Jessica Rose

Promotion of Physical Fitness and Prevention of Secondary Conditions for Children With Cerebral Palsy: Section on Pediatrics Research Summit Proceedings

Inadequate physical fitness is a major problem affecting the function and health of children with cerebral palsy (CP). Lack of optimal physical activity may contribute to the development of secondary conditions associated with CP such as chronic pain, fatigue, and osteoporosis. The purpose of this article is to highlight the content and recommendations of a Pediatrics Research Summit developed to foster collaborative research in this area. Two components of physical fitness—muscle strength and cardiorespiratory fitness—were emphasized. Although there is evidence to support the use of physical fitness interventions, there are many gaps in our current knowledge. Additional research of higher quality and rigor is needed in order to make definitive recommendations regarding the mode, intensity, frequency, and duration of exercise. Outcome measurements have focused on the body functions and structures level of the International Classification of Functioning, Disability and Health (ICF), and much less is known about effects at the activities and participation levels. Additionally, the influence of nutritional and growth factors on physical fitness has not been studied in this population, in which poor growth and skeletal fragility have been identified as serious health issues. Current intervention protocols and outcome measurements were critically evaluated, and recommendations were made for future research.

DEFINITION AND CLASSIFICATION OF HYPERKINETIC MOVEMENTS IN CHILDHOOD

Hyperkinetic movements are unwanted or excess movements that are frequently seen in children with neurologic disorders. They are an important clinical finding with significant implications for diagnosis and treatment. However, the lack of agreement on standard terminology and definitions interferes with clinical treatment and research. We describe definitions of dystonia, chorea, athetosis, myoclonus, tremor, tics, and stereotypies that arose from a consensus meeting in June 2008 of specialists from different clinical and basic science fields. Dystonia is a movement disorder in which involuntary sustained or intermittent muscle contractions cause twisting and repetitive movements, abnormal postures, or both. Chorea is an ongoing random‐appearing sequence of one or more discrete involuntary movements or movement fragments. Athetosis is a slow, continuous, involuntary writhing movement that prevents maintenance of a stable posture. Myoclonus is a sequence of repeated, often nonrhythmic, brief shock‐like jerks due to sudden involuntary contraction or relaxation of one or more muscles. Tremor is a rhythmic back‐and‐forth or oscillating involuntary movement about a joint axis. Tics are repeated, individually recognizable, intermittent movements or movement fragments that are almost always briefly suppressible and are usually associated with awareness of an urge to perform the movement. Stereotypies are repetitive, simple movements that can be voluntarily suppressed. We provide recommended techniques for clinical examination and suggestions for differentiating between the different types of hyperkinetic movements, noting that there may be overlap between conditions. These definitions and the diagnostic recommendations are intended to be reliable and useful for clinical practice, communication between clinicians and researchers, and for the design of quantitative tests that will guide and assess the outcome of future clinical trials.

Energy expenditure index of walking for normal children and for children with cerebral palsy.

Energy expenditure indices (EEI) based on oxygen uptake and heart rate were used to compare the economy of walking at various speeds by normal and cerebral‐palsied children. At low walking speeds, EEI values were high, indicating poor economy. At higher speeds the EEI values decreased until a range of maximum economy was reached. For normal children who were capable of walking beyond this range at higher speeds, the EEI increased again. This pattern was noted for both oxygen‐uptake and heart‐rate indices. Mean EEI values based on oxygen uptake and heart rate for normal children were significantly lower and occurred at faster walking speeds than values for children with cerebral palsy. EEI based on either oxygen uptake or heart rate can be used clinically to provide objective information to help evaluate the influence on gait function of surgical intervention, ambulatory aids or orthotics.

Neuromuscular activation and motor-unit firing characteristics in cerebral palsy

Muscle strength, neuromuscular activation, and motor-unit firing characteristics (firing rate, recruitment, and short-term synchronization) were assessed during voluntary contractions of the medial gastrocnemius (GAS) and tibialis anterior (TA) muscles of 10 participants with spastic diplegic or hemiplegic cerebral palsy (CP). The participants (six females, four males; age range 6 to 37y) walked with equinus gait at Gross Motor Function Classification System levels II to III. These were compared with 10 age-matched controls (five females; age range 7 to 35y). Neuromuscular activation was estimated by the ratio of surface electromyogram amplitude to M-wave amplitude elicited by supramaximal electrical nerve stimulation. Participants with CP produced significantly less torque (normalized by leg length) compared with controls (TA: mean 2.3, SD 1.6 vs mean 8.9, SD 3.4Nm/m; GAS mean 13.7, SD 7.1 vs mean 28.6, SD 5.1Nm/m, p < 0.001). Neuromuscular activation during maximum voluntary contraction was significantly reduced in the participants with CP compared with controls (mean 2.4, SD 1.5 vs mean 9.7, SD 2.7Nm/m for TA; mean 1.04, SD 0.41 vs mean 3.1, SD 1.2Nm/m for GAS, p < 0.001). When compared at the same submaximal level of neuromuscular activation, motor-unit recruitment and firing rates were not different between the groups, although short-term synchronization in TA was reduced in the participants with CP. These data indicate that weakness, known to be an important component of the motor deficit in CP, has a strong central component. Although the relation between recruitment and firing rate remained substantially intact at the low and moderate force contractions tested, results suggest that the participants with CP were unable to recruit higher threshold motor units or to drive lower threshold motor units to higher firing rates.

Postural balance in children with cerebral palsy

Postural control deficits have been suggested to be a major component of gait disorders in cerebral palsy (CP). Standing balance was investigated in 23 ambulatory children and adolescents with spastic diplegic CP, ages 5 to 18 years, and compared with values of 92 children without disability, ages 5 to 18 years, while they stood on a force plate with eyes open or eyes closed. The measurements included center of pressure calculations of path length per second, average radial displacement, mean frequency of sway, and Brownian random motion measures of the short-term diffusion coefficient, and the long-term scaling exponent. In the majority of children with CP (14 of 23) all standing balance values were normal. However, approximately one-third of the children with CP (eight of 23) had abnormal values in at least two of the six center of pressure measures. Thus, mean values for path length, average radial displacement, and diffusion coefficient were higher for participants with CP compared with control individuals with eyes open and closed (p<0.05). Mean values for frequency of sway and the long-term scaling exponent were lower for participants with CP compared with control participants (p<0.05). Increased average radial displacement was the most common (nine of 23) postural control deficit. There was no increase in abnormal values with eyes closed compared with eyes open for participants with CP, indicating that most participants with CP had normal dependence on visual feedback to maintain balance. Identification of those children with impaired standing balance can delineate factors that contribute to the patients gait disorder and help to guide treatment.

Energy cost of walking in normal children and in those with cerebral palsy: comparison of heart rate and oxygen uptake.

The rate of oxygen uptake can be used to assess energy expenditure during walking, but the necessary intrumentation is cumbersome, expensive, and usually unavailable in the clinical setting. Heart rate is an easily measured parameter, but its use as an index of energy expenditure in children has not been validated previously. We found that the relationship between oxygen uptake and heart rate was linear throughout a wide range of walking speeds for both children with cerebral palsy and normal children. There was no significant difference between the slope or the y-intercept of the lines for the two groups. These findings validate the use of heart rate as an index of energy expenditure for normal children and for children with cerebral palsy.

The energy expenditure index : a method to quantitate and compare walking energy expenditure for children and adolescents

We used heart rate and walking speed to calculate an energy expenditure index (EEI), the ratio of heart rate per meter walked, for 102 normal subjects, age 6-18 years. Heart rate was measured at self-selected slow, comfortable, and fast walking speeds on the floor and on a motor-driven treadmill. At slow walking speeds (37 +/- 10 m/min) the EEI was elevated (0.71 +/- 0.32 beats/m), indicating poor economy. At comfortable speeds (70 +/- 11 m/min) the EEI values decreased to the maximum economy (0.47 +/- 0.13 beats/m). At fast speeds (101 +/- 13 m/min), the EEI increased (0.61 +/- 0.17 beats/m), indicating poor economy relative to comfortable speeds. A graph of the EEI versus walking speed provides a way to evaluate and compare energy expenditure in a clinical setting.

Neonatal brain structure on MRI and diffusion tensor imaging, sex, and neurodevelopment in very‐low‐birthweight preterm children

The neurological basis of an increased incidence of cerebral palsy (CP) in preterm males is unknown. This study examined neonatal brain structure on magnetic resonance imaging (MRI) and diffusion tensor imaging (DTI) at term‐equivalent age, sex, and neurodevelopment at 1 year 6 months on the basis of the Amiel−Tison neurological examination, Gross Motor Function Classification System, and Bayley Scales of Infant Development in 78 very‐low‐birthweight preterm children (41 males, 37 females; mean gestational age 27.6wks, SD 2.5; mean birthweight 1021g, SD 339). Brain abnormalities on MRI and DTI were not different between males and females except in the splenium of the corpus callosum, where males had lower DTI fractional anisotropy (p=0.025) and a higher apparent diffusion coefficient (p=0.013), indicating delayed splenium development. In the 26 infants who were at higher risk on the basis of DTI, males had more abnormalities on MRI (p=0.034) and had lower fractional anisotropy and a higher apparent diffusion coefficient in the splenium (p=0.049; p=0.025) and right posterior limb of the internal capsule (PLIC; p=0.003; p=0.033). Abnormal neurodevelopment was more common in males (n=9) than in females (n=2; p=0.036). Children with abnormal neurodevelopment had more abnormalities on MRI (p=0.014) and reduced splenium and right PLIC fractional anisotropy (p=0.001; p=0.035). In children with abnormal neurodevelopment, right PLIC fractional anisotropy was lower than left (p=0.035), whereas in those with normal neurodevelopment right PLIC fractional anisotropy was higher than left (p=0.001). Right PLIC fractional anisotropy correlated to neurodevelopment (rho=0.371, p=0.002). Logistic regression predicted neurodevelopment with 94% accuracy; only right PLIC fractional anisotropy was a significant logistic coefficient. Results indicate that the higher incidence of abnormal neurodevelopment in preterm males relates to greater incidence and severity of brain abnormalities, including reduced PLIC and splenium development.

ENERGY COST INDEX AS AN ESTIMATE OF ENERGY EXPENDITURE OF CEREBRAL-PALSIED CHILDREN DURING ASSISTED AMBULATION

The energy expenditure of cerebral‐palsied children ambulating with bilateral quad canes and wheeled walkers was estimated, using average heart rate and speed of walking. An energy cost index (average number of heart rates per unit distance walked) was derived to allow for varying speeds among patients. Children were then categorized into those who had a lower energy cost index when using quad canes and those who had a lower index when using walkers. This made it possible to offer treatment recommendations based on objective data.

Postural sway reduction in aging men and women: relation to brain structure, cognitive status, and stabilizing factors.

Postural stability becomes compromised with advancing age, but the neural mechanisms contributing to instability have not been fully explicated. Accordingly, this quantitative physiological and MRI study of sex differences across the adult age range examined the association between components of postural control and the integrity of brain structure and function under different conditions of sensory input and stance stabilization manipulation. The groups comprised 28 healthy men (age 30-73 years) and 38 healthy women (age 34-74 years), who completed balance platform testing, cognitive assessment, and structural MRI. The results supported the hypothesis that excessive postural sway would be greater in older than younger healthy individuals when standing without sensory or stance aids, and that introduction of such aids would reduce sway in both principal directions (anterior-posterior and medial-lateral) and in both the open-loop and closed-loop components of postural control even in older individuals. Sway reduction with stance stabilization, that is, standing with feet apart, was greater in men than women, probably because older men were less stable than women when standing with their feet together. Greater sway was related to evidence for greater brain structural involutional changes, indexed as ventricular and sulcal enlargement and white matter hyperintensity burden. In women, poorer cognitive test performance related to less sway reduction with the use of sensory aids. Thus, aging men and women were shown to have diminished postural control, associated with cognitive and brain structural involution, in unstable stance conditions and with diminished sensory input.