Darcy Fehlings

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.

Practice Parameter: Pharmacologic treatment of spasticity in children and adolescents with cerebral palsy (an evidence-based review) Report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society

Objective: To evaluate published evidence of efficacy and safety of pharmacologic treatments for childhood spasticity due to cerebral palsy. Methods: A multidisciplinary panel systematically reviewed relevant literature from 1966 to July 2008. Results: For localized/segmental spasticity, botulinum toxin type A is established as an effective treatment to reduce spasticity in the upper and lower extremities. There is conflicting evidence regarding functional improvement. Botulinum toxin type A was found to be generally safe in children with cerebral palsy; however, the Food and Drug Administration is presently investigating isolated cases of generalized weakness resulting in poor outcomes. No studies that met criteria are available on the use of phenol, alcohol, or botulinum toxin type B injections. For generalized spasticity, diazepam is probably effective in reducing spasticity, but there are insufficient data on its effect on motor function and its side-effect profile. Tizanidine is possibly effective, but there are insufficient data on its effect on function and its side-effect profile. There were insufficient data on the use of dantrolene, oral baclofen, and intrathecal baclofen, and toxicity was frequently reported. Recommendations: For localized/segmental spasticity that warrants treatment, botulinum toxin type A should be offered as an effective and generally safe treatment (Level A). There are insufficient data to support or refute the use of phenol, alcohol, or botulinum toxin type B (Level U). For generalized spasticity that warrants treatment, diazepam should be considered for short-term treatment, with caution regarding toxicity (Level B), and tizanidine may be considered (Level C). There are insufficient data to support or refute use of dantrolene, oral baclofen, or continuous intrathecal baclofen (Level U).

Definition and Classification of Negative Motor Signs in Childhood

In this report we describe the outcome of a consensus meeting that occurred at the National Institutes of Health in Bethesda, Maryland, March 12 through 14, 2005. The meeting brought together 39 specialists from multiple clinical and research disciplines including developmental pediatrics, neurology, neurosurgery, orthopedic surgery, physical therapy, occupational therapy, physical medicine and rehabilitation, neurophysiology, muscle physiology, motor control, and biomechanics. The purpose of the meeting was to establish terminology and definitions for 4 aspects of motor disorders that occur in children: weakness, reduced selective motor control, ataxia, and deficits of praxis. The purpose of the definitions is to assist communication between clinicians, select homogeneous groups of children for clinical research trials, facilitate the development of rating scales to assess improvement or deterioration with time, and eventually to better match individual children with specific therapies. “Weakness” is defined as the inability to generate normal voluntary force in a muscle or normal voluntary torque about a joint. “Reduced selective motor control” is defined as the impaired ability to isolate the activation of muscles in a selected pattern in response to demands of a voluntary posture or movement. “Ataxia” is defined as an inability to generate a normal or expected voluntary movement trajectory that cannot be attributed to weakness or involuntary muscle activity about the affected joints. “Apraxia” is defined as an impairment in the ability to accomplish previously learned and performed complex motor actions that is not explained by ataxia, reduced selective motor control, weakness, or involuntary motor activity. “Developmental dyspraxia” is defined as a failure to have ever acquired the ability to perform age-appropriate complex motor actions that is not explained by the presence of inadequate demonstration or practice, ataxia, reduced selective motor control, weakness, or involuntary motor activity.

Reliability of the Tardieu Scale for assessing spasticity in children with cerebral palsy.

OBJECTIVE To measure the Tardieu Scales reliability in children with cerebral palsy (CP) when used by raters with and without experience in using the scale, before and after training. DESIGN Single-center, intrarater and interrater reliability study. SETTING Institutional ambulatory care. PARTICIPANTS Referred children with CP in the pretraining phase (n=5), during training (n=3), and in the posttraining phase (n=15). INTERVENTIONS The Tardieu Scale involves performing passive muscle stretch at 2 velocities, slow and fast. The rater derives 2 parameters; the Spasticity Angle X is the difference between the angles of arrest at slow speed and of catch-and-release or clonus at fast speed; the Spasticity Grade Y is an ordinal variable that grades the intensity (gain) of the muscle reaction to fast stretch. In phase 1, experienced raters without formalized training in the scale graded elbow, knee, and ankle plantar flexors bilaterally, without and with a goniometer. In phase 2, after training, the experienced and nonexperienced raters graded the same muscles unilaterally. MAIN OUTCOME MEASURES Intrarater and interrater reliability of the Tardieu Scale. RESULTS After training, nonexperienced raters had mean +/- SD intrarater and interrater agreement rates across all joints and parameters of 80%+/-14% and 74%+/-16%, respectively. For experienced raters, intrarater and interrater agreement rates before training were 77%+/-13% and 66%+/-15%, respectively, versus 90%+/-8% and 81%+/-13%, respectively, after training (P<.001 for both). Specific angle measurements at the knee were less reliable for the angles of catch measured at fast speed. Across all joints, agreement rates were similar using visual or goniometric measurements. CONCLUSIONS Both parameters of the Tardieu Scale have excellent intrarater and interrater reliability when assessed at the elbow and ankle joints of children with CP, with no difference noted between visual and goniometric measurements. Angle measurements were less reliable at the knee joints. Training was associated with a highly significant improvement in reliability.

Quality of life in spina bifida: importance of parental hope

BACKGROUND AND AIMS Prognosis in spina bifida (SB) is often based only on neurological deficits present at birth. We hypothesised that both parental hope and the neurophysical examination predict quality of life in children and adolescents with SB. METHODS A previously validated disease and age specific health related quality of life (HRQL) instrument was posted to families of children (aged 5–12 years) and adolescents (aged 13–20 years) with SB. We measured parental hope, determined the childs current physical function, and obtained retrospective data on the neonatal neurophysical examination (NPE). Regression analysis modelled HRQL firstly as a dependent variable on parental hope and NPE (“birth status”); and secondly on parental hope and current physical function (“current function”). RESULTS Response rates were 71% (137 of 194) for families of children, and 54% (74 of 138) for families of adolescents. NPE data were available for 121 children and 60 adolescents. In children, the birth status model predicted 26% of the variability (R2 hope 21%) compared with 23% of the variability (R2 hope 23%)in the adolescents. The current function model explained 47% of the variability (R2 hope 19%) in children compared with 31% of the variability (R2hope 24%) in the adolescents. CONCLUSIONS In both age groups, parental hope was more strongly associated with the HRQL than neonatal or current physical deficits. A prospective study is required to determine whether a causal relation exists between parental hope and HRQL of children and adolescents with SB.

How do changes in body functions and structures, activity, and participation relate in children with cerebral palsy?

Rehabilitation increasingly addresses the International Classification of Functioning, Disability and Healths (ICF) concepts of activity and participation, but little is known about associations between changes in body functions and structures, activity, and participation. We conducted a before‐and‐after study of 35 ambulatory children with spastic diplegia or hemiplegic cerebral palsy, mean age 5 years 6 months (SD 2y 2mo). Children were in Gross Motor Function Classification System (GMFCS) Levels I (n=11), II (n=12), or III (n=12). We assessed body functions and structures, activity, and participation at baseline and at 2 months and 6 months post‐botulinum toxin type A (BoNT‐A) injection. Repeated‐measures analysis of variance evaluated change, and linear regression assessed relationships. Baseline score relationships were moderate to strong but, despite similar directions of change at 2 months, change score relationships between measures of body functions and structures (spasticity and timed walk), activity (Gross Motor Function Measure and Pediatric Evaluation of Disability Inventory), and participation (Pediatric Outcomes Data Collection Instrument) at 2 months and 6 months were poor to fair (r<0.40). Predictor combinations accounted for <69% of variation in activity and participation change scores. Predictors often pertained to baseline score, GMFCS level, or age. Relationships between changes at different ICF levels are complex, and activity and participation gains post‐BoNT‐A are likely to be influenced by the child and environment factors.

Active Video Game Play in Children With Cerebral Palsy: Potential for Physical Activity Promotion and Rehabilitation Therapies

OBJECTIVE To evaluate the potential of active video game (AVG) play for physical activity promotion and rehabilitation therapies in children with cerebral palsy (CP) through a quantitative exploration of energy expenditure, muscle activation, and quality of movement. DESIGN Single-group, experimental study. SETTING Human movement laboratory in an urban rehabilitation hospital. PARTICIPANTS Children (N=17; mean age ± SD, 9.43±1.51y) with CP. INTERVENTION Participants played 4 AVGs (bowling, tennis, boxing, and a dance game). MAIN OUTCOME MEASURES Energy expenditure via a portable cardiopulmonary testing unit; upper limb muscle activations via single differential surface electrodes; upper limb kinematics via an optical motion capture system; and self-reported enjoyment via the Physical Activity Enjoyment Scale (PACES). RESULTS Moderate levels of physical activity were achieved during the dance (metabolic equivalent for task [MET]=3.20±1.04) and boxing (MET=3.36±1.50) games. Muscle activations did not exceed maximum voluntary exertions and were greatest for the boxing AVG and for the wrist extensor bundle. Angular velocities and accelerations were significantly larger in the dominant arm than in the hemiplegic arm during bilateral play. A high level of enjoyment was reported on the PACES (4.5±0.3 out of 5). CONCLUSIONS AVG play via a low-cost, commercially available system can offer an enjoyable opportunity for light to moderate physical activity in children with CP. While all games may encourage motor learning to some extent, AVGs can be strategically selected to address specific therapeutic goals (eg, targeted joints, bilateral limb use). Future research is needed to address the challenge of individual variability in movement patterns/play styles. Likewise, further study exploring home use of AVGs for physical activity promotion and rehabilitation therapies, and its functional outcomes, is warranted.

Cortical Reorganization After Modified Constraint-Induced Movement Therapy in Pediatric Hemiplegic Cerebral Palsy

Constraint-induced movement therapy improves motor function in the affected hand of children with hemiplegic cerebral palsy and results in cortical changes in adults with stroke. This study measured clinical improvement and cortical reorganization in a child with hemiplegia who underwent modified constraint-induced movement therapy for 3 weeks. Clinical, functional magnetic resonance imaging and magnetoencephalography measurements were done at baseline, after therapy, and 6 months after therapy. Modified constraint-induced movement therapy resulted in clinical improvement as measured by the Pediatric Motor Activity Log. Functional magnetic resonance imaging showed bilateral sensorimotor activation before and after therapy and a shift in the laterality index from ipsilateral to contralateral hemisphere after therapy. Magnetoencephalography showed increased cortical activation in the ipsilateral motor field and contralateral movement evoked field after therapy. Cortical reorganization was maintained at the 6-month follow-up. This is the first study to demonstrate cortical reorganization after any version of constraint-induced movement therapy in a child with hemiplegia.

Canadian stroke best practice recommendations: Stroke rehabilitation practice guidelines, update 2015:

Stroke rehabilitation is a progressive, dynamic, goal-orientated process aimed at enabling a person with impairment to reach their optimal physical, cognitive, emotional, communicative, social and/or functional activity level. After a stroke, patients often continue to require rehabilitation for persistent deficits related to spasticity, upper and lower extremity dysfunction, shoulder and central pain, mobility/gait, dysphagia, vision, and communication. Each year in Canada 62,000 people experience a stroke. Among stroke survivors, over 6500 individuals access in-patient stroke rehabilitation and stay a median of 30 days (inter-quartile range 19 to 45 days). The 2015 update of the Canadian Stroke Best Practice Recommendations: Stroke Rehabilitation Practice Guidelines is a comprehensive summary of current evidence-based recommendations for all members of multidisciplinary teams working in a range of settings, who provide care to patients following stroke. These recommendations have been developed to address both the organization of stroke rehabilitation within a system of care (i.e., Initial Rehabilitation Assessment; Stroke Rehabilitation Units; Stroke Rehabilitation Teams; Delivery; Outpatient and Community-Based Rehabilitation), and specific interventions and management in stroke recovery and direct clinical care (i.e., Upper Extremity Dysfunction; Lower Extremity Dysfunction; Dysphagia and Malnutrition; Visual-Perceptual Deficits; Central Pain; Communication; Life Roles). In addition, stroke happens at any age, and therefore a new section has been added to the 2015 update to highlight components of stroke rehabilitation for children who have experienced a stroke, either prenatally, as a newborn, or during childhood. All recommendations have been assigned a level of evidence which reflects the strength and quality of current research evidence available to support the recommendation. The updated Rehabilitation Clinical Practice Guidelines feature several additions that reflect new research areas and stronger evidence for already existing recommendations. It is anticipated that these guidelines will provide direction and standardization for patients, families/caregiver(s), and clinicians within Canada and internationally.

Designing action-based exergames for children with cerebral palsy

Children with cerebral palsy (CP) want to play fast-paced action-oriented videogames similar to those played by their peers without motor disabilities. This is particularly true of exergames, whose physically-active gameplay matches the fast pace of action games. But disabilities resulting from CP can make it difficult to play action games. Guidelines for developing games for people with motor disabilities steer away from high-paced action, including recommendations to avoid the need for time-sensitive actions and to keep game pace slow. Through a year-long participatory design process with children with CP, we have discovered that it is in fact possible to develop action-oriented exergames for children with CP at level III on the Gross Motor Function Classification Scale. We followed up the design process with an eight-week home trial, in which we found the games to be playable and enjoyable. In this paper, we discuss the design of these games, and present a set of design recommendations for how to achieve both action-orientation and playability.