Volume 90, Issue 6 , Pages 994-1003, June 2009
Functional Status of Patients With Cerebral Palsy According to the International Classification of Functioning, Disability and Health Model: A 20-Year Follow-Up Study After Selective Dorsal Rhizotomy
Article Outline
- Abstract
- Methods
- Results
- Discussion
- Conclusions
- Appendix 1
- Appendix 2
- References
- Copyright
Abstract
Langerak NG, Lamberts RP, Fieggen AG, Peter JC, Peacock WJ, Vaughan CL. Functional status of patients with cerebral palsy according to the International Classification of Functioning, Disability and Health Model: a 20-year follow-up study after selective dorsal rhizotomy.
Objective
To determine functional status of patients with cerebral palsy 20 years after they received selective dorsal rhizotomy (SDR).
Design
A prospective 20-year follow-up study.
Setting
Red Cross Children's Hospital (SDR operation and 1-year follow-up assessment) and at institutional or private locations nearby patients' homes (20-year follow-up assessment).
Participants
Referred sample of 14 patients with spastic diplegia (6 women, 8 men; mean age, 27y; range, 22–33y) who were preoperatively ambulant and fulfilled strict selection criteria for SDR operation in 1985.
Interventions
Patients were assessed before and 1 and 20 years after SDR.
Main Outcome Measures
Standardized assessments of function according to 2 dimensions of the International Classification of Functioning, Disability and Health (ICF) model: (1) body structure and function (muscle tone, joint stiffness, voluntary movement) and (2) activity (rolling, sitting, kneeling, crawling, standing, walking, transitions) were obtained. In addition, based on assessments and questionnaires, Gross Motor Function Classification System (GMFCS) levels were determined before and at 1 year after SDR retrospectively and currently at 20 years after SDR.
Results
One year after SDR, functional outcomes based on the 2 dimensions of the ICF model improved significantly, and these improvements were maintained at 20 years after surgery. Patients showed a shift in their GMFCS levels 1 and 20 years after SDR.
Conclusions
In line with our 20-year follow-up study with gait parameters as outcome measures, patients with spastic diplegia still show improvements in their functional status 20 years after SDR. We acknowledge the presence of possible confounding factors and a small sample size, but we argue that the improvements found in this study were caused mainly by SDR. Finally, changes in GMFCS levels suggest a possible role for this tool to detect changes after an intervention.
Key Words: Adult, Cerebral palsy, Follow-up studies, Rehabilitation, Rhizotomy, Spastic diplegia
List of Abbreviations: CP, cerebral palsy, GMFCS, Gross Motor Function Classification System, GMFM, Gross Motor Function Measure, ICF, International Classification of Functioning, Disability and Health, SDR, selective dorsal rhizotomy
DURING THE PAST few decades, the prevalence of CP has risen in developed countries from 1.5 to 2.5 per 1000 live births, and these patients now constitute the largest diagnostic group in pediatric rehabilitation.1 About 80% of these children are diagnosed with spasticity,1, 2 a condition that may disturb the normal growth process and can lead to secondary abnormalities such as deformities and pain.3, 4, 5, 6 Therefore, it is important to treat spasticity in these children to facilitate their optimal physical development.
One of the possible treatment options to reduce spasticity in children with CP is SDR. In the early 1980s, Peacock and Eastman7 reintroduced and refined this neurosurgical procedure in Cape Town, South Africa. During this operation, laminectomies are performed from L2 to S1, which exposes the cauda equina. After separating the dorsal roots from the anterior roots, the dorsal roots are electronically stimulated, and the electromyographic response of the corresponding muscle is recorded. Those dorsal nerve rootlets associated with a normal electromyographic response are left intact, whereas those associated with an abnormal response are divided. By transecting a percentage of dorsal lumbar sacral rootlets, the myotactic reflex arc is disrupted, which decreases muscle spasticity in the lower extremity.
Because the manifestation of CP and spasticity is complex, the ICF model, which was developed by the World Health Organization in 2001,8 can be used to quantify the outcomes and determinants of CP.4, 9, 10, 11 Steinbok12 published an extensive review of 63 articles that examined the outcomes of SDR in 2001 and reanalyzed his findings in 2007 in the context of the ICF model.13 All these studies supported the finding that SDR decreases spasticity and increases joint range of motion in the lower extremity (ICF model dimension: [1] body structure and function). Strong, although inconclusive, evidence was found for improvements in motor function (ICF model dimension: [2] activity), whereas only moderate evidence was found for a reduction in the level of disability (ICF model dimension: [3] participation).12, 13
Although these findings are promising, most conclusions are based on follow-up studies not longer than 5 years after SDR. Because the refined SDR operations were first performed in Cape Town, we are in the unique position of being able to provide insight into the long-term sequelae of this neurosurgical procedure.14 As a result, we recently published a 20-year follow-up study that showed the long-term benefits of SDR in locomotor function.15 Although gait analysis is a reliable and objective research method, there is nevertheless a demand for more detailed long-term functional outcomes after SDR. In an attempt to provide a complete picture of these patients' function, we conducted, in line with our 1-year follow-up study,16, 17 functional assessments 20 years after SDR. Accordingly, the aim of this study was to determine the functional status based on the following ICF model dimensions: (1) body structure and function and (2) activity in a group of patients with spastic diplegia who had already shown a continued improvement in their gait pattern 20 years after SDR.
Methods
Participants
The selection of participants was based on the following criteria: (1) diagnosed with spastic diplegia of congenital origin, (2) fulfilled strict selection criteria for SDR, (3) received SDR by a single neurosurgeon (W.J.P.) at the Red Cross Children's Hospital in Cape Town (South Africa) in 1985, (4) had access to intensive physiotherapy before and after surgery, and (5) ambulant preoperatively.
Fourteen patients met the inclusion criteria and had participated in the original 1-year follow-up study.16, 17 After an extensive search because of the absence of contact details and a nonexisting national CP registration system, we tracked down and contacted each of the 14 patients in 2005. Before entering the study, the aim and purpose of the research project was clearly explained to each patient, after which an informed consent was signed. This study was approved by the Human Ethics and Research Committee of the Faculty of Health Sciences of the University of Cape Town.
Procedures
Preoperatively in 1985, 2 days before surgery, a single occupational therapist assessed the participants' neuromuscular and functional status in a clinical room at the Red Cross Children's Hospital. In 1986, on average 1 year after surgery, the same investigator conducted the reassessments of each child by using the same protocol.16, 17
Because the SDR operation was performed on patients from all over South Africa, 8 of the 14 participants were not living in or close to Cape Town 20 years after SDR (in 2005). These patients were visited and tested either at home or at a suitable location nearby. The assessments were repeated by a single physiotherapist (N.G.L.) by using the original protocol.16 A second physiotherapist (R.P.L.) who recorded all assessments on video tape was consulted when a clear judgment could not be made by the principal researcher (N.G.L.). After the assessment, the videotape of each patient was reviewed by the 2 experienced physiotherapists (N.G.L., R.P.L.) to confirm the final scores chosen. The 2 therapists were blinded from any outcomes of previous studies during this process.
Assessment Tools
International Classification of Functioning, Disability and Health model: body structure and functionIn line with the original 1-year follow-up study, an ordinal assessment scale was used to quantify outcomes of the first dimension of the ICF model, body structure and function, which included the following items:16 for muscle tone, the limb was moved passively in a direction opposite to the action of the muscle group being tested, and the resistance to passive stretch was noted. This outcome measure is comparable to the Modified Ashworth Scale. However, outcomes of the muscle tone parameter were not identical and so it was not possible to convert to this worldwide accepted assessment tool. In appendix 1, the defined scores for muscle tone are presented in detail (scale from low [1] and normal [2] to most severe increase [5] in muscle tone).
To measure joint stiffness, each lower-limb joint was tested passively by moving the limb through the maximum range of movement and compared with the full range of joint movement in a normal limb. The score for this parameter was based on the maximum passive range of motion achieved, taking into account the manner wherein this movement was obtained. The score for joint stiffness varied from normal (1) to total limitation like an apparent fixed contracture (4) (see appendix 1).
For voluntary movement measurement, the patient was asked to stop and start a movement 8 times within his/her full available range of movement, counting with each movement. The patient was positioned to eliminate the effects of gravity. The joints proximal to the movement were stabilized in a position to inhibit mass patterns as far as possible.
Outcomes of voluntary movement consist of a combined assessment of selective motor control, muscle strength, and joint range of motion (scale from normal [1] to most severe limitation [5] [see appendix 1]).
Muscle tone, joint stiffness, and voluntary movement assessments were measured for the following 10 movements: hip flexion, extension, abduction, and adduction; knee flexion and extension; and ankle inversion, eversion, plantar, and dorsiflexion. To quantify the total outcome of each item optimally, a mean score for all 10 movements was calculated for both the right and left legs.
International Classification of Functioning, Disability and Health model: activityTo quantify the functional outcomes according to the second dimension of the ICF model, activity, the following item of the original ordinal assessment scale16 was used: the quality of functional movement was tested by asking the patient to assume and maintain various positions including rolling, long and side sitting, prone and half kneeling, kneel standing, crawling, standing, and walking. This assessment tool bears some resemblance to the well-known GMFM, which was developed in the early 1990s after our 1-year follow-up study. Therefore, we did not apply the GMFM outcome measures, but rather we used the comparable functional movement assessment tool as described by Berman.16 (scale from normal [1] to most severe limitation [5] [appendix 2]).
Each of these developmental movements allows researchers and clinicians to quantify a patient's ability to dissociate and align his/her body. Therefore, the outcomes of the functional movements were determined separately and as an overall mean for all 9 movements.
Questionnaire and Gross Motor Function Classification System
In addition to the primary functional assessments, a questionnaire was completed with the following topics: medical interventions received, current medical issues, use of assistive devices, employment and/or student status, and problems with daily activities.
Based on the current questionnaire and the assessment outcomes, each participant was classified into the GMFCS in 2005.18, 19 Although retrospective classification into GMFCS levels has not yet been validated, we used a detailed questionnaire plus the original clinical reports and the functional assessments from 1985 and 1986 to assign GMFCS levels for our 14 participants before and 1 year after SDR.
The GMFCS categorizes patients with CP, based on their gross motor performance, into levels ranging from level I (minor limitations) to level V (severe limitations).18 Although it is debatable whether the GMFCS can be used to measure changes over time,20 we decided to use this classification system to show a possible shift in gross motor function after SDR, as was done by Cole et al.21
Statistical Analysis
We used the nonparametric Friedman test to determine the influence of SDR over time for each of the functional outcome measures. If statistical significance between the preoperative and postoperative studies was reached, we used the nonparametric Wilcoxon matched-pairs test to determine where the significant difference appeared. Because the outcome measures were analyzed at 3 different time points (preoperatively and at 1 and 20 years postoperatively), this analysis resulted in 3 different paired comparisons. Bonferroni correction for multiple comparisons was used, which resulted in a P value of .05/3 equal to .017 that was used to determine statistical significance.22
Results
Characteristics of Participants
The mean age of the 14 patients with spastic diplegia was 28±4 years (range, 22–33y) 20 years after SDR. The cohort consisted of 6 women and 8 men from all socioeconomic backgrounds and races. Current GMFCS levels in the participants ranged from GMFCS level I (n=7), level II (n=3), level III (n=3), to level IV (n=1).The cognitive function of the participants before SDR varied from moderate (n=3) to mild (n=11) impairment. All participants attended a specialized school for children with CP and received intense physiotherapy (and if necessary occupational therapy) before and after surgery.
International Classification of Functioning, Disability and Health Model: Body Structure and Function
Muscle tone, joint stiffness, and voluntary movement scores all showed a significant difference when comparing before and after SDR (Friedman test, P<.001). As shown in the box plots (fig 1), the scores for these parameters improved as represented by a decrease in each score. The median score for muscle tone was significantly reduced from 3.1 to 2.1 at 1 year after surgery and to 2.0 at 20 years after SDR, which is equivalent to normal muscle tone (table 1, fig 1A). In addition, the median joint stiffness score was significantly reduced, from 1.9 to 1.2, at 1 year after surgery, and this improvement was maintained with a median score of 1.3 at 20 years after surgery (see table 1, fig 1B). The voluntary movement score was significantly decreased from a median score of 3.6 before surgery to 2.3 at 1 year after surgery and decreased further, after 20 years, to a median score of 1.9, which approached a normal score of 1.0 (see table 1, fig 1C). As presented in table 1, none of the outcome measures of the ICF model dimension body structure and function showed a significantly different score between 1 and 20 years after SDR. Note that the highest scores for muscle tone (2.8), joint stiffness (3.4), and voluntary movement (3.6) 20 years after SDR represented a single participant who was classified at GMFCS level IV.
Fig 1.
Outcome measures for the ICF dimension body structure and function before and at 1 and 20 years after surgery. Box plots show (A) muscle tone, (B) joint stiffness, and (C) voluntary movement, whereas whiskers represent the minimum and maximum values, excluding outliers. Outliers are values more than 1.5 box lengths from the upper or lower edge of the box and shown as circles. Note: the median of muscle tone scores at 1 and 20 years postoperatively are 2.1 and 2.0, respectively.
Table 1. P Values From Wilcoxon Matched-Pairs Test Tabulated for Scaled Scores of Neuromuscular and Functional Outcomes
| Outcome Measure | P | ||
|---|---|---|---|
| Pre-1y | Pre-20y | 1y-20y | |
| Muscle tone | <.001⁎ | <.001⁎ | .859 |
| Joint stiffness | .001⁎ | .019 | .972 |
| Voluntary movement | .001⁎ | .002⁎ | .021 |
| Functional movement | <.001⁎ | <.001⁎ | .328 |
 Long sitting | .001⁎ | .001⁎ | .074 |
| Side sitting | .005⁎ | .074 | .937 |
| Prone kneeling | .008⁎ | .005⁎ | .361 |
| Kneel standing | .001⁎ | .002⁎ | .463 |
| Half kneeling | .001⁎ | .003⁎ | .024 |
| Standing | .012⁎ | .028 | 1.000 |
| Rolling | .005⁎ | .004⁎ | .463 |
| Crawling | .028 | .008⁎ | .142 |
| Walking | .008⁎ | .050 | .208 |
⁎Significant difference achieved by a P value of 0.05/3 = 0.017. |
International Classification of Functioning, Disability and Health Model: Activity
The mean functional movements scores and the 9 separate assessment scores all showed a statistical effect in time (Friedman test, P<.001). The box plots show an improvement with a reduced score approaching the normal level of 1.0 at 1 and 20 years after surgery (fig 2). The preoperative scores decreased significantly from 3.1 to 1.9 and 1.8 at 1 and 20 years after surgery, respectively (see table 1). Figure 2 provides an overview of the median scores of the 9 different assessments before and after SDR. Note that the outlier with a mean functional movement score of 3.7 represented the participant who was classified at GMFCS level IV 20 years after SDR.
Fig 2.
Outcome measures for the ICF dimension activity before and at 1 and 20 years after surgery. (A) Box plots show 1st, 2nd (median), and 3rd quartiles, whereas whiskers represent the minimum and maximum values, excluding outliers. Outliers are values more than 1.5 box lengths from the upper or lower edge of the box and are shown as circles. (B) Line plots of median scores of 9 functional movements before and at 1 and 20 years after surgery.
The assessment of long sitting, prone kneeling, kneel standing, half kneeling, and rolling resulted in significant improvements at 1 year and 20 years after surgery (see table 1). The median score for crawling at 1 year after surgery was reduced compared with preoperative values, although not significantly. Twenty years after SDR, the score was further decreased, which resulted in a significant difference when compared with preoperative values. Side sitting, standing, and walking assessments resulted in a significantly different score 1 year after surgery, but 20 years later the score was not significantly improved when compared with the values before dorsal rhizotomy. None of the 9 movement scores changed significantly between 1 and 20 years after SDR (see table 1). Figure 3 shows one of the participants demonstrating long sitting before surgery and at 1 year and 20 years after surgery. These images show improved hip flexion and knee extension and better control of the head and trunk after dorsal rhizotomy.
Fig 3.
Patient with CP showing 1 of the functional movement outcome measures: long sitting before surgery (A), at 1 year (B), 20 years (C) after surgery.17
Questionnaire and Gross Motor Function Classification System
After surgery, 9 participants received at least 1 orthopedic surgery procedure, including muscle releases (Achilles' tendon: n=2, hamstring: n=3, rectus femoris: n=1) and/or osteotomies (foot: n=6, femur: n=1). However, none of them received an intrathecal baclofen pump or botulinum toxin injections, and only 1 participant (classified at GMFCS level IV) occasionally used oral antispasmodic medication after SDR.
Twenty years after SDR, 11 participants walked in- and outdoors independently, whereas 2 participants walked with a cane or crutch, and only 1 participant (classified at GMFCS level IV) became a wheelchair user. All participants reported problems with holding their balance, whereas the majority (n=10) experienced regular back pain and 1 person had a bladder dysfunction (which was controlled by medication). However, all participants answered “no” to the question “Do you need help with activities of daily living?” Eleven of the 14 participants were currently employed or were studying at college, whereas 5 actively participated in physical exercise.
Figure 4 provides an overview of the GMFCS levels of the 14 participants before and at 1 and 20 years after SDR. One year after surgery, 10 participants were classified at a lower GMFCS level (functional improvement), whereas 4 remained at the same level. Between 1 year and 20 years after SDR, 2 participants deteriorated from level II to III, 1 participant deteriorated from level III to IV and 1 participant improved from level II to I. In 2005, 20 years after surgery, 9 participants were still classified in a lower GMFCS level compared with their levels before SDR, whereas 4 participants remained unchanged and 1 participant deteriorated from GMFCS level III to IV.
Fig 4.
Distribution of GMFCS levels per patient (n=14) before and at 1 and 20 years after surgery.
Discussion
A modification of SDR was introduced in Cape Town in the early 1980s7, and became widespread after the relocation of Warwick Peacock from South Africa to Los Angeles in 1986. During the past 2 decades, dorsal rhizotomy has become an accepted method to release children with CP from their spasticity, and it has produced a positive influence in patients' function, activities, and participation in daily living during the first few years after surgery.12, 13 Besides our 20-year follow-up studies, no other studies have been performed to determine the long-term effects of SDR on patients with CP. In line with our gait study,15 outcomes of functional assessments based on the 2 dimensions of the ICF model, body structure and function and activity, showed improved values 20 years after SDR compared with preoperative values.
Outcomes Related to the 3 Dimensions of the International Classification of Functioning, Disability and Health Model
With regards to the first dimension of the ICF model, body structure and function, numerous studies have shown improvements in neuromuscular outcome measures after SDR. Steinbok12 concluded in his review article that dorsal rhizotomy results in a significant reduction in muscle tone in the first few years after surgery. Short-term follow-up studies, up to 5 years after SDR, by Gul23 and Mittal24 have shown improvements in muscle tone. In line with these outcomes, the present study shows a significant reduction in muscle tone 1 year after dorsal rhizotomy, which was maintained 20 years after SDR (see fig 1A).
When spasticity is not treated properly, it can lead to secondary abnormalities such as muscle contractures and decreased range of motion.3 Because dorsal rhizotomy reduces muscle tone in the lower limbs, it also improves the range of motion in these joints.12 Gul23 and Mittal24 showed an augmented or at least a stabilized range of motion in lower-limb joints between 1 and 5 years after SDR. Our Cape Town research group, which measured range of motion based on gait analysis, found improvements in range of motion up to 20 years after SDR.15, 25, 26, 27 This latter finding is in line with the current study, which found a decreased joint stiffness (a measure of range of motion during passive movement) 1 year after SDR that was maintained at 20 years after surgery (see fig 1B). This long-term result would appear to be important because a descriptive study in adults with CP who did not receive SDR showed that 77% of a cohort of 77 with spastic diplegia reported contractures in their extremities.5
Voluntary movement, which was assessed in this study, measured a combination of range of motion, motor control, and muscle strength. Steinbok's review article12 showed either an unchanged or increased muscle strength after SDR. A short-term follow-up study by Gul et al23 reported improved muscle strength in hip abductors, dorsiflexors, and hip extensors between 1 and 5 years after SDR in which the muscle strength of the quadriceps remained the same. These findings are in line with the initial and further improvements found in voluntary movements at 1 and 20 years after SDR (see fig 1C).
Related to the second dimension of the ICF model, activity, changes in functional movements were measured after SDR. Steinbok12 concluded in his review article that there is strong, although inconclusive, evidence that dorsal rhizotomy also leads to benefits in functional outcome measures. Short-term follow-up studies showed a significant improvement in GMFM24 and functional tasks and positions23, 24 1 year after SDR, with a further improvement 5 years after surgery. This current study shows that the improvements in 9 functional movements gained 1 year after SDR are maintained at 20 years (see fig 2).
A third dimension of the ICF model, participation, could unfortunately not be quantified in this study. The original questionnaire (used before and 1 year after SDR) only included a limited amount of questions, and so we were not able to get a complete and accurate picture of this dimension. Steinbok,12 however, reported improvements in participation measured through the Pediatric Evaluation of Disability Inventory and FIM for Children after SDR. Mittal et al28 found improvements in participation measured by the Pediatric Evaluation of Disability Inventory 3 and 5 years after SDR. Based on our questionnaire, which was completed in 2005, we noted that none of our 14 patients reported problems with daily activities 20 years after SDR. In addition, we found that 28% of our cohort actively participated in physical training, whereas 86% were employed and 14% were studying. In contrast, Andersson and Mattsson5 reported higher rates of physical training (66%), whereas a lower percentage of employment and study rates were found (47%–64% and 9%, respectively) in a cohort of 77 adults with spastic diplegia who did not receive SDR. However, because our questionnaire was not validated, the results of this part of the study should be interpreted with caution, and no definitive conclusion can be drawn regarding participation.
Gross Motor Function Classification System
The GMFCS system has been validated as a stable classification system in children29 and adults,30 and no changes in GMFCS level are normally expected after an intervention.20 However, our data suggest that this system can possibly measure changes in gross motor function over time after dorsal rhizotomy because 64% of our participants (n=10) improved 1 level in the GMFCS, whereas 29% (n=3) remained unchanged and 1 participant (7%) deteriorated from level III to IV 20 years after SDR (see fig 4). This is in accordance with a study by Cole et al21 who used the GMFCS to show functional improvements in a cohort of 19 patients (17 of whom were diagnosed with diplegia) 18 months after SDR. This cohort (GMFCS level II: n=6, level III: n=10, level IV: n=3) showed an improvement of at least 1 GMFCS level for 79% of their patients, whereas 21% remained at the same level.
Because the primary aim of our study (and the study of Cole) was not to determine if the GMFCS is a reliable and valid tool to measure changes in motor function after an intervention, further research is needed to establish whether the GMFCS could in the future play this important and practical role. In addition, the application of the GMFCS levels retrospectively still requires validation.
Study Limitations
Because this 20-year follow-up study was conducted only on a group of patients with spastic diplegia who were operated in 1985, our sample size was relatively small. Therefore, we were unable to account for confounding factors, which could have influenced our outcome measures. One of these possible factors is further orthopedic surgery after SDR, which is normally performed in 65% of the patients12 and is comparable to our incidence rate of 64%. Therefore, it is impossible to conclude that the improvements we found in neuromuscular outcomes (body structure and function) and functional outcomes (activity) are exclusively caused by dorsal rhizotomy. However, we would argue that SDR played a decisive and critical role in achieving these improvements.
A second limitation of the study is the absence of data with which to compare our results. Our study did not include a control group of patients who did not receive SDR, which would have allowed us to interpret the outcomes from a better perspective. In addition, most studies on adults with CP do not distinguish between the types of CP, which makes it impossible to compare our data with their outcomes. Furthermore, only short-term follow-up studies have been published comparing SDR with other interventions such as orthopedic surgery31 or intrathecal baclofen pump implantation,32 which limited us in comparing our results with other research groups and their long-term outcomes of different interventions.
A third and final limitation of this study was that we had no freedom in the selection of our assessment tools because the preoperative measurements were conducted in 1985. This is nevertheless inherent to long-term follow-up studies, whereas adapted or new assessment tools are always developed over time. The tests and ordinal assessment scales that we used were developed and described in detail by Berman et al16, 17 (and have been reproduced here in the appendices) but have never been tested for validity or reliability. The 2 physiotherapists (N.G.L., R.P.L.) who applied the assessment scales 20 years after SDR were nevertheless in agreement and had confidence in their judgments.
Conclusions
As presented in our 20-year follow-up study that focused on gait parameters, patients with spastic diplegia also showed improvements in their functional status based on 2 dimensions of the ICF model, body structure, and function and activity, 20 years after SDR. Although we could not control for all confounding factors, we argue that most of these improvements were because of SDR. Outcome measures of a third dimension of the ICF model, participation, were not directly measured but rather indications of active participation status found through a questionnaire. Although the GMFCS was originally developed as a prognostic classification tool, our study suggests that the GMFCS might play an important role in detecting changes over time after an intervention.
We encourage researchers to perform further long-term follow-up studies in patients with different types of CP. This would enable the comparison of a variety of treatment options in different diagnostic CP groups, which could ultimately assist clinicians, patients, and parents when considering the most effective treatment option.
Appendix 1
RATING CATEGORIES FOR OUTCOME MEASURE OF INTERNATIONAL CLASSIFICATION OF FUNCTIONING MODEL BODY STRUCTURE AND FUNCTION
| Muscle tone (increase) | |
| 1 Hypotonia | The limb feels floppy and is unable to resist gravity. |
| 2 Normal | A good balance between agonist and antagonist. Movement feels controlled. No resistance to passive movement. |
| 3 Mild | There is slight resistance to passive stretch and there is a slight decrease in joint mobility. The stretch reflex occurs when the muscle is in a lengthened position. |
| 4 Moderate | There is a greater resistance to passive movement than in 3 above and a greater decrease in mobility. The stretch reflex occurs in the middle of the range. |
| 5 Severe | There is a severe increase in resistance to passive stretch and a severe decrease in mobility. The stretch reflex occurs when the muscle is in a shortened position. |
| Joint stiffness (limitation) | |
| 1 Normal | No contracture. The joint can be moved freely and full range is easily attained. |
| 2 Mild | There is some stiffness. This can easily be released by passive movement. |
| 3 Moderate | There is a great deal of stiffness but this can be released with difficulty on passive movement. Full range can be attained. |
| 4 Total | Apparent fixed contracture or fixed contracture. Great limitation to passive movement. Full range of movement of the joint cannot be achieved before surgery but may be achieved after surgery. |
| Voluntary movement (limitation) | |
| 1 Normal | Able to initiate and inhibit (i.e. stop and start) a movement 7 to 8 times during one full range of movement. The movement is smooth. |
| 2 Moderate | Able to initiate and inhibit the movement 5 to 6 times. Can achieve full range, but movement is jerky and mass patterns are present. |
| 3 Moderate | Able to initiate and inhibit the movement 3 to 4 times. Can achieve middle or full range, but movement is not smooth. |
| 4 Moderate severe | Able to initiate and inhibit the movement 1 to 2 times. Movement is jerky or overshoots, or there is total movement and patient uses compensations in attempting the movement. |
| 5 Severe | Unable to initiate and inhibit movement. Range is totally limited, or there is a totally abnormal pattern. |
Appendix 2
RATING CATEGORIES FOR OUTCOME MEASURE OF INTERNATIONAL CLASSIFICATION OF FUNCTIONING MODEL ACTIVITY
| Rolling (limitation) | |
| 1 Normal | The patient can roll with good separation between the shoulders and the pelvis. In moving from prone to supine, the pelvis leads, and from supine to prone, the shoulders lead. There is good separation between the legs. |
| 2 Mild | The patient can roll with some separation between the shoulders and the pelvis. There is some separation of the legs and some pelvic mobility. |
| 3 Moderate | The patient rolls without separation between the shoulders and the pelvis but with dissociation between the legs, or with some degree of separation between the shoulders and the pelvis but without dissociation between the legs. |
| 4 Moderate severe | The patient rolls en bloc, without separation between the legs and without separation between the shoulders and the pelvis. |
| 5 Severe | The patient cannot roll independently or may roll only with facilitation by the therapist. |
| Side-sitting (limitation) | |
| 1 Normal | The patient can assume side-sitting and sits adequately, ie, protraction of the weight bearing hip. Lateral weight shift and elongation of the weight bearing side facilitates lateral righting of the trunk on that side. He is able to free his arms from the support surface. |
| 2 Mild | The patient can assume side-sitting with inadequate weight shift onto the weight bearing side (ie, he sits on both buttocks) due to inability to protract the weight bearing side. He lacks mobility at the pelvis. He can maintain this position. He can free his arms. |
| 3 Moderate | The patient can assume the position as in 2 above but is unstable and cannot maintain it. He may or may not be able to free his arms. |
| 4 Moderate severe | The patient cannot assume the side-sitting position but can maintain it if the therapist provides compression through the shoulder and elbow of the arm on the weight bearing side and assists with weight transference onto the weight bearing side. |
| 5 Severe | The patient can be placed in a side-sitting position but needs a lot of external support, and he cannot maintain this position. |
| Long-sitting (limitation) | |
| 1 Normal | Patient can long-sit independently. No rounding of the back. Good hip flexion and knee extension. He does not require his arms to “hang on.” |
| 2 Mild | The patient can long-sit and uses arm support but can let go momentarily. He long-sits with some rounding of the back. |
| 3 Moderate | The patient long-sits with some rounding of the back, poor hip flexion and leg extension, and some internal rotation of the legs. He can support himself on the side. |
| 4 Moderate | The patient long-sits but uses forward arm support. There is a lot of back rounding, poor hip flexion and knee extension, and marked internal rotation of the hips. |
| 5 Severe | The patient can long-sit only with assistance from the therapist. |
| Prone kneeling (limitation) | |
| 1 Normal | The quadruped position is assumed and maintained without a lordosis and with good arm support. The thighs are held at an angle of 90° to the trunk while the knees are kept at an angle of 90° to the thighs. |
| 2 Mild | The patient can assume and maintain a quadruped position but he has a lordosis and some lower leg abduction. He stabilizes his pelvis with active hip flexion. Arm support is good. |
| 3 Moderate | The patient can assume the quadruped position. He maintains hip flexion, adduction and internal rotation, and knee flexion. The lower legs are slightly abducted to stabilize the base. The feet are in a mass dorsiflexion pattern or rest on the medial borders. Arm support is fair to good. |
| 4 Moderate severe | The patient can assume the quadruped position only with difficulty. The hips remain semi-extended and internally rotated, with adduction or abduction. Mass dorsiflexion of feet may be present. Arm support is poor. |
| 5 Severe | The patient cannot assume this position and needs assistance to maintain it. The arms collapse as arm support may be poor. |
| Kneel-standing (limitation) | |
| 1 Normal | The patient can assume and maintain the kneel-standing position and maintain a neutral pelvic tilt. |
| 2 Mild | The patient can rise against gravity and may or may not have difficulty maintaining this position. He maintains an anterior pelvic tilt, a lordosis, an hip flexion with lower leg abduction. He has great difficulty shifting weight. He can perform an activity in this position. |
| 3 Moderate | As in 2 above, but the hips are adducted and internally rotated and there is lower-leg abduction. He can maintain this position for a few seconds with little support. He is unable to shift weight and has difficulty freeing his arms. |
| 4 Moderate severe | The patient assumes or is assisted to assume kneel-standing. He has an anterior pelvic tilt, a lordosis, hip adduction, internal rotation, and flexion. He can maintain this position but is unable to free his hands. |
| 5 Severe | He cannot assume this position but needs to be placed in the position and given full external support. |
| Half-kneeling (limitation) | |
| 1 Normal | The patient can assume and maintain a good half-kneeling position. He is able to free his arms for an activity. |
| 2 Mild | The patient can assume and maintain the half-kneeling position but internally rotates the forward leg and flexes the hip of the weight bearing leg. Legs separate easily and the patient can free his arms. |
| 3 Moderate | The patient can assume or is helped to assume half-kneeling. The legs separate but the patient needs support, ie, protraction of the pelvis on the weight bearing side and traction of the forward leg. He cannot free his arms or can do so for only a short while. |
| 4 Moderate severe | The patient can assume this position but can separate his legs with difficulty, and he cannot free his hands. If he cannot assume this position and is placed in it, he can only maintain it with difficulty. |
| 5 Severe | The patient cannot assume this position. There is great difficulty separating the legs when he is placed in this position. The therapist must maintain full external support and the patient collapses after a few seconds. |
| Crawling (limitation) | |
| 1 Normal | The patient crawls reciprocally with well-integrated righting and equilibrium reactions. His legs separate with ease. The weight is well distributed and arm support is good. |
| 2 Mild | The patient crawls with some reciprocation but uses side flexion instead of elongation of the weight bearing side. Arm support is good. |
| 3 Moderate | As in 2 above, but the legs shoot into extension on the non-weight bearing side or he crawls with a mass flexion pattern with legs widely abducted at the hips and with flexion at the knees. Arm support may vary. |
| 4 Moderate severe | The patient “bunny-hops,” moving both lower extremities together. Arm support may vary. |
| 5 Severe | The patient is unable to assume the four point kneeling position. He “mermaid” crawls by pulling his body forward with his arms while the legs remain in extension. There is very poor dissociation of the legs. Arm support is usually poor. |
| Standing (limitation) | |
| 1 Normal | The patient is able to assume and maintain a good standing posture. |
| 2 Mild | The patient can assume the standing position but stands with an anterior pelvic tilt. He can shift his weight using side flexion. He stands independently. Feet may or may not be in a valgus position. |
| 3 Moderate | As in 2 above, but he needs to hold on due to lack of stability. The patient has an anterior pelvic tilt, some internal rotation at the hips with slight flexion, and some knee flexion, The feet may be in a valgus position. |
| 4 Moderate severe | Contractures and deformities are developing at the hips, knees, and ankles. The patient can only assume standing for a few seconds. Feet may be in valgus position. |
| 5 Severe | As in 4 above. The patient cannot assume standing. The patient requires full external support to stand. Hips, knees, and ankles may have fixed deformities. Feet may be in a valgus position. |
| Walking (limitation) | |
| 1 Normal | The patient walks reciprocally. Starting from a stable standing posture, the body is propelled forward in a smoothly coordinated way. The gait cycle consists of a stance and a swing phase. The stance is a 5-phased activity, involving contact, loading response, mid-stance, terminal stance, and pre-swing. The swing is divided into initial mid-, and terminal thirds. |
| 2 Mild | The patient walks with an anterior pelvic tilt but has the necessary break-up of patterns. |
| 3 Moderate | The patient can walk, has the necessary break-up of patterns, but lacks rotation and has poor weight-shift. He uses upper trunk compensations to assist with gait. |
| 4 Moderate severe | The patient has poor break-up patterns and cannot take a few steps independently or has very poor balance. |
| 5 Severe | No reciprocal gait, no break-up of patterns, and no weight shift. The patient needs full external support. |
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Supported by the Science Foundation Ireland, the South African Medical Research Council, and the University of Cape Town. The study protocol was approved by the University of Cape Town's Human Ethics Committee (REC REF 139/2005). We also acknowledge the financial support of the South African Medical Research Council.
No commercial party having a direct financial interest in the results of the research supporting this article has or will confer a benefit on the authors or on any organization with which authors are associated.
Reprints are not available from the author.
PII: S0003-9993(09)00183-X
doi:10.1016/j.apmr.2008.11.019
© 2009 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.
Volume 90, Issue 6 , Pages 994-1003, June 2009
