Cerebral palsy: what parents and doctors want to knowBMJ 2003; 326 doi: https://doi.org/10.1136/bmj.326.7396.970 (Published 03 May 2003) Cite this as: BMJ 2003;326:970
- Peter Rosenbaum, professor of paediatrics ()
- CanChild Centre for Childhood Disability Research, McMaster University, Hamilton, ON, Canada L8S 1C7
- Accepted 6 March 2003
Cerebral palsy is the most common physical disability in childhood. Children with cerebral palsy usually survive into adulthood, and the condition is often poorly understood in adulthood. Recognising and managing cerebral palsy's many important comorbidities is as important as treating the motor disabilities. Recent advances in the understanding of cerebral palsy include new ways of thinking about disability; recognition of causal pathways; and improvements in measurement, classification, and prognostication. Challenges include ensuring the wellbeing of families as well as children; tackling the issues faced lifelong by people with cerebral palsy; and the continuing need for primary, secondary, and tertiary prevention of the effects of cerebral palsy on people's lives.
Cerebral palsies are neurodevelopmental conditions, are the commonest “physical” disabilities in childhood, and severely affect a child's development
Comorbidities include epilepsy, learning difficulties, behavioural challenges, and sensory impairments and are at least as important as the motor disabilities
Advances in research are increasing our understanding of causal pathways, opportunities for primary prevention, and the value of specific intervention strategies
Cerebral palsy cannot be cured, but a host of interventions can improve functional abilities, participation, and quality of life
These conditions need to be recognised as involving the whole family, and management should always occur in the context of family needs, values, and abilities
The needs of adults with cerebral palsy, who currently are underemployed and face major barriers in the community, must now be tackled
What is cerebral palsy?
Cerebral palsy is “an umbrella term covering a group of non-progressive, but often changing, motor impairment syndromes secondary to lesions or anomalies of the brain arising in the early stages of development.”1 A total of 2-2.5 of every 1000 live born children in the Western world have the condition2; incidence is higher in premature infants and in twin births. 3 4 Some causal pathways have been described, which make it possible to prevent, for example, athetoid cerebral palsy due to kernicterus associated with Rh isoimmunisation, and potentially to eliminate cerebral palsy associated with maternal iodine deficiency. 2 5 The common perception that perinatal asphyxia is an important cause of cerebral palsy almost certainly overstates the case6; occult infection or inflammation is increasing implicated.7 Often a cause cannot be found in the history of children with clear clinical evidence of cerebral palsy.
Many efforts are under way in the basic and clinical sciences to describe the cascades of pathophysiological events that cause neurological damage, particularly in compromised premature infants.8 As the mechanisms of injury to the developing central nervous system are better understood, neuroprotective agents are likely to play an increasing role in continuing efforts at primary prevention of cerebral palsy.
Cerebral palsy, except in its mildest forms, can be seen in the first 12 to 18 months of life. The condition presents when children fail to reach their motor milestones and when they show qualitative differences in motor development, such as asymmetric gross motor function or unusual muscle stiffness or floppiness. Cerebral palsy is usually characterised clinically by the parts of the body affected (box 1), although conventional terminology used to describe cerebral palsy is less precise or reliable than the terms imply. Descriptions of the predominant motor disorder refer to spastic, dystonic, athetotic, and ataxic features (box 2).9 Functional status can be categorised (with respect to gross motor activity) by using the five levels of the gross motor function classification system for cerebral palsy (box 3),10 a reliable and valid system with prognostic importance now available on the CanChild web page (box 4). 11 12
Box 1 : Topography of cerebral palsy
Hemiparesis (hemiplegia)—(predominantly) unilateral impairment of arm and leg on the same side
Diplegia—motor impairment primarily of the legs (usually with some relatively limited involvement of arms)
Triplegia—three limb involvement
Quadriplegia (tetraplegia)—all four limbs, in fact the whole body, are functionally compromised
Box 2 : European classification of (motor impairment in) cerebral palsy9
Spastic cerebral palsy is characterised by at least two of
Abnormal movement pattern of posture or movement
Increased tone (not necessarily constant)
Pathological reflexes (increased reflexes, hyperreflexia, or pyramidal signs—for example, Babinski response)
Spastic bilateral cerebral palsy is diagnosed if
Limbs on both sides of the body are involved
Spastic unilateral cerebral palsy is diagnosed if
Limbs on one side of the body are involved
Ataxic cerebral palsy is characterised by both
Abnormal pattern of posture and/or movement
Loss of orderly muscular coordination so that movements are performed with abnormal force, rhythm, and accuracy
Dyskinetic cerebral palsy is dominated by both
Abnormal pattern of posture or movement
Involuntary, uncontrolled, recurring, and occasionally stereotyped movements
Dystonic cerebral palsy is dominated by both
Hypokinesia (reduced activity—stiff movement)
Hypertonia (tone usually increased)
Choreoathetotic cerebral palsy is dominated by both
Hyperkinesia (increased activity—stormy movement)
Hypotonia (tone usually decreased)RETURN TO TEXT
Box 3 : Severity of cerebral palsy: gross motor function classification system (for children between 6 and 12 years)10
Level I—Walks without restrictions; limitations in more advanced gross motor skills
Level II—Walks without devices; limitations in walking outdoors and in the community
Level III—Walks with mobility devices; limitations in walking outdoors and in the community
Level IV—Self mobility with limitations; children are transported or use power mobility outdoors and in the community
Level V—Self mobility is severely limited even with the use of supporting technologyRETURN TO TEXT
Cerebral palsy is in many ways the prototype for developmental disabilities. By definition the problems stem from one of many impairments of the developing central nervous system.2 Cerebral palsy affects gross motor function to a varying extent. A child's resulting overall development, specifically in mobility and other aspects of development and learning, is compromised by relative deprivation of experience. Delayed or aberrant motor function affects the development of a child's capacity to explore actively and to learn about space, effort, independence, and the social consequences of moving, touching, and getting into mischief. Limits to a child's functioning can cause parents to perceive their child as damaged, impaired, or disabled (and therefore limited); parents may interact with their child differently than if the child had better function.
People with cerebral palsy are considerably more likely to have functional difficulties unrelated to movement but related to their central nervous system (including sensory, epileptic, learning, behavioural, and related developmental impairments).13 These impairments may begin early in life as difficulties in feeding, irritability, and disordered sleep patterns. These problems, when present, affect day to day life and can cause considerable distress to children, parents, and carers. These problems are not inevitable or intractable, but it is essential to ask about, identify, and intervene before problems become entrenched.
Children with chronic functional limitations have considerably more difficulties in the social and behavioural aspects of their lives than typical children.14 Intellectual and behavioural problems in children with hemiplegic cerebral palsy reported by teachers in mainstream schools indicate that such children are at high risk of rejection by peers, lack of friends, and victimisation.15 It is essential to recognise the coexistence of physical functional and neurobehavioural disabilities in children with cerebral palsy and to provide integrated services to tackle these manifestations.16
Parents' first questions about cerebral palsy
From the outset parents want to know how “bad” the cerebral palsy is and whether their child will walk. These questions can be difficult to answer, particularly for healthcare professionals with limited experience of children with the condition. The literature on truth disclosure and communication with patients and families calls unequivocally for honesty, openness, communication with both parents together, and sensitivity to the individual needs of each family.17 A reliable and validated functional classification system for cerebral palsy9 that carries prognostic information has made it possible to provide evidence based answers to inform both parents and service providers (fig 1).12 In future this information may also be used to develop intervention programmes appropriate to a child's age and stage.
Modern goals: treatment and management
Cerebral palsy cannot be cured. The World Health Organization's model of health and disease focuses on function and is an important framework to guide modern thinking about treatment for children with cerebral palsy (fig 2).18 The goals of management should be to use appropriate combinations of interventions (including developmental, physical, medical, surgical, chemical, and technical modalities) to promote function, to prevent secondary impairments and, above all, to increase a child's developmental capabilities.
Many of the conventional approaches used in developmental treatment more or less target the primary impairments that underlie the functional challenges faced by children with cerebral palsy. With increased emphasis on promoting function, two important issues should be considered. Firstly, there is a need to move beyond efforts to promote normal function in children with cerebral palsy (often an illusory goal) toward the achievement of functional abilities that facilitate independence. Secondly, the liberal use of adaptive equipment—for example, powered mobility or walkers—may support early development of capacities such as independent ability to move about with the important effect of improving overall development.
The common concern that making things too easy for children will inhibit normal function is unfounded; there is strong evidence that, for example, the provision of powered mobility to children with disabilities as young as 36 months can have pervasive impacts on social, language, and play skills as well as increase efforts to try independent movement.19 A similar argument can be made for the early introduction of augmentative communication systems for children with communicative difficulties—for example, sign language, and picture boards—which make communication possible and often help to promote the development of oral language. Parents are often initially reluctant to accept augmentative interventions, preferring to work toward normal function. Introducing new parents to others who have chosen to provide devices to their children can be helpful to let the experienced parents discuss how they made the decisions about the use of equipment and sharing their perceptions about the value (or not) of this special equipment.
The role of the family and how doctors can help
Modern services for child health are increasingly being offered within a framework that espouses family centred service.20 Parents and providers work together in a partnership quite different from the traditional management of a child's rehabilitation programme directed by a doctor. These new relationships are predicated on mutual respect, empowered parents, and appropriate sharing of information with which decisions can be made. Parents' experiences of family centred service and their satisfaction with services, as well as the stress they experience in their dealings with their child's treatment centre, are strongly correlated.21 There is also a measurable link between family centred service and parents' mental health.22
Parental values and goals can form an important component of the management programme that is created for a child. Goal setting should be a joint venture between parents (and older children) and healthcare providers. This approach has recently been shown to lead to more effective outcomes and to be more efficient in terms of the amount of intervention by professionals.23 From a developmental perspective this finding makes sense—parents and especially children are more likely to follow through with recommendations for treatment that tackle their goals and needs. (Some parents may wish professionals to make decisions for them; an active choice by parents to be advised what to do is still a family centred approach to delivering services.)
Cerebral palsy is a long term condition; parents (and people with cerebral palsy) will have questions and issues to resolve throughout their lives. Continuity in the relationship between parents and trusted counsellors is important; professionals such as family doctors and therapists are people who will listen, support, advocate, and be there when challenges arise. Challenges are especially likely at times of transition in the life of the child and family, such as at the time of diagnosis, starting primary or secondary school, leaving school, and when entry to the broader community is being considered. Continuous and consistent service is valued by parents.24
New developments in treatment
The array of biomedical and surgical innovations for the treatment of cerebral palsy is ever expanding, much of it aimed at the reduction of what can at times be disabling spasticity. These include the use of botulinum toxin for temporary relief of spasticity,25 selective dorsal rhizotomy for more permanent relief,26 and intrathecal baclofen as a titratable antispasticity agent.27 Recent work to promote strength training for people with cerebral palsy may provide important new avenues to improve function.28
At the same time as innovative treatments are being developed, complementary treatments have emerged.29 These approaches range from apparently sensible but untested methods of teaching, training, or treating children (such as conductive education based on educational principles, which is as effective as, but not better than, conventional approaches),30 to interventions based on anecdotal evidence and testimonials but usually no credible research. New treatments are greeted with an expectation of impact that rarely happens. One such innovation which has attracted a lot of attention in the past few years is the use of hyperbaric oxygen, an approach that has been clearly shown in a well designed randomised clinical trial to provide no benefit.31 Other essentially untested ideas include subthreshold electrical stimulation of muscles, intensive passive muscle manipulation (patterning), and the use of an astronaut suit to promote independent mobility. In each case the claims about the effectiveness of the treatments are unsupported by solid clinical trial based research.32
Doctors are often called on to advise about treatment approaches with which they are unfamiliar. Specialty organisations such as Scope in the United Kingdom and UCPA in the United States, and research groups such CanChild in Canada have well developed websites (box 4). These websites may provide information on topics that are not available in published literature about complementary treatment (because so little research on these modalities has been undertaken).
The future for research
Considerable research efforts are under way toward the primary prevention of brain injury in infants at high risk who are exposed to perinatal abuse. Although most of this work is still at the development stage, clinical trials will soon be under way to evaluate a host of strategies.
An important and growing concern is the unmet needs of young adults with cerebral palsy. Community services for adults are often ill prepared to understand, let alone to meet, the needs of today's young people with disabilities, who have generally grown up at home and in the community and have been more integrated than children with cerebral palsy in any previous generation.33 The challenge to be addressed by service providers, educators, prospective employers, policy makers, and others is to begin to anticipate and plan appropriately for the full incorporation of adults with cerebral palsy into the life of their community, a goal fully consistent with the World Health Organization's focus on participation. This challenge is one that must be addressed by the whole community, and should involve the imagination and political will of professionals and families from all areas of society. To do less would be to marginalise young people with cerebral palsy and to squander the developmental and functional gains they have made in their developing years.
Box 4 : Website resources for parents and doctors
CanChild Centre for Childhood Disability Research (www.fhs.mcmaster.ca/canchild)—Many downloadable resources for service providers and parents on its website and links to several other related websites
United Cerebral Palsy Association (www.ucpa.org/ucp_general.cfm/1/4)—Links to their Research and Educational Foundation on its website, where more than 100 fact sheets are posted on topics of interest to parents and service providers
Scope (www.scope.org.uk)—A UK based organisation concerned with the needs of people with cerebral palsy
Mac Keith Press (distributed by Cambridge University Press) (http://publishing.cambridge.org/stm/medicine/mackeith)—Publishes Developmental Medicine and Child Neurology, the most highly cited developmental journal in the world, and Clinics in Developmental Medicine, an ongoing series of more than 160 books on a wide variety of topics pertinent to developmental disabilities of childhood
American Academy for Cerebral Palsy and Developmental Medicine (www.aacpdm.org)—The pre-eminent multiprofessional childhood disability organisation in the world. Their website links to resources for parents as well as professionals. The academy's annual meeting draws several hundred professionals from around the world to attend symposiums and instructional courses and give papers on topics concerned with cerebral palsy and related developmental issues
European Academy of Childhood Disability (www.eacd-org.org)—Presents an annual conference tackling issues in childhood disability offering symposiums and workshops of interest to professionals
I thank Doreen Bartlett, Robert Palisano, Richard Stevenson, and Martin Bax. I also thank colleagues at CanChild Centre for Childhood Disability Research.
Contributors: PR reviewed contemporary information and concepts about cerebral palsy drawn from his clinical and research experience and the current literature.
Competing interests None declared.