What is Cerebral Palsy?
Cerebral palsy is a group of lifelong disorders that affects movement and activity. It’s caused by issues in the developing brain of a fetus or infant, making it the most common cause of disability in children. The way it affects movement and ability to function can vary based on the cause.
Causes can be traced back to issues before, during, or after birth. Those with cerebral palsy may often have other health conditions too, like epilepsy, problems with bones and muscles, intellectual disabilities, difficulties with eating, issues with vision or hearing, and problems with communication. It’s important to see a neurologist if there are early signs of these problems, especially if there’s a family history of them.
Once a diagnosis is made, comprehensive care is needed to maintain the best quality of life possible. Treating cerebral palsy requires a team of healthcare professionals because of the significant impact it has on a person’s ability to function.
What Causes Cerebral Palsy?
Cerebral palsy develops due to irregularities or harm to a baby’s brain either while it’s still in the womb, during birth, or after birth. The issues causing cerebral palsy don’t get worse over time, but the causes are often quite complex and involve several factors.
During the time in the womb, things like abnormalities in the brain’s development, infections, strokes, or chromosomal issues can contribute to cerebral palsy.
During birth, the baby’s brain could become deprived of oxygen, suffer from an infection that affects the brain, experience a stroke, or develop a rare condition called Kernicterus, all of which can cause cerebral palsy.
After birth, cerebral palsy may result from accidents or deliberate harm, infections that target the brain, strokes, or a lack of oxygen to the brain.
Being born prematurely is a significant risk factor for developing cerebral palsy. Some complications linked to premature birth that can lead to cerebral palsy include damage to the white matter of the brain, bleeding inside the brain, or stroke in the area surrounding the brain’s ventricles.
Other things that can increase the risk of cerebral palsy include twins or multiple birth pregnancies, slow growth within the womb, the mother’s substance abuse, high blood pressure in the mother, inflammation of the fetal membranes, issues with the placenta, aspiration of the first bowel movement, low blood sugar after birth, and genetic factors.
Risk Factors and Frequency for Cerebral Palsy
Cerebral palsy is the top cause of disability in children, affecting 1.5 to 2.5 out of every 1000 infants born. Premature infants have a much higher likelihood of developing this condition than those born full-term. The risk of cerebral palsy becomes even higher with earlier birth, specifically for infants born at less than 28 weeks of gestation. Babies who weigh less than 1500 grams also face an elevated risk.
- Cerebral palsy is the most common cause of disability in children.
- It affects 1.5 to 2.5 out of every 1000 newborns.
- Premature infants are at a significantly higher risk than full-term infants.
- Infants born earlier than 28 weeks gestation face the highest risk.
- Infants with a low birth weight (less than 1500 grams) are also at a higher risk – 5% to 15% of these infants develop cerebral palsy.
- About 80% of cerebral palsy cases are caused by events before birth, while about 10% are caused by events after birth.
Signs and Symptoms of Cerebral Palsy
Cerebral palsy is identified based on a patient’s personal history and a physical exam. The examination aims to find risk factors and likely causes of cerebral palsy. It’s essential to cover prenatal, birth, and development history. Particular focus is placed on the patient’s motor development, since delays are common in cerebral palsy. If the patient shows signs of regression in development, cerebral palsy might not be the cause. If several family members have similar issues with development or related neurological disorders, doctors might consider a genetic cause for the symptoms.
In addition to looking for cerebral palsy, healthcare providers also look for conditions that often go together with cerebral palsy, like epilepsy, problems with their musculoskeletal system, pain, issues with vision and hearing, problems with feeding, communication issues, and behavioral disorders.
The physical exam evaluates signs of cerebral palsy. This includes checks of the head size, mental status, muscle tone and strength, posture, reflexes, and way of walking. Symptoms could be a head size that’s too small or too big, being excessively irritable or less interactive, having inconsistent muscle tone, showing spasticity, dystonia, muscle weakness, keeping primitive reflexes, displaying abnormal or absent postural reflexes, being uncoordinated, and having brisk reflexes.
The physical exam can also identify the specific type of cerebral palsy. It can look at the type of irregularity in muscle tone and the location of motor abnormalities. There are several different kinds of cerebral palsy:
- Spastic diplegic: The patient displays increased muscle tension and difficulties in motor skills, with the legs being more affected than the arms.
- Spastic hemiplegic: The patient displays increased muscle tension and difficulties in motor skills affecting one side of the body, with the arms often more affected than the legs.
- Spastic quadriplegic: The patient displays increased muscle tension and difficulties in motor skills that affect all four limbs. Usually, the arms are more affected than the legs.
- Dyskinetic/hyperkinetic (choreoathetoid): The patient displays excessive, unplanned movements that resemble a combination of rapid, dance-like muscle contractions and slow wriggling movements.
- Dystonic: The patient has involuntary, sustained muscle contractions that result in twisting, repetitive movements.
- Ataxic: The patient displays unsteadiness, a lack of coordination, and low muscle tone.
Testing for Cerebral Palsy
Diagnosing cerebral palsy involves a few steps, including understanding the patient’s medical history, a physical exam, brain imaging, and standardized developmental assessments. Magnetic resonance imaging or MRI, which provides highly detailed images of the brain, is the preferred method for finding the cause of cerebral palsy.
Another valuable tool in diagnosis is an MRI, which is very efficient at spotting abnormal areas in the brain related to motor functions. Ultrasounds of the head, performed in newborns or infants, can also be informative. They can reveal conditions like intraventricular hemorrhage (bleeding into the brain’s ventricles), ventriculomegaly (enlarged ventricles), and periventricular leukomalacia (damage to the brain’s white matter).
Aside from imaging techniques, the General Movements Assessment (GM) is a standard tool used for children younger than 5 months. It involves observing the quality of an infant’s spontaneous movements when they are lying on their back. These observations can accurately predict cerebral palsy predictors. For older infants and toddlers between 2 and 24 months, the Hammersmith Infant Neurological Exam (HINE) is used. This assessment covers a physical exam, motor development documentation, and behavior state evaluation.
For patients who have had seizures, an electroencephalogram (EEG) – a brain wave test – is usually needed. In cases where a stroke led to cerebral palsy, a test for clotting abnormalities called thrombophilia screening may be recommended. It can identify these abnormalities in over half of this group of patients.
There are also many conditions that can be mistaken for cerebral palsy, and sometimes these conditions have treatments that can help manage or even stop their progression. That’s why it’s crucial to rule out conditions that mimic cerebral palsy when the patient’s history, physical exam, and brain scans seem abnormal. For example, if the patient has a family history of cerebral palsy or neurological disorders, shows regressing development, or experiences a rapid loss of neurological skills, especially during fasting or illness, further investigation is needed.
Additionally, patients showcasing these signs might need additional tests for inborn errors of metabolism or genetic testing, especially if they have physical deformities, a family history of cerebral palsy or consanguinity relationships. A lumbar puncture might also be necessary, particularly in patients with unexplained refractory seizures or movement disorders, to screen for neurotransmitter disorders and glucose transporter deficiency.
Treatment Options for Cerebral Palsy
Treating cerebral palsy is a team effort. The team is typically made up of a variety of medical professionals like physicians of different specialties, therapists who specialize in physical movement, occupation, and speech, mental health experts, social workers, case managers, and educational specialists. The aim is to provide the best possible quality of life for the patient and reduce the impact of disability. The patient, their family, and the team work together to set achievable goals, which are reviewed and updated regularly.
There are a number of different medications that can help manage cerebral palsy. Oral and injectable medications, like botulinum toxin, can help with abnormal muscle tone, pain, and other conditions that can occur alongside cerebral palsy, like epilepsy, excessive drooling, digestive issues, and behavioral disorders. Medications for muscle stiffness include benzodiazepines, baclofen, dantrolene, tizanidine, cyclobenzaprine, botulinum toxin, and phenol. Some medications used to treat involuntary movements include trihexyphenidyl, gabapentin, carbidopa-levodopa, and benztropine. For excessive drooling, treatments include glycopyrrolate, atropine drops, and scopolamine patches. If the patient has epilepsy, anti-seizure medications are used. Frequent constipation, a common issue in cerebral palsy, can be managed with stool softeners and agents that promote digestion. Pain can be dealt with using anti-inflammatory medications, and antidepressants are used for depression and anxiety.
There are also surgical options to manage cerebral palsy. These include implanting a pump that delivers baclofen (a muscle relaxer), carrying out a selective dorsal rhizotomy (a procedure that cuts certain nerve roots in the spinal cord to relieve spasticity), releasing tendons, rotating the hip, performing spinal fusion, fixing eye muscle issues, and stimulating the deep parts of the brain.
What else can Cerebral Palsy be?
Cerebral palsy can sometimes be confused with other medical conditions because the symptoms can appear similar. These include conditions like genetic disorders, metabolic disorders, spinal cord abnormalities, diseases affecting nerve and muscle function, disorders of movement, and tumors. Here’s a list of potential conditions that should be considered based on the primary symptoms:
If the main symptom is muscle stiffness (Spasticity):
- Inherited disorders causing muscle stiffness
- Spinal cord tethered or stuck to the spine
- Tumors on the spinal cord
- Adrenoleukodystrophy (a specific type of genetic disorder)
- Deficiencies of specific enzymes such as arginase and pyruvate dehydrogenase
- Rett syndrome and Lesch-Nyhan syndrome (genetic disorders)
- Conditions affecting brain’s white matter, like Pelizaeus-Merzbacher
- GLUT-1 transporter deficiency (which affects brain’s fuel supply)
If the primary symptom is abnormal muscle movements (Dystonia):
- Dopa-responsive dystonia (a specific type of movement disorder)
- Glutaric aciduria type 1 (a metabolic disorder)
- Genetic disorders like Lesch-Nyhan syndrome, Leigh disease, and Niemann-Pick type C
- GLUT-1 transporter deficiency
If the main symptom is low muscle tone (Hypotonia):
- Specific enzyme deficiency, like Holocarboxylase synthetase deficiency
- Conditions affecting brain and liver, like Zellweger syndrome
- Infantile Refsum disease (a genetic disorder that affects fat metabolism)
- Conditions affecting brain development such as Pontocerebellar hypoplasias
- Metachromatic leukodystrophy (a genetic disorder affecting nerves, muscles, and other organs)
If the main symptom is unsteady and clumsy movement (Ataxia):
- Conditions like Ataxia-telangiectasia or Angelman syndrome where balance and coordination are affected
- GLUT-1 transporter deficiency
- Leigh disease (a rare genetic disorder)
- Joubert syndrome (which affects brain’s coordination control centre)
If the main symptom is sudden, uncontrollable movements (Choreoathetosis):
- Conditions affecting brain’s white matter like Pelizaeus-Merzbacher
- Lesch-Nyhan syndrome
If the primary symptom is lack of strength (Weakness):
- Muscular dystrophies (a group of genetic diseases causing progressive weakness and loss of muscle mass)
- Metachromatic leukodystrophy
- Conditions affecting brain’s development like Pontocerebellar hypoplasias
What to expect with Cerebral Palsy
Most children with cerebral palsy live into adulthood. However, those severely affected may not live as long, often due to respiratory diseases such as aspiration pneumonia, which is a common cause of early death. The future of a child’s motor skills largely depends on the type of cerebral palsy, their rate of motor development, the presence of developmental reflexes, and their cognitive abilities.
Children who can walk independently usually achieve this milestone by the age of 3. Those who need support to walk might take up to 9 years to reach this point. If a child is not walking by the age of 9, it’s unlikely they will walk even with support. But, children with forms of cerebral palsy such as hemiplegic, choreoathetoid, and ataxic are likely to be able to walk eventually.
Certain signs can suggest a better chance of a child being able to walk on their own, including sitting by 24 months and crawling by 30 months. On the other hand, indicators of a poor outlook for walking include not being able to balance their head by 20 months, keeping their primitive reflexes, not having postural reflexes by 24 months, and not crawling by 5 years.
Possible Complications When Diagnosed with Cerebral Palsy
Cerebral palsy comes with a range of complications such as:
- Pain, affecting 50% to 75% of individuals
- Intellectual disability, seen in 50% of people
- Epilepsy, in 25% to 45% of cases
- Orthopedic disorders, including hip displacement, foot deformities, and scoliosis
- Speech impairment, in 40% to 50% of individuals
- Hearing impairment, affecting 10% to 20% of people
- Blindness, experienced by 10% of individuals
- Eye misalignment (strabismus), seen in 50% of people
- Behavioural disorders, in 25% of cases
- Growth failure
- Pulmonary disease
- Low bone density (osteopenia), affecting 77% of those moderately to severely affected
- Urologic conditions such as incontinence and neurogenic bladder, seen in 30% to 60% of cases
- Sleep disturbances, affecting 23% of individuals
- Dental abnormalities
Recovery from Cerebral Palsy
Studies have indicated that people benefit from cardio training, particularly rhythmic training. The frequency, intensity, and type of training often depend on factors such as initial fitness levels, available support, and goals. While muscle strengthening used to be avoided in those with muscle tightness, thinking it would worsen the condition, recent studies have shown its potential benefits, including reducing muscle tightness. However, ongoing studies are required to confirm these results. It’s preferred to carry out exercises involving multiple joints, but single-joint exercises can also be beneficial, especially for those who are significantly out of shape or tend to use compensations during multi-joint exercises.
Training for proper walking techniques has been proven as the best way to increase walking speed compared to others like strength, velocity, and feedback training. It’s vital because walking speed heavily influences overall life quality and functional mobility. Passive stretching was traditionally used to ease muscle tightness, and various different types are used. However, studies have showed limited evidence supporting its use for improving movement range or functionality. Though, long-stretched sessions might have more advantages, but further research is required to understand the impact of stretching.
Families’ involvement in therapy practices are crucial as research has shown improved results when therapies involve the patient’s family. It’s equally important to work together with the patient and their family or support system on the care plan.
Preventing Cerebral Palsy
Cerebral palsy is a term that refers to a range of conditions caused by a stable abnormality in the brain, which leads to problems with movement, muscle tone or posture. It is the leading cause of disability in children. Certain factors during pregnancy, at the time of birth, and after birth contribute to the development of cerebral palsy. Among the most significant of these risk factors are premature birth and low birth weight.
Other causes can include stroke, lack of oxygen to the brain, infections affecting the brain, and irregular development of the brain. Medical professionals diagnose cerebral palsy by examining the patient’s prenatal and birth history, performing a physical exam, and using techniques to visualize the brain.
The goal of treatment for cerebral palsy is to achieve the best possible functional outcome for the patient. Doctors take a team-based approach, with a focus on regular prenatal care and taking necessary measures to prevent premature birth, in order to reduce the risk of developing cerebral palsy.