NCBI Bookshelf. A service of the National Library of Medicine, National Institutes of Health.
National Guideline Alliance (UK). Cerebral palsy in under 25s: assessment and management. London: National Institute for Health and Care Excellence (NICE); 2017 Jan. (NICE Guideline, No. 62.)
Review question: In infants, children and young people with cerebral palsy, what are the clinical and developmental prognostic indicators in relation to: the ability to walk; the ability to talk; and life expectancy?
10.1. Introduction
Although the central nervous system lesion of cerebral palsy is not progressive, it affects the development of children and young people with cerebral palsy in different ways according to their age, severity of activity limitation, type of motor disorder and cognitive ability. Skills attained in early development can be ‘lost’ because of growth-associated factors such as muscle tightness, contracture formation and weakness. The parents of children usually want to know what the future holds for their child, and yet the development of key activities is usually unknown at diagnosis.
There are many areas of development that are crucial for independence in everyday life such as independence in transfers, being able to communicate meaningfully, and to have effective upper limb activity for carrying out all activities of daily living and for using mobility aids such as walkers and wheelchairs. However, parents particularly want to know if their child will ‘walk and talk’. Life expectancy is another area regularly discussed at an early point after diagnosis, particularly in children with a severe impairment.
Most children and young people with cerebral palsy live at home with their parents, and there are understandable concerns from families as to what arrangements can be made for when their children are older and they are no longer able to care for them. The clinical team needs to be able to provide prognostic information for families about these areas where possible.
The Committee agreed with the 3 main areas for review based on parental views, clinical experience and published literature to determine clinical and developmental prognostic indicators.
The aim of this review was to determine which clinical and developmental indicators are able to predict the future ability of a child with cerebral palsy to talk, walk, and their life expectancy, with the view to providing information for parents and/or carers. Other reviews within this guideline and the NICE clinical guideline on Spasticity in under 19s provided more information in the area of independent mobility and communication, which were felt by the Committee to be as important in informing future management.
The quality of each study was assessed using the NICE methodology checklist (2012) for prognostic studies.
10.2. Description of clinical evidence
10.2.1. Prognosis for walking
Three studies were included for the prognosis of walking: two applied a prospective cohort design (Beckung 2008, Wu 2004) and 1 applied a retrospective cohort design (Trahan & Marcoux 1994).
10.2.2. Prognosis for talking
Two studies were included for the prognosis of talking: 1 applied a prospective cohort design (Chen 2013) and 1 analysed a cohort in the Northern Ireland Cerebral Palsy Register (Parkes 2010). It is important to note that the prospective cohort study (Chen 2013) had a short follow-up period of 6 months only.
10.2.3. Life expectancy
Four studies were included for the prognosis of life expectancy: 2 had a prospective cohort design (Blair 2001, Westbom 2011) and two had a retrospective cohort design (Strauss 2007, Touyama 2013).
For full details, see the review protocol in Appendix D. See also the study selection flow chart in Appendix F, study evidence tables in Appendix J and the exclusion list in Appendix K.
10.2.4. Summary of included studies
A summary of the studies that were included in this review is presented in Table 34, Table 35 and Table 36.
Table 34
Summary of included studies for prognosis of walking.
Table 35
Summary of included studies for prognosis of talking.
Table 36
Summary of included studies for prognosis of life expectancy.
10.3. Clinical evidence results
Table 37, Table 38 and Table 39 below summarise the results from the clinical evidence review on the prognostic indicators for walking, talking and life expectancy, respectively.
Table 37
Prognostic indicators for walking.
Table 38
Prognostic indicators for talking.
Table 39
Prognostic indicators for life expectancy.
10.4. Economic evidence
This review question is not relevant for economic analysis because it does not involve a decision between alternative courses of action.
No economic evaluations on the clinical and developmental prognostic indicators in relation to walking, talking or life expectancy were identified in the literature search conducted for this guideline. Full details of the search and economic article selection flow chart can be found in Appendix E and Appendix F, respectively.
10.5. Evidence statements
10.5.1. Prognostic indicators for walking
High-quality evidence from 1 study with 9,012 children with cerebral palsy suggests that children with unilateral spastic cerebral palsy with IQ < 50 are more likely to be unable to walk compared with children with unilateral spastic cerebral palsy and IQ > 50.
High-quality evidence from 1 study with 9,012 children with cerebral palsy suggests children with bilateral spastic cerebral palsy with IQ < 50 are more likely to be unable to walk compared with children with bilateral spastic cerebral palsy and IQ > 50. Moderate-quality evidence from 1 study with 187 infants with cerebral palsy suggests that there was no significant difference in risk of not being able to walk in children classified as having diplegic (bilateral LL>UL) or quadriplegic (bilateral LL+UL) cerebral palsy at 12 months of age.
Moderate-quality evidence from 1 study with 2,295 children showed that children without spastic quadriplegia (bilateral spastic LL+UL), are more likely to achieve full ambulation (defined as being able to walk well alone at least 20 feet without assistive devices) compared with those children with spastic quadriplegic distribution of cerebral palsy.
Moderate-quality evidence from 1 study with 2,295 children with cerebral palsy suggests that children who roll but do not sit without support at 2 years of age are more likely of being capable of full ambulation at 14 years of age (defined as being able to walk well alone at least 20 feet without assistive devices) compared with those children who do not roll at 2 years.
No evidence was found for the indicator: severity of functional disability (GMFCS levels). However, evidence was found in children who have quadriplegia (bilateral spastic LL+UL), who are generally of GMFCS level IV to V.
10.5.2. Prognostic indicators for talking
Low-quality evidence from 1 study with 78 children with cerebral palsy suggests that an increase in severity of functional disabilities (denoted by GMFCS levels) is associated with a decrease in ‘language’, assessed using the Comprehensive Developmental Inventory for Infants and Toddlers (CDIIT).
Moderate-quality evidence from 1 study with 1,357 children with cerebral palsy suggests that bilateral spastic cerebral palsy is associated with an increased odds of speech impairment compared with unilateral spastic cerebral palsy and non-spastic cerebral palsy.
Moderate-quality evidence from 1 study with 1,357 children with cerebral palsy suggests that an increase in severity of functional disabilities (GMFCS levels) is associated with an increase in speech impairment.
Moderate-quality evidence from 1 study with 1,357 children with cerebral palsy suggests that moderate cognition impairment (IQ 50–70) and severe cognition impairment (IQ < 50) are associated with an increase in speech impairment compared to children with ‘no intellectual impairment’ (IQ >70).
No evidence was found for the indicators: uncontrolled epilepsy and swallowing difficulties and/or /dysphagia, including the need for enteral tube feeding.
10.5.3. Prognostic indicators for life expectancy
Very low-quality evidence from 1 study with 2,014 children with cerebral palsy suggests an increase in severity of motor impairment (categorised as: minimal, mild, moderate or severe) is associated with an increased risk of mortality.
Very low-quality evidence from 1 study with 2,014 children with cerebral palsy suggests a decrease in IQ (from IQ >85 to IQ <20) is associated with an increased risk of mortality.
Moderate-quality evidence from 1 study with 6,277 children with severe cerebral palsy (unable to crawl, walk or self-feed) and 22,236 children with ‘not-severe’ cerebral palsy suggests that a feeding tube is associated with an increased risk of mortality.
Low-quality evidence from 1 study with 580 children with cerebral palsy suggests that severe functional disability (GMFCS level V) is associated with an increased risk of mortality.
Moderate-quality evidence from 1 study with 708 children with cerebral palsy suggests that severe functional disability (GMFCS level V) is associated with an increased risk of mortality, taking into account gastrostomy.
Moderate-quality evidence from 1 study with 708 children with cerebral palsy suggests that gastrostomy is associated with an increased risk of mortality, taking into account severity of functional disability.
No evidence was found for the indicator: comorbidities (epilepsy, scoliosis and chest infections).
10.6. Evidence to recommendations
10.6.1. Relative value placed on the outcomes considered
All outcomes in this review (ability to walk, talk and life expectancy) in relation to clinical indicators listed in the protocol were considered critical outcomes.
10.6.2. Consideration of clinical benefits and harms
10.6.2.1. Prognosis for walking
The Committee acknowledged the evidence presented and agreed that no additional studies meeting the protocol criteria were missed.
There was some evidence that showed that, in children who were non-ambulatory at 2 years, full ambulation at 6 years, defined as being able to walk well alone at least 20 feet without assistive devices, was even less likely if they also did not roll at 2 years of age compared to those who were able to roll but did not sit. The Committee agreed that this evidence supported their observations in clinical practice. Conversely, the Committee recommended that it was important to advise parents that if a child could not sit and could not roll at 2 years of age they would be unlikely to be able to walk later in life. This recommendation was based on the Committee’s clinical experience and the information provided by development of the GMFCS levels and was therefore agreed by consensus.
The Committee agreed that the evidence showed that more severe cognitive and physical abnormality in function was associated with increased odds of being unable to walk at 5 years of age. The Committee noted that the disparity between normal and abnormal developmental profiles, as outlined in GMFCS at an early stage, led to difficulties in clearly assessing long-term functional outcomes on assessment before 12 months of age.
The Committee agreed to consider an additional paper (Rosenbaum 2002) that did not meet the review protocol criteria due to non-comparative and unadjusted analysis, but which constituted supplementary evidence on the matter of prognosis for walking in children with cerebral palsy. Although no adjusted relative effects were reported, gross motor prognostic curves for children and young people with cerebral palsy were presented. This provided an association between Gross Motor Function Measure (GMFM) assessment, which measures gross motor activity, including a child’s ability to walk forward 10 steps unsupported (item 69 of the GMFM) and GMFCS levels enabling a prediction of walking ability and reported that GMFM decreased as GMFCS (level of severity) increased. The GMFM scores gross motor function in lying, crawling, kneeling, sitting, standing and walk-run-jump activities. Divergence between GMFCS levels in terms of gross motor development curves becomes more recognisable between 12 months and 2 years of age. Therefore, based on this and their experience, the Committee recommended to advise parents and/or carers that if a child with cerebral palsy could sit at 2 years of age, it was likely but not certain that the child would be able to walk independently without adult assistance.
10.6.2.2. Prognosis for talking
The evidence showed an association between severity in terms of GMFCS levels and decreased cognition with poor prognosis for language at around 4 years (mean age after follow-up not specified) and speech impairment at 5 years. Additionally, there was evidence that showed that children with bilateral spastic cerebral palsy have increased speech impairment compared with unilateral spastic cerebral palsy, and that non-spastic types of cerebral palsy (dyskinetic and ataxic motor patterns) have increased speech impairment compared with unilateral spastic cerebral palsy. However, the Committee agreed it was important to note that the detail regarding the assessment methods of both language and speech impairment was not reported in the included studies.
The Committee agreed that parents and/or carers should be advised that, with more severe physical, function and/or cognitive impairment, the greater the possibility was of difficulties with talking. Additionally, the Committee agreed parents and/or carers should be advised that children with bilateral spastic cerebral palsy are more likely to have a speech impairment compared to unilateral spastic cerebral palsy and that dyskinetic or ataxic types of cerebral palsy are likely to have increased speech impairment compared with unilateral spastic cerebral palsy.
Supplementary evidence from Cockerill 2014 that was not included in the review (this paper did include a multivariate analysis) was considered by the Committee. This study reported an association (p<0.001) between current epilepsy and speech impairment at 16 to 18 years and was considered by the Committee at the meeting as no other evidence for epilepsy and talking was found. The Committee recognised from their clinical experience that the presence of epilepsy in children with cerebral palsy was more likely to be associated with learning and speech difficulties but that this did not necessarily mean a cause and effect. Parents and/or carers should be advised that the presence of epilepsy may have an additional adverse effect on comorbidities in a child or young person with cerebral palsy.
10.6.2.3. Prognosis of life expectancy
The evidence showed that increased severity in terms of GMFCS levels and decreased cognition was associated with a decreased prognosis for life expectancy. The Committee agreed to advise parents and/or carers that the more severe the physical, functional and cognitive impairment, the greater the likelihood for reduced life expectancy.
The evidence also showed that tube feeding (reported as feeding tube and gastrostomy) was associated with reduced life expectancy. However, the Committee noted that children and young people who need tube feeding often have swallowing problems associated with increased severity of cerebral palsy. It was, therefore, a marker of severity and risk of aspiration because of poor swallow safety rather than life expectancy directly.
The Committee noted that, despite retrieving no evidence for life expectancy associated with the presence of comorbidities, two studies included in the evidence review (Blair 2001, Westbom 2011) reported comorbidities (epilepsy, scoliosis and chest infections, particularly pneumonia) as a cause of death. In 1 study (Blair 2001), out of n=151 deaths reported, 16.6% was due to aspiration pneumonia and 37.1% due to other pneumonia, with deaths due to aspiration pneumonia increasing from 1967, 1976 and 1986. It was also reported that deaths due to aspiration pneumonia were associated with profound intellectual deficit, particularly for deaths after 5 years of age (Blair 2001). Another study (Westbom 2011) reported that, of the 30 who had died, 26 had epilepsy, 12 had scoliosis and pneumonia was reported as the cause of death in 8. As with the presence of a feeding tube, the Committee recognised that aspiration pneumonia as a cause of death was likely to be a reflection of poor swallow safety.
The Committee considered that it was important to highlight that the major reported cause of early death was respiratory problems and especially infection. They also agreed these factors were a significant cause of morbidity and reduction in quality of life. They highlighted the importance of actively recognising and managing respiratory health factors in children and young people with cerebral palsy, such as minimising the risk of aspiration, monitoring and dealing with scoliosis and considering the use of prophylactic antibiotics as and when appropriate. They agreed it was also vital to ensure that children and young people with cerebral palsy receive immunisations against seasonal flu and pneumococcus.
10.6.3. Consideration of economic benefits and harms
This review question is not relevant for economic analysis because it does not involve a decision between alternative courses of action. As an aside, the Committee noted that children and young people with increased severity of cerebral palsy may need interventions to optimise their nutritional status. The resource and cost use regarding such interventions is discussed in Appendix G.
10.6.4. Quality of evidence
Quality of evidence in studies ranged from moderate to very low as there was variable adjustment for confounders in the statistical models of the studies. Confounders that were assessed for adjustment in the statistical model for walking and talking were: severity of functional disability, type of motor disorder, cognition and age. Confounders that were assessed for adjustment in the statistical model for life expectancy were: severity of functional disability, type of motor disorder, age, cognition and enteral tube feeding. If the statistical model adjusted for all confounders that were listed, no downgrading of quality was applied. If some confounders were adjusted for, quality was downgraded by 1; and if only 1 was adjusted for, then quality was downgraded by two.
Two studies included in the review (Chen 2013, Touyama 2013) reported evidence from a cerebral palsy population in Taiwan and Japan, respectively. Possible indirectness of the evidence was noted, yet it was decided that the quality of evidence was not to be downgraded as the aetiology and distribution of cerebral palsy does not largely differ in these countries. Of these studies, 1 was included for the prognosis of life expectancy and it was also noted in discussion that the life expectancy in Japan does not greatly differ from the UK.
10.6.5. Other considerations
The Committee noted that cognitive impairment, reported in terms of IQ in the studies included, was a proxy for the severity of the brain injury in people with cerebral palsy.
The recommendations related to this evidence review were based on the evidence and the Committee’s clinical experience.
10.6.6. Key conclusions
Walking
There is indication from the evidence that decreased cognition or not being able to roll at 2 years may indicate poor prognosis for walking.
Talking
There is indication from the evidence that type of cerebral palsy, decreased cognition and increased severity may indicate poor prognosis for speech and language.
The presence of epilepsy not controlled by medication can have a negative impact on speech development.
Life expectancy
There is indication from the evidence that decreased cognition, severe cerebral palsy and need for a feeding tube may indicate poor prognosis of life expectancy. However, the Committee agreed that the need for a feeding tube tends to be correlated with severity of cerebral palsy and problems with swallowing.
The Committee agreed it was important to consider that individual life expectancy should be adjusted for associated comorbidities.
10.7. Recommendations
- 32.
Provide the following information to parents or carers about the prognosis for walking for a child with cerebral palsy:
- The more severe the child’s physical, functional or cognitive impairment, the greater the possibility of difficulties with walking.
- If a child can sit at 2 years of age it is likely, but not certain, that they will be able to walk unaided by age 6.
- If a child cannot sit but can roll at 2 years of age, there is a possibility that they may be able to walk unaided by age 6.
- If a child cannot sit or roll at 2 years of age, they are unlikely to be able to walk unaided.
- 33.
Recognise the following in relation to prognosis for speech development in a child with cerebral palsy, and discuss this with parents or carers as appropriate:
- Around 1 in 2 children with cerebral palsy have some difficulty with elements of communication (see recommendation 132).
- Around 1 in 3 children have specific difficulties with speech and language.
- The more severe the child’s physical, functional or cognitive impairment, the greater the likelihood of difficulties with speech and language.
- Uncontrolled epilepsy may be associated with difficulties with all forms of communication, including speech.
- A child with bilateral spastic, dyskinetic or ataxic cerebral palsy is more likely to have difficulties with speech and language than a child with unilateral spastic cerebral palsy.
- 34.
Provide the following information to parents or carers, as appropriate, about prognosis for life expectancy for a child with cerebral palsy:
- The more severe the child’s physical, functional or cognitive impairment, the greater the likelihood of reduced life expectancy.
- There is an association between reduced life expectancy and the need for enteral tube feeding, but this reflects the severity of swallowing difficulties and is not because of the intervention.
10.8. Research recommendations
None identified for this topic.
- Prognosis for walking, talking and life expectancy - Cerebral palsy in under 25s...Prognosis for walking, talking and life expectancy - Cerebral palsy in under 25s: assessment and management
- Homo sapiens heterogeneous nuclear ribonucleoprotein D (HNRNPD), transcript vari...Homo sapiens heterogeneous nuclear ribonucleoprotein D (HNRNPD), transcript variant 1, mRNAgi|1519315555|ref|NM_031370.3|Nucleotide
Your browsing activity is empty.
Activity recording is turned off.
See more...