Cerebral Palsy

Purpose. Motor skills can be learned in an explicit or an implicit manner. Explicit learning places high demands on working memory capacity, but engagement of working memory is largely circumvented when skills are learned implicitly. We propose that individuals with impaired movement dynamics may benefit from implicit learning methods when acquiring sports-related motor skills. Method. We discuss converging evidence that individuals with cerebral palsy and children born prematurely have compromised working memory capacity. This may in part explain the difficulties they encounter when learning motor and other skills. We also review tentative evidence that older people, whose movement dynamics deteriorate, can implicitly learn sports-related motor skills and that this results in more durable performance gains than explicit learning. Results. Individuals with altered movement dynamics and compromised working memory can benefit from implicit motor learning. Conclusion. We conclude with an appeal for more extensive evaluation of the merits of implicit motor learning in individuals with impaired movement dynamics.

Purpose. The aim of this study was to describe the physical activity levels of 5- and 7-year-old children with cerebral palsy (CP, n = 97), to compare their physical activity levels with those of typically developing peers (TD, n = 57) and the Dutch recommendation for physical activity, and to investigate the associated factors. Method. The level of physical activity (hours spent on sports and physical activity per week) and contextual factors were assessed with standardised questionnaires. Results. Mean duration of self-reported physical activity for children with CP was 3.4 (+/- 1.9) h/week, which was significantly less than the 5.8 (+/- 2.3) h/week for TD-peers. Ninety-three percent of the children with CP were insufficiently physically active according to the Dutch recommendation for physical activity. Multiple regression analyses showed that younger age and lower educational level of the mother were significantly associated with lower levels of physical activity for children with CP, while severity of CP was not associated with physical activity levels. Twenty-two percent of the parents reported that more facilities in sport and games are required for children with CP. Conclusion. Physical activity is low in young children with CP and needs to be promoted at an early stage.

BACKGROUND: This study was performed to identify the issues of concern of parents of patients with cerebral palsy before single event multilevel surgery (SEMS). METHODS: The parental concerns of 64 patients [34 males, 30 females, mean age 15 y and 5 mo (SD 8 y and 10 mo)] with cerebral palsy were obtained using a questionnaire preoperatively. The issues of parental concern were evaluated using 59 items specifically designed for this study. The individual items were scored using a 5-point Likert scale (1 to 5). RESULTS: The top 5 issues of concern were as follows: postoperative rehabilitation, duration of rehabilitation, immediate postoperative pain, general anesthesia, and medical cost. Sporting activities and poor compliance to wearing orthosis were included in the top 5 issues of parental concern in patients with unilateral involvement. The overall parental concern in patients with unilateral involvement was lower than that in bilateral involvement (P=0.054). Parents of younger patients showed significantly higher concern score than those of older patients (P=0.020). There was no significant difference in the overall concern score between the parents of patients scheduled for bony procedures and those of patients scheduled for soft tissue procedures (P=0.298). Multiple regression analysis revealed the patient’s age (P=0.018) and responding parent (father vs. mother, P=0.025) to be the factors that significantly affected the overall concern score (adjusted R(2)=0.281). CONCLUSIONS: An understanding of the issues of concern will enhance communication between physicians and parents, which would assist in preoperative discussion. LEVEL OF EVIDENCE: Diagnostic level II.

BACKGROUND: Percutaneous adductor longus tenotomy (PAT) is a frequently used procedure, yet no study has ever compared its effectiveness and safety with those of open adductor longus tenotomy (OAT). We conducted this prospective study to describe the effects of PAT and to compare them with those of OAT. METHODS: This consisted of a cross-over randomized controlled trial including 50 consecutive hips from 27 patients with cerebral palsy scheduled for adductor tenotomy in the setting of multilevel tendon lengthening/release procedures or hip surgery (femoral or Dega osteotomy) in a university hospital. A pediatric orthopaedic surgeon conducted a PAT. Another surgeon extended the wound to explore what had been cut during the PAT, and completed the tenotomy if necessary. Hip abduction (HA) was assessed by a third surgeon immediately before PAT, after PAT, and then after OAT, using a goniometer, in a standardized reproducible manner. All 3 surgeons were blinded to the others' findings. Primary end-points included the percentage of tendon/muscle portion sectioned percutaneously, and the HA measure. Comparison between HA after PAT and OAT was done using a paired t-test with a 95% confidence interval. The influence of anatomic variants of adductor longus origin was also assessed. RESULTS: Mean HA (hips flexed) measured 40.36 degrees preoperatively and increased to 50.04 degrees after PAT (P<0.0001). After OAT, HA averaged 53.32 degrees with no statistical gain compared with that observed after PAT (P=0.2). The tendinous portion of adductor longus was cut to an average of 98% by PAT (completely in 46 cases and more than 75% in only 4 cases). The muscular portion of adductor longus origin was cut to an average of 83.7% (completely in only 15 cases, cut to more than 75% in 26 cases, and approximately 50% in 9 cases). The gain in HA positively correlated with the extent of the tendinous portion divided (P=0.03) but not with the extent of muscular portion divided. Results were independent of the anatomic variants of adductor longus origin. Partial section of adductor brevis after PAT was encountered in 6 cases. No major iatrogenic lesion was observed (obturator nerve, major vessels). CONCLUSIONS: This is the only prospective study concerning the effects of PAT. The anatomic factor associated with gain in HA seems to be the extent of the section of the tendinous portion of adductor longus origin, which was found to be cut to more than 90% in all cases after PAT. The extent of muscular portion section does not seem to influence the gain in HA. The researchers detail the technique of percutaneous adductor tenotomy and show that when done correctly, PAT is a fast and simple procedure, as reliable and effective as the open release and without any major risks. LEVEL OF EVIDENCE: Level II therapeutic study-prospective comparative study.

BACKGROUND: The aim of this study was to examine both the tendon and muscle components of the medial gastrocnemius muscle-tendon unit in children with cerebral palsy (CP) and equinus gait, with or without contracture. We also examined a small number of children who had undergone prior surgical lengthening of the triceps surae to address equinus contracture. METHODS: Ultrasound was used to measure Achilles tendon length and muscle-tendon architectural parameters in children of ages 5 to 12 years. Muscle and tendon parameters were compared among 4 groups: Control group (N=40 limbs from 21 typically developing children), Static Equinus group (N=23 limbs from 15 children with CP and equinus contracture), Dynamic Equinus group (N=12 limbs from 7 children with CP and equinus gait without contracture), and Prior Surgery group (N=10 limbs from 6 children with CP who had prior gastrocnemius recession or tendo-achilles lengthening). The groups were compared using analysis of variance and Scheffe post hoc tests. RESULTS: The CP groups had longer Achilles tendons and shorter muscle bellies than the Control group (P<0.001). Normalized tendon length was also longer in the Prior Surgery group compared with the Static Equinus group (P<0.001). The Prior Surgery group had larger pennation angles than the CP groups (P< or =0.009) and tended to have shorter muscle fascicle lengths (P< or =0.005 compared with Control and Static Equinus, P=0.08 compared with Dynamic Equinus). Similar results were observed for pennation angles and normalized muscle fascicle lengths throughout the range of motion. CONCLUSIONS: Children with spastic CP and equinus gait have longer-than-normal Achilles tendons and shorter-than-normal muscle bellies. These characteristics are observed even in children with dynamic equinus, before contracture has developed. Surgery further lengthens the tendon, restoring dorsiflexion but not normal muscle-tendon architecture. These architectural features likely affect function, possibly contributing to functional deficits such as plantarflexor weakness after surgery. LEVEL OF EVIDENCE: Level II, prospective comparative study.

BACKGROUND: Hamstring stretching is an integral component in the treatment for knee flexion contracture in cerebral palsy (CP). As hamstrings span across hip and knee joints, passive stretching of hamstrings applies force to the hip that is often dysplasia in CP. The purpose of this study is to measure the dynamic displacement of femoral head produced by passive stretching of hamstrings and to determine the factors associated with the phenomenon. METHODS: Children with spastic CP were studied using computerized tomography (CT) of the pelvis. Two sets of CT studies were carried out, one with the knee flexed and the hip flexed (resting) and the other with the knee extended and the hip flexed to simulate manual hamstrings stretching. The distance from pelvic baseline to the posterior margin of femoral epiphysis was measured on the CT images and compared between resting and stretching for dynamic displacement. The dynamic displacement of the femoral head was expressed by a ratio to femoral epiphysis diameter. RESULTS: Twenty-seven CP children had CT studies at a mean age of 6.8 years (range: 4.5 to 9.6 y). Ten children were ambulators with or without devices and the other 17 children were nonambulators. On plain radiographs of the pelvis, the mean Reimer's migration percentage was 39% (range: 13% to 92%). On CT scan, dynamic displacement by stretching was 4.7% (range: -3.8% to 16.1%) of femoral epiphysis diameter (P<0.001 by paired t test). The displacement in the 33 hips with migration percentage of greater than 30% was significantly greater than the displacement in the other 21 hips with migration percentage of less than 30% (7.4% vs. 0.5%, P=0.001). CONCLUSIONS: Passive stretching of spastic hamstrings in the hip flexion position resulted in dynamic posterior displacement of the femoral head. Medical professionals should be aware of this fact, especially in spastic CP children with a higher migration percentage on radiograph. LEVEL OF EVIDENCE: Diagnostic II.

Gillette Gait Index (GGI) is a very useful tool to assess gait abnormalities. However, it seems that it has only been validated in children with cerebral palsy. Nevertheless, the parameters used to compute GGI are not specific to children population. Our aim is to demonstrate that GGI could also be used to evaluate adults gait abnormalities. 44 adults (25 healthy and 19 pathological) participated to this study. Pathological subjects had a diagnosis of central nervous system pathology (6 with spinal cord injury and 13 with brain injury). We first, compared the kinematic parameter values of our healthy adult group to healthy children group in previous studies. It appears that those parameters’ variability is a bit lower in adults, which makes the GGI more sensitive. Moreover, the GGI in adults is too much dependent on one parameter among the 16 proposed by Schutte et al. (2000), the “Time of Peak Flexion”. Finally, the Edinburgh Visual Gait Score (EVGS) is correlated to GGI in children. To emphasize the relevance of GGI in adults, we have evaluated the correlation between EVGS and GGI in our pathological group. Those two parameters are indeed highly correlated. All these results allow us to conclude that the GGI computed with the 15 remaining parameters is a useful tool to assess gait abnormalities in adults. Copyright © 2010 Elsevier B.V. All rights reserved.

ABSTRACT: BACKGROUND: Knowledge of sitting and standing performance in a total population of children with cerebral palsy (CP) is of interest for health care planning and for prediction of future ability in the individual child. In 1994, a register and a health care programme for children with CP in southern Sweden were initiated. In the programme information on how the child usually sits, stands, stands up and sits down, together with use of support or assistive devices, is recorded annually. Material and method A cross-sectional study was performed, analysing the most recent report of all children with CP born 1990-2005 and living in southern Sweden during 2008. All 562 children (326 boys, 236 girls) aged 3-18 years were included in the study. The degree of independence, use of support or assistive devices to sit, stand, stand up and sit down was analysed in relation to the Gross Motor Function Classification System (GMFCS), CP subtype and age. Result A majority of the children used standard chairs (57%), could stand independently (62%) and could stand up (62%) and sit down (63%) without external support. Adaptive seating was used by 42%, external support to stand was used by 31%, to stand up by 19%, and to sit down by 18%. The use of adaptive seating and assistive devices increased with GMFCS levels (p < 0.001) and there was a difference between CP subtypes (p < 0.001). The use of support was more frequent in preschool children aged 3-6 (p < 0.001). CONCLUSION: About 60% of children with CP, aged 3-18, use standard chairs, stand, stand up, and sit down without external support. Adding those using adaptive seating and external support, 99% of the children could sit, 96% could stand and 81% could stand up from a sitting position and 81% could sit down from a standing position. The GMFCS classification system is a good predictor of sitting and standing performance.

Aim The aim of this study was to assess the rate of fracture before and after a 1-year course of intravenous pamidronate in children with spastic quadriplegic cerebral palsy (CP) who had previously experienced fractures. Method Twenty-five children (nine males, 16 females) with quadriplegic CP in Gross Motor Function Classification System (GMFCS) level IV or V who were treated with intravenous pamidronate for approximately 1 year were identified. All participants had previously experienced at least one non-traumatic fracture. Each received 15 doses of pamidronate over a mean of 13.6 months. Post-treatment observation ranged from 1 to 10 years 6 months (mean 4y 1mo). The fracture rate before and after commencement of treatment was calculated using the person-years method. Results The participants had experienced a total of 86 fractures before treatment began, occurring over 280.6 person-years, giving a fracture rate of 30.6% per year. During the post-treatment observation period, totalling 107.5 person-years, 8 of the 25 children experienced a total of 14 fractures. This fracture rate of 13.0% per year is a statistically significant decrease (p=0.02). Interpretation Pamidronate treatment lowered the rate of fracture, and a 1-year course appears to provide a protective effect after treatment ends. For the majority of participants, this effect lasted 4 years or longer. However, a subset of children suffered a fracture soon after the drug was discontinued. In these children, a longer course of treatment appears to have been necessary.

We describe a 78-year-old Japanese woman with early-stage progressive supranuclear palsy (PSP). She had a 3-week history of postural instability and gait disturbance. On examination, upper vertical gaze palsy, akinesia, hyperreflexia with pathological reflexes, hesitation, and postural instability were observed. Rigidity and resting tremors were not apparent. Brain MRI revealed atrophy of the frontotemporal lobes and dilatation of the third ventricle. A month later, she died of cerebral infarction. The total duration of her clinical course was approximately 2 months. The brain weighed 1180 g after fixation. Macroscopically, mild atrophy of the frontal lobes and mild depigmentation of the substantia nigra were observed. The conspicuous findings included degeneration confined to the subthalamic nucleus and substantia nigra and widespread but infrequent tau-positive neurofibrillary tangles/pretangles and glial fibrillary tangles (tuft-shaped astrocytes, coiled bodies and argyrophilic threads) in the brain. It has been reported that the most affected areas in PSP are the globus pallidus, subthalamic nucleus and substantia nigra. We suggest that degeneration in PSP would start with involvement of the substantia nigra and subthalamic nucleus.