Bone health in boys with Duchenne Muscular Dystrophy on long-term daily deflazacort therapy

Quality of life in Duchenne Muscular Dystrophy (DMD) has improved significantly with corticosteroid treatment. However, corticosteroids decrease bone mass and increase vertebral fragility fracture risk. We report on bone health in 39 boys with DMD on long-term deflazacort (0.9 mg/kg/day) therapy. Bone health was defined by lumbar (L₁–L₄) bone mineral density (BMD), long-bone and/or symptomatic vertebral fractures. Lumbar BMD was reported as height-adjusted Z-scores at initiation of deflazacort (T₀) and 1–2 year intervals thereafter. Subcapital body fat percentage and ambulatory status were recorded. At T₀, 39 boys, aged 6.6 ± 1.6 years had height-adjusted BMD Z-score −0.5 ± 0.8, and 23.5 ± 5.0% body fat. Height-adjusted Z-scores remained stable with years of deflazacort until loss of ambulation and accrual of body fat. Nine long-bone fractures occurred in eight ambulating boys, two before T₀. Seven vertebral fractures occurred in six non-ambulatory boys after ⩾5 years of deflazacort with height-adjusted Z-score −1.8 ± 0.7, and 47.8 ± 12% body fat. Bone health in DMD is influenced by disease progression, corticosteroids, BMD Z-scores and fat mass accumulation. Adjustments for short stature must be considered during BMD interpretation. Percent body fat and ambulatory status are useful bone health indicators. Routine use of height adjusted Z-scores is advocated for use in routine clinical practice.     

Assessment of intramuscular lipid and metabolites of the lower leg using magnetic resonance spectroscopy in boys with Duchenne muscular dystrophy

The purpose of this study was to use proton magnetic resonance spectroscopy to assess intramuscular lipid and metabolites of lower leg muscles in boys with Duchenne muscular dystrophy (DMD) and determine its relationship with strength and functional ability. Spectroscopic measurements were obtained from four muscles of the lower leg in 25 boys with DMD (9.2 ± 3.1 years) and 10 healthy boys (10.2 ± 2.6 years). Lipid fractions and metabolite concentrations were also determined. Muscle strength, a timed functional test, and the Modified Brooke Lower Extremity Functional Scale were also determined. Lipid fractions were higher (p < 0.01) for the DMD group than healthy subjects for all muscles, and lipid fraction was found to be greater in the older DMD boys. The peroneal muscle demonstrated a significant difference in lipid fraction in all DMD age groups. Lipid fractions in all muscles correlated with functional measures (r = 0.52–0.70, p < 0.001), with smaller inverse correlations with the strength measure (r = −0.36 to −0.56, p < 0.05). These findings provide quantifiable information regarding intramuscular lipid and metabolite levels of different muscles across various age groups in boys with DMD and may be used in determining the effect of interventions in future clinical trials.     

In utero exposure to fluoride and cognitive development delay in infants

The objective of this study was to evaluate the association between in utero exposure to fluoride (F) and Mental and Psychomotor Development (MDI and PDI) evaluated through the Bayley Scale of Infant Development II (BSDI-II) in infants. The sample included 65 mother-infant pairs. Environmental exposure to F was quantified in tap and bottled water samples and F in maternal urine was the biological exposure indicator; samples were collected during the 1st, 2nd and 3rd trimester of pregnancy. The mean values of F in tap water for the 1st, 2nd and 3rd trimester were 2.6 ± 1.1 mg/l, 3.1 ± 1.1 mg/l and 3.7 ± 1.0 mg/l respectively; above to 80% of the samples exceeded the reference value of 1.5 mg/l (NOM-127-SSA1-1994). Regarding F in maternal urine, mean values were 1.9 ± 1.0 mg/l, 2.0 ± 1.1 mg/l and 2.7 ± 1.1 mg/l for the 1st, 2nd and 3rd trimester respectively. The infants with MDI and PDI scores less than 85 points were 38.5% and 20.9% respectively. After adjusting for potential confounding factors (gestational age, age of child, marginalization index and type of water for consumption), the MDI showed an inverse association with F levels in maternal urine for the first (β = −19.05, p = 0.04) and second trimester (β = −19.34, p = 0.01). Our data suggests that cognitive alterations in children born from exposed mothers to F could start in early prenatal stages of life.     

Implicit and explicit motor learning: Application to children with Autism Spectrum Disorder (ASD)

Aims and objectivesThis study aims to determine whether children with Autism Spectrum Disorder (ASD) are capable of learning a motor skill both implicitly and explicitly.MethodsIn the present study, 30 boys with ASD, aged 7–11 with IQ average of 81.2, were compared with 32 typical IQ- and age-matched boys on their performance on a serial reaction time task (SRTT). Children were grouped by ASD and typical children and by implicit and explicit learning groups for the SRTT.ResultsImplicit motor learning occurred in both children with ASD (p = .02) and typical children (p = .01). There were no significant differences between groups (p = .39). However, explicit motor learning was only observed in typical children (p = .01) not children with ASD (p = .40). There was a significant difference between groups for explicit learning (p = .01).DiscussionThe results of our study showed that implicit motor learning is not affected in children with ASD. Implications for implicit and explicit learning are applied to the CO-OP approach of motor learning with children with ASD.     

Lack of differential motor outcome with subthalamic nucleus region stimulation in Parkinson’s disease

Deep brain stimulation (DBS) has emerged as a viable therapy for Parkinson’s disease (PD). The impact of subthalamic nucleus (STN) lead placement (lateral versus medial) on motor outcome, however, has not been systematically evaluated. Forty-eight patients with PD underwent STN-DBS surgery and were evaluated postoperatively for 48 weeks for motor improvement as measured by the Unified Parkinson’s Disease Rating Scale (UPDRS) part III (standardized motor examination) and levodopa equivalent daily dose (LEDD). Postoperative MRI was used to identify the location of the active stimulating contact and motor outcome was analyzed. STN-DBS was associated with significant improvement in motor outcome as determined by a reduction in the UPDRS part III subscore from 34.44 ± 1.29 at baseline to 18.76 ± 1.06 at end visit (p < 0.0001) and a reduction in LEDD from 1721 ± 152 mg/day at baseline to 1134 ± 119 mg/day at end visit (p = 0.0024). Patients with stimulating contacts in the medial STN compared to the lateral STN did not demonstrate any significant differences in motor outcome (UPDRS, p = 0.5811; LEDD, p = 0.7341). No significant differences were found in motor outcome between patients with STN stimulation compared to stimulation of surrounding fiber tracts (p = 0.80). No significant difference in stimulation voltage was noted with respect to lead location. Our study did not find a significant effect for the location of active contact and motor outcome neither within the subregions of the STN nor between the STN and surrounding fibers. Further research is needed to better understand the neurophysiological basis for these results.     

Understanding physical activity and motivations for children with Developmental Coordination Disorder: An investigation using the Theory of Planned Behavior

Developmental coordination disorder (DCD) is a neurodevelopmental condition, affecting approximately 5–6% of children. Previous research has consistently found children with DCD being less physically active compared to typically-developing (TD) children; however, the psychosocial factors associated with physical activity for children with DCD are poorly understood. The purpose of this study was to examine how theory-based physical activity cognitions impacts physical activity behaviors for children with and without DCD. Participants included a sample of boys (N = 61, M_age = 13.25 ± .46) with DCD (n = 19) and without DCD (n = 42), drawn from a larger prospective cohort study. A questionnaire with psychosocial measures was first administered, and accelerometers were used to assess their physical activity behavior over the subsequent week. Findings indicate that DCD was significantly associated with lower physical activity (F(1,58) = 6.51, p < .05), and poorer physical activity cognitions (F(4,56) Wilks Lambda = 2.78, p < .05). Meditational analyses found attitudes (B = .23, p < .05) and subjective norms (B = .31, p < .05) partially mediating the relationship between DCD and physical activity. Overall, this study further confirms that the activity deficit that exists among boys with DCD, and that the relationship is partially mediated through some physical activity cognitions. Interventions should target the perceived approval of influential people, and the personal evaluations of physical activity for boys with motoric difficulties. These findings further emphasizes the discrepancy in physical activity that exist between boys with DCD and TD boys, and highlight the need to better understand the psychological factors related to physical activity for children with DCD.     

A comparison of the oxygen cost and physiological responses to running in children with and without Developmental Coordination Disorder

The aim of this study was to compare the oxygen cost of running in boys with and without Developmental Coordination Disorder (DCD). Fourteen boys with DCD (9.1 ± 1.4 yr) and 16 typically developing (TD) controls (9.4 ± 1.3 yr) were tested on two separate occasions at least a week apart. On the first visit, motor proficiency, body composition and maximal aerobic capacity were established. On the second visit, oxygen consumption was determined via indirect calorimetry while participants ran at three submaximal speeds (7.2 km/h, 8.0 km/h and 8.8 km/h) on a motorised treadmill for 4 min each. Additional physiological responses such as blood lactate, respiratory exchange ratio (RER), heart rate, salivary alpha amylase and pain threshold were monitored at baseline and after each submaximal effort. Although there were no differences in the oxygen cost of running at all three speeds, the boys with DCD had higher blood lactate concentration (7.2 km/h, p = 0.05; 8.0 km/h p = 0.019), heart rate (p ≤ 0.001), RER (8.0 km/h, p = 0.019; 8.8 km/h, p = 0.001), salivary alpha amylase (8.0 km/h, p = 0.023; 8.8 km/h, p = 0.020) and a lower pain threshold (p < 0.01). The higher overall metabolic cost of running in boys with DCD as indicated by the higher RER, heart rate and blood lactate concentrations, together with the higher levels of sympathoadrenal medullary activity and sensitivity to pain, may be deterring factors for participation in physical activity in this population.     

Effects of overweight and obese body mass on motor planning and motor skills during obstacle crossing in children

Little is known about how obesity relates to motor planning and skills during functional tasks. We collected 3-D kinematics and kinetics as normal weight (n = 10) and overweight/obese (n = 12) children walked on flat ground and as they crossed low, medium, and high obstacles. We investigated if motor planning and motor skill impairments were evident during obstacle crossing. Baseline conditions showed no group differences (all ps > .05). Increased toe clearance was found on low obstacles (p = .01) for the overweight/obese group and on high obstacles (p = .01) for the normal weight group. With the crossing leg, the overweight/obese group had larger hip abduction angles (p = .01) and medial ground reaction forces (p = .006) on high obstacles and high anterior ground reaction forces on low obstacles (p = .001). With the trailing leg, overweight/obese children had higher vertical ground reaction forces on high obstacles (p = .005) and higher knee angles (p = .01) and anterior acceleration in the center of mass (p = .01) on low obstacles. These findings suggest that differences in motor planning and skills in overweight/obese children may be more apparent during functional activities.     

Aerobic exercise improves self-reported sleep and quality of life in older adults with insomnia

ObjectiveTo assess the efficacy of moderate aerobic physical activity with sleep hygiene education to improve sleep, mood and quality of life in older adults with chronic insomnia.MethodsSeventeen sedentary adults aged ?55 years with insomnia (mean age 61.6 [SD ± 4.3] years; 16 female) participated in a randomized controlled trial comparing 16 weeks of aerobic physical activity plus sleep hygiene to non-physical activity plus sleep hygiene. Eligibility included primary insomnia for at least 3 months, habitual sleep duration <6.5 h and a Pittsburgh Sleep Quality Index (PSQI) score >5. Outcomes included sleep quality, mood and quality of life questionnaires (PSQI, Epworth Sleepiness Scale [ESS], Short-form 36 [SF-36], Center for Epidemiological Studies Depression Scale [CES-D]).ResultsThe physical activity group improved in sleep quality on the global PSQI (p < .0001), sleep latency (p = .049), sleep duration (p = .04), daytime dysfunction (p = .027), and sleep efficiency (p = .036) PSQI sub-scores compared to the control group. The physical activity group also had reductions in depressive symptoms (p = .044), daytime sleepiness (p = .02) and improvements in vitality (p = .017) compared to baseline scores.ConclusionAerobic physical activity with sleep hygiene education is an effective treatment approach to improve sleep quality, mood and quality of life in older adults with chronic insomnia.     

Requirements for and impact of a serious game for neuro-pediatric robot-assisted gait training

We investigated whether children with neurological gait disorders who walked in a driven gait orthosis could adjust their participation level according to the demands of a newly developed rehabilitation game. We further investigated if cognitive capacity and motor impairment influenced game performance. Nineteen children with neurological gait disorders (mean age: 13.4y, 42% girls) participated. To quantify game participation, electromyographic muscle activity (M. rectus femoris) and heart rate were compared in a demanding part and a less demanding part of the game. Cognitive capacity was assessed with the Test of Nonverbal Intelligence (TONI-4). Furthermore, the Functional Independence Measure for Children (WeeFIM), Manual Muscle Tests and a therapist-derived score of how well the child was able to train were assessed. Results showed that muscle activity and heart rate were higher during the demanding part of the game (30.7 ± 22.6 μV; 129.4 ± 15.7 bpm) compared to the less demanding part (16.0 ± 13.4 μV; 124.1 ± 15.9 bpm; p < 0.01 for both measures). Game performance correlated moderately with the TONI-4 (r = 0.50, p = 0.04) and the cognition subscale of the WeeFIM (ρ = 0.59, p = 0.01). The therapist-derived score correlated significantly with game performance (p = 0.75, p < 0.01) and the ability to modify muscle activity to the demands of the game (p = ?0.72, p < 0.01). Receiver operating characteristic analyses revealed that the latter factor differentiated well between those children suitable for the game and those not. We conclude that children with neurological gait disorders are able to modify their activity to the demands of the VR-scenario. However, cognitive function and motor impairment determine to which extent. These results are important for clinical decision-making.     

Adult separation anxiety disorder in complicated grief: an exploratory study on frequency and correlates

IntroductionComplicated grief (CG) has been the subject of increasing attention in the past decades but its relationship with separation anxiety disorder (SEPAD) is still controversial. The aim of the current study was to explore the prevalence and clinical significance of adult SEPAD in a sample of help-seeking individuals with CG.Methods151 adults with CG, enrolled in a randomized controlled trial comparing the effectiveness of (CG) treatment to that of interpersonal therapy, were assessed by means of the Inventory of Complicated Grief (ICG), the Structured Clinical Interview for DSM-IV, the Hamilton Rating Scale for Depression (HAM-D), the Work and Social Adjustment Scale (WSAS), the Adult Separation Anxiety Questionnaire (ASA-27), the Grief Related Avoidance Questionnaire (GRAQ), the Peritraumatic Dissociative Experiences Questionnaire (PDEQ), and the Impact of Events Scale (IES).Results104 (68.9%) individuals with CG were considered to have SEPAD (ASA-27 score ≥22). Individuals with SEPAD were more likely to have reported a CG related to the loss of another close relative or friend (than a parent, spouse/partner or a child) (p = .02), as well as greater scores on the ICG (p = <.001), PDEQ (p = .004), GRAQ (p < .001), intrusion (p < .001) and avoidance (p = <.001) IES subscales, HAM-D (p < .001) and WSAS (p = .006). ASA-27 total scores correlated with ICG (p < .0001), PDEQ (p < .001) GRAQ (p < .0001) scores and both the IES intrusion (p < .0001) and IES avoidance (p < .0001) subscale scores. People with SEPAD had higher rates of lifetime post-traumatic stress disorder (PTSD) (p = .04) and panic disorder (PD) (p = .01).ConclusionsSEPAD is highly prevalent among patients with CG and is associated with greater symptom severity and impairment and greater comorbidity with PTSD and PD. Further studies will help to confirm and generalize our results and to determine whether adult SEPAD responds to CG treatment and/or moderates CG treatment response.     

The relationship between motor abilities and early social development in a preschool cohort of children with cerebral palsy

AimTo investigate the relationship between motor ability and early social development in a cohort of preschool children with cerebral palsy (CP).DesignPopulation-based cohort study.MethodsParticipants were 122 children with CP assessed at 18, 24 and 30 months, corrected age (ca). Motor ability was measured by the Gross Motor Function Classification System (GMFCS) with classification assigned by physiotherapists. The sample was representative of a population-based cohort (I = 48, 38.4%, II = 19, 15.2%, III = 17, 13.6%, IV = 22, 17.6% and V = 19, 15.2%). Social development was measured by the Paediatric Evaluation of Disability Inventory (PEDI) and included capabilities in social interaction, social communication, interactive play and household/community tasks.ResultsCross-sectional analyses indicated a significant relationship between motor ability and social development at 18 months, F(4, 56) = 11.44, p < .0001, η² = .45, at 24 months, F(4, 79) = 15.66, p < .0001, η² = .44 and at 30 months, F(4, 76) = 16.06, p < .0001, η² = .49. A longitudinal analysis with a subset of children (N = 24) indicated a significant interaction between age at assessment and GMFCS, F(2, 21) = 7.02, p = .005, η² = .40. Comparison with community norms indicated that at 18 months corrected age, 44.3% of the cohort was greater than two standard deviations below the mean (>2SD) for social development and a further 27.9% of the cohort was greater than one standard deviation below the mean (>1SD).InterpretationThere is a relationship between motor ability and social development in preschool children with CP. Children with CP may require support for social development in additional to physical interventions, from as early as 18 months.     

Ambulatory capacity and disease progression as measured by the 6-minute-walk-distance in Duchenne muscular dystrophy subjects on daily corticosteroids

In order to understand contemporary natural history of Duchenne muscular dystrophy (DMD), we report 6-minute walk distance (6MWD) and its change over time from a large single centre population of corticosteroid treated DMD boys. Sixty-five boys on daily corticosteroid treatment were identified with a mean (SD) age of 9.5 (2.3) years at first observation. 6MWD was described for 1 year age groupings. In addition, changes in 6MWD at 1, 1.5 and 2 years (±12 weeks) of follow-up were evaluated. The same evaluations were applied to 6MWD data converted to percent predicted values based on the Geiger equation. 6MWD showed an increase from age group 4.5–5.5 years to age group 6.5–7.5 years, followed by a decline, which became precipitous from 12.5 years onwards. From 15.5 years, all boys were unable to perform the 6-min test. Changes in 6MWD demonstrated a mean (median, SD) decline of ?43 (?14, 90) m at 1 year (N = 25, mean baseline age 9.5 years), ?64 (?56, 99) m at 1.5 years (N = 18, mean baseline age 9.6 years), ?125 (?106, 139) m at 2 years (N = 14, mean baseline age 10.0 years). Conversion to percent predicted values showed the same pattern of evolution.This study provides data on the ambulatory capacity and its changes over time in a homogenous cohort of 65 DMD boys on daily corticosteroids. The variability, the age-related aspects and the slope of decline of the 6MWD should be considered in the design and interpretation of therapeutic trials in ambulant DMD patients.     

Pulmonary capillary wedge pressure and pulmonary arterial pressure in heart failure patients with sleep-disordered breathing

BackgroundThere is a high prevalence of central sleep apnea (CSA) in patients with chronic heart failure (CHF). The present study investigates the hypotheses that CSA in CHF patients reflects heart failure severity as measured by cardiac index (CI), pulmonary artery pressure (PAP) and pulmonary capillary wedge pressure (PCWP).MethodsIn 105 patients with stable CHF (NYHA ? II, LV-EF ? 40%) cardiorespiratory polygraphy and simultaneous right and left heart catheterization was performed.ResultsCSA was present in 58% and obstructive sleep apnea (OSA) in 23% of patients. In CSA patients, PAP and PCWP were significantly higher when compared to patients without SDB. In CSA patients, but not in OSA patients, PCWP showed a significant correlation with apnea–hypopnea index (AHI; r = 0.41, p = 0.005), apnea index (AI; r = 0.44, p = 0.003) and central AI (cAI; r = 0.358, p = 0.015). Cardiac index was more impaired in CSA (1.93 ± 0.5 l/min/m²) than in OSA patients (2.55 ± 1.0 l/min/m²) or those without SDB (2.22 ± 0.4 l/min/m²). A negative correlation of CI and cAI (r = ?0.344, p = 0.008), AI (r = ?0.31, p = 0.02) and AHI (r = ?0.21, p < 0.05) was documented exclusively in CSA patients.ConclusionThe present study supports the hypotheses that the occurrence and severity of CSA in CHF patients reflects heart failure severity.     

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.     

Cross sectional area reference values for sonography of peripheral nerves and brachial plexus

ObjectiveUltrasound measurements of the cross sectional area (CSA) variability have been recently introduced to quantify pathological changes in peripheral nerves (PN).MethodsReference values from 75 healthy subjects and their correlation to age, height, weight and sex are reported.ResultsThe mean values in PN were: (1) intranerve CSA-variability: median 1.05 (SD ± 0.13), ulnar 1.53 (SD ± 0.51), fibular 1.33 (SD ± 0.37), tibial 1.39 (SD ± 0.39), (2) internerve CSA-variability 1.76 (SD ± 0.37), (3) intraplexus CSA-variability 1.52 (SD ± 0.37), (4) side-to-side difference ratio of the CSA-variability: median 1.21 (SD ± 0.04), ulnar 1.2 (SD ± 0.25), fibular 1.19 (SD ± 0.23), tibial 1.28 (SD ± 0.24) and brachial plexus 1.19 (SD ± 0.23). CSA did not correlate with height in PN, but correlated with weight in the ulnar nerve [Guyon’s canal, r = 0.411, p = 0.0237, elbow r = 0.409, p = 0.0248]. Significant changes between sex were found only in the ulnar (Guyon’s canal, p = 0.0265), fibular (popliteal fossa, p = 0.0336) and sural nerve (p = 0.048). CSA decreased with age in the median (axilla, p = 0.0236), and radial nerve (spiral groove, p = 0.0037) and increased in the tibial nerve (ankle, p < 0.0001).ConclusionsThe CSA reference values reported seem to correlate at certain sites with age, weight and sex but not with height.SignificanceThe new CSA variability measures may be helpful in investigating pathologies of the PN.     

Influence of a two-year steroid treatment on body composition as measured by dual X-ray absorptiometry in boys with Duchenne muscular dystrophy

Steroids are nowadays routinely used as a long-term treatment in Duchenne muscular dystrophy (DMD). Their effects on body composition were assessed using dual X-ray absorptiometry. The study followed over 2 years 29 genetically confirmed DMD patients: 21 in the steroid-treated group and 8 in the steroid-naïve group. After 2 years of steroid treatment, the lean tissue mass values increased significantly (p < 0.0001), the percentage of body fat mass remained practically constant (p = 0.94) in comparison with the initial visit. In the steroid-naïve patients, there were no significant increases in the lean tissue mass but deterioration in body composition confirmed by a significant increase in the percentage of body fat mass. Besides, significant negative correlations were found between the percentage of body fat mass and the MFM total score (R = −0.79, n = 76, p < 0.0001). A 2-year steroid treatment improves significantly body composition of boys with DMD through a significant increase in lean tissue mass. We suggest that a thorough check of body composition should be carried out before steroid treatment discontinuation in case of overweight gain.     

Variabilidad en la exploración motora de la enfermedad de Parkinson entre el neurólogo experto en trastornos del movimiento y la enfermera especializada

Introduction and objectiveIn clinical practice, assessing patients with Parkinson's disease (PD) is a complex, time-consuming task. Our purpose is to provide a rigorous and objective evaluation of how motor function in PD patients is assessed by neurologists specialising in movement disorders, on the one hand, and by nurses specialising in PD management, on the other.MethodsWe conducted an observational, cross-sectional, single-centre study of 50 patients with PD (52% men; mean age: 64.7  ±  8.7 years) who were assessed between 5 January 2016 and 20 July 2016. A neurologist and a nurse evaluated motor function in the early morning hours using the Unified Parkinson's Disease Rating Scale (UPDRS) parts III and IV and Hoehn & Yahr (H&Y) scale. Tests were administered in the same PD periods (in 48 patients during the ‘off’ time and in 2 patients during the ‘on’ time). Inter-rater variability was estimated with the intraclass correlation coefficient (ICC).ResultsForty-nine patients (98%) were classified in the same H&Y stage by both raters. Assessment times were similar for both raters. ICC for UPDRS-IV and UPDRS-III total scores were 0.955 (P<.0001) and 0.954 (P<.0001), respectively. The greatest variability was found for UPDRS-III item 29 (gait; ICC = 0.746; P<.0001) and the lowest, for item 30 (postural stability; ICC = 0.918; P<.0001).ConclusionsMotor function assessment of PD patients by a trained nurse is equivalent to that made by an expert neurologist and takes the same time to complete.     

Correlation between motor performance scales, body composition, and anthropometry in patients with duchenne muscular dystrophy

The aim of this study is to investigate the relationship between body composition, anthropometry, and motor scales in patients with Duchenne muscular dystrophy (DMD). Twenty six patients with DMD were evaluated by Expanded Hammersmith Functional Motor Scale (HFMSE), gross motor function classification system (GMFCS), multifrequency bioelectrical impedance analysis, and anthropometric measurements. Seventeen healthy children served as control group. There were 26 patients with a mean age of 9.5 ± 4.8 years. Ages and anthropometric measurements did not differ between groups. Of the 26 patients, nine were level I, seven were level II, two were level III, seven were level IV, and one was level V, according to the GMFCS. Despite the similar percentage of total body water, extracellular water/intracellular water ratio was significantly elevated in DMD patients (p = 0.001). Increased values of fat percentage and body fat mass index (BFMI) correlated positively with elevated GMFCS levels (r = 0.785 and 0.719 respectively). Increased fat-free mass index (FFMI) correlated negatively with elevated GMFCS levels (r = ?0.401). Increased fat percentage and BFMI correlated negatively with HFMSE scores (r = ?0.779 and ?0.698, respectively). Increased values of FFMI correlated positively with HFMSE scores. There was also a negative correlation between increased skin fold measurements from triceps and scapula and HFMSE scores (r = ?0.618 and ?0.683, respectively). Increased skin fold values from the same regions correlated positively with elevated GMFCS levels (r = 0.643 and 0.712, respectively). Significant body composition changes occur in patients with DMD. Anthropometric and multifrequency bioelectrical impedance analyses measurements show good correlation between motor function scales. These results may also be helpful to evaluate the effects of new treatment strategies.     

Predictors for changes in various developmental outcomes of children with cerebral palsy—A longitudinal study

We aimed to identify predictors for the changes of various developmental outcomes in preschool children with cerebral palsy (CP). Participants were 78 children (49 boys, 29 girls) with CP (mean age: 3 years, 8 months; SD: 1 year, 7 months; range: 1 year to 5 years, 6 months). We examined eight potential predictors: age, sex, CP subtype, Gross Motor Function Classification System (GMFCS) level, selective motor control, Modified Ashworth Scale, and the spinal alignment (SA) and range of motion subscales of the Spinal Alignment and Range of Motion Measure (SAROMM). Developmental outcomes for cognition, language, self-help, and social and motor functions were measured at baseline and a 6-month follow-up with the Comprehensive Developmental Inventory for Infants and Toddlers. Regression model showed GMFCS level was a negative predictor for change of language (adjusted r² = 0.30, p < .001), motor function (adjusted r² = 0.26, p < .001), social function (adjusted r² = 0.07, p = 0.014), and self-help (adjusted r² = 0.26, p < .001). Age was a negative predictor for change of cognition (adjusted r² = 0.21, p < .001) and language functions (adjusted r² = 0.26, p < .001). SAROMM-SA was a negative predictor for cognitive change (adjusted r² = 0.30, p < .001). The GMFCS levels and age are robust negative predictors for change of most developmental domains in these children.