Impairment of motor functioning is the major symptom of cerebral palsy and may present itself at various levels of the motor ability spectrum, from gross motor functioning to fine motor functioning. In general, the cerebral palsied person suffers limited mobility and abnormal (sometimes involuntary) movements. Specifically, such motor impairment can affect locomotion, communication (especially speech) and manual ability. Classification systems for the extent of motor dysfunction in cerebral palsy patients have been established.
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BACKGROUND: The Gross Motor Function Classification System (GMFCS) has become an important tool to describe motor function in children with Cerebral Palsy (CP). The Manual Ability Classification System (MACS) was developed recently as a corresponding classification of manual ability. The aim of this study was to describe the association between gross motor function and manual ability in a total population of children with cerebral palsy. METHODS: 365 children, born 1992 to 2001, who were registered in a population-based health care programme (CPUP) for children with CP living in the south of Sweden were included in the study. GMFCS was evaluated by the child's physiotherapist and MACS by the occupational therapist. CP diagnosis and subtype were determined by the neuropaediatrician at or after the age of four. RESULTS: GMFCS levels were available in all 365 children, MACS levels in 359 (98%). There was a poor overall correlation between gross motor function and manual ability. However, different associations between gross motor function and manual ability were found in the different diagnostic subtypes. Children with spastic hemiplegia generally had a lower level of manual ability than gross motor function (p < 0.001). The reverse association was generally found in children with spastic diplegia (p < 0.001). Children with dyskinetic CP had large limitations in both gross motor function and manual ability, with no significant discrepancy between GMFCS and MACS levels. CONCLUSION: Gross motor function and manual ability are often discrepant in children with CP, and the patterns seem to vary across the different subgroups based on the predominant neurological findings. To give a complete clinical picture when evaluating these children, both aspects have to be described. The GMFCS and the MACS seem to work well in this context and seem very useful in population-based studies, in health care registers for children with CP, and in clinical practice.
[Carnahan, K. D., Arner, M. & Hagglund, G. (2007). Association between gross motor function (GMFCS) and manual ability (MACS) in children with cerebral palsy. A population-based study of 359 children. BMC Musculoskeletal Disorders, 8, 50.]
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The aim of this study was to describe the motor function of a population of children at age 5 years enrolled on the South Australian Cerebral Palsy Register. Among children born between 1993 and 1998, there were 333 with confirmed cerebral palsy (prevalence rate 2.2 per 1000 live births), in whom 247 assessments (56.7% males, 43.3% females) were completed. The distribution by Gross Motor Function Classification System (GMFCS) level was: Level I, 50.6%; Level II, 18.2%; Level III, 9.3%; Level IV, 9.7%; Level V, 12.1%. The most common topographical classification was spastic diplegia (38.5%), followed by spastic hemiplegia (34.8%) and spastic quadriplegia (14.6%). Abnormal movements occurred at rest or with intention in 19.4% of children. A high proportion of the population with relatively mild gross motor impairments have difficulty with everyday bimanual tasks, reinforcing the need to assess upper limb function independently of gross motor function. The use of ankle-foot orthoses was common, particularly across GMFCS levels II to IV. Further refinement is indicated for this population's motor dataset, to include more recently described classification measures as well as future novel measures to better describe the presence of both spasticity and dystonia.
[Rice, J., Russo, R., Halbert, J., van Essen, P. & Haan, E. (2008). Motor function in five-year-old children with cerebral palsy in the South Australian population. Developmental Medicine and Child Neurology [epublication ahead of print].]
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BACKGROUND AND PURPOSE: Physical therapists frequently use the 66-item Gross Motor Function Measure (GMFM-66) with the Gross Motor Function Classification System (GMFCS) to examine gross motor function in children with cerebral palsy (CP). Until now, reference percentiles for this measure were not available. The aim of this study was to improve the clinical utility of this gross motor measure by developing cross-sectional reference percentiles for the GMFM-66 within levels of the GMFCS. SUBJECTS AND METHODS: A total of 1,940 motor measurements from 650 children with CP were used to develop percentiles. These observations were taken from a subsample, stratified by age and GMFCS, of those in a longitudinal cohort study reported in 2002. A standard LMS (skewness-median-coefficient of variation) method was used to develop cross-sectional reference percentiles. RESULTS: Reference curves were created for the GMFM-66 by age and GMFCS level, plotted at the 3rd, 5th, 10th, 25th, 50th, 75th, 90th, 95th, and 97th percentiles. The variability of change in children's percentiles over a 1-year interval also was investigated. DISCUSSION AND CONCLUSION: The reference percentiles extend the clinical utility of the GMFM-66 and GMFCS by providing for appropriate normative interpretation of GMFM-66 scores within GMFCS levels. When interpreting change in percentiles over time, therapists must carefully consider the large variability in change that is typical among children with CP. The use of percentiles should be supplemented by interpretation of the raw scores to understand change in function as well as relative standing.
[Hanna, S. E., Bartlett, D. J., Rivard, L. M. & Russell, D. J. (2008). Reference curves for the Gross Motor Function Measure: Percentiles for clinical description and tracking over time among children with cerebral palsy. Physical Therapy, 88(5), 596-607.]
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The gross motor function and disabilities in children with cerebral palsy in southern Sweden were investigated and related to clinical features. The study covered the birth year period 1990-1993 and comprised 167 children, 145 of them born in Sweden and 22 born abroad. The clinical features and gross motor function were analysed at a mean age of 6.8 y. Clinical features were obtained from a continuing healthcare follow-up programme. Gross motor function was classified according to the Gross Motor Function Classification System (GMFCS). Walking independently was possible for 86% of the hemiplegic, 63% of the pure ataxic, 61% of the diplegic and 21% of the dyskinetic children. None of the tetraplegic children was able to walk. The classification of gross motor function revealed that 59% of the children were categorized into levels I and II (mildly disabled), 14% into level III (moderately disabled) and 27% into levels IV and V (severely disabled). Children born abroad were more severely disabled. CONCLUSION: The standardized age-related classification system GMFCS enabled a specific description of gross motor function in relation to clinical features. Significant differences between GMFCS levels and subgroups of diagnosis, aetiology. intellectual capacity, epilepsy and visual impairment were found.
[Nordmark, E., Hagglund, G. & Lagergren, J. (2001). Cerebral palsy in southern Sweden II. Gross motor function and disabilities. Acta Paediatrica [Norway], 90(11), 1277-82.]
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OBJECTIVE: The purpose of this study was to evaluate the relationship between cerebral palsy (CP) diagnoses as measured by the topographic distribution of the tone abnormality with level of function on the Gross Motor Function Classification System (GMFCS) and developmental performance on the Bayley Scales of Infant Development II (BSID-II). It was hypothesized that (1) the greater the number of limbs involved, the higher the GMFCS and the lower the BSID-II Motor Scores and (2) there would be a spectrum of function and skill achievement on the GMFCS and BSID-II Motor Scores for children in each of the CP categories. METHODS: A multicenter, longitudinal cohort study was conducted of 1860 extremely low birth weight (ELBW) infants who were born between August 1, 1995 and February 1, 1998, and evaluated at 18 to 22 months' corrected age. Children were categorized into impairment groups on the basis of the typography of neurologic findings: spastic quadriplegia, triplegia, diplegia, hemiplegia, monoplegia, hypotonic and/or athetotic CP, other abnormal neurologic findings, and normal. The neurologic category then was compared with GMFCS level and BSID-II Motor Scores. RESULTS: A total of 282 (15.2%) of the 1860 children evaluated had CP. Children with more limbs involved had more abnormal GMFCS levels and lower BSID-II scores, reflecting more severe functional limitations. However, for each CP diagnostic category, there was a spectrum of gross motor functional levels and BSID-II scores. Although more than 1 (26.6%) in 4 of the children with CP had moderate to severe gross motor functional impairment, 1 (27.6%) in 4 had motor functional skills that allowed for ambulation. CONCLUSIONS: Given the range of gross motor skill outcomes for specific types of CP, the GMFCS is a better indicator of gross motor functional impairment than the traditional categorization of CP that specifies the number of limbs with neurologic impairment. The neurodevelopmental assessment of young children is optimized by combining a standard neurologic examination with measures of gross and fine motor function (GMFCS and Bayley Psychomotor Developmental Index). Additional studies to examine longer term functional motor and adaptive-functional developmental skills are required to devise strategies that delineate therapies to optimize functional performance.
[Vohr, B. R., Msall, M. E., Wilson, D., Wright, L. L., McDonald, S. & Poole, W. K. (2005). Spectrum of gross motor function in extremely low birth weight children with cerebral palsy at 18 months of age. Pediatrics, 116(1), 123-9.]
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BACKGROUND AND PURPOSE: Development of gross motor function in children with cerebral palsy (CP) has not been documented. The purposes of this study were to examine a model of gross motor function in children with CP and to apply the model to construct gross motor function curves for each of the 5 levels of the Gross Motor Function Classification System (GMFCS). SUBJECTS: A stratified sample of 586 children with CP, 1 to 12 years of age, who reside in Ontario, Canada, and are known to rehabilitation centers participated. METHODS: Subjects were classified using the GMFCS, and gross motor function was measured with the Gross Motor Function Measure (GMFM). Four models were examined to construct curves that described the nonlinear relationship between age and gross motor function. RESULTS: The model in which both the limit parameter (maximum GMFM score) and the rate parameter (rate at which the maximum GMFM score is approached) vary for each GMFCS level explained 83% of the variation in GMFM scores. The predicted maximum GMFM scores differed among the 5 curves (level I=96.8, level II=89.3, level III=61.3, level IV=36.1, and level V=12.9). The rate at which children at level II approached their maximum GMFM score was slower than the rates for levels I and III. The correlation between GMFCS levels and GMFM scores was (.91. Logistic regression, used to estimate the probability that children with CP are able to achieve gross motor milestones based on their GMFM total scores, suggests that distinctions between GMFCS levels are clinically meaningful. CONCLUSION AND DISCUSSION: Classification of children with CP based on functional abilities and limitations is predictive of gross motor function, whereas age alone is a poor predictor. Evaluation of gross motor function of children with CP by comparison with children of the same age and GMFCS level has implications for decision making and interpretation of intervention outcomes.
[Palisano, R. J., Hanna, S. E., Rosenbaum, P. L., Russell, D. J., Walter S. D., Wood, E. P., Raina, P. S. & Galuppi, B. E. (2000). Validation of a model of gross motor function for children with cerebral palsy. Physical Therapy, 80(10), 974-85.]
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The aim of this study was to explore motor development in children with cerebral palsy (CP) using developmental curves for CP, subtypes, and the five severity levels of the Gross Motor Function Classification System (GMFCS). The Gross Motor Function Measure (GMFM) and the GMFCS were applied to 317 children (145 females, 172 males) with CP, aged between 1 and 15 years. The CP type distribution was spastic diplegia in 157 (49%), spastic hemiplegia in 101 (33%), spastic tetraplegia in 11 (3%), dyskinesia in 38 (12%), and ataxia in 10 (3%). Forty-five physiotherapists were trained in the GMFM and intra- and interrater reliability was tested. The GMFM was measured prospectively every 6 months up to the age of 4 years and once a year thereafter. Developmental curves were constructed for 258 children with spastic CP. About three-quarters of the children at GMFCS Level I reached 90% of the maximum GMFM score at 5 years of age. The performance peaked at 7 years of age. Children at GMFCS Level II reached 90% at a median age of 5 years, which was also the upper limit, reached by about three-quarters at 7 years of age. The majority of children at GMFCS Level III reached 80% of the GMFM by 7 years of age and most of the children at GMFCS Level IV reached 30% at 5 years and remained there. The median score for children at GMFCS Level V was 20%. The intra- and interrater reliability for the GMFM 88 among physiotherapists were Spearman's rank correlation coefficient 0.91 and 0.99 respectively. There were 931 measurements with a median of 2 (1-11) per child. The gross motor development was demonstrated for the five GMFCS levels in children with spastic CP. These kind of curves may be useful for monitoring and predicting motor development, for planning treatment, and for evaluating outcome after interventions.
[Beckung, E., Carlsson, G., Carlsdotter, S. & Uvebrant, P. (2007). The natural history of gross motor development in children with cerebral palsy aged 1 to 15 years. Developmental Medicine and Child Neurology, 49(10), 751-6.]
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The aim of this study was to describe and analyze gross and fine motor function and accompanying neurological impairments in children with cerebral palsy (CP) born between 1991 and 1998 in western Sweden. A population-based study comprised 411 children with a diagnosis of CP ascertained at 4 to 8 years of age. Gross Motor Function Classification System (GMFCS) levels were documented in 367 children (205 males, 162 females). Bimanual Fine Motor Function (BFMF) classification levels of 345 of the children and information on learning disability, epilepsy, visual and hearing impairments, and hydrocephalus from 353 children were obtained. For spastic CP, a new classification according to the Surveillance of Cerebral Palsy in Europe of uni- and bilateral spastic CP was applied. GMFCS was distributed at Level I in 32%, Level II in 29%, Level III in 8%, Level IV in 15%, and Level V in 16%. The corresponding percentages for BFMF were 30.7%, 31.6%, 12.2%, 11.9%, and 13.6% respectively. Learning disability was present in 40%, epilepsy in 33%, and severe visual impairment in 19% of the children. Motor function differed between CP types. More severe GMFCS levels correlated with larger proportions of accompanying impairments and, in children born at term, to the presence of adverse peri/neonatal events in the form of intracranial haemorrhage/stroke, cerebral infection, and hypoxic-ischaemic encephalopathy. GMFCS Level I correlated positively to increasing gestational age. We conclude that the classification of CP should be based on CP type and motor function, as the two combine to produce an indicator of total impairment load.
[Himmelmann, K., Beckung, E., Hagberg, G. & Uvebrant, P. (2006). Gross and fine motor function and accompanying impairments in cerebral palsy. Developmental Medicine and Child Neurology, 48(6), 417-23.]
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Objective: Limited resources in terms of elementary functions may be a limiting factor for functional activities. The objective of the study was to examine basic hand motor capacities in young children with bilateral spastic cerebral palsy (BSCP) and to compare with deficits in functional activities. Method: Eighty-eight children with BSCP, 3-6 years of age, manipulated a grip object (200g) equipped with a uniaxial force sensor. Basic motor capacity was assessed based upon (1) maximal grip strength and (2) production of fast repetitive grip force changes (FFC) while holding the object on the table. Subjects' performance on this task was compared to the grip force amplitude and force rate assessed while the subject was lifting the same object. Results were compared between different degrees of manual ability according to the Manual Ability Classification System (MACS). Results: In children with BSCP, even in high-functioning children with MACS 1, fast grip force changes and grip strength were 2 SDs and more below the mean of controls. Differences increased from MACS 2 to 4 but not between MACS 1 and 2. During lifting children with BSCP used considerable proportions of their maximum grip strength (40-90%) and of their grip force rates during 70% vs. 86%. In some children with low manual abilities (MACS 3/4), grip force rates during lifting were higher than during FFC. Conclusion: In children with BSCP, basic motor capacity may influence manual ability, particularly in children with MACS 3 and 4. In some of these children, the underlying processes during lifting may also differ qualitatively.
[Blank, R. & Hermsdorfer, J. (2008). Basic motor capacity in relation to object manipulation and general manual ability in young children with spastic cerebral palsy. Neuroscience Letters [epublication ahead of print].]
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This study evaluated the hypothesis that upper extremity function and range of motion can be quantified reliably in children with cerebral palsy (CP) in a busy clinical setting. The specific aim was to determine the inter- and intrarater reliability of a modified House Functional Classification (MHC) system to evaluate upper extremity function and a standardized instrument to document upper extremity range of motion (Upper Extremity Rating Scale [UERS]). Sixty-five children with CP (43 males, 22 females, mean age 9y 2mo, SD 4y 1mo) with spasticity involving the upper extremity (quadriplegia n=22; hemiplegia n=36; diplegia n=7; Gross Motor Functional Classification System Levels I n=41, II n=6, III n=3, IV n=5, V n=10) were evaluated independently by occupational therapists and orthopedic surgeons using both instruments at several visits. Inter- and intrarater reliability were determined for both instruments by calculating measures of agreement (weighted kappa values and intraclass correlation coefficients [ICCs]). Interrater agreement (ICC=0.94) and intrarater agreement (ICC=0.96) on the MHC were good to excellent. Similarly, inter-rater agreement (kappa 0.66-0.81) and intrarater agreement (kappa 0.64-0.88) on the UERS was either good or excellent. The MHC and the UERS provide standardized, reliable, reproducible, and efficient instruments that can be used by occupational therapists and orthopedic surgeons to evaluate the upper extremities of children with CP.
[Koman, L. A., Williams, R. M., Evans, P. J., Richardson, R., Naughton, M. J., Passmore, L. & Smith, B. P. (2008). Quantification of upper extremity function and range of motion in children with cerebral palsy. Developmental Medicine and Child Neurology, 50(12), 910-7.]
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To describe the impact of periventricular leukomalacia (PVL) on gross motor function, data on 59 children (37 males, 22 females) with a gestational age (GA) of 34 weeks or less with cerebral palsy (CP) due to PVL grade I (n=20), II (n=13), III (n=25), and IV (n=1) were studied; (mean GA 29 wk 4d [SD 4 wk 6d]; mean birthweight 1318 g [SD 342]). Two independent raters used the Gross Motor Function Classification System (GMFCS) at four time points: T1, mean corrected age (CA) 9 months 15 days (SD 2 mo 6d); T2, mean CA 16 months (SD 1 mo 27 d); T3, mean CA 24 months 27 days (SD 2 mo 3d); and T4, median age 7 years 6 months (range 2 y 2 mo-16 y 8 mo). Interrater reliability and stability across time with respect to the total cohort were kappa>or=0.86 and rho>or=0.74 respectively. The association between PVL and gross motor outcome at T4 was strong (positive and negative predictive values 0.92 and 0.85 respectively). The proportion of children who remained in the same GMFCS level increased from 27% (T1-T4) to 53% (T2-T4) and 72% (T3-T4). PVL grade I to II, as diagnosed in the neonatal period, has a better functional mobility prognosis than PVL grade III-IV. These findings have implications for habilitation counselling and intervention strategies.
[van Haastert, I. C., de Vries, L. S., Eijsermans, M. J., Jongmans, M. J., Helders, P. J. & Gorter, J. W. (2008). Gross motor functional abilities in preterm-born children with cerebral palsy due to periventricular leukomalacia. Developmental Medicine and Child Neurology, 50(9), 684-9.]
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