Thickened saliva after effective management of drooling with botulinum toxin A

Aim The aim of this study was to evaluate the rheological properties of saliva after submandibular botulinum toxin type A (BoNT‐A) injections. Method We enrolled 15 children (11 males and six females; age range 3–17y, mean age 9y 10mo) diagnosed with spastic (n=9) or dyskinetic (n=6) quadriplegic cerebral palsy (CP); Gross Motor Function Classification System level IV or V; and two children with intellectual disability (IQ <70) who experienced moderate to severe drooling. Salivary flow rate and drooling quotient were measured at baseline and at different times after BoNT‐A injections up to 24 weeks. The mucin concentration of saliva was analysed before and after BoNT‐A treatment. Results Both submandibular salivary flow rate (baseline 0.38mL/min; 24wks after injection 0.26mL/min) and drooling quotient (baseline 42.5%; 24wks 28.80%) were substantially reduced, with a concomitant increase in mucin concentration within 8 weeks after BoNT‐A injection (from 0.612 to 1.830U/mL). The parents of nine children observed thickened saliva. Swallowing and chewing were problematic in seven children. Two of these children needed treatment with mucolytics because of pooling of thickened saliva in the throat. Interpretation When making decisions about the use of BoNT‐A, the risk of problems with masticatory and swallowing functions as a result of thickening of saliva after BoNT‐A treatment should be taken into account.     

Determinants of responsiveness to botulinum toxin,casting, and bracing in the treatment of spastic equinus in children with cerebral palsy

Aim The objective was to determine whether specific intrinsic (age, pattern of cerebral palsy [CP], child’s motivation) and extrinsic (number of treatments, parenting stress) characteristics were associated with responsiveness to botulinum toxin A (BoNT‐A) injections in children with CP 3 months after injection into the gastrocnemius muscle. Method Children with hemiplegia or diplegia recruited from a BoNT‐A programme were evaluated before and 3 months following injection of BoNT‐A into the gastrocnemius. Outcome measures included muscle tone, range of motion, gait pattern, level of ambulation, gross motor function, and functional independence. Determinants of responsiveness to BoNT‐A considered were age, number of treatments, distribution of CP, parenting stress, and motivation. Results Thirty‐one children were recruited (17 males, 14 females) – 22 with hemiplegia and nine with diplegia. Twenty‐eight were classified at Gross Motor Function Classification System (GMFCS) level I and three at level III. The mean age was 6 years 4 months (SD 2y 11mo). Younger age (p=0.015) and fewer number (p=0.024) of BoNT‐A treatments were associated with greater change in gross motor function. Child’s motivation and parenting stress were significantly associated with improvements in muscle tone (p=0.006–0.017), passive range of motion (p=0.008–0.033), gait pattern (p=0.005–0.042), level of ambulation (p=0.001–0.043), and functional independence (p=0.004–0.027). Interpretation The results indicate that child, family, and treatment characteristics influence the degree of responsiveness to BoNT‐A treatment. The contribution of contextual factors (personal and environmental) on responsiveness may be underappreciated in children with CP.     

The impact of botulinum toxin A and abduction bracing on long-term hip development in children with cerebral palsy

Aim To study the long‐term impact of 3 years of botulinum toxin A (BoNT‐A) injections and abduction bracing on hip development in children with bilateral spastic cerebral palsy (CP). We wanted to know if early treatment improved hip development and reduced the need for surgery. Method A long‐term review of hip morphology and surgery requirements in children who participated in a multicentre, randomized controlled trial. The trial investigated short‐term effects of BoNT‐A injections combined with an abduction brace, compared with usual care, on hip displacement in children with bilateral spastic CP. Results Forty‐six children with bilateral spastic CP (31 males, 15 females; 10 with diplegia, 36 with quadriplegia; mean age at enrolment of 3y 2mo, mean age at most recent clinical review 13y 11mo [range 10y 6mo–16y 8mo]; three children in Gross Motor Function Classification System level II, 11 in level III, 20 in level IV, 12 in level V) were followed for a mean of 10 years 10 months from recruitment to the trial. Mean migration percentage was 15.9% in the BoNT‐A group and 15.2% in the comparison group (t=0.26, p=0.79). Eighty‐nine percent of hips in the treatment group and 91% hips in the comparison group had satisfactory development, using a valid scale (Mann–Whitney U test=867.50, z=?1.59, p=0.11). Forty children had preventive surgery (21 treatment group, 19 comparison group) and 18 children had reconstructive surgery (10 treatment, 8 comparison). Interpretation In children with bilateral spastic CP, early treatment with BoNT‐A and hip abduction bracing does not reduce the need for surgery or improve hip development at skeletal maturity.     

Oromotor dysfunction and communication impairments in children with cerebral palsy: a register study

Aim To report the prevalence, clinical associations, and trends over time of oromotor dysfunction and communication impairments in children with cerebral palsy (CP). Method Multiple sources of ascertainment were used and children followed up with a standardized assessment including motor speech problems, swallowing/chewing difficulties, excessive drooling, and communication impairments at age 5 years. Results A total of 1357 children born between 1980 and 2001 were studied (781 males, 576 females; median age 5y 11mo, interquartile range 3–9y; unilateral spastic CP, n=447; bilateral spastic CP, n=496; other, n=112; Gross Motor Function Classification System [GMFCS] level: I, 181; II, 563; III, 123; IV, 82; IV, 276). Of those with ‘early‐onset’ CP (n=1268), 36% had motor speech problems, 21% had swallowing/chewing difficulties, 22% had excessive drooling, and 42% had communication impairments (excluding articulation defects). All impairments were significantly related to poorer gross motor function and intellectual impairment. In addition, motor speech problems were related to clinical subtype; swallowing/chewing problems and communication impairments to early mortality; and communication impairments to the presence of seizures. Of those with CP in GMFCS levels IV to V, a significant proportion showed a decline in the rate of motor speech impairment (p=0.008) and excessive drooling (p=0.009) over time. Interpretation These impairments are common in children with CP and are associated with poorer gross motor function and intellectual impairment.     

Botulinum toxin A versus B in sialorrhea: A prospective,randomized, double‐blind,crossover pilot study in patients with amyotrophic lateral sclerosis or Parkinson's disease

Background: Either botulinum toxins (BoNTs) A and B have been used for improving drooling in different neurological conditions. Methods: Consecutive patients affected by Amyotrophic Lateral Sclerosis (ALS) or Parkinson's Disease (PD) accompanied by severe drooling were randomized to receive botulinum neurotoxin type A (BoNT‐A) or B (BoNT‐B) injections into the salivary glands. Following the first treatment, when sialorrhea returned to baseline (at least three months after the first injection), subjects were re‐treated with the other serotype. Ultrasound‐guided injections into parotid and submandibular glands were bilaterally performed: total doses were 250 U BoNT‐A (Dysport) and 2500 U BoNT‐B (Neurobloc). Objective (cotton roll weight) and subjective (ad hoc clinical scales) evaluations were performed at baseline, after 1 and 4 weeks, and every 4 weeks until drooling returned to baseline. Results: Twenty‐seven patients (15 ALS and 12 PD) were enrolled, fourteen completed the study. BoNT‐A and BoNT‐B treatments gave both subjective and objective improvements in all patients. The latency was significantly shorter after BoNT‐B treatments (3.2 ± 3.7 days) compared to BoNT‐A (6.6 ± 4.1 days; P = 0.002). The mean benefit duration was similar at 75 and 90 days for BoNT‐A and BoNT‐B, respectively (P = NS). The only toxin‐related side effect was a change to saliva thickness. Conclusions: Either 250 U Dysport or 2500 U Neurobloc have similar effectiveness and safety in controlling sialorrhea. BoNT‐B has a shorter latency and comparable duration. Cost analysis, considering the doses used in this study protocol favored BoNT‐B treatment. © 2011 Movement Disorder Society     

Effectiveness of sedation using nitrous oxide compared with enteral midazolam for botulinum toxin A injections in children

This randomized, double‐blind, placebo‐controlled study compared the efficacy of inhaled nitrous oxide (N₂O) with enteral midazolam for sedation of children with cerebral palsy (CP) undergoing botulinum toxin A (BoNT‐A) injections. Fifty children (29 males, 21 females; mean age 8y 2mo [SD 4y 5mo]; range 1–16y) were randomized to sedation with N₂O (n=25) or midazolam (n=25). Groups were similar in type of CP (diplegia, 11; triplegia, three; quadriplegia, 16; hemiplegia, 16; other, three) and Gross Motor Function Classification System level (Level I, 4; II, 24; III, 4; IV, 13; V, 5). Both groups were equally sedated at time of injection (p=0.661), but those in the midazolam group were more sedated at time of discharge (p<0.001). N₂O was more effective in reducing pain compared with midazolam as measured using the Face, Legs, Activity, Cry, Consolability (FLACC) scale (p=0.010), parental estimate of pain (p=0.009), and nursing estimate of pain (p=0.007). Parents in the N₂O group rated it better than prior sedation with midazolam for BoNT‐A injections (p=0.031). Physicians and nurses reported no difference in ease of procedure between the groups. One child in the midazolam group and eight in the N₂O group had adverse effects, all of which resolved promptly. N₂O appears to be an effective means of sedation for children undergoing outpatient BoNT‐A injections.     

Botulinum toxin type B for sialorrhoea in children with cerebral palsy: a randomized trial comparing three doses

Aim The aim of the present study was to evaluate the efficacy and safety of three doses of botulinum toxin type B (BoNT‐B) in reducing persistent sialorrhoea in children with cerebral palsy (CP). Method Children with CP and refractory sialorrhoea were randomized to one of four groups: a control group and three experimental groups receiving a low (1500 mouse units [MU]), medium (3000MU), or high (5000MU) dose of BoNT‐B respectively, into bilateral salivary glands. Drooling was measured using the Thomas‐Stonell rating scale, and the weight and the number of bibs used per day were counted in all children at baseline, 4, and 12 weeks after BoNT‐B injection. Results Twenty‐seven children (15 males, 12 females; mean age 7y 10mo, SD 1y 6mo; range 5–15y) were randomized into a control (seven children: four males, three females) and experimental groups receiving low (six children: four males, two females), medium (seven children: four males, three females), and high (seven children: three males, four females) doses of BoNT‐B respectively. All children had mixed neurological disorders consisting of spastic paraparesis, tetraparesis, dystonic movements, and ataxia. Gross Motor Function Classification System levels ranged from III to V, and all children had moderate or severe intellectual disability. Estimated means with their standard errors (SEM) of drooling were at baseline, 4, and 12 weeks respectively, as follows: control group, 12.1 (2.1), 11.9 (2.1), 11.8 (2.2), p for trend 0.992; low dose group, 13.8 (2.3), 11.4 (2.3), 13.9 (2.3), p for trend 0.952; medium dose group, 13.9 (2.1), 6.7 (2.1), 7.1 (2.1) p for trend 0.008; and for the high dose group 14.4 (2.1), 5.0 (2.1), 5.6 (2.1), p for trend 0.002. Side effects included dense saliva, xerostomia, and difficulty in swallowing, and were more frequent in the high‐dose group. Interpretation A 3000MU injection of BoNT‐B into the salivary glands significantly improved the frequency and severity of sialorrhoea in children with CP. The lower dose was ineffective, and the higher dose produced no greater benefit and more side effects.     

An internet‐based physical activity intervention for adolescents with cerebral palsy: a randomized controlled trial

Aim To determine the effectiveness of an 8‐week internet‐based, lifestyle physical‐activity intervention for adolescents with cerebral palsy (CP). Method A randomized controlled trial using concealed allocation with blinded assessments at baseline, 10, and 20 weeks. Forty‐one adolescents with CP participated (26 males, 15 females; mean age 13y 7mo, SD 1y 8mo, range 11–17y; Gross Motor Function Classification System levels: I, n=21; II, n=17; III, n=3; unilateral distribution n=16, bilateral n=25). Primary outcome was physical activity (NL‐1000 accelerometers and self‐report [Multimedia Activity Recall for Children and Adolescents: MARCA]). Secondary outcomes were exercise knowledge (a purpose‐designed scale), attitudes, intention and self‐efficacy (Lifestyle Education for Activity Program II scales), self‐reported sedentary behaviour (MARCA), and functional capacity (6‐min walk test). Results At 10 weeks the intervention group showed no increased physical activity compared with the comparison group (weekly steps: change of +2420 vs ?12189 steps p=0.06; weekly moderate‐to‐vigorous physical activity: change of +70 vs +8min, p=0.06; weekly distance walked: change of +3 vs ?9.1km, p=0.05) and exercise knowledge (12% vs 1% improvement, p=0.08). There were no statistically significant differences for these outcomes at 20 weeks, or in self‐reported physical activity at 10 or 20 weeks. Interpretation There was a positive short‐term pattern for improvement in physical activity and knowledge. Internet‐based programs may offer an alternative for participants unable to attend regular face‐to‐face physical activity programs.     

Prevalence and predictors of drooling in 7‐ to 14‐year‐old children with cerebral palsy: a population study

Aim To establish a prevalence estimate for drooling and explore factors associated with drooling in a population sample of children with cerebral palsy (CP) aged 7 to 14 years living in Victoria, Australia. Method A self‐report questionnaire was used to collect data on drooling from parents of children born between 1996 and 2001, and registered with the Victorian Cerebral Palsy Register. Results A total of 385 children (231 males, 154 females; mean age 10y 9mo [SD 1y 7mo], range 8–14y) were studied. The clinical type and distribution of CP were spastic (341), ataxic (16), dyskinetic (17), hypotonic (10), and unknown (1). Distribution in Gross Motor Function Classification System (GMFCS) levels was I (103), II (98), III (52), IV (63), V (61), and unknown (8). After adjustment for topographical pattern of motor impairment and GMFCS level, 40% were reported to have experienced drooling between 4 years of age and the time of completing the questionnaire. A significantly higher prevalence of drooling was found in children with poor gross motor function and in those with more severe presentations of CP, including poor head control, difficulty with eating, and inability to sustain lip closure (p<0.001 for each). Drooling was shown to be significantly associated with both intellectual disability and epilepsy in this group of children (p<0.001 for both). Interpretation With a prevalence of 40%, drooling is an important comorbidity in CP. It was considered severe in 15% of children. Poor oromotor function was associated with drooling and could be the target of interventions for this under‐researched problem.     

The Drooling Impact Scale: a measure of the impact of drooling in children with developmental disabilities

Aim To describe the development and clinimetric properties of a new scale to evaluate changes in the impact of drooling in children with developmental disabilities. Method After examining the properties of potential items, 10 items were retained for inclusion in the final Drooling Impact Scale. The clinimetric properties of the scale were evaluated using data from two convenience samples of children attending a saliva‐control clinic: a stable group (n=31, 22 males, nine females; mean age 10y 7mo, SD 4y 5mo, range 3y 6mo–18y 3mo; cerebral palsy [CP] n=17, intellectual disability n=10; non‐ambulatory n=13, nonverbal n=12) and an intervention group (n=49, 29 males, 20 females; mean age 11y, SD 3y 6mo, range 3y 4mo–16y 10mo; CP n=31, intellectual disability n=15; non‐ambulatory n=27, nonverbal n=28). To assess validity, changes in scores on the Drooling Impact Scale over time were compared with a carer’s global rating of change using Pearson’s correlations and t‐tests. A concordance correlation coefficient was used to compute the level of agreement between assessments 1 month apart in stable children. Effect size, standardized response mean, Guyatt responsiveness statistic, and an unpaired t‐test were used to estimate responsiveness. Results The correlation between the global rating and change in Drooling Impact Scale scores was 0.69 (p<0.001). The concordance correlation coefficient was 0.85. An effect size of 1.8, standardized response mean of 1.5, Guyatt responsiveness statistic of 1.4, and mean group difference of 23.5 (95% confidence interval 17.4–29.6) were obtained. Interpretation The Drooling Impact Scale is a valid and reliable subjective measure that is responsive to change.     

Modified constraint-induced therapy for children with hemiplegic cerebral palsy: a randomized trial

Aim Conventional constraint‐based therapies are intensive and demanding to implement, particularly for children. Modified forms of constraint‐based therapies that are family‐centred may be more acceptable and feasible for families of children with cerebral palsy (CP)‐but require rigorous evaluation using randomized trials. The aim of this study was to determine the effects of modified constraint‐induced therapy compared with intensive occupational therapy on activities of daily living and upper limb outcomes in children with hemiplegic CP. Method In this assessor‐blinded pragmatic randomized trial, 50 children (27 males, 23 females; age range 19mo–7y 10mo) with hemiplegic CP were randomized using a concealed allocation procedure to one of two 8‐week interventions: intensive occupational therapy (n=25), or modified constraint‐induced therapy (n=25). Manual Ability Classification System (MACS) levels of the participants were, level I n=2, II n=37, III n=8, and level IV n=1; Gross Motor Function Classification System (GMFCS) levels were, level I n=33, level II n=15, and level III n=1. Participants were recruited through three specialist CP centres in Australia and randomized between January 2008 and April 2010. Children randomized to modified constraint‐induced therapy wore a mitt on the unaffected hand for 2 hours each day, during which time the children participated in targeted therapy. The primary outcome was the Canadian Occupational Performance Measure (COPM – measured on a 10‐point scale) at completion of therapy. Other outcome measures were Goal Attainment Scaling, Assisting Hand Assessment, Pediatric Motor Activity Log, Modified Ashworth Scale, Modified Tardieu Scale, and a parent questionnaire. Assessments were carried out at 10 weeks and 6 months following randomization. Results All participants were included in the analysis. Between‐group differences for all outcomes were neither clinically important nor statistically significant. The mean difference in COPM was 0.3 (95% confidence interval [CI] −0.8 to 1.4; p=0.61) and mean difference in COPM satisfaction was 0.1 (95% CI −1.1 to 1.2; p=0.90). Minor adverse events were reported by five of the 25 participants in the modified constraint‐induced therapy group and by one of the 25 in the intensive occupational therapy group. All adverse events were related to participants’ lack of acceptance of therapy. Interpretation Modified constraint‐induced therapy is no more effective than intensive occupational therapy for improving completion of activities of daily living or upper limb function in children with hemiplegic CP.     

Attentional and executive impairments in children with spastic cerebral palsy

Aim Children with cerebral palsy (CP) are reported to have learning and social problems. The aim of the present study was to examine whether children with CP have impairments in attention or executive function. Method We examined attention and executive function with standardized neuropsychological measures in a group of children with unilateral (n=15) or bilateral (n=18) spastic CP (14 females, 19 males, mean age 11y 4mo, SD 1y 1mo, range 9y 1mo–13y 7mo; Gross Motor Function Classification System level I n=22, II n=3, III n=6, and IV n=2). Performance was compared with test norms. Results Verbal cognitive functioning fell within the normal range, whereas sustained (p=0.001) and divided attention (p<0.001) were found to be impaired. Greater impairment was observed in executive function in general (p<0.001) and in inhibition (p=0.038) and shifting (p<0.001) in particular. No significant difference was found between types of CP (unilateral and bilateral). Performance of all timed tasks was slower than the test norm (p<0.00). Interpretation The finding of slower performances across tasks may indicate a general impairment in efficiency of information processing in relation to white‐matter lesions. Impairments in attention and executive functions are present in children with CP and may help to explain why these children have increased social and learning problems.     

Characterization of spasticity in cerebral palsy: dependence of catch angle on velocity

Aim To evaluate spasticity under controlled velocities and torques in children with cerebral palsy (CP) using a manual spasticity evaluator. Method The study involved 10 children with spastic CP (six males, four females; mean age 10y 1mo, SD 2y 9mo, range 7–16y; one with quadriplegia, six with right hemiplegia, three with left hemiplegia; Gross Motor Function Classification System levels I [n=2], II [n=3], III [n=2], IV [n=2], and V [n=1]; Manual Ability Classification System levels II [n=5], III [n=4], and V [n=1]) and 10 typically developing participants (four males, six females; mean age 10y 3mo, SD 2y 7mo, range 7–15y). Spasticity and catch angle were evaluated using joint position, resistance torque, and torque rate at velocities of 90°, 180°, and 270° per second, controlled using real‐time audio‐visual feedback. Biomechanically, elbow range of motion (ROM), stiffness, and energy loss were determined during slow movement (30°/s) and under controlled terminal torque. Results Compared with typically developing children, children with CP showed higher reflex‐mediated torque (p<0.001) and the torque increased more rapidly with increasing velocity (p<0.001). Catch angle was dependent on velocity and occurred later with increasing velocity (p=0.005). Children with CP showed smaller ROM (p<0.05), greater stiffness (p<0.001), and more energy loss (p=0.003). Interpretation Spasticity with velocity dependence may also be position‐dependent. The delayed catch angle at higher velocities indicates that the greater resistance felt by the examiner at higher velocities was also due to position change, because the joint was moved further to a stiffer position at higher velocities.     

Effects of constraint-induced movement therapy on neurogenesis and functional recovery after early hypoxic-ischemic injury in mice

Aim Constraint‐induced movement therapy (CIMT) has emerged as a promising therapeutic strategy for improving affected upper limb function in children with hemiplegic cerebral palsy (CP). However, little is known about the changes in the brain that are induced by CIMT. This study was designed to investigate these changes and behavioural performance after CIMT intervention in mice with neonatal hypoxic‐ischemic brain injury. Method We utilized the neonatal hypoxic‐ischemic brain injury model established in mice pups. Three weeks after the injury, the mice were randomly assigned to the following three groups: the control group (n=15), the enriched‐environment group (n=17), and the CIMT with an enriched‐environment group (CIMT‐EE, n=15). 5‐bromo‐2‐deoxyuridine (BrdU) was injected daily to label proliferating cells during the 2 weeks of intervention. Results The CIMT‐EE group showed better fall rate in the horizontal ladder rung walking test (mean 5.4%, SD 3.6%) than either the control (mean 14.3%, SD 7.3%; p=0.001) or enriched‐environment (mean 12.4%, SD 7.7%; p=0.010) groups 2 weeks after the end of intervention. The CIMT‐EE group also showed more neurogenesis (mean 7069 cells/mm³, SD 4017 cells/mm³) than either the control group (mean 1555 cells/mm³, SD 1422 cells/mm³; p<0.001) or enriched‐environment group (mean 2994 cells/mm³, SD 3498 cells/mm³; p=0.001) in the subventricular zone. In the striatum, neurogenesis in the CIMT‐EE group (mean 534 cells/mm³, SD 441 cells/mm³) was greater than in the control group (mean 95 cells/mm³, SD 133 cells/mm³; p=0.001). Interpretation There was CIMT‐EE enhanced neurogenesis in the brain along with functional benefits in mice after early hypoxic‐ischemic brain injury. This is the first study to demonstrate the effects of CIMT on neurogenesis and functional recovery after experimental injury to an immature brain.     

Mechanical properties of the plantarflexor musculotendinous unit during passive dorsiflexion in children with cerebral palsy compared with typically developing children

Aim To examine the passive length–tension relations in the myotendinous components of the plantarflexor muscles of children with and without cerebral palsy (CP) under conditions excluding reflex muscle contraction. Method A cross‐sectional, non‐interventional study was conducted in a hospital outpatient clinic. Passive torque–angle characteristics of the ankle were quantified from full plantarflexion to full available dorsiflexion in 26 independently ambulant children with CP (11 females, 15 males; mean age: 6y 11mo, range 4y 7mo–9y 7mo) and 26 age‐matched typically developing children (18 females, 8 males; mean age 7y 2mo, range 4y 1mo–10y 4mo). In the children with CP, the affected (hemiplegia; n=21) or more affected (diplegia; n=5) leg was tested; in typically developing children, the leg tested was randomly selected. Gross Motor Function Classification System levels were I (n=15) and II (n=11). Care was taken to eliminate active or reflex muscle contribution to the movement, confirmed by the absence of electromyographic activity. Results There were small but significant differences between the two groups for maximum ankle dorsiflexion (p=0.003), but large and significant differences in the torques required to produce the same displacement (p<0.001). Further, the hysteresis of the average loading cycle in the children with CP was over three times that of the typically developing children (p<0.001). Interpretation We believe that the plantarflexor muscles of children with CP are stiffer and intrinsically more resistant to stretch, even though they retain near normal excursion. This increased stiffness is a non‐neurally‐mediated feature demonstrated by these children. The extent to which it influences function and predisposes the children to development of soft tissue contracture is unknown.     

Correlates of decline in gross motor capacity in adolescents with cerebral palsy in Gross Motor Function Classification System levels III to V: an exploratory study

Aim To explore associations between clinical variables and decline in motor capacity in adolescents with cerebral palsy (CP). Method Participants included 76 males and 59 females, whose mean age at the beginning of the study was 14 years 6 months (SD 2.4, range 11.6–17.9); 51 at Gross Motor Function Classification System (GMFCS) level III, 47 at level IV, and 37 at level V. Ninety‐six participants had tetraplegia, 32 had diplegia, and one had hemiplegia. Types of motor disorder were spastic n=98; mixed, n=11; dystonic, n=9; hypotonic, n=7; and ataxic n=3 (seven participants were not classified). Reliable raters collected data annually for 4 years on anthropometric characteristics, the Spinal Alignment and Range of Motion Measure, as well as the Gross Motor Function Measure, 66 items (GMFM‐66); participants or their parents reported on health status (using the Health Utilities Questionnaire), pain, and exercise participation (using measures developed for this study). The predicted drop in GMFM‐66 scores after childhood was calculated using data on the same children from an earlier study. Correlations were calculated between the drop in GMFM‐66 scores and the average and change scores of the clinical variables (the alpha level for statistical significance of this exploratory study was 0.10). Results The drop in GMFM‐66 score was significantly correlated with limitations in range of motion (r=0.42) and spinal alignment (r=0.28), and pain (r=0.16). Increases in triceps skinfold (r=?0.19), mid‐arm circumference (r=?0.23), and the ratio of mid‐arm circumference to knee height (r=?0.23) were associated with less decline. Interpretation Preventing range‐of‐motion limitations and pain experiences and optimizing nutrition might contribute to less decline in the gross motor capacity of adolescents with CP. Further investigation is required to clarify the role other factors that contribute to maintained function over time.     

Salivary gland botulinum toxin injections for drooling in children with cerebral palsy and neurodevelopmental disability: a systematic review

Aim The aim of this paper was to systematically review the efficacy and safety of botulinum toxin (BoNT) injections to the salivary glands to treat drooling in children with cerebral palsy and neurodevelopmental disability. Method A systematic search of The Cochrane Central Register of Controlled Trials, PubMed, CINAHL (Cumulative Index to Nursing and Allied Health Literature), EMBASE, and the Physiotherapy Evidence Database (PEDro) was conducted (up to 1 October 2011). Data sources included published randomized controlled trials (RCTs) and prospective studies. Results Sixteen studies met inclusion criteria. Three outcome measures support the effectiveness of BoNT for drooling. One RCT found an almost 30% reduction in the impact of drooling on patients’ lives, as measured by the Drooling Impact Scale (mean difference ?27.45; 95% confidence interval [CI] ?35.28 to ?19.62). There were sufficient data to pool results on one outcome measure, the Drooling Frequency and Severity Scale, which supports this result (mean difference ?2.71; 95% CI ?4.82 to ?0.60; p<0.001). There was a significant reduction in the observed number of bibs required per day. The incidence of adverse events ranged from 2 to 41%, but was inconsistently reported. One trial was terminated early because of adverse events. Interpretation BoNT is an effective, temporary treatment for sialorrhoea in children with cerebral palsy. Benefits need to be weighed against the potential for serious adverse events. More studies are needed to address the safety of BoNT and to compare BoNT with other treatment options for drooling.     

Development of the Hypertonia Assessment Tool (HAT): a discriminative tool for hypertonia in children

Aim The aim of this study was to develop a tool to identify paediatric hypertonia subtypes. Method Items generated by experts were subscaled (spasticity, dystonia, rigidity). The tool was administered to 34 children (19 males, 15 females, mean age 8y 2mo, range 2y 5mo–18y 7mo) with hypertonia and cerebral palsy (CP) in Gross Motor Function Classification System (GMFCS) levels: I, n=7; II, n=5; III, n=7 level IV, n=7; and level V, n=8 level. Kuder–Richardson Formula 20 determined internal consistency. To assess reliability, two physicians administered the tool to 25 additional children with CP (15 males, 10 females; mean age 10y 8mo; GMFCS levels I, n=4; II, n=3; III, n=7; IV, n=4; and V, n=7) on two occasions, 2 weeks apart. To evaluate validity, a third physician diagnosed the hypertonia by neurological examination. Results The internal consistency of the spasticity items was moderate (α=0.58), and dystonia was high (α=0.79). Item reduction eliminated seven of the 14 original items. The agreement of the spasticity and rigidity subscales was adequate (prevalence‐adjusted bias‐adjusted kappa [PABAK] ranging from moderate [0.57] to excellent [1.0]) for validity, test–retest reliability, and interrater reliability. For dystonia agreement was lower, with PABAK ranging from fair (0.30) to good (0.65). Eighty‐seven per cent had spasticity and 78% had dystonia. Interpretation The Hypertonia Assessment Tool has good reliability and validity for identifying spasticity and the absence of rigidity, and moderate findings for dystonia.     

Long‐term survival of children with cerebral palsy in Okinawa,Japan

Aim The aim of this study was to describe the survival prognosis of children with cerebral palsy (CP) in Okinawa, Japan. Method A cohort study was conducted on all children with CP born between 1988 and 2005 in Okinawa, Japan. Survival proportions were determined with a life table and Kaplan–Meier survival curves were plotted. The effect of each predictor variable was estimated using Cox regression analysis. Results This study included 580 children with CP (332 males, 248 females). In the cohort, 119 (20.5%) children were classified in Gross Motor Function Classification System (GMFCS) level I, 65 (11.2%) were classified in level II, 40 (6.9%) in level III, 189 (32.6%) in level IV, 166 (28.6%) in level V and GMFCS level was unknown for one. Of the 34 children who died, 29 were classified in GMFCS level V and GMFCS level was unknown for one. Mean age at start of follow‐up was 24.5 months (SD 2.6mo); mean length of follow‐up was 8 years 8 months (standard error of the mean 0.214y). The 5 year‐ and 18‐year survival percentages of the entire cohort were 98% and 89% respectively. In children with CP, significantly lower survival rates were associated with multiple factors, including a birthweight of at least 2500g (p=0.009), a gestational age of at least 37 weeks (p=0.004), and the most severe gross motor limitation, GMFCS level V (p<0.001). However, multivariate analysis showed GMFCS level V was the only significant predictor variable (p<0.001) for survival of CP. Interpretation This study is the first to describe survival of children with CP in Japan. Our results are similar to those previously reported in other countries. These results are important in planning adequate provision of social and medical services for individuals with CP.