Remo Nunzio Russo

Upper-limb botulinum toxin A injection and occupational therapy in children with hemiplegic cerebral palsy identified from a population register: a single-blind, randomized, controlled trial

OBJECTIVES. The purpose of this work was to assess the effect of botulinum toxin A and occupational therapy compared with occupational therapy alone on body structure, activities participation, and self-perception in a sample of children (aged 3–16 years) with hemiplegic cerebral palsy recruited from a statewide register. PATIENTS AND METHODS. Participants of this single-blind, randomized, controlled trial identified from a population-based cerebral palsy register received either an individually prescribed and localized injection of botulinum toxin A with 4 sessions of occupational therapy over 4 weeks (intervention) or occupational therapy alone (control). Outcomes were assessed from 2 domains of the World Health Organization International Classification of Functioning, Disability, and Health: body structure (Modified Ashworth Scale and Tardieu Scale) and activities participation (Assessment of Motor and Process Skills, Goal Attainment Scale, Pediatric Evaluation of Disability Inventory, and Pediatric Quality of Life Inventory). Self-perception was also measured. RESULTS. All of the participants (intervention: n = 21; control: n = 22) provided data at baseline and 3 and 6 months. Mean age was 8.6 years; 23 were boys and 20 were girls. At 3 months, children allocated to receive the intervention performed significantly better in terms of body structure and activities participation. They reported improvements in self-perception for the global self-worth domain. At 6 months, the differences between the intervention and control groups persisted for the measures of body structure but not for activities participation or self-perception. CONCLUSION. Botulinum toxin A injection combined with a low-intensity occupational therapy program achieves significant improvements in body structure, activity participation, and self-perception.

Botulinum toxin assessment, intervention and follow‐up for paediatric upper limb hypertonicity: international consensus statement

The primary objective of this paper was to evaluate the published evidence of efficacy and safety of botulinum neurotoxin (BoNT) injections in paediatric upper limb hypertonia (PULH). Secondary objectives included the provision of clinical context, based on evidence and expert opinion, in the areas of assessment, child and muscle selection, dosing, and adjunctive treatment. A multidisciplinary panel of authors systematically reviewed, abstracted, and classified relevant literature. Recommendations were based on the American Academy of Neurology (AAN) evidence classification. Following a literature search, 186 potential articles were screened for inclusion, and 15 of these met the criteria and were reviewed. Grade A evidence was found to support the use of BoNT to reach individualized therapeutic goals for PULH. There is grade B evidence (probably effective) for tone reduction following BoNT injections and grade U evidence (inconclusive) for improvement in upper limb (UL) activity and function. BoNT injections were generally found to be safe and well tolerated with the most common side effect identified as a transient decrease in grip strength.

Self-esteem, self-concept, and quality of life in children with hemiplegic cerebral palsy.

OBJECTIVES To investigate self-esteem, self-concept and quality of life in children with hemiplegic cerebral palsy (HCP) compared with typically developing peers. STUDY DESIGN Cross-sectional evaluation of 86 children (3-16 years; 54 boys; mean age 9.4 +/- 3.7 years) with HCP and age and sex-matched peers. Self-esteem/concept was measured with the Self-Perception Profile for Children (age 8-16; n = 55 pairs) and the Pictorial Scale of Perceived Competence and Social Acceptance for Young Children (age 3-7 years; n = 31 pairs). Quality of life was measured with the Pediatric Quality of Life Inventory, version 4. RESULTS Significant differences in mean scores ([95%CI] P < .05) favoring the peer group were found for physical competence (HCP 2.8 [2.5, 3.0]; peer 3.2 [3.1, 3.3]), athletic competence (HCP 2.7 [2.5, 2.9]; peer 3.1 [3.0, 3.3]), and scholastic competence (HCP 2.8 [2.6, 3.0]; peer 3.1 [3.0, 3.3]), but favored children with HCP for maternal acceptance (HCP 3.1 [2.9, 3.3]; peer 2.7 [2.5, 3.0]). Quality of life was significantly higher for the peer group for both parent (HCP 54.5 [51.1, 58.0]; peer 80.6 [78.3, 82.9]) and child (HCP 67.6 [62.7, 72.6]; peer 80.6 [78.1, 83.1]) scales. CONCLUSIONS Children with HCP experience reduced quality of life and self-concept compared with typically developing peers.

Pain characteristics and their association with quality of life and self-concept in children with hemiplegic cerebral palsy identified from a population register.

ObjectivesTo describe and characterize the prevalence and quality of pain in a population-based community sample of children with hemiplegic cerebral palsy. MethodsOutcomes were assessed from 2 domains of the World Health Organization International Classification of Functioning, Disability and Health: body structure/function (upper limb spans, modified Ashworth scale, Tardieu scale, sensory function), activities-participation (Assessment of Motor and Process Skills), Pediatric Quality of Life Inventory (PedsQL), and self-perception (Harter Self-Perception Profile). ResultsThere were 107 participants of age (mean, 95% confidence interval) 8.94 years (8.23 to 9.65); 61 (57%) were boys and 58 (54%) had hemiplegia affecting the right side. Fifty-one (48%) reported pain. Pain mostly affected the involved side (n=28, 55%) and lower limb (n=19, 37%). Eighteen (35%) reported moderate to severe pain. Thirty-five (69%) said pain impacted on movement/activity and was of an aching quality (n=29, 57%). Mean (95% confidence interval) quality of life according to Pediatric Quality of Life Inventory was significantly lower for children experiencing pain compared with children experiencing no pain [parent 50.2 (45.9 to 54.5) vs. 60.1 (55.1 to 65.1), P<0.01; child 60.5 (55.4 to 65.6) vs. 75.8 (68.4 to 83.2), P<0.01]. The self-perception domains of Scholastic Competence [no pain, 3.02 (2.78 to 3.26); pain, 2.55 (2.31 to 2.79) P<0.01] and Behavioral Competence [no pain, 3.33 (3.07 to 3.60); pain, 2.88 (2.70 to 3.06) P<0.01] were significantly lower in children with pain aged ≥8 years. DiscussionPain is common in children with hemiplegic cerebral palsy with qualities suggesting a nociceptive origin. Pain is associated with lower quality of life and self-perception. Results suggest clinicians should assess and actively manage pain in this population.

The effectiveness of robotic-assisted gait training for paediatric gait disorders: systematic review

BackgroundRobotic-assisted gait training (RAGT) affords an opportunity to increase walking practice with mechanical assistance from robotic devices, rather than therapists, where the child may not be able to generate a sufficient or correct motion with enough repetitions to promote improvement. However the devices are expensive and clinicians and families need to understand if the approach is worthwhile for their children, and how it may be best delivered.MethodsThe objective of this review was to identify and appraise the existing evidence for the effectiveness of RAGT for paediatric gait disorders, including modes of delivery and potential benefit. Six databases were searched from 1980 to October 2016, using relevant search terms. Any clinical trial that evaluated a clinical aspect of RAGT for children/adolescents with altered gait was selected for inclusion. Data were extracted following the PRISMA approach. Seventeen trials were identified, assessed for level of evidence and risk of bias, and appropriate data extracted for reporting.ResultsThree randomized controlled trials were identified, with the remainder of lower level design. Most individual trials reported some positive benefits for RAGT with children with cerebral palsy (CP), on activity parameters such as standing ability, walking speed and distance. However a meta-analysis of the two eligible RCTs did not confirm this finding (p = 0.72). Training schedules were highly variable in duration and frequency and adverse events were either not reported or were minimal. There was a paucity of evidence for diagnoses other than CP.ConclusionThere is weak and inconsistent evidence regarding the use of RAGT for children with gait disorders. If clinicians (and their clients) choose to use RAGT, they should monitor individual progress closely with appropriate outcome measures including monitoring of adverse events. Further research is required using higher level trial design, increased numbers, in specific populations and with relevant outcome measures to both confirm effectiveness and clarify training schedules.

CP or Not CP? A Review of Diagnoses in a Cerebral Palsy Register

The purpose of this study was to document the inaccuracy rate of diagnosis of cerebral palsy recorded on the South Australian Cerebral Palsy Register. A total of 402 children born in South Australia from 1993 to 2002 and notified to the Register as having cerebral palsy were identified through the Register database, and 21 children (5.2%) were later identified to have a noncerebral palsy diagnosis. Of these, 5 had either a metabolic or a neurodegenerative disorder and 2 had a syndromic disorder (1 Joubert syndrome and 1 Sotos syndrome); the remaining 14 children had one of the following final diagnoses: developmental delay, gross motor delay, perinatal myositis, spinal subdural and subarachnoid arteriovenous malformation, and Erbs palsy. In 16 of 21 children (76%), the diagnosis was changed at 5 years of age or older. Studies based on population registers may need to take into account the possibility of misclassification, estimated to be at least 5.2% in this study. A complete clinical assessment at the time of diagnosis followed by regular reassessment would enable the clinician to exclude children with alternative diagnoses, which has important implications for clinical management and research based on cerebral palsy registers.

Motor function in 5-year-old children with cerebral palsy in the South Australian population.

The aim of this study was to describe the motor function of a population of children at age 5 years enrolled on the South Australian Cerebral Palsy Register. Among children born between 1993 and 1998, there were 333 with confirmed cerebral palsy (prevalence rate 2.2 per 1000 live births), in whom 247 assessments (56.7% males, 43.3% females) were completed. The distribution by Gross Motor Function Classification System (GMFCS) level was: level I, 50.6%; level II, 18.2%; level III, 9.3%; level IV, 9.7%; level V, 12.1%. The most common topographical classification was spastic diplegia (38.5%), followed by spastic hemiplegia (34.8%) and spastic quadriplegia (14.6%). Abnormal movements occurred at rest or with intention in 19.4% of children. A high proportion of the population with relatively mild gross motor impairments have difficulty with everyday bimanual tasks, reinforcing the need to assess upper limb function independently of gross motor function. The use of ankle–foot orthoses was common, particularly across GMFCS levels II to IV. Further refinement is indicated for this population’s motor dataset, to include more recently described classification measures as well as future novel measures to better describe the presence of both spasticity and dystonia.

Determination of interventions for upper extremity tactile impairment in children with cerebral palsy: a systematic review

This study reviewed interventions suitable for treating tactile dysfunction in children with cerebral palsy (CP).

Upper limb orthoses and assistive technology utilization in children with hemiplegic cerebral palsy recruited from a population register

Objective: To describe the use of upper limb orthoses and assistive technologies (AT) in a representative community-based sample of children with hemiplegic cerebral palsy (HCP) and compare children on the basis of their prescription. Method: Survey of all children with HCP entered on a population register. Outcome measures included a clinical examination; Assessment of Motor and Process skills; Self-perception Profile for Children; PedsQL version 4; Paediatric Evaluation of Disability Inventory. Results: Of 107 respondents, 56% were prescribed upper limb orthoses and 46% AT; 48% of children prescribed upper limb orthoses were using them, compared to 98% of children prescribed AT. Greater tone at the wrist was associated with orthotic prescription (OR [95% CI] 39.6 [4.8, 323]). Conclusion: Children with HCP prescribed orthoses and AT are a more severely affected group. AT have higher utility than upper limb orthoses in children with HCP, with orthoses having a high rate of abandonment.

Identification and measurement of dystonia in cerebral palsy

To establish the prevalence and severity of dystonia in a population of children with cerebral palsy (CP) with hypertonia assessment and measurement tools.