What is Cerebral Palsy?
Cerebral palsy (CP) is a chronic motor disorder involving posture with or without movement that results from a non-progressive injury to the developing brain. Although the underlying brain injury is non-progressive, the clinical features of Cerebral Palsy may change over time as the child acquires new motor functions and as the cerebrum starts to exhibit control over sub-cortical centres of the brain.
Many children with CP function at a high educational and vocational level, without any sign of the type of cognitive dysfunction CP is commonly associated with. This means that a child with Cerebral Palsy may not show some signs until the child begins to grow; this does not mean that the disease is increasing but it is due to the fact that some centers of the brain that were initially affected were not being used when the child was a baby but as the child is growing and there is need for those centers to function, only then will the deficiency of the centers be manifested.
Cerebral Palsy Epidemiology
CP is the most common form of chronic motor disability that begins in childhood with a prevalence of 2/1000. This means that out of 1000 children, 2 are found with Cerebral Palsy. The condition was first described by Dr. Little, an orthopaedic surgeon about 170 years ago. He suggested that the primary causes of Cerebral Palsy were birth trauma and birth asphyxia as well as prematurity; he suggested that improved obstetric care would reduce the incidence of CP. Unfortunately, despite recent considerable advances in obstetric and neonatal care in developed countries; there has been virtually no change in the incidence of CP.
What causes Cerebral Palsy?
Cerebral Palsy is caused by a broad group of factors that can be grouped according to developmental factors, genetic factors, metabolic factors, ischaemic, infectious, and many others. The basic way to understand the cause of Cerebral Palsy is to know that anything that can cause injury to the brain of a baby that is developing will cause cerebral palsy. It is worthy of note that cerebral palsy involves injury to a DEVELOPING brain and not to an ALREADY developed brain.
Cerebral Palsy Causes (Etiology)
- Maternal infections can cause damage to the baby right in the womb. This is the reason why seeking Antenatal Care (ANC) is very important. ANC helps for early diagnosis and treatment of any infections without complications.
- Chorioamnionitis This is an inflammation of the membranes that enclose the baby in the womb. Any Pelvic infection during pregnancy can cause chorioamnionitis.
- Antepartum haemorrhage: bleeding of the pregnant before delivery predisposes the baby to risk of developing Cerebral Palsy.
- Eclampsia: severe hypertension during pregnancy that causes convulsion of the pregnant woman.
- Maternal drug abuse during pregnancy can cause Cerebral Palsy
- Trauma to the abdomen may injure the umbilical cord that that causes insufficient oxygen supply and nutrients to the brain of the baby while in the womb.
- Abdominal irradiation: use of ionizing radiations to the abdomen during pregnancy
- Congenital Central Nervous System (CNS) malformations
- Other congenital anomalies especially abnormalities of development of the babys heart (cardiovascular system abnormalities)
- Congenital infections affecting the baby
- Umbilical Cord accidents
- Prolonged obstructed labour can cause severe stress to the baby during delivery
- Precipitate labour: delivery of a baby within a short period of time such as within 2 hours of labour predisposes the child to Cerebral Palsy because of the abrupt and forceful delivery
- Instrumental delivery: use of instruments to deliver a baby can place the baby at risk especially when the doctor carrying out this is not skilled. Injury may occur in the process.
- Early separation of the placenta
- Severe Birth Asphyxia (Hypoxic Ischemic Encephalopathy)
- Prematurity/Low Birth Weight (LBW): a premature baby stands the risk of having Cerebral Palsy for so many reasons such as predisposition of the premature baby to developing infections, inability to control body temperature and lack of energy. These factors are in themselves risks for developing Cerebral Palsy and the premature infant is prone to them.
- Multiple births: such as giving birth to twins, triplets or more.
- Intracranial haemorrhage
- Birth trauma: any injury during delivery especially in women that give birth at home are predisposing their children to developing Cerebral Palsy
- Bilirubin encephalopathy: this occurs as a result of excess bilirubin in the blood of the baby that is not treated in time leading to damage to the brain. Initially, the warning sign for this is yellowish eye colour of a baby that persists even for few days is sufficient to cause Cerebral Palsy.
- Hypoglycaemia: low blood glucose (or what is commonly referred by non-medical persons as low blood sugar level) is an important cause of Cerebral Palsy. This is the reason why breastfeeding a baby immediately after birth (within 30 minutes is very important)
- Meningitis: a severe infection affecting the coverings of the brain of the baby can cause Cerebral Palsy. This meningitis can be caused by different microorganisms and can also be a complication of some diseases.
- Encephalitis: inflammation of the brain itself as result of infections
- Inborn errors of metabolism: errors of the digestive system being unable to digest or process some specific elements found in food such as lactose, phenylalanine etc.
- Near drowning
- Hypertensive stroke
- Severe shock
- Acute cerebral syndrome/Sickle cell stoke
Types and Classifications of Cerebral Palsy
CP may be classified by a description of the motor handicap in terms of physiological, topographical, etiological categories and functional capacity. The physiological classification identifies the major motor abnormality; Topographical taxonomy indicates the involved extremities. The International and Edinburgh classifications are perhaps the most acceptable clinical classification of Cerebral Palsy as it combines both physiological and topographical characteristics
Physiological Classification of Cerebral Palsy Types
- Spastic Cerebral Palsy
- Athetoid Cerebral Palsy
- Rigid Cerebral Palsy
- Ataxic Cerebral Palsy
- Tremor Cerebral Palsy
- Atonic Cerebral Palsy
- Mixed Cerebral Palsy
- Unclassified Cerebral Palsy
Topographical Classification of Types of Cerebral Palsy
- Double hemiplegia
Functional Classification of Cerebral Palsy
- Class I- with no limitation of activity
- Class II- with slight to moderate limitation
- Class III- with moderate to great limitation
- Class IV- with no useful physical activity
International classification of Cerebral Palsy Types
Edinburgh Classification of Types of Cerebral Palsy
- Spastic Hemiplegia
- Bilateral (double) Spastic Hemiplegia
- Spastic Diplegia
Clinical features (Symptoms and Signs Cerebral Palsy Types)
Spastic hemiplegia symptoms and signs
- Spastic CP constitute 70-80% of all Cerebral Palsy
- Infants with spastic hemiplegia have decreased spontaneous movements on the affected side and show hand preference at a very early age.
- The upper limb is often more involved than the lower limb and difficulty in hand manipulation is obvious by 1 year of age.
- Walking is usually delayed until 1824 months and a circumductive gait is apparent.
- Examination of the extremities may show growth arrest, particularly in the hand and thumbnail.
- Spasticity is apparent in the affected extremities, particularly the ankle, causing an equinovarus deformity of the foot.
- An affected child often walks on tiptoe because of the increased tone, and the affected upper extremity assumes a dystonic posture when the child runs.
- Ankle clonus and a Babinski sign may be present, the deep tendon reflexes are increased, and weakness of the limbs is evident.
- About one third of patients with spastic hemiplegia have a seizure disorder that usually develops in the 1st year or 2.
- About 25% have cognitive abnormalities including intellectual disability.
- Focal cerebral infarction (stroke) secondary to intrauterine or perinatal thromboembolism is a common cause
- Other causes include Severe birth asphyxia, Intraventricular haemorrhage, head trauma, and stroke from other pathologies
Bilateral spastic hemiplegia symptoms and signs
- A tetraplegia with the upper limbs more affected than the lower limbs
- Uncommon, about 5% of all CP
- Usually associated with congenital CNS malformations, meningitis & head injury
- Presents as hemiplegia, though bilateral
- Additional involvement of bulbar musculature with feeding difficulties & speech defect
- Usually associated with seizure disorder
- Patients tend to die early in life from aspiration pneumonia and intercurrent infections
Spastic diplegia Cerebral Palsy symptoms and signs
Spastic diplegia is bilateral spasticity of the legs greater than in the arms. The first indication of spastic diplegia is often noted when an affected infant begins to crawl.
- The child uses the arms in a normal reciprocal fashion but tends to drag the legs behind more as a rudder (commando crawl) rather than using the normal four-limbed crawling movement.
- If the spasticity is severe, application of a diaper is difficult because of the excessive adduction of the hips.
- If there is paraspinal muscle involvement, the child may be unable to sit.
- Examination of the child reveals spasticity in the legs with brisk reflexes, ankle clonus, and a bilateral Babinski sign.
- When the child is suspended by the axillae, a scissoring posture of the lower extremities is maintained.
- Walking is significantly delayed, the feet are held in a position of equinovarus, and the child walks on tiptoe.
- Severe spastic diplegia is characterized by disuse atrophy and impaired growth of the lower extremities and by disproportionate growth with normal development of the upper torso.
- The prognosis for normal intellectual development is excellent for these patients, and the likelihood of seizures is minimal.
- The most common neuro-pathologic finding is periventricular leukomalacia, particularly in the area where fibers innervating the legs course through the internal capsule.
- Common causes include congenitssal malformations, multiple births, and prematurity or Low birth weight
Dyskinesia Cerebral Palsy symptoms and signs
Also called choreoathetoid or extrapyramidal Cerebral Palsy. It is less common than spastic cerebral palsy and accounts for 10-15% of all CPs.
- Affected infants are characteristically hypotonic with poor head control and marked head lag and develop increased variable tone with rigidity and dystonia over several years.
- Feeding may be difficult, and tongue thrust and drooling may be prominent.
- Speech is typically affected because the oropharyngeal muscles are involved.
- Speech may be absent or sentences are slurred, and voice modulation is impaired.
- High frequency deafness or even total hearing loss is common
- Generally, upper motor neuron signs are not present, seizures are uncommon, and intellect is preserved in many patients.
- This form of CP is the type most likely to be associated with birth asphyxia.
- Dyskinetic CP can also be caused by kernicterus secondary to high levels of bilirubin
- The main pathological changes are found in the basal ganglia.
- Involuntary muscle movement & tone changes seen in dyskinetic CP include: Dystonia, Athetosis, Chorea, Tremor and Tension
Ataxic Cerebral Palsy Symptoms and Signs
Least common type of CP, less than 5% and common causes include congenital malformations, meningitis and encephalitis
- Hypotonia which usually persists into early childhood
- Common features include delayed motor development, dysarthria, intention tremor, ataxic stance and gait disorders.
- Intelligence is normal in most cases
- Main neuropathological lesion is mal-development of the cerebellum
Mixed forms of Cerebral palsy
- In some cases of CP, the presentation may show features of more than one clinical type
- Accounts for 10-15% of all CPs
Associated Disabilities (Complications) of Cerebral Palsy
CP is commonly associated with a spectrum of developmental disabilities which are listed below
- mental retardation
- Seizure disorder
- Visual disorders
- Hearing impairment
- Speech disorders
- Cognitive disability
- Behavioural abnormalities
- Attention Deficit Hyperactivity Disorder (ADHD)
- Drooling of saliva
The motor handicap may be the least of the child’s problems.
Cerebral Palsy Diagnosis
Diagnosis of Cerebral Palsy is primarily made clinically. Early recognition of a child with CP is very important. Active search for presence of risk factors for cerebral palsy such as Severe Neonatal Jaundice, Prematurity, Hypoglycaemia (as a result of prolonged delay in feeding), Central Nervous System infections (meningitis, encephalitis). The absence of risk factors does not exclude the fact that a child can still develop cerebral palsy
Newborn examination should be carried out to check for:
- Lethargy: lack of energy of the baby
- Poor feeding
- Persistence of primitive reflexes
- Serial measurement of head circumference for easy diagnosis of any abnormal development
- Movement of the limbs: hand dominance before 1yr may point to cerebral palsy
- Thorough search for congenital malformations (whether of CNS or extracranial malformations)
- Eye examination of the baby for cataract, retinitis or optic atrophy
A thorough history and physical exam should preclude a progressive disorder of the CNS, including degenerative diseases, metabolic disorders, spinal cord tumour, or muscular dystrophy.
Investigations that may help in diagnosis of cerebral palsy
- Skull X-ray may show intracranial calcifications
- An MRI scan of the brain is indicated to determine the location and extent of structural lesions or associated congenital malformations.
- Additional studies may include tests of hearing and visual function.
Cerebral Palsy Treatment
A multidisciplinary team of physicians from various specialties, as well as occupational and physical therapists, speech therapists, social workers, educators, and developmental psychologists provide important contributions to the treatment of these children.
Counselling is very important as soon as the diagnosis of cerebral palsy is made; Both parents should be present and the nature of the illness should be explained to them such as the cause, they should know that there is no cure yet for cerebral palsy, and also know that it will involve long-term management with Family support being crucial.
Parents should be taught how to work with their child in daily activities such as feeding, carrying, dressing, bathing, and playing in ways that limit the effects of abnormal muscle tone.
They also need to be instructed in the supervision of a series of exercises designed to prevent the development of contractures, especially a tight Achilles tendon.
There is no proof that physical or occupational therapy prevents development of CP in infants at risk or that it corrects the neurologic deficit, but evidence shows that therapy optimizes the functioning of children to achieve their maximum potential
Some drugs used in management of cerebral palsy
Several drugs have been used to treat spasticity, including oral dantrolene sodium, the benzodiazepines, and baclofen.
Botulinum toxin injected into salivary glands may also help reduce the severity of drooling of saliva, which is seen in 1030% of patients with cerebral palsy and has been traditionally treated with anticholinergic agents.
Patients with rigidity, dystonia, and spastic quadriparesis sometimes respond to levodopa, and children with dystonia may benefit from carbamazepine.
Anticonvulsants may be used for recurrent seizures.
Cerebral Palsy Prognosis
Long term outcome depends largely on the severity of the motor disability, and the presence with severity of intellectual disability. Other factors that determine the prognosis include: other associated disabilities, level of motivation of the family, Support from the Family, community and society, type and intensity of facilities available for rehabilitative intervention.
Cerebral Palsy Prevention
- Good Antenatal Care
- Well supervised pregnancy by skilled health provider
- Appropriate investigations
- Early identification of high risk pregnancies
- Immunization against congenital infections such as Toxoplasmosis, Rubella, Herpes
- Avoid home delivery
- Well trained birth attendants
- Efficient resuscitation of asphyxiated babies
- Good referral system
- Newborn babies should be properly examined to check for any condition or abnormality that may predispose the baby to developing Cerebral Palsy
- Neonatal screening of newly born babies for G6PD deficiency and Rhesus isoimmunisation
- Good Neonatal Intensive Care Unit in order to properly manage babies that are Preterm, Low birth weight, babies with Severe Jaundice (yellowing of the eyes), Birth trauma and Intracranial haemorrhage
Cerebral palsy may manifest differently from one person to another. They need our support and love.