Cerebral Palsy Introduction


Original Editor - Roelie Wolting as part of the Enablement Project Top Contributors - Michelle Lee, Naomi O'Reilly, Admin, Laura Ritchie, Kim Jackson, Simisola Ajeyalemi, Vidya Acharya, Mande Jooste, Pranay Jindal, WikiSysop, Rucha Gadgil, 127.0.0.1, Lucinda hampton, Jess Bell, Rachael Lowe, Olajumoke Ogunleye, Evan Thomas, Scott Buxton and George Prudden


Introduction

The information on this page has developed for you from the expert work of Roelie Wolting alongside the Enablement Cerebral Palsy Project and Handicap International Group.

Definition

Cerebral Palsy (CP) is a disorder of movement and posture that appears during infancy or early childhood resulting from damage to the brain. The damage to the brain is permanent and cannot be cured but the earlier we start with intervention the more improvement can be made.Any non-progressive central nervous system (CNS) injury occurring during the first 2 (some say 5) years of life is considered to be CP. There are several definitions of Cerebral Palsy within the literature, although these may all vary slightly in the way they are worded they are all similar and can be summarised to:

Cerebral Palsy is a group of permanent, but not unchanging, disorders of movement and/or posture and of motor function, which are due to a non-progressive interference, lesion, or abnormality of the developing/immature brain.

This definition specifically excludes progressive disorders of motor function, defined as loss of previously acquired skills in the first 5 years of life.

Time Frame of Brain Injury

We only talk about cerebral palsy if the brain damage arises during one of the following periods:

Prenatal Period:

• Conception to the onset of labour

Perinatal Period:

• 28 weeks intrauterine to 7 days

Postnatal Period: 

• First two (and some say five) years of life

After the age of 5 we speak of stroke or traumatic brain injury.

Epidemiology

The incidence of CP has not declined despite the improved perinatal and obstetric care. Even at centres where optimal conditions exist for perinatal care and birth asphyxia is relatively uncommon, the incidence of CP in term babies has remained the same.

The overall prevalence worldwide has increased during the last decades because of increased survival rates.Here are some facts on the epidemiology of Cerebral Palsy:

• The incidence is 2-2,5/1000 live births in Western Countries
• The prevalence varies between 1-5/1000 babies in different countries.
• There are no reliable statistics from Asian countries.
• Some affected children do not survive

Aetiology

Improved medical care has decreased the incidence of CP among some children. Medical advances have also resulted in the survival of children who previously would have died at a young age.

The type of cerebral palsy has also changed:

• In the 60’s Athetoid / Dyskinetic Cerebral Palsy accounted for approximately 20% of children with Cerebral Palsy.
• Today only 5 to 10% have this type, with Spastic Cerebral Palsy now accounting for 80-90% of children with Cerebral Palsy.
• This decrease is mainly due to advances in the treatment of hyperbilirubinamia.
• The increase of Spastic Cerebral Palsy is predominantly a result of higher survival rates for (very small premature) babies.
• The most common cause of Cerebral Palsy is idiopathic which means that the cause of damage to the brain during pregnancy is not known.

Risk Factors

There are different risk factors for each stage at which a child might develop CP. These can be broken down into Prenatal, Perinatal and Postnatal.

Prenatal:

• Prematurity (gestational age less than 36 weeks)
• Low birth weight (less than 2500 g), which could be due to poor nutritional status of the mother
• Maternal epilepsy
• Hyperthyroidism
  
• Infections (TORCH = Toxoplasmosis, Other (syphilis, varicella-zoster, parvovirus B19,)Rubella, Cytomegalovirus (CMV), Herpes simplex virus)
• Severe toxemia, eclampsia
• Drug abuse
• Trauma
• Multiple pregnancies
• Placental insufficiency

Perinatal:

• Premature rupture of membranes
• Prolonged and difficult labour
• Vaginal bleeding at the time of admission for labour
• Bradycardia

Postnatal (0-2 years):

• Central Nervous System infection (encephalitis,meningitis)
• Hypoxia
• Seizures
• Coagulopathies
• Neonatal hyperbilirubinemia
• Head trauma

There is no way to predict which child’s brain will be damaged by one of these factors or to what the extent of the damage will be. None of these factors always results in brain damage and even when brain damage occurs, the damage does not always result in CP.

For example: Some children may have an isolated hearing loss from their meningitis, others will have severe intellectual disability and some will have CP.

Sub Types of Cerebral Palsy

There are 3 sub-types of CP and these are:

• Spastic
• Dyskinetic
• Ataxic

Even though they are not clinically precise and may lack reliability, amongst observers these terms can be conceptually useful. 

Spastic CP

Spastic Cerebral Palsy is characterised by at least two of the following symptoms, which may be unilateral (hemiplegia) or bilateral:

• Abnormal pattern of posture and/or movement
• Increased tone (not necessarily constantly)
• Pathological reflexes (hyper-reflexia or pyramidal signs e.g. Babinski response)
  

Dyskinetic CP

Dyskinetic Cerebral Palsy is Characterised by the following Symptoms:

• Abnormal pattern of posture and/or movement, and
• Involuntary, uncontrolled, recurring, occasionally stereotyped movements of affected body parts

Dyskinetic Cerebral Palsy may be either:

• Dystonic Cerebral Palsy, dominated by both hypokinesia and hypertonia.

or

• Choreo-athetotic Cerebral Palsy, dominated by both hyperkinesia and hypotonia
  

Ataxic CP

Ataxic Cerebral Palsy is characterised by the following symptoms:

• Abnormal pattern of posture and/or movement
• Lost of orderly muscular co-ordination, so that movements are performed with abnormal force, rhythm and accuracy

Classification Tree for Sub-types of CP

Many children with cerebral palsy have a mixed form of cerebral palsy.

In this page the definition and classification used as agreed on in Europe. More information on definitions, outcome of some studies, exchange of information on clinical practice on CP in Europe you can find, after registering here. 
The Surveillance of Cerebral Palsy in Europe was established in 1998.

Anatomical Classifications

Anatomical classification are as follows:

• Unilateral:  one side of the body is affected
• Bilateral:  both sides of the body are affected
• Spastic CP: are used to distinguish between quadriplegia, diplegia and hemiplegia. Diplegia mainly affects the upper limbs in comparison the lower limbs.

Spastic CP is either bilateral or unilateral.

• Unilateral CP: we refer to as hemiplegia.
• Dyskinetic CP and Ataxic CP: always involve the whole body (bilateral).
  

Spacticity 

Spasticity is defined as an increase in the physiological resistance of muscle to passive motion. It is part of the upper motor neuron syndrome characterised by hyperreflexia, clonus, extensor plantar responses and primitive reflexes. Spastic CP is the most common form of CP. Approximately 80% to 90% of children with CP have spastic CP.

Traditionally we recognised three types of spastic CP:

Hemiplegia	Diplegia	Quadriplegia
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Hemiplegia (unilateral) CP

With hemiplegia, one side of the body is involved with the upper extremity generally more affected than the lower. Seizure disorders, visual field deficits, astereognosis, and proprioceptive loss are likely. Twenty percent of children with spastic CP have hemiplegia. A focal traumatic, vascular, or infectious lesion is the cause in many cases. A unilateral brain infarct with posthemorrhagic porencephaly can be seen on magnetic resonance imaging (MRI).

Diplegia (bilateral) CP

With diplegia, the lower extremities are severely involved and the arms are mildly involved. Intelligence usually is normal, and epilepsy is less common. Fifty per cent of children with spastic CP have diplegia. A history of prematurity is usual. Diplegia is becoming more common as more low- birth-weight babies survive. MRI reveals mild periventricular leukomalacia (PVL).

Quadriplegia (bilateral) CP

With quadriplegia, all four limbs, the trunk and muscles that control the mouth, tongue and pharynx are involved. Thirty percent of children with spastic CP have quadriplegia. More serious involvement of lower extremities is common in premature babies. Some have perinatal hypoxic ischemic encephalopathy. MRI reveals periventricular leukomalacia (PVL).

Dyskinetic CP

Abnormal movements that occur when the child initiates movement are named dyskinesias. Dysarthria, dysphagia and drooling accompany the movement problem. Intellectual development is generally normal, however severe dysarthria makes communication difficult and leads the outsider to think that the child has intellectual impairment. Sensorineural hearing dysfunction also impairs communication. Dyskinetic CP accounts for approximately 10% to 15 % of all cases of CP. Hyperbilirubinemia or severe anoxia causes basal ganglia dysfunction and results in dyskinetic CP.

Ataxic CP

Ataxia is loss of balance, coordination and fine motor control. Ataxic children cannot coordinate their movements. They are hypotonic during the first 2 years of life. Muscle tone becomes normal and ataxia becomes apparent toward the age of 2 to 3 years. Children who can walk have a wide-based gait and a mild intention tremor (dysmetria). Dexterity and fine motor control is poor. Ataxia is associated with cerebellar lesions. Ataxia is often combined with spastic diplegia. Most ataxic children can walk but some need walkers.

Mixed CP

Children with a mixed type of CP commonly have mild spasticity, dystonia and/or athetoid movements. Ataxia may be a component of the motor dysfunction in children in this group. Ataxia and spasticity often occur together. Spastic ataxic diplegia is a common mixed type that often is associated with hydrocephalus.

The Brain

Here is some of the clinical terminology used when talking about CP:

• Tonus
• Lesion Site
• Spastic
• Cortex 
• Dyskinetic
• Basal Ganglia - Extrapyramidal System 
• Hypotonic / Ataxic
• Cerebellum
• Mixed
• Diffuse

Knowing were the damage could be located will not influence your interventions. Look at the following pictures of the brain to understand the relationship between the location of the damage and the symptoms.

Problems Regularly Seen with Children with CP

This table highlights the problems that children with CP experience within different areas.

Neurological	Musculoskeletal	Associated Problems
Muscle weakness Abnormal muscle tone Balance problems Loss of selective control Pathological reflexes Loss of sensation Swallowing difficulty	Contractures Deformities	Intellectual impairment Epilepsy Visual problems Hearing loss Speech and communication problems Feeding difficulty, failure to thrive Respiratory problems Incontinence

This image gives a nice pictorial overview of the problems experience by children with CP.

Associated Problems

Cerebral Palsy in itself can significantly impact upon the child. Many assocaited conditions also need to be managed. As a Health Care Professional it is essential to also understand these associated conditions and think about how these might impact or influence your management strategies when working with the child. You can find out more about these associated conditions here.

Diagnosis

Cerebral palsy is one of the main causes of childhood disabilities, with many different signs, symptoms and challenges. There is no one test to confirm if a child has cerebral palsy or something else. There is no blue print of interventions for a child with cerebral palsy and
each child is different and unique. Classification of gross motor, fine motor and communication will help medical professionals and the family to better understand the abilities of the child and what to focus on for interventions.

The diagnosis of cerebral palsy is based on a clinical description. The diagnosis is not based on the result of a (biological) test or on imaging findings. Consequently, the diagnosis can be subject to some degree of variability. This means that two paediatricians may disagree on a diagnosis of CP for the same child. It is sometimes difficult for even professionals to differentiate between bilateral spastic CP and dyskinetic CP.

Ideally a paediatrician or neurologist will give the diagnosis but some children with CP in developing countries have never seen a doctor. There are also many children in developing countries with CP who have seen many doctors previously but with no good explanation of the meaning and consequences of the diagnoses.

Life Expectancy

Mortality in CP is extremely variable. Life expectancy is normal in most diplegic and hemiplegic children who receive adequate medical care and have strong family support. Some severely affected quadriplegics die of malnutrition, infections or respiratory problems before reaching adolescence.

In some very poor and poor resource areas children with cerebral palsy may not reach the age of 5 years.

Interventions with CP 

The aim and types of interventions are unique for each child with CP because their needs are all different depending on the level of disability. This table gives a great overview of the aims of treatment / interventions for each level of disability.

Mild	Moderate	Severe
Intervention will focus of appearance and integration	Intervention will focus on independence and self-care skill	Intervention will focus on comfort and enhanced care

Communication

Communication is necessary to express thoughts, feelings and needs. Every individual with CP needs a way to communicate to be part of the family and the community. If the child can produce comprehensible sounds and syllables by 2 years of age, they will probably have normal verbal communication but considerations need to be made for those whose communication skills are severely affected. Alternative methods such as communication methods such as simple communication boards in children who have difficulty speaking.

Activities of Daily Living (ADL)

Activities of daily living are self-care activities such as feeding, toileting, bathing, dressing, and grooming in addition to meal preparation and household maintenance. Dyskinetic and total body involved children have problems of dexterity and fine motor control that prevent independence in activities of daily living. Hemiplegic and diplegic children can become functional in these areas. They sometimes need help of (occupational) therapy. Family attitude is a critical factor determining the level of independence of a child. Overprotection results in a shy and passive individual who has not gained self-care abilities.

Mobility

Children have to explore their surroundings to improve their cognitive abilities. Mobilisation is crucial for the young child with disability to prevent secondary mental deprivation. The use of wheelchairs or other mechanical assertive devices can help to promote independent mobility in the community if the child cannot mobilise through walking. Mobility is important to function in the fast paced societies and people who have difficulty moving are always at a disadvantage. In the adult, becoming an independent member of the society and earning a living depends upon independent mobility. Families view ambulation as the most important issue during childhood. Every effort must be made to increase the child’s ability to walk; however, walking depends more on the extent of the child’s neurological impairment rather than the amount of physical therapy, surgery or bracing that they receive. The child can achieve his/her own maximum potential with practice.

Focus on ambulation should not result in neglecting communication and cognitive development. Priorities change in adolescence as they need education, independence, and an active social life. Although ambulation is still important, it is needed less to function. Learning how to use computers may benefit the adolescent in the long term rather than being able to take a few assisted steps.
Mobility is important for the child, whereas social identity and independence are more valuable for the adolescent.

Additional Resources 

Here Are Some Additional Resources Related to CP:

• Child Development and Development Delay
• Cerebral Palsy

References

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