New muscle power test in neuromuscular disease. Feasibility and reliability

We studied the feasibility, reliability, and reproducibility of an "all-out" 30-second cycling or arm cranking test of muscle power and muscle endurance (the Wingate Anaerobic Test) in 66 girls and boys aged 5 to 18 years old who had cerebral palsy or a myopathy. The arm and leg tests, given in duplicate, were feasible in 94% and 61% of subjects, respectively. Reliability coefficients exceeded .95 in patients with spastic cerebral palsy and myopathies and were somewhat lower in those with athetotic cerebral palsy. Means and SDs were similar in both trials. We conclude that the Wingate Anaerobic Test is feasible, highly reliable, and reproducible in these patients.     

A Review of the Quality of Upper Extremities Skills Test (QUEST) for Children with Cerebral Palsy

Disorder of movement is the main feature of cerebral palsy. Yet, until recently, no easy-to-use validated measure of upper limb quality of movement has been available for children with cerebral palsy. The Quality of Upper Extremities Skills Test (QUEST) was developed to meet this need. The QUEST was developed for children aged 18 months to 8 years and this review finds that initial reliability and validity data are promising. The ability of the test to detect change, however, is less clear and there is little support for its discriminative properties. Some suggestions are made regarding administration and scoring procedures. Notwithstanding these limitations, the QUEST is identified as an important first step to the measurement of upper extremity quality of movement for children with cerebral palsy.     

Nutritional status of mentally retarded children in north-west Spain. I. Anthropometric indicators

AIM: To evaluate the nutritional status of mentally retarded children in the region of Galicia in north-west Spain, on the basis of anthropometric variables. METHODS: The following variables were determined in a sample of 128 mentally retarded children (81 M, 47 F): birthweight, bodyweight, height/length, head circumference, mid-arm circumference, mid-arm muscle circumference, triceps skinfold thickness, subscapular skinfold thickness, arm area, arm muscle area, arm fat area, arm lean-to-fat ratio, Shukla's nutrition index and Quetelet's body mass index (BMI). A preliminary statistical analysis indicated that most information content could be maintained considering only eight of these variables. A factor analysis of the resulting 8 x 128 (variables by subjects) data matrix was performed, identifying two factors (FA1 and FA2) that together explained 82% of total variance. Taking these factors as indicators of nutritional status, the data were analysed for possible effects of age, gender, socioeconomic and family environment, intelligence quotient (IQ), presence/absence of cerebral palsy, quality of diet, appetite and antiepileptic use. RESULTS: The analysis suggested that most subjects were in the normal nutrition range, but about 33% showed either borderline or definite malnutrition. Mean score on FA2 showed a significant negative correlation with age. Children with cerebral palsy had lower mean scores on both factors, and scores on both factors varied with IQ. Children with definite malnutrition had a significantly lower IQ than those in the normal nutrition range. Mean score on both factors varied with appetite and quality of diet. The mean FAI score of children from inland-rural areas was significantly lower than that of children from coastal or urban areas. Mean FA1 score increased with increasing age of the parents. The prevalence of obesity was 13% when obesity was defined on the basis of Shukla's nutrition index, and 19% when defined on the basis of FA1 score. CONCLUSION: Malnutrition as revealed by anthropometric variables occurs with a high prevalence among mentally retarded children. The prevalence increases with age, increasing IQ deficit and cerebral palsy.     

高选性周围神经肌支切断术治疗痉挛性脑瘫

目的：痉挛性脑瘫多以大腿内收肌群、后群和小腿后肌张力增高为主，从而与相对应的拮抗肌群形成不平衡状态，导致尖足、足内翻与剪刀步等畸形与功能异常。针对此病因，通过选择性切断高张力肌群的部分神经束，可矫正失衡状态，改善功能。方法：在周围神经应用解剖研究的基础上，对38例因肌张力增高失主要原因引起畸形或功能障碍的患儿，采用了高选性周围神经切断术，其中闭孔神经21例，坐骨神经27例，胫神经26例。确定高张力肌群是以临床病理生理改变为依据，在电刺激监测下确定切断高敏感的神经束支。按肌张力增高程度确定切断神经束的比例。结果：本组获随访的31例，时间3-30个月，功能改善有效率为100％。31例术后患儿肌张力均下降1例，按评定标准，优22例，良6例，一般2例，差1例。结论：该术式具有针对性强，创伤小，疗效确切、并发症少等优点。     

The effect of pre-exercise carbohydrate supplementation on anaerobic exercise performance in adolescent males

Carbohydrate (CHO) consumption before anaerobic exercise was studied in 13 adolescent boys (15.2 ± 0.9 yrs). A within subjects design was employed where subjects consumed a 22% CHO or volume-matched placebo (PL) beverage 30-min before anaerobic exercise on two separate days. Exercise consisted of a Wingate Anaerobic Test (WAnT), ten by 10-s-sprints, and a second WAnT. Fatigue index and peak power (PP) were similar while mean power (MP) was higher (p < .025) in CHO trial; however this difference was ascribed to initial WAnT performance. PP and MP for the 10-s sprints were similar between trials. Intravenous blood glucose and insulin concentrations were higher (p < .05) in the CHO trial while lactate and catecholamine concentrations were similar. Improved performance on a single WAnT was apparent with CHO consumption before exercise; however, this strategy did not attenuate fatigue over time in adolescent boys.     

Optimizing the Wingate Anaerobic Cycling Test for youth with juvenile idiopathic arthritis

The Wingate Anaerobic Test (WAnT) can assess muscle function in youth with juvenile idiopathic arthritis (JIA). Our objective was to compare peak power (PP) and mean power (MP) when the WAnT is performed with a standard vs. an optimized braking force. Eight patients with JIA between the ages of 8 and 18 participated in two sessions. Optimal braking force was determined with a series of 15-s force-velocity tests performed against braking forces ranging from 3.5 to 8.5% of body weight. Participants then performed two randomized WAnTs against the standard (4.5%) and optimal braking forces. PP tended to be greater in the optimized vs. standard WAnT (12.5 ± 2.6 vs. 10.8 ± 1.0 W/kg, respectively; p = .07). No differences were observed for MP (standard: 6.2 ± 0.9 vs. optimized: 6.2 ± 1.1 W/kg; p = .9). Optimization of the WAnT tended to increase PP by 10-28% in youth with JIA.     

Attenuated maximal muscle strength and peak power in children with sickle cell disease

Dominant hand maximal handgrip strength evaluated with a handgrip dynamometer and peak power evaluated with a force plate, adjusted for body size and composition, were compared in African-American children aged 5 to 13 years, with and without type SS sickle cell disease (SCD-SS). Children with SCD-SS (n = 35; age, 9.0 ± 2.0 y) compared with healthy control children (n = 103; age, 8.6 ± 1.8 y) did not differ by age, sex, or pubertal status, yet had significantly lower Z scores for height, weight, body mass index, upper arm muscle area, upper arm fat area, fat mass-for-height and lean mass-for-height. Children with SCD-SS had significantly lower handgrip strength (12.7 ± 3.3 vs. 15.2 ± 5.1 kg, P < 0.008), peak power (882 ± 298 vs. 1167 ± 384 W, P < 0.001), and growth and body composition adjusted Z scores for handgrip strength (0.6 ± 1.3 standard deviations, P < 0.004) and peak power (male children = 1.0 ± 0.8 standard deviations, P < 0.0002; female children = 1.0 ± 1.7 standard deviations, P < 0.006). Maximal muscle strength and peak power are attenuated in children with SCD-SS compared with healthy control children beyond expectation for growth and body composition deficits suggesting that additional factors contribute to attenuation in anaerobic performance.     

虚拟现实训练对痉挛型双瘫脑瘫患儿肢体运动功能的影响

目的 观察虚拟现实（VR）训练对痉挛型双瘫脑瘫患儿上肢精细运动和下肢粗大运动的影响。方法 选取痉挛型双瘫脑瘫患儿35 例,随机分为VR 训练组（n=19）和常规训练组（n=16）,常规训练组给予3 个月的常规运动疗法和作业疗法训练;VR 训练组给予3 个月的VR 训练和作业疗法训练。采用Peabody 运动发育量表的抓握、视觉-运动整合分测试对患儿治疗前后精细运动进行评价,采用88 项粗大运动功能量表（GMFM-88）的D 区及E 区、改良Ashworth 量表（MAS）、Berg 平衡量表（BBS）对患儿治疗前后粗大运动进行评价。结果 治疗前两组患儿抓握、视觉-运动整合、精细运动发育商、GMFM-88 之D 区、E 区评分、MAS评分、BBS 评分无明显差异（P > 0.05）;治疗后,VR 训练组抓握、视觉-运动整合、精细运动发育商、GMFM-88D 区评分、E 区评分、BBS 评分、MAS 评分较常规训练组明显改善（P 结论 VR 训练可有效提高痉挛型双瘫脑瘫患儿上肢精细运动功能和下肢粗大运动功能。     

Relationships between lower limb muscle strength and locomotor capacity in children and adolescents with cerebral palsy who walk independently

This study aimed to quantify relationships between lower limb muscle strength and locomotor capacity for children and adolescents with cerebral palsy (CP) to identify key muscle groups for strength training. Fifty 6- to 16-year-olds with CP (Gross Motor Function Classification System level I or II) participated. Isometric muscle strength of hip flexor and abductor, knee flexor and extensor, and ankle dorsiflexor muscles was measured using hand-held dynamometry. Ankle plantar flexor concentric muscle strength was assessed as the maximal number of unilateral heel rises. Locomotor capacity was evaluated by the 6-min walk test (6MWT), 10-meter Shuttle Run Test (10mSRT), and Timed Up and Down Stairs Test (TUDS). With control for age, sex, and height, hip flexor and ankle plantar flexor strength explained 47.8% of the variance in the 6MWT and 32.9% of variance in the TUDS and hip abductor isometric strength explained 43.5% of the variance in the 10mSRT. Avenues for future research include randomized controlled trials that specifically target hip flexor muscles, as this has not previously been done, and determining factors other than strength that are likely related to locomotor capacity of children and adolescents with CP.     

Relationships Between Lower Limb Muscle Strength and Locomotor Capacity in Children and Adolescents with Cerebral Palsy Who Walk Independently

ABSTRACT

This study aimed to quantify relationships between lower limb muscle strength and locomotor capacity for children and adolescents with cerebral palsy (CP) to identify key muscle groups for strength training. Fifty 6- to 16-year-olds with CP (Gross Motor Function Classification System level I or II) participated. Isometric muscle strength of hip flexor and abductor, knee flexor and extensor, and ankle dorsiflexor muscles was measured using hand-held dynamometry. Ankle plantar flexor concentric muscle strength was assessed as the maximal number of unilateral heel rises. Locomotor capacity was evaluated by the 6-min walk test (6MWT), 10-meter Shuttle Run Test (10mSRT), and Timed Up and Down Stairs Test (TUDS). With control for age, sex, and height, hip flexor and ankle plantar flexor strength explained 47.8% of the variance in the 6MWT and 32.9% of variance in the TUDS and hip abductor isometric strength explained 43.5% of the variance in the 10mSRT. Avenues for future research include randomized controlled trials that specifically target hip flexor muscles, as this has not previously been done, and determining factors other than strength that are likely related to locomotor capacity of children and adolescents with CP.     

Neuromuscular electrical stimulation for children with cerebral palsy: a review

The aim of this review paper is to consider the application of neuromuscular electrical stimulation (NMES) to improve gait or upper limb function in children with cerebral palsy (CP). Although most NMES research has been directed at adults with neurological conditions, there is a growing body of evidence supporting its use in children with CP. In line with a recent meta-analysis, the use of electrical stimulation to minimise impairment and activity limitations during gait is cautiously advocated. A detailed commentary on one of the most common lower limb NMES applications, tibialis anterior stimulation (either with or without gastrocnemius stimulation) is given. Although there is a lack of randomised controlled trials and a predominance of mainly small studies, this review further concludes that the balance of available evidence is in favour of upper limb exercise NMES offering benefits such as increased muscle strength, range of motion and function in children with CP. The use of dynamic splinting with NMES has been shown to be more effective than either treatment on its own in improving function and posture. There is at present little published work to support the application of botulinum toxin type A to temporarily reduce muscle tone as an adjunct intervention to NMES in this population, although the presence of parallel applications to manage similar symptoms in other muscular disorders is noted.     

Six-year follow-up of early physiotherapy intervention in very low birth weight infants

Eighty very low birth weight infants assigned to neurologically normal or at-risk groups on the basis of a neurodevelopmental score were previously described. Infants were assigned to physiotherapy or control groups, and the effect of physiotherapy was assessed at 1 year. At-risk infants had a significantly lower developmental quotient than the normal group and no beneficial effect of physiotherapy was shown. Of the original 80 subjects, 49 were reassessed at a mean age of 74.7 months. As observed previously, physiotherapy until 1 year did not influence subsequent outcome in either normal or at-risk children. At-risk and normal children had similar mean developmental quotients at 6 years, but the locomotor score of at-risk children was significantly below that of normal children. Cerebral palsy occurred in 6 of 24 at-risk vs 0 of 25 normal subjects (P less than .01) and remedial therapy was recommended in 17 of 24 at-risk subjects vs 6 of 25 normal subjects (P less than .001). These results confirm that the neurodevelopmental score predicts a risk for either cerebral palsy or soft neurological problems, and early physiotherapy is of questionable benefit in preventing such problems.     

Adaptive motor strategy for squatting in spastic diplegia.

Motor strategies, defined by kinetic, kinematic and/or muscle activation patterns, reflect neural planning of movement, which takes into account central as well as peripheral constraints. Major alteration is expected in cerebral palsy, a condition characterized by abnormal posture and movement secondary to early lesion of the brain. The objective of this study was to characterize the motor strategies involved in disruption of posture in cerebral palsy of the spastic diplegia type and compare them with normal controls. The optoelectronic ELITE system was used to record and analyse the movement of squatting from the standing position with the arms extended forward in 11 children with spastic diplegia aged between 3 and 12 years and 11 age-matched normal controls. Normal children maintained gaze and arm horizontality and trunk verticality throughout the movement. The knee followed an oblique trajectory. Its angular velocity profile showed a short, single-peaked, ascending phase. The onset of movement was preceded by deactivation of the semimembranous muscle. In diplegic children, gaze and arm horizontality and trunk verticality were lost. The ankle was rigidified, resulting in spatial fixation of the knee. The ascending phase of the knee velocity profile was prolonged and multi-peaked. There was widespread muscle co-contraction from the outset of movement. No anticipatory deactivation was evidenced, but anticipatory bursts appeared in the soleus. Patients with cerebral palsy have to organize a limited motor repertoire from a restricted neural potential. Consequent motor strategies presently demonstrated in spastic diplegia are distinct and appear as an original alternative to those of normal subjects.     

Lower limb standards in newborns

To define standards for lower limb measurements in the newborn, 198 full-term and preterm infants (range, 27 to 41 gestational weeks) were examined. The gestational age was determined chronologically and clinically, and the total length of the lower limb and the leg and foot lengths were measured by two observers with the use of standard measurement techniques. Normal values were determined by plotting the mean +/- 2 SDs for each gestational week v gestational age.     

Involuntary and voluntary muscle activation in children with unilateral cerebral palsy – Relationship to upper limb activity

BackgroundSpasticity and reduced strength are both primary neuromuscular impairments associated with cerebral palsy (CP). However, it is unclear whether spasticity or reduced strength is the strongest contributor to activity limitations.AimTo study whether involuntary activation of the biceps brachii muscle, in addition to reduced strength, contributes to limitations in upper limb activity in children with CP.MethodFifteen children with unilateral CP (9 males and 6 females, age range 8–17 years) participated in this study. Involuntary activation, reflecting spasticity, was studied as biceps brachii activity during passive elbow extension at four isokinetic velocities (10, 90, 180 and 300°/s). Elbow flexion peak torque, reflecting strength, was measured during maximal voluntary isometric contraction, and concurrent biceps brachii activity was registered reflecting voluntary muscle activation. Bimanual upper limb activity was assessed in the performance domain using the Assisting Hand Assessment (AHA).ResultsBoth involuntary and voluntary muscle activation were related to activity, the former negatively, but voluntary activation showed the strongest relationship (Spearmans rho = .84). Involuntary muscle activation at 10, 90 and 180°/s was negatively related to muscle strength (Spearmans rho = −.63, −.58 and −.62, respectively).ConclusionsOur results do not indicate that spasticity affects upper limb activity in addition to strength. Most likely, muscle weakness and spasticity jointly contribute to activity limitations, reflected by the strong relationship between the ability to voluntarily activate a muscle and activity performance.     

Continuous intrathecal baclofen infusion for children with cerebral palsy: a pilot study

OBJECTIVES: To determine whether continuous intrathecal baclofen infusion (CIBI) would decrease spasticity and improve function in children with spastic cerebral palsy. DESIGN: Prospective study with measurement of changes from baseline assessed at regular intervals for 6 months following the intervention. PATIENTS: Two subjects aged 8 and 9 years with cerebral palsy. Inclusion criteria included severe spasticity and age greater than 4 years. STUDY CENTRE: Department of Rehabilitation, The Children's Hospital at Westmead, Westmead, New South Wales, Australia. RESULTS: Both subjects had clinically significant reductions in lower limb spasticity from a single intrathecal dose of baclofen (screening procedure), and had intrathecal pumps implanted. An intensive physical therapy programme was provided for both subjects in the 1-3-month period after commencing CIBI, in order to maximize functional gains. Modified Ashworth Scale scores remained reduced during the 6-month study period. There was a clinically significant increase in upper limb function for Subject 1. Gross Motor Function Measure scores decreased from 22% to 19% for Subject 1, and increased from 6% to 10% for Subject 2 over the study period. Paediatric Evaluation of Disability Inventory scores for Subject 1 showed a reduction in the level of caregiver assistance required, while Subject 2 showed significant improvement in the functional mobility domain. The major changes noted in the parent questionnaires were reduction in tone, increased range of motion and reduced time taken helping with activities of daily living such as toilet and dressing. No significant side-effect was seen after the screening procedure or after continuous intrathecal infusion. Both subjects' parents felt their child was improved following the intervention. CONCLUSIONS: Children with spastic cerebral palsy can have their spasticity effectively reduced with CIBI. In this study of two children, the clinical improvements were encouraging, and it is proposed that CIBI may be of benefit to those patients whose level of spasticity severely interferes with function. Further studies using multidimensional assessment approaches, with larger numbers of children, are warranted.     

Analysis of power output time series in response to supramaximal exercise: an approach through dynamic factor analysis

The responses to supramaximal exercise testing have been traditionally analyzed by means of standard parametric and nonparametric statistics. Unfortunately, these statistical approaches do not allow insight into the pattern of variation of a given parameter over time. The purpose of this study was to determine if the application of dynamic factor analysis (DFA) allowed discriminating different patterns of power output (PO), during supramaximal exercise, in two groups of children engaged in competitive sports: swimmers and soccer players. Data derived from Wingate testing were used in this study. Analyses were performed on epochs (30 s) of upper and lower body PO obtained from twenty two healthy boys (11 swimmers and 11 soccer players) age 11-12 years old. DFA revealed two distinct patterns of PO during Wingate. Swimmers tended to attain their peak PO (upper and lower body) earlier than soccer players. As importantly, DFA showed that children with a given pattern of upper body PO tend to perform similarly during lower body exercise.     

Functional assessment of a multicenter very low-birth-weight cohort at age 5 years. Newborn Lung Project

BACKGROUND: Very low-birth-weight newborns (birth weight < or = 1500 g) experience serious neonatal complications, but long-term outcomes are not completely known. Most studies reflect an era of neonatal care that was fundamentally different from the present. OBJECTIVES: To compare the functional level of very low-birth-weight children before and after surfactant introduction and to relate functional level to clinical and socioeconomic factors. DESIGN: Inception cohort followed up from birth to an average age of 5 years. SETTING: Six regional neonatal intensive care units in a contiguous geographic area. PARTICIPANTS: Four hundred twenty-five very low-birth-weight children, born between August 1, 1988, and June 30, 1991, of 438 located among 626 whose parents provided follow-up information before neonatal intensive care unit discharge. INTERVENTIONS: None. MAIN OUTCOME MEASURES: Diagnosis of cerebral palsy and standardized scores for self-care, mobility, and social function from the Pediatric Evaluation of Disability Inventory. RESULTS: Cerebral palsy was present in 12.6% of the children, with no change after surfactant introduction. Intraventricular hemorrhage (odds ratio, 2.3 per grade; 95% confidence interval, 1.8-2.8) and bronchopulmonary dysplasia (odds ratio, 2.3; 95% confidence interval, 1.2-4.6) were independently predictive of cerebral palsy and of functional outcome. For self-care, mobility, and social function, 11.7%, 29.5%, and 10.7% of the children, respectively, scored at least 2 SDs below the normative means. Social function was 0.25 to 0.50 normative SDs lower after general surfactant availability than before general surfactant availability. Conclusions: While there was no increase in major disability after surfactant introduction, there may have been a decrease in social function associated with the lower neonatal mortality. Most very low-birth-weight children functioned within the normal range in everyday tasks. Several predictors of outcome were identified.     

Lower urinary tract dysfunction in cerebral palsy.

The clinical features and management of 27 children with cerebral palsy referred with symptoms of lower urinary tract dysfunction were reviewed. The mean age at referral was 9.9 years. Daytime urinary incontinence was the commonest presenting symptom. Videourodynamic studies were abnormal in 23 patients (85%). Only two children had evidence of upper renal tract damage. Treatment was determined by urodynamic findings, and led to improvement in symptoms in all patients for whom there was follow up information. Urinary incontinence may be improved or cured in children with cerebral palsy. These children would therefore benefit from early referral for assessment and treatment.     

Lower urinary tract dysfunction in cerebral palsy

The clinical features and management of 27 children with cerebral palsy referred with symptoms of lower urinary tract dysfunction were reviewed. The mean age at referral was 9.9 years. Daytime urinary incontinence was the commonest presenting symptom. Videourodynamic studies were abnormal in 23 patients (85%). Only two children had evidence of upper renal tract damage. Treatment was determined by urodynamic findings, and led to improvement in symptoms in all patients for whom there was follow up information. Urinary incontinence may be improved or cured in children with cerebral palsy. These children would therefore benefit from early referral for assessment and treatment.