A diagnosis of ‘cerebral palsy’ in childhood is relatively common. Abnormalities of the upper cervical spine causing spinal cord compression are rare, but can be a cause of symptoms and signs that may otherwise be attributed to brain injury acquired during development. We present an interesting case of a congenital abnormality of the atlas causing severe cervical spinal cord compression in a 9-year-old child, together with a discussion of the relevant aspects of spinal development and a review of the literature.

OBJECTIVE: To investigate the validity of MTI accelerometer as a physical activity (PA) measurement instrument for children with cerebral palsy (CP).

DESIGN: Participants were classified within Gross Motor Function Classification System I to III and took part in 2 activity sessions: (1) a structured activity protocol with increasing intensities and (2) a free play session. Concurrent measurements of activity counts, heart rate, and observed physical activity were performed.

SETTING: Data were collected on normal school days in special schools within the participants’ 30-minute break period.

PARTICIPANTS: Convenience sample of children with CP (N=31; 17 girls, 14 boys) age between 6 and 14 years (mean +/- SD, 9.71+/-2.52 y).

INTERVENTIONS: Not applicable.

MAIN OUTCOME MEASURES: MTI measured activity counts, a monitoring device measured heart rate, and the System for Observing Fitness Instruction Time (SOFIT) was used for direct PA observation.

RESULTS: There were strong relationships between MTI and SOFIT (r=.75; R(2)=.56; P<.001) and heart rate monitor (HRM) and SOFIT (r=.65; R(2)=.43; P<.001) data in structured activities, but the difference between these 2 correlation coefficients was not significant (P=.46). In free play activities, the association between MTI and SOFIT data (r=.67; R(2)=.45; P<.001) was significantly stronger (P=.01) than that between heart rate and SOFIT data (r=.14; R(2)=.02; P<.001) . Bland-Altman plots showed better agreement between observed SOFIT and MTI-predicted SOFIT data than observed SOFIT and HRM-predicted SOFIT data from the linear regression analysis.

CONCLUSIONS: The findings suggest that the MTI appears to be a valid instrument for measuring raw activity volume among children with CP and is suitable for use in studies attempting to characterize the PA of this population.

OBJECTIVES: To develop a questionnaire that specifically and comprehensively measures mobility limitations in children with cerebral palsy (CP) and to investigate certain psychometric properties (scalability, unidimensionality, internal consistency) of this questionnaire.

DESIGN: Cross-sectional study.

SETTING: Private physical therapy practices and outpatient departments of hospitals and rehabilitation centers.

PARTICIPANTS: Children with CP undergoing physical therapy or rehabilitation. The Mobility Questionnaire, 47-item (MobQues47), was completed by the mothers of these children (N=323; mean age +/- SD, 7.1+/-2.9y; 57% boys; Gross Motor Function Classification levels: I [48%], II [26%], III [19%], IV [7%]).

INTERVENTIONS: Not applicable.

MAIN OUTCOME MEASURES: Mokken scale analysis was used to investigate (1) scalability, indicating that the items form a scale; (2) unidimensionality, indicating that the items measure only 1 concept; and (3) internal consistency, indicating the degree of interrelatedness of the items.

RESULTS: The MobQues47, made up of 47 items, was developed on the basis of 3 pilot studies and careful operationalization of the concept (or construct) of mobility limitations. The scalability (coefficient H=.70), unidimensionality, and internal consistency (coefficient rho=.99) of the MobQues47 were found to be very good.

CONCLUSIONS: The MobQues47 is a unidimensional scale with excellent internal consistency that can be used to measure caregiver-reported mobility limitations in children with CP.

BACKGROUND AND OBJECTIVES: Patients with cerebral palsy (CP) frequently receive drugs for the treatment of concomitant diseases, such as seizures. Midazolam is a benzodiazepine with hypnotic action most often used as pre-anesthetic medication and its drug interactions in patients with CP are unknown. The objective of the present study was to evaluate the effect of midazolam as pre-anesthetic drug on the BIS of patients with CP undergoing chronic treatment with anticonvulsant agents.

METHOD: Three groups of patients were assessed: CP without anticonvulsant treatment, CP undergoing treatment with anticonvulsant and a group with no disease and no medication use (control group). On the day before the surgery, with the patients conscious and in dorsal decubitus, the BIS monitor was placed and the basal BIS values were recorded. On the following day, 40 minutes before the surgery, the patients received 0.6 mg*kg(-1) of midazolam orally. Before the start of the anesthetic procedure, the same procedure for BIS recording was carried out after midazolam administration.

RESULTS: A total of 107 patients were studied – 39 patients from the Control Group (CG) and 68 with a diagnosis of CP. Among these, 17 used anticonvulsant drugs. Regarding the mean BIS value after the midazolam administration, there was no difference between patients from the CG and those in the CP group that did not take anticonvulsant drugs, whereas the ones who took anticonvulsants exhibited a difference (p = 0.003). The possibility of decrease in the BIS after midazolam use increases according to the number of anticonvulsant drugs used by the patient.

CONCLUSIONS: The chronic use of anticonvulsants associated to oral midazolam as pre-anesthetic medication can lead to the decrease in the BIS values, which configures deep level of hypnosis.

Crigler Najjar syndrome type II is related to a defect of bilirubin conjugation due to partial deficiency of the enzyme uridine diphosphate-glucuronyl transferase. Usually has a benign course, unlike Crigler Najjar type I, where the enzyme deficiency is total and the affected patients usually die at early ages. We present the case of a teenager with indirect hyperbilirubinemia, seizures and cerebral palsy. A good clinical history with pedigree and appropriate functional tests allowed us to determine the definitive diagnosis. This is an autosomal recessive disorder, has a very low prevalence worldwide, and is a diagnostic challenge for physicians in general.

Sätilä H, Huhtala H: Botulinum toxin type A injections for treatment of spastic equinus in cerebral palsy: A secondary analysis of factors predictive of favorable response.

OBJECTIVE: To investigate markers predicting favorable botulinum toxin injection outcomes in treating spastic equinus gait in children with cerebral palsy.

DESIGN: Thirty-six children (21 boys and 15 girls, aged 1.5-9.6 yrs; 20 with unilateral and 16 with bilateral cerebral palsy levels I to IV with the Gross Motor Function Classification System) with 49 treated lower limbs were included. Passive range of movement, selective dorsiflexion, dynamic muscle length (modified Tardieu Scale), calf tone (modified Ashworth Scale), attainment of anticipated gait pattern (Goal Attainment Scale), and video gait analysis (Observational Gait Scale) were assessed before and 1, 2, and 4 mos after intervention. Participants were classified as poor or good responders, according to the Goal Attainment Scale response at 2 mos.

RESULTS: Good response was noted in 23 (64%) children and 29 (59%) legs, whereas 13 (36%) children and 20 (41%) legs were defined as poor responders. In a multivariate regression analysis, the best predictors of good response per child were higher initial Observational Gait Scale total scores, one-level treatment, and normal cognition; and the best predictors per leg were good initial selective motor control in the ankle and capability of occasional flatfoot contact at pretreatment.

CONCLUSIONS: These results suggest that children with less severe functional deficit, fair to good selective motor control in the ankle, and mild equinus gait respond best to focal botulinum toxin type A treatment, with an eye to flatfoot or heel strike contact. Likewise, children with higher cognitive level seem to derive functional benefit from the treatment.

Objective To evaluate the relationships among cerebral palsy (CP) phenotypes and bronchopulmonary dysplasia (BPD) severity and, in the process, to generate hypotheses regarding causal pathways linking BPD to CP. Study design We studied 1047 infants born before the 28th week of gestation. Receipt of supplemental oxygen at 36 weeks postmenstrual age (PMA), with or without the need for mechanical ventilation (MV) at 36 weeks PMA, defined two levels of BPD. At 24 months, the children underwent neurologic examinations and CP diagnoses were made using an algorithm based on topographic localisation. Results The 536 infants with BPD were at increased risk of all three CP phenotypes. In time-oriented multivariable analyses that adjusted for potential confounders, receipt of supplemental oxygen without MV at 36 weeks PMA (BPD) was not associated with increased risk of any CP phenotype. In contrast, BPD accompanied by MV at 36 weeks PMA (BPD/MV) was associated with a nearly sixfold increased risk of quadriparesis and a fourfold increased risk of diparesis. Conclusions Combined treatment with both MV and supplemental oxygen at 36 weeks PMA strongly predicts the more common bilateral CP phenotypes. BPD without MV at 36 weeks PMA was not significantly associated with any form of CP.

We developed a secure, web-based system for parent-professional and inter-professional communication. The aim was to improve communication in the care of children with cerebral palsy. We conducted a six-month trial of the system in three Dutch health-care regions. The participants were the parents of 30 cerebral palsy patients and 120 professional staff involved in their care. Information about system usage was extracted from the system’s database. The experience of the parents and professionals was evaluated by a questionnaire after six months. The system proved to be technically robust and reliable. A total of 21 parents (70%) and 66 professionals (55%) used the system. The parents submitted 111 questions and 59 responses, with a mean of 5 questions (range 1-17) and 3 responses (range 1-9) per parent. The professionals submitted 79 questions and 237 responses, with a mean of 2 questions (range 1-8) and 4 responses (range 1-23) per professional. Most parents (95%) and some professionals (30%) reported value in using the system, which ranged from efficiency and accessibility to flexibility and transparency. The web-based communication system was technically feasible and produced improved parent-professional and inter-professional communication. It may be especially valuable if frequent interventions or consultations about a child’s care are required, involving complex care networks of different professionals and organisations.

ABSTRACT This systematic review focused on the common conventional physiotherapy interventions used with children with cerebral palsy (CP), aged 4 to 18 years, and critically appraised the recent evidence of each of these interventions using the Oxford Centre for Evidence-Based Medicine Levels of Evidence. The search strategy yielded 34 articles after inclusion and exclusion criteria were applied. The investigated physiotherapy interventions included strength and functional training, weight-supported treadmill training (WBSTT), and neurodevelopmental treatment (NDT). A category of treatment dosage was also included. Strength training was the most studied intervention with significant improvements found in the strength of selected muscle groups using dynamometry, with fewer studies showing significant improvement in function. Functional training showed improvements in gross motor function, endurance, and temperospatial measures, such as gait speed and stride length. Nonsignificant trends of improvement on the Gross Motor Function Measure (GMFM) and gait velocity were found for WBSTT by a few studies with low levels of evidence (case series). Of three studies that evaluated NDT, one high-level evidence study, i.e., randomized controlled trial (RCT) found significant improvements on the GMFM. All studies reviewing treatment dosage had high levels of evidence (RCTs), yet found no significant differences for different intensities of treatment. These results indicate that the levels of evidence for physiotherapy interventions, particularly strengthening and to a lesser extent functional training, in school-aged children with CP has improved; however, further high-level evidence is needed for other interventions.

ABSTRACT The Pediatric Evaluation of Disability Inventory (PEDI) assesses functional status in children with disabilities aged 0.5-7.5 years. The purpose of this study was to examine if the Danish version of the PEDI was able to discriminate between nondisabled children and children with cerebral palsy (CP) or juvenile idiopathic arthritis (JIA). Convenience sampling was used to select the clinical samples that consisted of 22 children with CP and 14 children with JIA aged 1.5-7.5 years. An additional sample, consisting of 224 nondisabled children aged 1.0-5.9 years, was obtained on the basis of random sampling. The PEDI was administered as a questionnaire completed by the parents. Results from receiver operating characteristics (ROC) analysis showed that in the case of discrimination between children with CP and nondisabled children, the sensitivity and specificity were 77.3 and 97.8%, respectively. In the discrimination between children with JIA and nondisabled children, the sensitivity and specificity were 99.8 and 81.7%, respectively. Study results indicate that the Danish version of the PEDI shows good discriminative validity. Further studies of the discriminative validity are recommended.