What do we need to know about the condition known as cerebral palsy?
Cerebral palsy is the most common physical disability in childhood. The term ‘cerebral palsy’ describes a group of disorders that are neither contagious, nor progressive, but which cause physical disability of body movement, balance and posture, due to damage to the child’s brain.1 This condition becomes visible in early life and has many complex symptoms, with various types and degrees of motor impairment.2,3,4,5
Cerebral palsy is a neurological condition resulting from brain injury occurring before brain development is complete, as the brain is still developing in the first two years of life. Cerebral palsy can result from brain injury occurring before, during or after birth.6,7
Key developments in research
William John Little (1810-1894) observed many cases of the condition amongst patients in his clinics, which led to the name ‘Little’s disease’.8 Little related cerebral palsy to perinatal anoxia, injury to the head and neck at birth and difficulty in labour.9
William Osler (1849-1919) published ‘The Cerebral Palsies of Children’.10 His work has been crucial in providing an excellent classification system for the different forms of cerebral palsy. Osler believed that trauma leading to meningeal haemorrhage and compression of brain and spinal cord was the major cause of cerebral palsy. At the same time, he stressed that it was nearly impossible to be certain of the cause of cerebral palsy.11
Sigmund Freud (1856-1939) agreed with Little that asphyxia and birth trauma could lead to brain damage. He went one step further, suggesting that diplegia may be of congenital origin. He also produced a thoroughly comprehensive classification system of cerebral palsy.12
During the past 50 years, cerebral palsy research has advanced once again with population studies such as those conducted by the National Institute of Neurological Disorders and Stroke (NINDS), who found complications during birth and labour accounting for only 10 % of the infants born with cerebral palsy.13
New research and knowledge about risk factors have included infections,14 German measles (rubella), oxiplasmosis, herpes simplex virus,15 and Rh incompatibility disease.16,17 Advancements in brain imaging and analysis have discovered areas of damage in the brain related to cerebral palsy.18,19,20 Genetic studies have identified genetic abnormalities associated with cerebral palsy.21,22 Therapies in the form of counselling have also proved to help people with cerebral palsy deal more effectively with their condition.23
Cerebral palsy: symptoms, effects and types
The symptoms of cerebral palsy include an early decrease in muscle tone and increasing muscle stiffness, known as hypotonia or spasticity. Although cerebral palsy is divided into different types, there are a number of physical symptoms that each type has in common, which include abnormal muscle tone, increased reflexes, spasticity and defects in muscle control. Movements may be jerky or slow.24
Cerebral palsy can be divided into four major forms: spastic, dyskinetic, ataxic and mixed presentations.25 The spastic forms of cerebral palsy, having stiff muscles, are the most common and include a loss of sensation or motor control to one or more limbs, as in monoplegia, diplegia, triplegia and quadriplegia. Hemiplegia is where one side of the body is affected and includes the trunk and abdomen. The dyskinetic forms of cerebral palsy include athetoid, choreoathetiod, and dystonic and are identified by slow, involuntary writhing movements, difficulties with maintaining a sitting position, lack of co-ordination and proprioception. Ataxic cerebral palsy affects balance and depth perception, co-ordination and often sight and hearing are affected too. Two or more forms of cerebral palsy may combine to cause mixed cerebral palsy.26,27
Incidence and prevalence of cerebral palsy
Research studies have suggested different cerebral palsy rates of 2 to 3 per every 1,000 live births.28,29,30,31,32,33 However, the incidence and type of cerebral palsy is closely related to the child’s gestational age (GA) at birth.34 Whilst the total number of children with cerebral palsy increased from the late 1960s to the mid 1990s, there was a consistent rise in the risk of cerebral palsy associated with pre-term and very pre-term infants. 35-39 The overall increase in cerebral palsy rates reflected the increased survival rates in very premature babies (possibly as early as 26 weeks), who have been placed in intensive care units.40,41,42 However, since the late 1990s and especially since the year 2000, the rates of neonatal morbidity have decreased with evidence also of a decrease in the rates of cerebral palsy.43
Cerebral palsy is most commonly linked to prematurity and low birth weight, where the brain is underdeveloped.44 Research findings point to perinatal asphyxia accounting for between 6% and 8% of cerebral palsy.45,46,47 Prenatal and largely unknown causes are thought to be responsible for 75 to 80 % of all cases of cerebral palsy.48 Estimated numbers of postnatally acquired cerebral palsy approximate 10%.49,50
Theories and causes of cerebral palsy
The causes of cerebral palsy can be categorised according to whether they occur during prenatal, perinatal or postnatal periods.51
Main causes of cerebral palsy in the prenatal stage are congenital brain malformations. These usually develop in the first or second trimester of pregnancy,52 and may be caused by infections or toxins transmitted from mother to infant, which affect the development of the brain.52 Problems in labour and delivery are defined as perinatal causes such as interruptions to blood flow causing anoxia, or asphyxia caused by twisted umbilical cords, which can cause cerebral palsy. 53 Infections and physical injuries including haemorrhaging are responsible for most cases of postnatally acquired cerebral palsy.54 Risk factors may be present before and during pregnancy, during labour and birth, and in the period shortly after birth.55
Accidental injuries, near-drowning episodes and non-accidental injuries may also result in cerebral palsy.56
A focus on children and the condition
Children are physically affected by the motor impairments in cerebral palsy,57 but they may have average or above average intelligence. Survival in cerebral palsy has improved over the past 50 years, and most children live to age 20 years plus, depending on the severity of impairments.58,59,60
Improving the child’s ability to walk or perform activities can be done by physiotherapy and exercise regimes involving repeated movement of skeletal muscles that result in exerting energy, and seek to improve or maintain levels of physical fitness.61
Children with cerebral palsy are at an increased risk for difficulties in their social development due to their physical disabilities, rather than cognitive abilities62 and have problems engaging in social interactions.63,64
Cerebral palsy can be stressful in the lives of parents. The stress of taking care of a child with cerebral palsy has been shown to affect the emotional and physical wellbeing of parents.65,66 Emotional consequences of this nature can hinder positive interactions between parent and child and it is difficult for parents to come to terms with a more limited expectation of their child. Barriers to childcare and information, lack of consultation and poor support have also been shown to add to the experiences of parental stress.67
Research has explored the employment prospects of young people with cerebral palsy. Studies have shown that between a quarter to half of those with cerebral palsy were competitively employed, because intelligence is rarely affected, although there may be some speech difficulties due to weak muscle tone and control.68,69,70,71,72
Diagnosis of cerebral palsy
It has been suggested by clinicians that diagnosing cerebral palsy too soon should be avoided. It should be assigned with caution before the age of 24 months, unless the disorder is exceptionally severe.73 The minimal age has been suggested as 3 years, but preference is for a child to reach 5 years of age. 74 This is because some early signs of motor impairment similar to cerebral palsy symptoms are known to be resolved later in childhood.
The diagnosis of cerebral palsy is made largely through clinical observations and not based solely on laboratory testing or neuroimaging. The approach that appears to be most widely used by clinicians is to rate activity limitation based on the standardised measure of motor function; The Gross Motor Function Classification System (GMFCS).75
To confirm a diagnosis of cerebral palsy, clinicians will use brain imaging techniques, such as EEG and MRI, which allow them to see brain abnormalities. These tests provide clues to the pattern and severity of brain injury underlying cerebral palsy and the motor problems that may occur. The particular test which is used will be based on an assessment of the patient’s symptoms.
Cranial Ultrasound (CUS) methods are used on preterm infants. Magnetic resonance imaging (MRI) provides the highest sensitivity to abnormalities in cerebral palsy. Computerised tomography (CT) scanning is based on the passing of an x-ray beam through a sample at a series of angles.
Prognosis for cerebral palsy
One of the most difficult aspects of cerebral palsy for both parents and clinicians is the prediction of the future for a child, as most people with cerebral palsy have normal intelligence but impaired communication skills, this has the potential to affect the child’s success, both academically and socially. Survival and quality of life vary but are associated with the severity and number of functional disabilities.76 There is a relationship between diminished life expectancy and the severity of the overall disabilities of the child. Contributing factors, which reflect severity of disability, include mental retardation, severe language disability, feeding by tube and the presence and severity of seizures.77
Information on related conditions
The related conditions to cerebral palsy include epilepsy, mental impairment, muskuloskeletal impairments, vision related problems, hearing related problems, speech related problems, drooling, swallowing difficulties, respiratory problems, growth problems, bowel and bladder problems and depression. All of these will vary in symptom and severity for each individual with cerebral palsy.78,79
Treatment options
The types of treatment available for patients with cerebral palsy depend on the patient’s specific symptoms and may include behavioural therapy, physiotherapy, speech therapy, medication, surgical treatments, orthoses, and aids.
Behavioural therapy helps to coach the patient to improve physical, mental and communicative skills to enhance social integration. The therapy itself varies greatly depending on the individual’s needs, age, and level of disability. Treatments such as the ‘Bobath method’ are common cerebral palsy treatments.80
Drugs are usually prescribed for cerebral palsy patients to control seizures caused by the condition. These drugs vary widely, as the source of the seizures can be attributed to a number of different causes. Different combinations of these drugs may be required in each individual case. Drug therapy may also be used to control the spasticity caused by cerebral palsy. Common drugs used are baclofen and botulinum toxin.
Surgical treatment is sometimes used, but only in extreme cases. Surgical treatment options include non-invasive abduction bracing, soft tissue releases, and major reconstructive femoral and pelvic osteotomies.
External aids such as orthoses are commonly used in conjunction with physical therapy, botulinum toxin, baclofen, and surgery to prevent inappropriate joint movements.
Sources of support and management
There is no cure for cerebral palsy, but the main goal is to increase the individual’s functioning, capabilities and to sustain health in relation to locomotion, cognitive development, social interaction, and independence. The best outcomes are shown to result from early, intensive management.81 A team approach focusing on the whole person achieves the best results.
Treatment programmes encompass physical and behavioural therapy, drug therapy and surgical treatments, mechanical aids, and management of associated medical conditions. In physical, occupational, speech, and behavioural therapies, the goals include enhancing patient and caregiver interactions, whilst providing family support.
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