Adverse events and health status following botulinum toxin type A injections in children with cerebral palsy

Aim The aim of this study was to assess changes in health status before and after, as well as adverse events after, botulinum toxin type A (BoNT‐A) injections in children with cerebral palsy (CP). Method A total of 334 children (190 male; 144 female) aged 1y 6mo to 19y 4mo (mean 9y 2mo, SD 4y) with CP who were undergoing BoNT‐A injections (596 injection courses in total) were clinically audited over a 16‐month period. Of the 334 children, 62 were classified at Gross Motor Function Classification System (GMFCS) level I, 52 of whom had unilateral CP and 10 of whom had bilateral CP. Eighty‐six children were classified at GMFCS level II, 39 of whom had unilateral CP and 47 of whom had bilateral CP. Forty‐four children were classified at GMFCS level III, two of whom had unilateral CP and 42 of whom had bilateral CP. Sixty‐six of the 334 children were classified at GMFCS level IV and 76 as level V. All the children classified as level IV or V had bilateral involvement. The health status of the children in the month before and a prospective audit of health status and adverse events in the month after BoNT‐A injections were examined in order to assess the effects of the toxin. Results The data gathered for the month before administration of BoNT‐A indicated that children with CP had significant background morbidities. After injection of BoNT‐A, adverse events occurred in 23.2% of children. All adverse events were temporary and there were no deaths. Interpretation The results of this audit indicate that there is insufficient evidence to warrant restriction of the administration of BoNT‐A in children with CP at any GMFCS level in our service.     

Stability of the Gross Motor Function Classification System after single‐event multilevel surgery in children with cerebral palsy

Aim There are conflicting reports about the stability of the Gross Motor Function Classification System (GMFCS) in children with cerebral palsy (CP) after orthopaedic surgery. We studied the stability of the GMFCS in children with bilateral spastic CP after single‐event multilevel surgery, using the Gait Profile Score (GPS) as the primary outcome measure. Method This was a retrospective cohort study of 107 children (46 females, 61 males) with bilateral spastic CP, classified as GMFCS level II or III, who underwent surgery at a single tertiary institution between 1997 and 2008. The mean age at surgery was 10 years 7 months (SD 2y 8mo). The primary outcome measure was the GPS. Changes in GMFCS level were studied at multiple time points before and after intervention. Results Gait dysfunction was partially corrected, with a mean improvement of 28% in the GPS. The GMFCS remained stable and unchanged in 95% of children and improved by one level in 5% of children. The improvement in GPS was three times the minimal clinically important difference. The mean age at final postoperative GPS assessment was 11 years 10 months (SD 2y 10mo) and at final GMFCS assessment was 15 years 7 months (SD 3y 9mo). Interpretation Stability of the GMFCS was confirmed in the majority of children with bilateral spastic CP after single‐event multilevel surgery, despite statistically and clinically significant improvements in gait dysfunction and functional mobility. This information is important in realistic goal‐setting and in counselling families.     

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.     

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.     

Self‐care and mobility skills in children with cerebral palsy,related to their manual ability and gross motor function classifications

Aim The aim of this study was to investigate the acquisition of self‐care and mobility skills in children with cerebral palsy (CP) in relation to their manual ability and gross motor function. Method Data from the Pediatric Evaluation of Disability Inventory (PEDI) self‐care and mobility functional skill scales, the Manual Ability Classification System (MACS), and the Gross Motor Function Classification System (GMFCS) were collected from 195 children with CP (73 females, 122 males; mean age 8y 1mo; SD 3y 11mo; range 3–15y); 51% had spastic bilateral CP, 36% spastic unilateral CP, 8% dyskinetic CP, and 3% ataxic CP. The percentage of children classified as MACS levels I to V was 28%, 34%, 17%, 7%, and 14% respectively, and classified as GMFCS levels I to V was 46%, 16%, 15%, 11%, and 12% respectively. Results Children classified as MACS and GMFCS levels I or II scored higher than children in MACS and GMFCS levels III to V on both the self‐care and mobility domains of the PEDI, with significant differences between all classification levels (p<0.001). The stepwise multiple regression analysis verified that MACS was the strongest predictor of self‐care skills (66%) and that GMFCS was the strongest predictor of mobility skills (76%). A strong correlation between age and self‐care ability was found among children classified as MACS level I or II and between age and mobility among children classified as GMFCS level I. Many of these children achieved independence, but at a later age than typically developing children. Children at other MACS and GMFCS levels demonstrated minimal progress with age. Interpretation Knowledge of a child’s MACS and GMFCS level can be useful when discussing expectations of, and goals for, the development of functional skills.     

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.     

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.     

Reference values for anaerobic performance and agility in ambulatory children and adolescents with cerebral palsy

Aim The aim of this study was to provide reference values of anaerobic performance and agility in a group of children and adolescents with spastic cerebral palsy (CP). Method A total of 300 children (184 males, 116 females) with spastic CP were recruited from 26 rehabilitation centres in six different countries. Of these, 215 were classified at GMFCS level I (mean age 11y 2mo, SD 3y, range 6–18y) and 85 were classified at GMFCS level II (mean age 11y; SD 3y 1mo, range 6–18y). The children performed the Muscle Power Sprint Test (MPST) and the 10×5m sprint test in a standardized manner. To establish reference values, reference curves were created using generalized additive models for location, scale, and shape. Results Height‐related reference curves were created based on performance on the two tests. Interpretation This study provides height‐related reference values for anaerobic performance and agility for children and adolescents with CP classified at GMFCS levels I and II. These curves are clinically relevant and provide a user‐friendly method in the interpretation of anaerobic performance and agility for children with spastic CP.     

The natural history of hip development in cerebral palsy

Aim The purpose of this study was to evaluate a population‐based radiographic hip surveillance programme for children with cerebral palsy (CP) and to assess the natural history of hip displacement. Method The study comprised 335 children (188 males, 147 females), born during 2002 to 2006 in the 10 south‐eastern counties in Norway. Their mean age at the first radiograph was 3 years (range 6mo–7y 11mo) and the mean age at the most recent follow‐up was 5 years 5 months. Distribution according to CP type was spastic hemiplegia in 38%, diplegia in 27%, quadriplegia in 21%, dyskinesia in 10%, and ataxia in 3%; Gross Motor Function Classification System (GMFCS) levels I to V were, 44%, 14%, 8%, 11%, and 23% respectively. Migration percentage (MP), acetabular index, and pelvic obliquity were measured on the radiographs. Results Hip displacement (MP>33%) occurred in 26% of all children (subluxation in 22% and dislocation in 4%) and in 63% of those in GMFCS levels IV or V. Dislocation occurred in 14 children at a mean age of 4 years 5 months (range 1y 10mo–9y 7mo). The mean migration percentage was 20.4% at the initial radiographs and 34.0% at the last follow‐up. Mean progression in migration percentage increased markedly with decreasing functional level, from 0.2% per year at GMFCS level I to 9.5% at level V. Interpretation There is a pronounced trend towards hip displacement in nonambulant children. Close surveillance from age 1 to 2 years is needed to find the appropriate time for preventive surgery. Since 12% of the nonambulant children developed dislocation, our routines for hip surveillance need improvement.     

Quantitative diffusion tensor tractography of the motor and sensory tract in children with cerebral palsy

Aim The aim of this study was to compare the findings of quantitative diffusion tensor tractography of the motor and sensory tracts in children with cerebral palsy (CP) and typically developed comparison individuals, and also to evaluate the correlation with gross motor function. Method Thirty‐four children with CP (mean age 2y 2.mo, SD 2y 0mo; 19 with spastic diplegia, eight with hemiplegia, six with spastic quadriplegia, and one with spastic triplegia) and 21 healthy comparison children (mean 2y 1.68mo, SD 2y 8.64mo) were evaluated. The distribution of Gross Motor Function Classification System (GMFCS) levels in the CP group was as follows: level I, 7; level II, 14; level III, 5; level IV, 3; and level V, 5. The following three diffusion tensor imaging (DTI) parameters including tractography were evaluated for each tract (corticospinal tract [CST] and posterior thalamic radiation [PTR]): number of fibres, tract‐based fractional anisotropy, and region of interest (ROI)‐based fractional anisotropy. We compared each value between the two groups, and correlated each value with the GMFCS level. Results The number of fibres and ROI‐based fractional anisotropy values of both tracts were significantly lower in children with CP than in the comparison group (p<0.05–0.001). Additionally, there was significant negative correlation between GMFCS level and motor–sensory parameters (p<0.001–0.05). Interpretation DTI parameters of the CST and PTR in children with CP were significantly lower than in comparison children. In addition, these parameters were significantly correlated with GMFCS level.     

Rasch analysis of items from two self-report measures of motor function: determination of item difficulty and relationships with children's ability levels

Aim The aim of this article was to determine item measurement properties of a set of items selected from the Gillette Functional Assessment Questionnaire (FAQ) and the Pediatric Outcome Data Collection Instrument (PODCI) using Rasch analysis, and to explore relationships between the FAQ/PODCI combined set of items, FAQ walking scale level, Gross Motor Function Classification System (GMFCS) levels, and the Gait Deviation Index on a common measurement scale. Method Rasch analysis was performed on data from a retrospective chart review of parent‐reported FAQ and PODCI data from 485 individuals (273 males; 212 females; mean age 9y 10mo, SD 3y 10mo) who underwent first‐time three‐dimensional gait analysis. Of the 485 individuals, 289 had a diagnosis of cerebral palsy (104 GMFCS level I, 97 GMFCS level II, 69 GMFCS level III, and 19 GMFCS level IV). Rasch‐based person abilities and item difficulties based on subgroups defined by the FAQ walking scale level, Gait Deviation Index, and the GMFCS level were compared. Results The FAQ/PODCI item set demonstrated necessary Rasch characteristics to support its use as a combined measurement scale. Item groupings at similar difficulty levels were consistent with the mean person abilities of subgroups based on FAQ walking scale level, Gait Deviation Index, and GMFCS level. Interpretation Rasch‐derived person ability scores from the FAQ/PODCI combined item set are consistent with clinical measures. Rasch analysis provides insights that may improve interpretation of the difficulty of motor functions for children with disabilities.     

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.     

Should children with cerebral palsy and normal imaging undergo testing for inherited metabolic disorders?

Aim  For the 9% to 16% of children with cerebral palsy (CP) who have normal brain imaging, further testing for metabolic and/or genetic conditions has been recommended. This study aimed to identify a cohort of children with CP with normal magnetic resonance imaging (MRI), clinically review and describe the cases, and assess the value of testing for inherited metabolic disorders in these children. Method  Children with congenital CP born from 1999 to 2005 were selected from a population register. Normal MRI reports were identified and the scans reassessed. Children whose scans were performed before 18 months were excluded, as were children with spastic CP (Gross Motor Function Classification System [GMFCS] level I). The remainder were reviewed clinically and offered investigations. Results  Of 730 children identified, 515 had available imaging and 54 were confirmed as normal. Cases with non‐spastic CP and those with milder clinical severity were more likely to have normal imaging. Twenty‐three children (17 males, six females; mean age 6y 11mo, SD 1y 10mo, range 3y 0mo to 10y 0mo) were reviewed clinically and offered investigations. Twelve children had spasticity (11 with diplegia, one quadriplegia), three had dyskinesia, five ataxia, and three hypotonia. Two children functioned in GMFCS level I, 11 in level II, seven in level III and three in level IV. Four children with spasticity had unusual features. No alternative diagnoses were made. Interpretation  Although important to consider in individual cases, comprehensive metabolic testing failed to clarify the aetiology of CP further in this large cohort of children with normal MRIs, even those with atypical features.     

Brain magnetic resonance imaging and motor and intellectual functioning in 86 patients born at term with spastic diplegia

Aim To investigate the association between magnetic resonance imaging (MRI) patterns and motor function, epileptic episodes, and IQ or developmental quotient in patients born at term with spastic diplegia. Method Eighty‐six patients born at term with cerebral palsy (CP) and spastic diplegia (54 males, 32 females; median age 20y, range 7–42y) among 829 patients with CP underwent brain MRI between 1990 and 2008. The MRI and clinical findings were analysed retrospectively. Intellectual disability was classified according to the Enjoji developmental test or the Wechsler Intelligence Scale for Children (3rd edition). Results The median ages at diagnosis of CP, assignment of Gross Motor Function Classification System (GMFCS) level, cognitive assessment, and MRI were 2 years (range 5mo–8y), 6 years (2y 8mo–19y), 6 years (1y 4mo–19y), and 7 years (10mo–30y) respectively. MRI included normal findings (41.9%), periventricular leukomalacia, hypomyelination, and porencephaly/periventricular venous infarction. The frequency of patients in GMFCS levels III to V and intellectual disability did not differ between those with normal and abnormal MRI findings. Patients with normal MRI findings had significantly fewer epileptic episodes than those with abnormal ones (p=0.001). Interpretation Varied MRI findings, as well as the presence of severe motor dysfunction and intellectual disability (despite normal MRI), suggest that patients born at term with spastic diplegia had heterogeneous and unidentified pathophysiology.     

Tibial length growth curves for ambulatory children and adolescents with cerebral palsy

Aim The aim of this study was to generate growth curves for ambulatory children and adolescents with cerebral palsy (CP) using tibial lengths and to determine if they differed according to sex or Gross Motor Function Classification System (GMFCS) level. Method Growth data were studied from a cohort of 750 participants (442 males, 308 females [1199 visits]; mean age 10y 9mo, SD 3y 4mo, range 4–21y) with CP (hemiplegia, n=163; diplegia, n=573; triplegia, n=11; quadriplegia n=2; GMFCS levels I–III), and 165 typically developing children (96 males, 115 females; [211 visits]) mean age 10y 9mo, SD 4y 2mo, range 4–19y). Tibial length measurements calculated from data collected during routine gait analyses were validated using anthropometric tibial length measurements and were used to generate growth curves for males and females classified as GMFCS level I, II, or III. Growth was compared in participants of different sexes and GMFCS levels using the median curves. Results Growth curves for males and females (GMFCS levels I–III) with estimate lines for 3rd, 10th, 25th, 50th, 75th, 90th, and 97th centiles were generated. Mean tibial length was greater in males than in females in all GMFCS levels. Tibial lengths were shorter in participants classified as GMFCS level III than in those classified as GMFCS level I or II. Interpretation To our knowledge this is the first large‐scale investigation of bone growth in ambulatory children and adolescents with CP. The large sample made it possible to generate growth curves and to provide insight into growth trends. The study findings serve as a basis for analysis of the relationships between growth, function, and treatment outcomes.     

Efficacy of botulinum toxin A in children with cerebral palsy in Gross Motor Function Classification System levels IV and V: a systematic review

Aim Previous studies have shown the efficacy of botulinum toxin type A (BoNT‐A) in the management of ambulant individuals with cerebral palsy (CP). There is little evidence on its use in non‐ambulant children with CP. This review aimed to investigate indications and efficacy for BoNT‐A use in managing pain, care, and comfort, and improving function in children with CP in Gross Motor Function Classification System (GMFCS) levels IV and V. Method Electronic databases were searched from the earliest available date to June 2012 using a combination of subject headings and free text. Inclusion criteria consisted of studies with (1) participants aged 18 or under, (2) participants with CP in GMFCS levels IV and V, (3) participants receiving BoNT‐A treatment, and (4) studies published in English‐language peer‐reviewed journals. Results The search resulted in a total of 814 studies, of which 19 met the inclusion criteria. Eighteen studies provided level IV or V evidence and one level I evidence according to the American Academy for Cerebral Palsy and Developmental Medicine guidelines for the development of systematic reviews. Most of the studies were of weak to moderate methodological quality. Interpretation The evidence that BoNT‐A is effective in reducing postoperative pain in children with CP in GMCFS levels IV and V is limited, with only one level I study identified. Remaining indications were general pain reduction, maintaining hip integrity, achieving functional changes, and goal attainment. A high percentage of participants in the studies showed positive changes in these areas. With the poor level of evidence of the included studies, no definite conclusion could be drawn on the indications for BoNT‐A use in children with CP in GMCFS levels IV and V. Further investigation by rigorous studies is required.     

No change in calf muscle passive stiffness after botulinum toxin injection in children with cerebral palsy

Aim Stiffness and shortening of the calf muscle due to neural or mechanical factors can profoundly affect motor function. The aim of this study was to investigate non‐neurally mediated calf‐muscle tightness in children with cerebral palsy (CP) before and after botulinum toxin type A (BoNT‐A) injection. Method Sixteen children with spastic CP (seven females, nine males; eight at Gross Motor Function Classification System level I, eight at level II; age range 4–10y) and calf muscle spasticity were tested before and during the pharmaceutically active phase after injection of BoNT‐A. Measures of passive muscle compliance and viscoelastic responses, hysteresis, and the gradient of the torque–angle curve were computed and compared before and after injection. Results Although there was a slight, but significant increase in ankle range of motion after BoNT‐A injection and a small, significant decrease in the torque required to achieve plantigrade and 5° of dorsiflexion, no significant difference in myotendinous stiffness or hysteresis were detected after BoNT‐A injection. Interpretation Despite any effect on neurally mediated responses, the compliance of the calf muscle was not changed and the muscle continued to offer significant resistance to passive motion of the ankle. These findings suggest that additional treatment approaches are required to supplement the effects of BoNT‐A injections when managing children with calf muscle spasticity.     

Functional decline in children undergoing selective dorsal rhizotomy after age 10

Aim To compare function and gait in a group of children older than most children who received selective dorsal rhizotomy (SDR) with age‐ and function‐matched peers who received either orthopedic surgery or no surgical intervention. Method A retrospective study examined ambulatory children with diplegic cerebral palsy, aged between 10 years and 20 years and categorized in Gross Motor Function Classification System (GMFCS) levels I or II. Three groups were considered: (1) children who had selective dorsal rhizotomy (n=8; two females, six males; mean age 15y 4mo at SDR, 16y 8mo at follow‐up); (2) children who had orthopedic surgery (n=9; three females, six males; mean age 14y 6mo at SDR, 15y 1mo at follow‐up), and (3) children who had no surgical intervention (n=9; two females, seven males; mean age 15y 6mo at follow‐up). Longitudinal measures of gait analysis (velocity, gait deviation index, and gait variable scores) and gross motor function (GMFCS level, Gross Motor Function Measure scores, and centiles) were examined. Results No significant differences were found between changes in gait comparing rhizotomy with orthopedic surgery; however, the group who received orthopedic surgery demonstrated improved gait compared with the group without surgical intervention. Longitudinal comparisons of gross motor function demonstrated a decrease in the group who received SDR. Between‐group analysis of outcomes also demonstrated worse outcomes of the SDR group compared with the orthopedic surgery group and with the no surgical intervention group. Interpretation Rhizotomy in older children was associated with functional declines compared with similar children who had no surgery and with those who underwent orthopedic surgery. This suggests that age greater than 10 years might be a contraindication for SDR if the goal is to improve motor skills.     

Survival of individuals with cerebral palsy receiving continuous intrathecal baclofen treatment: a matched‐cohort study

Aim To determine whether intrathecal baclofen (ITB) changes mortality risk in persons with cerebral palsy (CP). Method Records were reviewed for all persons with CP who were managed with ITB for hypertonicity at a specialty hospital in Minnesota between May 1993 and August 2007. A comparison cohort was randomly selected from clients of the California Department of Developmental Services who were initially evaluated between 1987 and 1990 and were matched to those with ITB for age, sex, Gross Motor Function Classification System (GMFCS) level, presence or absence of epilepsy, and feeding‐tube use. Survival probabilities were estimated using the Kaplan–Meier method, and differences were tested via log‐rank. Results Three hundred and fifty‐nine persons with CP (202 males, 157 females) receiving ITB for hypertonicity (mean age 12y 8mo, SD 7y 9mo, range 3y 1mo to 39y 9mo) were matched to 349 persons without ITB pumps (195 males, 154 females; mean age 12y 7mo, SD 8y 4mo, range 2y 7mo to 40y). The proportion of patients at different GMFCS levels in the ITB and in the non‐ITB cohorts, respectively, was as follows: level II 3% and 3%, level III 16% and 16%, level IV 38% and 37%, and level V 43% and 44%. Survival at 8 years of follow‐up was 92% (SD 1.9%) in the ITB cohort and 82% (SD 2.4%) in the non‐ITB cohort (p<0.001). After adjustment to account for recent trends in improved survival in CP, 8‐year survival in the non‐ITB cohort was 88%, which was not significantly different from the ITB cohort (p=0.073). Interpretation ITB therapy does not increase mortality in individuals with CP and may suggest an increase in life expectancy.     

Speech, expressive language, and verbal cognition of preschool children with cerebral palsy in Iceland

Aim The aim of this study was to describe speech, expressive language, and verbal cognition of children with cerebral palsy (CP). Method A population study included 152 Icelandic children with congenital CP (74 males, 78 females; mean age 5y 5mo, range 4y–6y 6mo). Children who spoke in sentences, phrases, or one‐word utterances were categorized as verbal. Speech was classified as normal, mild dysarthria, or severe dysarthria. Cognition was reported as IQ (Wechsler Preschool and Primary Scale of Intelligence – Revised) or developmental quotient (DQ). Results Most children (81%) had spastic CP and bilateral symptoms (76%); 74 (49%) were at Gross Motor Function Classification System (GMFCS) level I, 27% at levels II and III, and 24% at levels IV and V (p<0.001). One hundred and twenty‐eight children (84%) communicated verbally whereas 24 were nonverbal. Nonverbal status and severe dysarthria were associated with greater motor impairment (GMFCS; p<0.001). Twenty‐five children (16%) had severe dysarthria. Most (88%) of the nonverbal children had multiple disabilities compared with 18% of the verbal group (p<0.001). Median (interquartile range) verbal IQ was 93 (73–104) and performance IQ 77 (61–94; p<0.001). Sixty‐eight children (45%) had normal verbal cognition and almost a quarter of the children with severe dysarthria had a full‐scale IQ/DQ of 70. Interpretation Most children with CP express sentences and almost half of them have normal verbal IQ. Nonverbal status frequently indicates multiple impairments whereas severe dysarthria may be associated with normal cognition.