Kinematic movement strategies in primary school children with 22q11.2 Deletion Syndrome compared to age- and IQ-matched controls during visuo-manual tracking

The present study focused on the mechanism subserving the production of kinematic patterns in 21 children with 22q11.2DS (mean age = 9.6 ± 1.9; mean FSIQ = 73.05 ± 10.2) and 21 age- and IQ-matched control children (mean age = 9.6 ± 1.9; mean FSIQ = 73.38 ± 12.0) when performing a visuo-manual tracking task in which they had to track a cursor rhythmically between 2 target zones. Children with 22q11.2DS moved faster (overall) and reached their maximum velocity sooner when compared to controls. However, the number of corrective submovements to attain the target did not differ. Children with 22q11.2DS seem to adopt a young ballistic movement strategy, with a fast ballistic first movement phase, followed by a second movement phase with very little online corrections to attain the target. Children with 22q11.2DS are not able to process the incoming feedback during the second movement phase to maximize the accuracy of the ongoing movement and use this phase to prepare the following. The fact that the parietal cortex and cerebellum are involved in action prediction and internal representation and are implicated in children with 22q11.2DS provides a possible neurological basis for their problems with prospective control and tracking behavior.     

Evidence of gray matter reduction and dysfunction in chromosome 22q11.2 deletion syndrome

Chromosome 22q11.2 deletion syndrome (22q11DS) is associated with cognitive deficits and morphometric brain abnormalities in childhood and a markedly elevated risk of schizophrenia in adolescence/early adulthood. Determining the relationship between neurocognition and neuroimaging findings would yield crucial information about childhood neurodevelopment and provide a basis for the study of the trajectory that occurs on the pathway to psychosis. We compared morphometric brain findings between non-psychotic children with 22q11DS (n = 22) and healthy controls (n = 16), and examined the association between neurocognitive functioning and morphometric brain findings. Volumetric regional gray matter differences between the 22q11DS and control subjects were measured, and correlations of the regional gray matter volumes and neurocognition were performed. Children with 22q11DS demonstrated reductions in gray matter in several brain regions, chiefly the frontal cortices, the cingulate gyrus and the cerebellum. The volumetric reductions in these salient areas were associated with poor performance in sustained attention, executive function and verbal memory; however, the relation of brain volume with cognitive performance did not differ between the patient and control groups. Thus, children with 22q11DS demonstrate gray matter reductions in multiple brain regions that are thought to be relevant to schizophrenia. The correlation of these volumetric reductions with poor neurocognition indicates that these brain regions may mediate higher neurocognitive functions implicated in schizophrenia.     

Parental adjustment,parenting attitudes and emotional and behavioral problems in children with selective mutism

The present study investigated emotional and behavioral problems in children with selective mutism (SM) along with the psychological adjustment and parenting attitudes of their mothers and fathers. Participants included 26 children with SM (mean age = 8.11 ± 2.11 years), 32 healthy controls (mean age = 8.18 ± 2.55 years) and the parents of all children. Children with SM displayed higher problem scores than controls in a variety of emotional and behavioral parameters. They predominantly displayed internalizing problems, whereas aggressive and delinquent behavior was described among a subsample of the children. Significant differences existed between the SM and control groups only in paternal psychopathology, which included anxiety and depression. They did not differ with respect to maternal psychological distress or mother or father reported parental attitudes. Another important result of the present study was that the severity of emotional and behavioral problems of children with SM was correlated with maternal psychopathology but not paternal psychopathology.     

Association of IL-12p70 and IL-6:IL-10 ratio with autism-related behaviors in 22q11.2 deletion syndrome: A preliminary report

22q11.2 deletion syndrome (22q11DS) is a genetic disorder that conveys a significant risk for the development of social behavior disorders, including autism and schizophrenia. Also known as DiGeorge syndrome, 22q11DS is the second most common genetic disorder and is characterized by an elevated risk for immune dysfunction, up to 77% of individuals have an identifiable immune deficiency. We hypothesize that this immune dysfunction could contribute to the elevated risk of impaired social behavior seen in 22q11DS. The current study begins to elucidate these immune deficits and link them with the behavioral alterations associated with the disorder. Serum concentrations of a series of cytokines were examined, using a multiplex immunoassay, in sixteen individuals with 22q11DS and screened for autism-related behavior using the Autism Diagnostic Interview-Revised (ADI-R). This preliminary study examined correlations between specific immune proteins and each of the ADI-R algorithm scores (social, communication, and repetitive behavior). The inflammatory cytokine IL-1β, as well as the ratio between the inflammatory cytokine IL-6 and the anti-inflammatory cytokine IL-10, were correlated with social scores (r = 0.851, p = 0.004; r = 0.580, p = 0.018). In addition, the inflammatory cytokines interferon gamma and IL-12p70 were correlated with repetitive behaviors (r = 0.795, p = 0.033; r = 0.774, p = 0.002). Interestingly, IL-12 has been reported to be increased in autistic children. These data show a positive association between severity of autism-related behaviors and level of serum concentrations of inflammatory cytokines in individuals with 22q11DS, providing a basis for further inquiry.     

Test–retest reliabilities of hand-held dynamometer for lower-limb muscle strength in intellectual disabilities

The primary purpose of this study was to investigate the test–retest reliabilities of hand-held dynamometer (HDD) for measuring lower-limb muscle strength in intellectual disabilities (ID). The other purposes were to: (1) compare the lower-limb muscle strength between children with and without ID; (2) probe the relationship between the muscle forces and agility performance in ID; and (3) explore the factors associated with muscle strength in ID.Sixty-one participants (30 boys and 31 girls; mean age = 14.1 ± 3.3 year) were assessed by the HDD using a “make” test. The comparative group consisted of 63 typically developing children (33 boys and 30 girls; mean age = 14.9 ± 2.1 year). The ID group demonstrated lower muscle groups than in typically developing group. Except for the ankle plantarflexors (ICC = 0.69, SEM = 0.72), test–retest analysis showed good intrarater reliability with ICC ranging from 0.81 to 0.96, and intrarater SEM values ranged from 0.40 to 0.57. The HDD has the potential to be a reliable tool for strength measurement in ID. Muscle strength was positively related to agility performance. Regression analysis indicated that height, weight, BMI, and activity level were significant predictors of muscle strength in ID.     

Visual tasks and postural sway in children with and without autism spectrum disorders

We investigated the influences of two different suprapostural visual tasks, visual searching and visual inspection, on the postural sway of children with and without autism spectrum disorder (ASD). Sixteen ASD children (age = 8.75 ± 1.34 years; height = 130.34 ± 11.03 cm) were recruited from a local support group. Individuals with an intellectual disability as a co-occurring condition and those with severe behavior problems that required formal intervention were excluded. Twenty-two sex- and age-matched typically developing (TD) children (age = 8.93 ± 1.39 years; height = 133.47 ± 8.21 cm) were recruited from a local public elementary school. Postural sway was recorded using a magnetic tracking system (Flock of Birds, Ascension Technologies, Inc., Burlington, VT). Results indicated that the ASD children exhibited greater sway than the TD children. Despite this difference, both TD and ASD children showed reduced sway during the search task, relative to sway during the inspection task. These findings replicate those of Stoffregen et al. (2000), Stoffregen, Giveans, et al. (2009), Stoffregen, Villard, et al. (2009) and Prado et al. (2007) and extend them to TD children as well as ASD children. Both TD and ASD children were able to functionally modulate postural sway to facilitate the performance of a task that required higher perceptual effort.     

Severity of depressive symptomatology and functional impairment in children and adolescents with temporal lobe epilepsy

ObjectiveDepression is a frequent psychiatric disorder in children with temporal lobe epilepsy (TLE). However, severity of depressive symptoms (DS) is frequently neglected in these patients. This study aimed to determine severity of DS and global functioning by using quantitative measures and to establish their correlation with patients’ demographics and clinical variables.Methods31 children (mean age of 11.8 ± 2.3 years) with TLE were assessed with K-SADS-PL for axis I DSM-IV diagnosis. Severity of DS was measured by Children Depression Rating Scale-Revised – CDRS-R. Global functional impairment was evaluated with Child Global Assessment Scale-CGAS.Results25 patients (56% boys; 12 ± 2.3 years) had current DS, moderate or severe in 84% according to CDRS-R T-Score. Severity of DS was not correlated with age (p = 0.377), gender (p = 0.132), seizure control (p = 0.936), age of onset (p = 0.731), duration of epilepsy (p = 0.602) and the presence of hippocampal sclerosis (p = 0.614). Patients had moderate to major functional impairment measured by CGAS (48.7 ± 8.8), being adolescents more impaired than children (p = 0.03). Impairment of global functioning was not associated with epilepsy variables (p > 0.05).ConclusionChildren with TLE had moderate to severe DS early in the course of their disease with a relevant impact on their global functional activities, especially considering adolescents. Epilepsy severity seems not to be correlated to the severity of DS, contradicting the idea of a cause–consequence relationship. More systematic research is needed to better understand the association of depressive disorders in children and adolescents with TLE.     

Virtual reality as means to improve physical fitness of individuals at a severe level of intellectual and developmental disability

Individuals with intellectual and developmental disabilities (IDD) are in need of effective and motivating physical fitness training programs. The aim was to test the effectiveness of a virtual reality (VR)-based exercise program in improving the physical fitness of adults with severe IDD when implemented by on-site caregivers. A research group (N = 20; mean age ± standard deviation = 47.9 ± 8.6 years; severe IDD level) was matched for age, IDD level and functional abilities with a comparison group (N = 24, mean age = 46.2 ± 9.3 years; severe IDD level). An 8-week fitness program consisting of 2–3 30-min sessions per week included game-like exercises provided by the IREX/GX video capture VR system. Changes in physical fitness were monitored by changes in heart rate at rest. A significant (P < 0.005) reduction in heart rate was demonstrated for the research group. No change in heart rate was monitored in the comparison group. Despite statistically significant improvements in heart rate, the results are not strong enough functionally to claim that this program improved physical fitness of individuals with severe intellectual disability.     

Differences in counter-movement jump between boys with and without intellectual disability

The purpose of this study was to describe the performance and biomechanical parameters of the counter-movement jump for young people with ID, in comparison with age matched individuals without ID. Volunteers with ID (n = 13; age = 15.3 ± 1.6 years; Wisk III intelligence quotient 55.6 ± 11.2) and without ID (n = 13; age = 15.4 ± 1.2 years) performed maximal counter-movement jumps, while knee kinematics, vertical ground reaction forces and electromyographic activity of the vastus lateralis and biceps femoris were captured. Individuals without ID jumped higher than their counterparts with ID and had lower take-off velocity, lower knee joint power and stiffness, lower knee joint extension angle and angular velocity, longer braking and propulsion phase, lower agonist and higher antagonist EMG activity. These results give an insight on possible causes for the performance deficit in CMJ in persons with ID and give the potential for such individuals to improve their ability to jump higher and more efficiently.     

Visual-motor integration,visual perception,and fine motor coordination in a population of children with high levels of Fetal Alcohol Spectrum Disorder

BackgroundVisual-motor integration (VMI) skills are essential for successful academic performance, but to date no studies have assessed these skills in a population-based cohort of Australian Aboriginal children who, like many children in other remote, disadvantaged communities, consistently underperform academically. Furthermore, many children in remote areas of Australia have prenatal alcohol exposure (PAE) and Fetal Alcohol Spectrum Disorder (FASD), which are often associated with VMI deficits.MethodsVMI, visual perception, and fine motor coordination were assessed using The Beery-Buktenica Developmental Test of Visual-Motor Integration, including its associated subtests of Visual Perception and Fine Motor Coordination, in a cohort of predominantly Australian Aboriginal children (7.5–9.6 years, n = 108) in remote Western Australia to explore whether PAE adversely affected test performance. Cohort results were reported, and comparisons made between children i) without PAE; ii) with PAE (no FASD); and iii) FASD. The prevalence of moderate (≤16th percentile) and severe (≤2nd percentile) impairment was established.ResultsMean VMI scores were ‘below average’ (M = 87.8 ± 9.6), and visual perception scores were ‘average’ (M = 97.6 ± 12.5), with no differences between groups. Few children had severe VMI impairment (1.9%), but moderate impairment rates were high (47.2%). Children with FASD had significantly lower fine motor coordination scores and higher moderate impairment rates (M = 87.9 ± 12.5; 66.7%) than children without PAE (M = 95.1 ± 10.7; 23.3%) and PAE (no FASD) (M = 96.1 ± 10.9; 15.4%).ConclusionsAboriginal children living in remote Western Australia have poor VMI skills regardless of PAE or FASD. Children with FASD additionally had fine motor coordination problems. VMI and fine motor coordination should be assessed in children with PAE, and included in FASD diagnostic assessments.     

Catechol-O-methyltransferase Val158Met polymorphism and hyperactivity symptoms in Egyptian children with autism spectrum disorder

Catechol-O-methyltransferase (COMT) plays an important role in the catabolism of brain dopamine and norepinephrine, which have been implicated in the pathogenesis of Autism spectrum disorder (ASD) as well as in other neuropsychatric disorders. We aimed to investigate the association of COMT Val158Met gene polymorphism with ASD and to examine the influence of such genotypes on hyperactivity symptoms in ASD patients. Eighty ASD patients (mean age 9 ± 1.9 years) and 100 control children (mean age 8.9 ± 1.9 years) were examined. COMT Val58Met polymorphism was genotyped using Tetra-primer ARMS-PCR method. The clinical diagnosis of ASD and ADHD were confirmed according to the DSM-IV criteria for research. We found no significant difference in genotypes or alleles’ frequencies of COMT Val158Met polymorphism between ASD patients and control group. There was a significant association between COMT (Val/Val) genotype and both increasing CARS (p = 0.001) and hyperactivity scores (p = 0.006). Regarding Conner's Score, the DSM-IV hyperactive impulsive were significantly higher in Val/Val genotype than both Met/Val and Met/Met genotypes (p = 0.03). Our data suggested an association between COMT Val58Met polymorphism and hyperactivity symptoms in Egyptian children with ASD.     

Increased incidence and size of cavum septum pellucidum in children with chromosome 22q11.2 deletion syndrome

Chromosome 22q11.2 deletion syndrome (22q11.2DS) is a result of a hemizygotic microdeletion that results in a variety of impairments in children including greater risk for psychiatric ailments in adulthood. We used high-resolution magnetic resonance imaging to accurately quantify the length and, for the first time, volume, of the cavum septum pellucidum (CSP) in children aged 7 to 14 years with 22q11.2DS and typically developing (TD) controls. Significantly greater anteroposterior length and greater CSP volumes were found in children with 22q11.2DS compared with controls. Furthermore, the largest CSP were found only in the 22q11.2DS group and with a much higher incidence than previously reported in the literature. Given the significant midline anomalies in the brains of those affected by 22q11.2DS, large CSP may be a biomarker of atypical brain development. The implication of these larger CSP for cognitive and behavioral development is a topic in need of further investigation.     

Cognitive and behavioral trajectories in 22q11DS from childhood into adolescence: A prospective 6-year follow-up study

Patients with 22q11DS are at risk of behavioral problems and cognitive impairment. Recent studies suggest a possible intellectual decline in 22q11DS children. To date it is unknown if cognitive development is related to the behavioral problems in 22q11DS. We studied 53 children with 22q11DS who underwent cognitive and behavioral assessments at 9.5 years (T1) and 15.3 years (T2). In about one third, IQ data obtained at 7.5 years (T0) were also available. Results showed that internalizing behaviors intensified while externalizing behaviors decreased. Simultaneously, in about a third a significant decline in IQ was found, which, surprisingly, was unrelated to the behavioral changes. It can be concluded that children with 22q11DS follow a unique developmental trajectory. Cognitive deterioration is severe in some but does not appear to predict behavioral problems in early adolescence.     

Motor functioning,exploration, visuospatial cognition and language development in preschool children with autism

In order to understand typical and atypical developmental trajectories it is important to assess how strengths or weaknesses in one domain may be affecting performance in other domains. This study examined longitudinal relations between early fine motor functioning, visuospatial cognition, exploration, and language development in preschool children with ASD and children with other developmental delays/disorders. The ASD group included 63 children at T1 (Mage = 27.10 months, SD = 8.71) and 46 children at T2 (Mage = 45.85 months, SD = 7.16). The DD group consisted of 269 children at T1 (Mage = 17.99 months, SD = 5.59), and 121 children at T2 (Mage = 43.51 months, SD = 3.81). A subgroup nested within the total sample was randomly selected and studied in-depth on exploratory behavior. This group consisted of 50 children, 21 children with ASD (Mage = 27.57, SD = 7.09) and 29 children with DD (Mage = 24.03 months, SD = 6.42). Fine motor functioning predicted language in both groups. Fine motor functioning was related to visuospatial cognition in both groups and related to object exploration, spatial exploration, and social orientation during exploration only in the ASD group. Visuospatial cognition and all exploration measures were related to both receptive and expressive language in both groups. The findings are in line with the embodied cognition theory, which suggests that cognition emerges from and is grounded in the bodily interactions of an agent with the environment. This study emphasizes the need for researchers and clinicians to consider cognition as emergent from multiple interacting systems.     

The bone tissue of children and adolescents with Down syndrome is sensitive to mechanical stress in certain skeletal locations: A 1-year physical training program study

The systemic complications of Down syndrome (DS) attenuate the osteogenic response to physical activity in DS patients. Through an interventional study we showed the effects of physical training on development of bone mineral content (BMC) and density (BMD) as well as on quantitative bone ultrasound (QUS) parameters in individuals with DS. A total of 42 children with DS were randomly assigned to either an exercising (DS-E, n = 20, age 16 ± 1.8 years) or non-exercising group (DS-NE, n = 22, age 16.9 ± 1.5 years). DS-E group was assigned to a program of osteogenic activities with 60 min sessions twice a week, over 12 month period. Bone mass measures were performed by dual X-ray absorpsiometry (DXA) at the spine and hip, and ultrasound attenuation (BUA) and velocity (SOS) assessed from the calcaneus by QUS device. All bone parameters had evolved with age, except for neck BMD. One year of training increased BMC values at lumbar spine (7%, p < .005) and total hip (10%, p < .05), and BMD values only at lumbar spine (4%, p < .05). Changes in BUA and SOS values were not evident following training. Trained individuals increased their motor skills measured through Eurofit tests.It was concluded that a program of osteogenic physical training may induce bone improvement in children with DS, but with a lower magnitude than that reported in the specialized literature for individuals without DS.     

Evaluation of health-care utilization in patients with Dravet syndrome and on adjunctive treatment with stiripentol and clobazam

Dravet syndrome (DS) is a rare, severe childhood epilepsy syndrome that imposes a substantial burden on patients and their caregivers. This study evaluated health-care utilization over a 2-year period in patients with DS at an outpatient clinic of a German epilepsy center. Data on the course of epilepsy, anticonvulsant treatment, and direct costs were recorded using the electronic seizure diary Epivista® and patients' files.We enrolled 13 patients with DS (6 females, mean age: 12.3 ± 7.5 years) between 2007 and 2010 and evaluated them during a 1-year baseline. All patients had drug-resistant epilepsy and their seizures failed to improve with a mean number of 6.7 ± 3.4 anticonvulsants. They had an overall mean seizure frequency of 102.1 seizures per year (median: 31, range: 3–538) with 43.2 GTCSs per year (median: 14, range: 0–228). We estimated the annual total direct costs at €6506 ± 3974 (range: €1174–11,783) per patient with hospitalization (68.9% of total direct costs) as the major cost factor ahead of costs for anticonvulsants (24.0%). For the 1-year follow-up period, less severely affected patients were continued on conventional anticonvulsants (n = 4) or switched to adjunctive treatment with stiripentol and clobazam (n = 9). In the latter group, six patients (67%) were long-term responders, with between 25% and 100% seizure reduction with respect to either GTCSs or the overall seizure frequency. This reduction in seizure frequency was associated with a shift in the distribution of cost components towards higher medication costs and decreased hospitalization costs. The total direct costs increased by 42.7%, mainly due to the newly introduced stiripentol, with an annual cost of €6610.This study showed that direct costs of patients with DS were above the average European costs of drug-resistant epilepsy in children. Treatment with new anticonvulsants resulted in reduction of seizures and inpatient admissions.     

Motor control of the lower extremity musculature in children with cerebral palsy

The aim of this investigation was to quantify the differences in torque steadiness and variability of the muscular control in children with cerebral palsy (CP) and typically developing (TD) children. Fifteen children with CP (age = 14.2 ± 0.7 years) that had a Gross Motor Function Classification System (GMFCS) score of I-III and 15 age and gender matched TD children (age = 14.1 ± 0.7 years) participated in this investigation. The participants performed submaximal steady-state isometric contractions with the ankle, knee, and hip while surface electromyography (sEMG) was recorded. An isokinetic dynamometer was used to measure the steady-state isometric torques while the participants matched a target torque of 20% of the subject's maximum voluntary torque value. The coefficient of variation was used to assess the amount of variability in the steady-state torque, while approximate entropy was used to assess the regularity of the steady-state torque over time. Lastly, the distribution of the power spectrum of the respective sEMG was evaluated. The results of this investigation were: 1) children with CP had a greater amount of variability in their torque steadiness at the ankle than TD children, 2) children with CP had a greater amount of variability at the ankle joint than at the knee and hip joint, 3) the children with CP had a more regular steady-state torque pattern than TD children for all the joints, 4) the ankle sEMG of children with CP was composed of higher harmonics than that of the TD children.     

Early screening and identification of psychological comorbidities in pediatric epilepsy is necessary

Youth with epilepsy often have co-occurring psychological symptoms that are due to underlying brain pathology, seizures, and/or antiepileptic drug side effects. The primary study aim was to compare the psychological comorbidities of youth with new-onset epilepsy versus chronic epilepsy. Primary caregivers of youth with either new-onset (n = 82; M_age = 9.9 ± 2.9) or chronic epilepsy (n = 76; M_age = 12.8 ± 3.3) completed the Behavioral Assessment Scale for Children—2nd Edition. Compared to those with new-onset epilepsy, the chronic group had significantly higher depressive and withdrawal symptoms, as well as lower activities of daily living. A higher proportion of youth with chronic epilepsy exhibited at-risk/clinically elevated depressive symptoms and difficulties with activities of daily living compared to the new-onset group. Proactive screening in youth with epilepsy to ensure timely identification of psychological symptoms and to guide early psychological intervention is warranted.     

Developmental Coordination Disorder in children with specific language impairment: Co-morbidity and impact on quality of life

Co-morbidity of Developmental Coordination Disorder (DCD) in children with specific language impairment (SLI) and the impact of DCD on quality-of-life (QOL) was investigated in 65 5–8 year old children with SLI (43 boys, age 6.8 ± 0.8; 22 girls, age 6.6 ± 0.8). The prevalence of DCD was assessed using DSM-IV-TR criteria (American Psychiatric Association (APA), 2000) operationally defined in the clinical practice guideline (CPG): movement ABC scores below 15th percentile, scores on DCDQ and/or MOQ-T below 15th percentile, absence of medical condition according to paediatric-neurological exam. Quality of life (QOL) was measured with the TNO-AZL-Child-Quality-Of-Life (TACQOL) Questionnaire filled out by parents for the SLI group with and without DCD, and compared to a reference group (N = 572; age 6.9 ± 0.9). The TACQOL covers 7 QOL domains: physical, motor, cognitive and social functioning, autonomy, positive and negative moods. Prevalence of DCD in children with SLI was 32.3%. In children with SLI, mean QOL scores were significantly lower in the autonomy, cognitive, social and positive moods domains compared to the reference group. Children with SLI and DCD differed from children with SLI without DCD by significantly lower mean overall-, motor-, autonomy-, and cognitive domain-QOL scores. Clinicians should be aware that about one third of children with SLI can also be diagnosed with DCD. Assessment of QOL is warranted in order to assess which domains are affected in children with SLI with or without DCD.     

Motor and cognitive assessment of infants and young boys with Duchenne Muscular Dystrophy: results from the Muscular Dystrophy Association DMD Clinical Research Network

Therapeutic trials in Duchenne Muscular Dystrophy (DMD) exclude young boys because traditional outcome measures rely on cooperation. The Bayley III Scales of Infant and Toddler Development (Bayley III) have been validated in developing children and those with developmental disorders but have not been studied in DMD. Expanded Hammersmith Functional Motor Scale (HFMSE) and North Star Ambulatory Assessment (NSAA) may also be useful in this young DMD population. Clinical evaluators from the MDA-DMD Clinical Research Network were trained in these assessment tools. Infants and boys with DMD (n = 24; 1.9 ± 0.7 years) were assessed. The mean Bayley III motor composite score was low (82.8 ± 8; p ? .0001) (normal = 100 ± 15). Mean gross motor and fine motor function scaled scores were low (both p ? .0001). The mean cognitive comprehensive (p = .0002), receptive language (p ? .0001), and expressive language (p = .0001) were also low compared to normal children. Age was negatively associated with Bayley III gross motor (r = ?0.44; p = .02) but not with fine motor, cognitive, or language scores. HFMSE (n = 23) showed a mean score of 31 ± 13. NSAA (n = 18 boys; 2.2 ± 0.4 years) showed a mean score of 12 ± 5. Outcome assessments of young boys with DMD are feasible and in this multicenter study were best demonstrated using the Bayley III.