References included in this Seminar were identified by the authors based on their respective areas of expertise and supplemented by unsystematic database searches.
SeminarCerebral palsy
Introduction
In cerebral palsy's milder forms, individuals present with mild spasticity and contracture in one arm and leg on one side of the body, which interferes with fluid movement and fine manual dexterity. The individual might have some sensory inattention to that side of the body and to that visual field, and might have focal epilepsy. At the other end of the spectrum, an individual can present with involvement of the four limbs, with a mixed picture of spasticity and dyskinesia. The individual can have substantial contractures and scoliosis, and therefore require a wheelchair for mobility. They might also have associated severe learning difficulties, cortical visual impairment, and be prone to chest infections.
Cerebral palsy is a syndrome of motor impairment that results from a lesion occurring in the developing brain; the disorder varies in the timing of the lesion, the clinical presentation, and the site and severity of the impairments. The earliest description of the disorder is attributed to the orthopaedic surgeon William Little in 1862.1 Several attempts to define and classify the syndrome have been made. Recently, the International Executive Committee for the Definition of Cerebral Palsy, proposed the following definition: “Cerebral palsy describes a group of permanent disorders of the development of movement and posture, causing activity limitation, that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain. The motor disorders of cerebral palsy are often accompanied by disturbances of sensation, perception, cognition, communication and behaviour, by epilepsy, and by secondary musculoskeletal problems”. This definition is supplemented by a full explanation of the terms used in the definition.2
The complexity of the syndrome is clear from its various classifications; cerebral palsy can be defined according to the anatomical site of the brain lesion (cerebral cortex, pyramidal tract, extrapyramidal system, or cerebellum); clinical symptoms and signs (spasticity, dyskinesia [dystonic and choreo-athetotic forms], or ataxia); topographical involvement of extremities (diplegia, quadriplegia, or hemiplegia); timing of presumed insult (prepartum, intrapartum, or postneonatal); and classification of degree of muscle tone (isotonic, hypotonic, or hypertonic).3
Standard classifications are essential for research and transfer of knowledge. The 9th and 10th International Classifications of Disease include many categories of cerebral palsy and substantial inconsistency exists in how clinicians interpret these guidelines.4 A straightforward classification is needed that can be applied reliably by clinicians and used in registers. Such a classification (with categories of unilateral spastic, bilateral spastic, dyskinetic, and ataxic) and an associated decision tree was developed by the Surveillance of Cerebral Palsy in Europe (European network SCPE) and is now widely adopted.5
Section snippets
Epidemiology
Development of registers for cerebral palsy, with an emphasis on a shared definition of the syndrome and efforts to ensure complete identification of cases, has shown a cerebral palsy prevalence of 2·0–3·5 per 1000 livebirths.6, 7, 8, 9 Prevalence in developing countries seems to be similar, but data sources are not well established.10, 11 Cerebral palsy prevalence is inversely associated with gestational age and birthweight, with a prevalence ranging from 90 cases per 1000 neonatal survivors
Pathogenesis
Although many mechanisms have been proposed to explain the cause, nature, and timing of the definitive cerebral insult, adverse factors might have been present for some time during pregnancy. For every gestation and for each type of cerebral palsy, an optimum birthweight exists; the high rates of cerebral palsy observed in preterm births occur when gestational birthweight deviates from this optimum (figure 2).27 This optimum birthweight effect is especially pronounced when fetal assessment of
MRI
About 85% of children with cerebral palsy have an abnormal MRI scan. An MRI scan can provide an estimate of the timing of the lesion and assist in determination of whether the lesion is responsible for the motor impairment or is an incidental finding.52 An abnormal scan finding is not a prerequisite for diagnosis of cerebral palsy but scans are recommended to assist clinical management.53 Findings could provide families with a more complete explanation of the cause of their child's cerebral
Life expectancy
To estimate life expectancy, a register of all cases is needed with dates of birth and regular updates of deaths to allow actuarial analysis. The severities of mental, manual, ambulatory, and visual impairments are significant factors in survival. If all impairment domains are not severe, survival is only marginally less than that of individuals without cerebral palsy.54, 55, 56 If severe impairments are present, then life expectancy is reduced approximately in proportion to the number and
Quality of life
In the past decade, studies of individuals with cerebral palsy have been done within the framework of the International Classification of Functioning, Disability and Health (ICF).61 The ICF recognises three constructs: body structure and function, activity limitation, and participation. Each construct can influence the others, there is not a causative progression from an impairment of structure to participation. Furthermore, their interactions are influenced by the context in which an
Improving outcomes
As the ICF and life-course frameworks predict, a divergence exists between trying to make the body of the individual to function more normally and accepting the person as they are and concentrating on environmental adjustments. Many physical therapies are available, but little evidence exists on which to choose. Therapy combined with medical and surgical interventions offers benefit, at least in the short term. Seeking normalisation of physical impairment in childhood only achieves gains in the
Overview
Two factors are key in management of individuals with cerebral palsy. First, all interventions must be planned, done, and validated by a multidisciplinary service with the choices of the child and family at the core of decision making. Second, difficulties encountered are not restricted to an individual's motor disorder but also to the variety of comorbidities.
The social model of disability,100 in which problems in participation are attributed to failure of society to accept and adapt to the
Conclusions
In the next decade, the brain lesions described by the umbrella term cerebral palsy might be classified by their causal factors coupled with a full description of impairments to body structure and functions they produce. Improved understanding of brain plasticity will probably lead to new treatments or at least better application of existing ones. Management will increasingly focus on promotion of participation and quality of life, with an expanding range of new technologies directed both to
Search strategy and selection criteria
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