous system integrity. Even in the first months of life, there are substantial differences in cognitive, language, and motor development in association with sleep characteristics.

Early Spontaneous Movements and Sensory Processing in Preterm Infants.

IMPORTANCE: Preterm infants are at higher risk of motor development abnormalities and sensory processing difficulties. Few studies have examined both movement development and sensory processing in the early months of life, and the results are controversial. OBJECTIVE: In this cross-sectional study, we investigated (1) differences in early spontaneous movements and sensory processing between preterm infants born at <32 wk gestation and those born at 32 to 36 wk gestation when they reached corrected (postterm) age 3 to 5 mo and (2) the relationship between early spontaneous movements and sensory processing. PARTICIPANTS: We included 50 preterm infants born at <32 wk gestation and 61 preterm infants born at 32 to 36 wk gestation. OUTCOMES AND MEASURES: We assessed early spontaneous movements, including fidgety movements, using the General Movements Assessment (GMA), which provides the Motor Optimality Score (MOS), and sensory processing using the Infant Sensory Profile-2. RESULTS: The preterm infants born at <32 wk gestation had lower MOS results (p = .035) and more sensory processing difficulties (p = .006) than those born at 32 to 36 wk gestation. We found no significant relationship between early spontaneous movements and sensory processing (p > .05). CONCLUSIONS AND RELEVANCE: Preterm infants born at <32 wk gestation are at increased risk for motor development abnormalities and sensory processing difficulties. What This Article Adds: Assessment of both motor development and sensory processing can play a crucial role in identifying infants who need early intervention.

Assessing Joint Hypermobility, Proprioception, and Developmental Functioning in Toddlers Born Preterm.

OBJECTIVE: The aims of this study were to explore (i) the joint hypermobility, proprioception, and developmental functioning in toddlers born preterm, (ii) differences in the proprioception and developmental functioning between toddlers with and without joint hypermobility, and (iii) the relationship between them. MATERIALS AND METHODS: One hundred twelve toddlers born preterm between 24 and 42 months of age were included in this observational study. Beighton Score for joint hypermobility assess- ment and the Bayley Scales of Infant and Toddler Development, Third Edition (Bayley-III) for developmental functioning were applied in all toddlers born preterm; however, propriocep- tion assessment could be applied on 55 of 112 (49.11%) toddlers born preterm due to lack of cooperation. RESULTS: Of 112 toddlers, 30 (26.79%) had joint hypermobility. There were no differences in coop- eration rate (P = .629) and success rate (P = .887) in the proprioception assessment between toddlers with and without joint hypermobility (55 toddlers born preterm), which is similar to the cognitive domain (P = .430), language domain (P = .062), and motor domain (P = .619) in the Bayley-III. Additionally, none of them were related to each other (P > .05). CONCLUSION: Our study findings showed that joint hypermobility has no effect on propriocep- tion and motor development in toddlers born preterm between 24 and 42 months of age, and there is no relationship between them. The possibility of these results might be that movement repetition and not only proprioception but also other sensory systems could be important in this early period of life.

Early motor repertoire and developmental function outcomes in infants with West syndrome: a case series.

BACKGROUND: West syndrome (WS), also known as infantile spasm, is a rare form of severe epilepsy that begins during early infancy. This case series aimed to describe the early motor repertoire and examine the developmental function outcomes of infants with WS. CASE DESCRIPTIONS: Three infants (one female) with WS were assessed for early motor repertoire using the General Movement Assessment (GMA) which determined General Movement Optimality Scores (GMOS) at 4 post-term weeks of age, and Motor Optimality Scores (MOS) at 12 post-term weeks of age. Cognitive, language, and motor development were evaluated with the Bayley Scales of Infant and Toddler Development - Third Edition (Bayley-III) at 3, 6, 12, and 24 months of age. OUTCOMES: At 4-weeks post-term, one infant showed poor repertoire movements, while the other two showed cramped-synchronized movements with their GMOS ranging from 6 to 16 (out of 42). All infants showed sporadic/absent fidgety movements at 12 weeks post-term with their MOS ranging from 5 to 9 (out of 28). All sub-domain scores of Bayley-III were <2 SD at all follow-up assessments, that is <70, indicating severe developmental delay. CONCLUSION: These infants with WS had less than optimal scores of early motor repertoire, and developmental delay at a later age. Early motor repertoire might be an early sign for developmental function outcome at a later age in this population suggesting the need for additional research.

Sensory processing and sleep characteristics in preterm infants in the early period of life.

OBJECTIVE: The present study aimed to investigate the following: (i) differences in sensory processing and sleep characteristics between preterm infants born at < 32 weeks', vs. those born at ≥ 32 weeks' gestation; (ii) differences in sleep characteristics between preterm infants with typical vs. atypical sensory processing; and (iii) relationship between sensory processing and sleep characteristics in preterm infants at 3 months of age. METHODS: A total of 189 preterm infants, 54 born at < 32 weeks' gestation (26 females; mean gestational age [standard deviation (SD)], 30.1 [1.7] weeks), and 135 born at ≥ 32 weeks' gestation (78 females; mean gestational age [SD], 34.9 [0.9] weeks) were included in the present study. Sleep characteristics were evaluated using the Brief Infant Sleep Questionnaire, and sensory processing was assessed using the Infant Sensory Profile-2. RESULTS: There were no significant differences in sensory processing (P > 0.05) or sleep characteristics (P > 0.05) between the preterm groups; however, more infants snored in the <32 weeks' gestation group (P = 0.035). Preterm infants with atypical sensory processing showed lower nighttime (P = 0.027) and total sleep durations (P = 0.032), and higher rates of nocturnal wakefulness (P = 0.038) and snoring (P = 0.001) than preterm infants with typical sensory processing. A significant relationship, therefore, was observed between sensory processing and sleep characteristics (P < 0.05). CONCLUSIONS: Sensory processing patterns may play an important role in understanding sleep problems in preterm infants. The early detection of sleep problems and sensory processing difficulties are necessary for early intervention.

Early spontaneous movements and spatiotemporal gait characteristics in preterm children.

This study aimed to analyze spatiotemporal gait characteristics of preterm children from 3 to 4 years of age according to different gestational age groups and to examine the relationship between the detailed general movements assessment and spatiotemporal gait characteristics. A total of 74 preterm children, 32 extremely preterm and very preterm (EP-VP, < 32 weeks gestational age) and 42 moderate to late preterm (MLP, 32 to < 37 weeks gestational age), were included in this prospective study, along with 38 term children. Early spontaneous movements of preterm children were assessed from videos at 9-20 weeks post-term according to the general movements assessment, which determines the Motor Optimality Score-Revised (MOS-R). The spatiotemporal gait characteristics of all children were evaluated using the GAITRite®electronic walkway at self-selected walking speeds. EP-VP children walked with shorter step lengths (p = 0.039), and MLP children walked with greater step length variability (p = 0.003) than their term peers. The MOS-R results were related to step length (r = 0.36, p = 0.042), step length variability (r = -0.56, p = 0.001), and base of support (r = -0.37, p = 0.038) in EP-VP children. The MOS-R subcategories, age-adequate movement repertoire, and postural patterns were related to some of the spatiotemporal gait characteristics, including step length, step length variability, and base of support (p < 0.05).  Conclusion: EP-VP and MLP children might catch up to their term peers at 3 to 4 years of age in terms of most gait parameters. In addition to the MOS-R, age-adequate movement repertoire and postural patterns of preterm children without cerebral palsy in early life may be a marker of later neurodevelopmental dysfunction. What is Known: • Preterm children walk with a wider step width, a greater step length asymmetry and step time, and a shorter stride length at 18 to 22 months of age compared with term children at a self-selected speed, while these differences disappear in children 4.5-5 years old and older. What is New: • Early spontaneous movements were related to some spatiotemporal gait characteristics. • Preterm children might catch up to term children at 3-4 years of age in spatiotemporal gait characteristics while walking at a self-selected speed.

Early Spontaneous Movements and Upper Extremity Movement Score in Infants With all Narakas Types of Obstetric Brachial Plexus Palsy.

BACKGROUND: Obstetric brachial plexus palsy (OBPP) is a birth injury that affects upper extremity performance. However, some children with OBPP might have central nervous system disorder or developmental disabilities. This study aimed to investigate (1) the early spontaneous movements using General Movements Assessment (GMA) in infants with OBPP according to the Narakas classification, (2) the differences from typical infants, and (3) the relationship between the GMA and the affected upper extremity movements score. METHODS: Fifty-six infants with OBPP (39 females; median gestational age 40 weeks, range = 34 to 42) and 50 typical infants (20 females; median gestational age 38 weeks, range = 37 to 41) were assessed at age three to five months using the GMA, which is a determined Motor Optimality Score (MOS) for fidgety movements and concurrent motor repertoire, and using the Active Movement Scale (AMS) for affected upper extremity movements. RESULTS: There were no differences in MOS and its subcategories between Narakas types in infants with OBPP (P > 0.05); however, infants with OBPP had a higher degree of aberrant fidgety movements and a lower score in MOS and its subcategories than typical infants (P < 0.05). There was no relationship between AMS scores, and MOS and its subcategories. CONCLUSIONS: Infants with OBPP, except Narakas type I, might have an increased risk of central nervous disorder and developmental problems in addition to peripheral nerve injury. Each test, GMA and AMS, contributes to the identification of their own specific risk in these infants.

Developmental Functioning Outcomes in Infants With Cystic Fibrosis: A 24- to 36-Month Follow-Up Study.

OBJECTIVE: This study aimed to examine early motor repertoire using Prechtl General Movement Assessment (GMA) and later developmental functioning of infants with cystic fibrosis (CF). METHODS: Early motor repetoire was evaluated using Prechtl GMA, and developmental functioning was assessed using Bayley Scales of Infant and Toddler Development-Third Edition (Bayley-III) in infants with CF and their peers who were neurotypical, as the control group. RESULTS: Twelve infants with CF clinically stable and 12 infants who were neurotypical, with respective median post-term ages of 14 and 13 weeks, were assessed using GMA. At 24 to 36 months, the Bayley-III was applied to the CF group (median post-term age = 27.5 months) and the control group (median post-term age = 27.0 months). Fidgety movements were absent in 5 infants with CF, whereas all infants who were neurotypical had normal fidgety movements. The Motor Optimality Score was significantly lower in the CF group (median = 18.5) compared with the control group (median = 26). The CF group had significantly lower composite scores in the Bayley-III cognition, language, and motor domains compared with the control group. CONCLUSION: Cognitive, language, and motor development was delayed in infants with CF. Developmental functioning of infants with CF should be assessed as early as possible and monitored, and age-specific early intervention programs should be considered when necessary. IMPACT: Infants with CF may have motor, cognitive, and language developmental delays compared with peers who are neurotypical during early childhood, and hospitalization was negatively correlated with motor development at 24 to 36 months of age. This study highlights the importance of early assessment of developmental functioning and age-specific, early intervention programs when necessary in infants with CF. LAY SUMMARY: It is important to assess developmental functioning as early as possible in infants with CF and to consider age-specific early intervention programs when necessary.

Early Spontaneous Movements and Postural Patterns in Infants With Extremely Low Birth Weight.

BACKGROUND: Extremely-low-birth-weight (ELBW) infants are at high risk of morbidity, mortality, and long-term neurodevelopmental disorders. Evaluating infants' early spontaneous movements and postural patterns could facilitate the early detection of neurological dysfunction. This study aimed to analyze the results of global-and detailed-General Movements Assessment (GMA) in ELBW infants at a corrected age of three to five months and to compare with normal-birth-weight (NBW) infants. METHODS: Fifty-two ELBW infants (median birth weight = 915.5 g) and 50 NBW infants were included. All infants were assessed according to GMA using Motor Optimality Score for 3- to 5-Month-Old Infants-Revised score sheet (MOS-R). In addition, later diagnoses of ELBW infants with atypical development were presented. RESULTS: Fidgety movements were observed in 36 (69.2%) of ELBW infants and all NBW infants. MOS-R scores were lower in the ELBW group (median = 24) compared with the NBW group (median = 26). The ELBW infants scored lower than NBW infants in all MOS-R subcategories. Twenty-three (44.2%) of ELBW infants were diagnosed as atypical in the later period, although all control infants had normal development. CONCLUSIONS: The study indicated that ELBW might increase the risk of atypical development in infants. The MOS-R could help us to find the risk of atypical development in infants with ELBW.

The early spontaneous movements, and developmental functioning and sensory processing outcomes in toddlers born preterm: A prospective study.

BACKGROUND: Preterm infants may present sensory processing difficulties as well as developmental disorders. However, studies investigating both early spontaneous movements, and later sensory processing and development functioning are limited. AIMS: To examine; (1) early spontaneous movements between the ages of 3 and 5 months, (2) the differences of sensory processing between the ages of 24 and 35 months in infants who had normal and aberrant fidgety movements between 3 and 5 months corrected age, and (3) the relationship between sensory processing and both early spontaneous movements and developmental functioning. STUDY DESIGN: A prospective observational study design. SUBJECTS: Eighty-eight preterm infants (median gestational age 32 weeks, range 23-36) were included. OUTCOME MEASURES: Early spontaneous movements, including fidgety movements, were assessed according to the General Movements Assessment (GMA), which determines the Motor Optimality Score (MOS). Developmental functioning was assessed using the Bayley Scales of Infant and Toddler Development, Third-Edition (Bayley-III) and sensory processing was assessed with Toddler Sensory Profile-2 between the ages of 24 and 35 months. RESULTS: Sixteen preterm infants (18.1%) displayed aberrant fidgety movements. Median MOS was 25. Infants who displayed aberrant fidgety movements had a lower Bayley-III score in cognitive, language, and motor domains (p = 0.001, p = 0.006, p < 0.001, respectively) and showed more atypical movement sensory processing (p = 0.016) and touch sensory processing (p = 0.018). Fidgety movements were related to typical/atypical movement processing (p = 0.004, r = 0.300). CONCLUSION: In addition to motor assessment, sensory processing assessment in preterm infants might play a crucial role due to sensory processing difficulties from the early-period of life.

The Effects of Different Exteroceptive Experiences on the Early Motor Repertoire in Infants With Down Syndrome.

OBJECTIVE: Down syndrome is a chromosomal abnormality in which muscle tone, motor development, and sensory systems are affected. The objectives of this study were to examine the changes in movements and postures of 3- to 5-month-old infants with Down syndrome and infants who were neurotypical controls during exposure to a rough-textured surface and to compare the differences occurring before and during an exteroceptive condition that was different between the 2 groups. METHODS: In this quasi-experimental study, participants were 20 infants with Down syndrome (8 female infants; age range = 10-18 weeks, mean [SD] = 12 weeks 2 days [2 weeks 2 days]) and 20 age-matched infants with typical development (8 female infants; age range = 9-17 weeks, mean (SD) = 12 weeks 6 days [1 week 5 days]). The movements and postures of the infants, including fidgety movements, were assessed according to the General Movements Assessment, which determines the Motor Optimality Score (MOS), on 2 surfaces. RESULTS: The MOS outcomes of the infants with Down syndrome (median = 21.5, range = 6-28) were significantly lower than those of the infants who were neurotypical (median = 28, range = 23-28) on a standard mattress. The postures were found to be significantly better in infants with Down syndrome during exposure to a rough-textured surface. The MOS did not change due to the different exteroceptive experiences in infants with Down syndrome and infants who were neurotypical. CONCLUSION: The different exteroceptive experiences caused only postural alterations, which might play a crucial role in early intervention programs for infants with Down syndrome. IMPACT: This is the first study, to our knowledge, on the effects of different exteroceptive conditions, such as a coco coir mattress covered with muslin fabric, on the early motor repertoire in infants with Down syndrome. The findings showed that infants with Down syndrome had heterogeneity in their fidgety movements and large variability of the MOS, and the MOS results from infants with Down syndrome were lower than those of infants who were neurotypical. Fidgety movements and the MOS did not change due to the different exteroceptive experiences in either group. Significant improvements in posture were observed during exposure to a rough-textured surface. The improvement of posture is essential; clinicians might consider the positive effect of putting an infant with Down syndrome on a rough-textured surface and include this strategy in early intervention programs.

The general movements assessment and effects of an early intervention in an infant with Cri du chat syndrome: a case report.

BACKGROUND: Cri du chat syndrome (CdCS) is a rare orphan genetic disorder. Infants with CdCS have a neurodevelopmental dysfunction, but there are limited studies on their spontaneous movements or effect of the early interventions in children with CdCS. This study aimed to describe early spontaneous movements and investigate the effects of an early intervention in an infant with the CdCS. CASE: We analyzed the detailed general movements assessment (GMA) of an infant with CdCS at 14 weeks, and the Bayley Scales of Infant and Toddler Development-third edition (Bayley-III) were used for the determining and the follow-up of developmental functioning at 14 weeks, 6 months and 12 months. The infant was included in an early intervention beginning from 14 weeks. Fidgety movements were absent. The motor repertoire appeared significantly reduced, and the movement character was monotonous at 14 weeks. Although the infant achieved some developmental milestones with the early intervention program, the improvements were not reflected in the Bayley-III composite score. CONCLUSIONS: As a consequence, abnormal GMA results, including fidgety movements and concurrent movement patterns, seen in CdCS can be associated with early signs of neurodevelopmental dysfunction. Early intervention programs in infants with genetic disorders could help enable the early achievement of motor milestones.

General Movements and Developmental Functioning in an Individual with Rhizomelic Chondrodysplasia Punctata within the First Months of the Life: A Case Report.

AIMS: Rhizomelic chondrodysplasia punctata (RCDP) is an autosomal recessive inherited disorder. Individuals with RCDP have a wide range of neurodevelopmental outcomes, but there are limited descriptions of their early motor development before 5 months of age. This study aimed to describe in detail the age-specific spontaneous movements and examine the developmental functioning in an individual with RCDP. METHODS: A female infant (born at 39 weeks' gestation), diagnosed with RCDP at 3 weeks of age, was assessed at 4 and 16 weeks for general movements (GMs) and concurrent motor repertoire; the Bayley Scales of Infant and Toddler Development-Third Edition (Bayley-III) was also applied at the same ages. RESULTS: At 4 weeks, the infant showed poor repertoire GMs, with a detailed General Movement Optimality Score of 16/42. At 16 weeks, age-specific fidgety movements were absent, and the movement character was monotonous and stiff; the detailed Motor Optimality Score was severely reduced (7/28). All Bayley-III scores were <2 SD, that is <70 indicating severe developmental delay. CONCLUSION: Functional assessments such as the GM assessment and age-specific detailed assessment could be complementary to neuroimaging assessments to predict the neurodevelopmental outcomes in infants with RCDP.

Psychometric properties of the Turkish version of Participation and Environment Measure for Children and Youth.

BACKGROUND: Participation in daily activities provides many opportunities for children with and without disabilities to improve cognitive, physical and communication abilities; to develop social relationships and to promote adaptive behaviours. The aim of this study is to examine the psychometric properties of the Participation Environment Measure for Children and Youth (PEM-CY) in Turkish children and youth with and without a disability. METHODS: A total of 410 parents of children with (n = 232) and without (n = 178) disabilities, aged 5-17 years, were included in this study. Cronbach's alpha (α) and intraclass correlation coefficients (ICCs) were evaluated for internal consistency and test-retest reliability, respectively. Discriminant validity was determined by comparing the differences in participation and environment scores for disability groups and age intervals with the two-way analysis of variance (ANOVA) followed by post-hoc analyses when results were statistically significant. RESULTS: Internal consistency (0.67-0.80) and test-retest reliability (0.67-0.93, p < 0.0001) ranged from moderate to very strong for different summary scores. Discriminant validity of the Turkish version of PEM-CY was supported by significant differences between children with and without disabilities on participation and environment scales (p < 0.05). We identified some significant age differences, but they did not follow consistently. CONCLUSIONS: The Turkish version of the PEM-CY is a valid and reliable tool to determine the participation and environmental factors in the home, at school and in community settings in Turkish children and youth aged 5-17 years, with and without disabilities.

The forgotten sixth sense in cerebral palsy: do we have enough evidence for proprioceptive treatment?

Purpose: The aim of this study was to evaluate the proprioception treatment approaches as well as to investigate the effect of these approaches in individuals with Cerebral Palsy.Materials and methods: A systematic review was performed using American Academy of Cerebral Palsy and Developmental Medicine Methodology. PubMed, PEDro, ScienceDirect, The Cochrane Library, Scopus and Web of Science database were searched. All the articles included were evaluated based on their level of evidence and conduct.Results: Five articles met the inclusion criteria, children and adults with Cerebral Palsy. The effectiveness of different approaches has been examined in all studies and some studies showed effectiveness of treatment on proprioception or on motor performance. However, there was no superiority in between treatment approaches.Conclusions: The reasons that limits the studies analyzed in this review were small sample sizes and insufficient heterogeneity of groups included. Because of the significance of proprioception on movement and motor performance, it should be included in the evaluation and treatment programs of individuals with Cerebral Palsy.Implications for rehabilitationIt has been found that the various treatment methods applied appear to have a positive effect on proprioception with children and adults Cerebral Palsy.No treatment appears to be superior to the others.Treatment was found to be better as Gross Motor Function Classification System level severity decreased in adults with Cerebral Palsy.Treatments used in children with Cerebral Palsy were shown to have effects especially on gait parameters associated with proprioception improvement.