Cerebral Palsy – Causes, Symptoms, Diagnosis, Treatment

Cerebral Palsy is a group of neurological non-progressive/ permanent movement disorders that appear in early childhood or before, during, or after birth due to brain injury or abnormal development of the brain or injury of the brain & central nervous system.  Often, symptoms include poor coordination, stiff muscles, weak muscles, tre, more, and maybe problems with sensation, vision, hearing, swallowing, and speaking.

Cerebral palsy is a broad range of static, non-progressive motor disabilities that present from birth or early childhood as a result of injury to neuromotor components of the central nervous system. Motor performance is normally coordinated via communication between the cerebral cortex, thalamus, basal ganglia, brain stem, cerebellum, and spinal cord, and communicating sensory-motor pathways. This complex network lends itself to injury at many different levels. Etiologies are numerous and can occur during the prenatal, perinatal, and postnatal periods. The severity of the neurologic deficit and the clinical manifestations are varied depending on the time, location, and nature of the original injury. To approach cerebral palsy systematically, the primary health care practitioner must be prepared to recognize neuromotor deficits, diagnose and classify the type of disorder, and implement a methodical treatment plan. The purpose of this article is to review the etiology, pathophysiology, diagnostic classification (Swedish system), clinical manifestations, and therapeutic management of cerebral palsy and prepare the advanced practice nurse to care for the individual and family.

 

Types of Cerebral Palsy

Geographical classification of cerebral palsy.

Physiologically, cerebral palsy can be divided into a spastic type, which affects the corticospinal (pyramidal) tracts, and an extrapyramidal type, which affects the other regions of the developing brain. The extrapyramidal types of cerebral palsy include athetoid, choreiform, ataxia, rigid, and hypotonic.

The specific forms of cerebral palsy are determined by the extent, type, and location of a child’s abnormalities. Doctors classify CP according to the type of movement disorder involved — spastic (stiff muscles), athetoid (writhing movements), or ataxic (poor balance and coordination) — plus any additional symptoms, such as weakness (paresis) or paralysis (plegia). For example, hemiparesis (Hemi = half) indicates that only one side of the body is weakened. Quadriplegia (quad = four) means all four limbs are affected.

Spastic cerebral palsy – is the most common type of disorder. People have stiff muscles and awkward movements. Forms of spastic cerebral palsy include:

• Spastic hemiplegia/hemiparesis – typically affects the arm and hand on one side of the body, but it can also include the leg. Children with spastic hemiplegia generally walk later and on tip-toe because of tight heel tendons. The arm and leg of the affected side are frequently shorter and thinner. Some children will develop an abnormal curvature of the spine (scoliosis). A child with spastic hemiplegia may also have seizures. The speech will be delayed and, at best, maybe competent, but intelligence is usually normal.
• Spastic diplegia/diparesis – involves muscle stiffness that is predominantly in the legs and less severely affects the arms and face, although the hands may be clumsy. Tendon reflexes in the legs are hyperactive. The toes point up when the bottom of the foot is stimulated. Tightness in certain leg muscles makes the legs move like the arms of a scissor. Children may require a walker or leg braces. Intelligence and language skills are usually normal.
• Spastic quadriplegia/quadriparesis – is the most severe form of cerebral palsy and is often associated with moderate-to-severe intellectual disability. It is caused by widespread damage to the brain or significant brain malformations. Children will often have severe stiffness in their limbs but floppy necks. They are rarely able to walk. Speaking and being understood is difficult. Seizures can be frequent and hard to control.
• Dyskinetic cerebral palsy (also includes athetoid, choreoathetosis, and dystonic cerebral palsies) – is characterized by slow and uncontrollable writhing or jerky movements of the hands, feet, arms, or legs. Hyperactivity in the muscles of the face and tongue makes some children grimace or drool. They find it difficult to sit straight or walk. Some children have problems hearing, controlling their breathing, and/or coordinating the muscle movements required for speaking. Intelligence is rarely affected in these forms of cerebral palsy.
• Athetoid – Athetoid cerebral palsy or dyskinetic cerebral palsy (sometimes abbreviated ADCP) is primarily associated with damage to the basal ganglia in the form of lesions that occur during brain development due to bilirubin encephalopathy and hypoxic-ischemic brain injury. ADCP is characterized by both hypertonia and hypotonia, due to the affected individual’s inability to control muscle tone. Clinical diagnosis of ADCP typically occurs within 18 months of birth and is primarily based on motor function and neuroimaging techniques. Athetoid dyskinetic cerebral palsy is a non-spastic, extrapyramidal form of cerebral palsy. Dyskinetic cerebral palsy can be divided into two different groups; choreoathetosis and dystonic. Choreoathetotic CP is characterized by involuntary movements most predominantly found in the face and extremities. Dystonic ADCP is characterized by slow, strong contractions, which may occur locally or encompass the whole body.
• Ataxic cerebral palsy – affects balance and depth perception. Children with ataxic CP will often have poor coordination and walk unsteadily with a wide-based gait. They have difficulty with quick or precise movements, such as writing or buttoning a shirt, or a hard time controlling voluntary movements such as reaching for a book.
• Mixed types  – of cerebral palsy refer to symptoms that don’t correspond to any single type of CP but are a mix of types. For example, a child with mixed CP may have some muscles that are too tight and others that are too relaxed, creating a mix of stiffness and floppiness.

Causes of Cerebral Palsy

Muscle control takes place in a part of the brain called the cerebrum. The cerebrum is the upper part of the brain. Damage to the cerebrum before, during, or within 5 years of birth can cause cerebral palsy.

• The cerebrum is also responsible for memory, the ability to learn, and communication skills. This is why some people with cerebral palsy have problems with communication and learning. Cerebrum damage can sometimes affect vision and hearing.
• Some newborns are deprived of oxygen during labor and delivery. In the past, it was thought that this lack of oxygen during birth led to brain damage.
• However, during the 1980s, research showed that fewer than 1 in 10 cases of cerebral palsy stem from oxygen deprivation during birth. Most often, the damage occurs before birth, probably during the first 6 months of pregnancy.

There are at least three possible reasons for this.

Periventricular leukomalacia (PVL)

• PVL is a kind of damage that affects the brain’s white matter because of a lack of oxygen in the womb. It may occur if the mother has an infection during pregnancy, such as rubella or German measles, low blood pressure, preterm delivery, or if she uses an illegal drug.

Abnormal development of the brain

• Disruption of brain development can affect the way the brain communicates with the body’s muscles and other functions. During the first 6 months of pregnancy, the brain of the embryo or fetus is particularly vulnerable. Damage can stem from mutations in the genes responsible for brain development, certain infections such as toxoplasmosis, a parasite infection, herpes and herpes-like viruses, and head trauma.

Intracranial hemorrhage

• Sometimes, bleeding inside the brain happens when a fetus experiences a stroke. Bleeding in the brain can stop the supply of blood to vital brain tissue, and this tissue can become damaged or die. The escaped blood can clot and damage surrounding tissue.

Several factors can cause a stroke in a fetus during pregnancy

• A blood clot in the placenta blocks the flow of blood
• A clotting disorder in the fetus
• Interruptions in arterial blood flow to the fetal brain
• Untreated pre-eclampsia in the mother
• Inflammation of the placenta
• Pelvic inflammatory infection in the mother

During delivery, the risk is increased by the following factors:

• Emergency cesarean
• The second stage of labor is prolonged
• Vacuum extraction is used during delivery
• Fetal or neonatal heart anomalies
• Umbilical cord abnormalities

Anything that increases the risk of preterm birth or low birth weight also raises the risk of cerebral palsy.

Factors that may contribute to a higher risk of cerebral palsy include:

• Multiple births, for example, twins
• Damaged placenta
• Sexually transmitted infections (STIs)
• Consumption of alcohol, illegal drugs, or toxic substances during pregnancy
• Malnourishment during pregnancy
• Random malformation of the fetal brain
• Small pelvis in the mother
• Breech delivery

Brain damage after birth

• A small proportion of cases happen because of damage after birth. This can happen because of an infection such as meningitis, a head injury, a drowning accident, or poisoning.
• When damage occurs, it will do so soon after the birth. With age, the human brain becomes more resilient and able to withstand more damage.

Effects of Cerebral Palsy

Symptoms of Cerebral Palsy

Signs and symptoms can vary greatly. Movement and coordination problems associated with cerebral palsy may include:

• Variations in muscle tone, such as being either too stiff or too floppy
• Stiff muscles and exaggerated reflexes (spasticity)
• Stiff muscles with normal reflexes (rigidity)
• Lack of muscle coordination (ataxia)
• Tremors or involuntary movements
• Slow, writhing movements (athetosis)
• Delays in reaching motor skills milestones, such as pushing up on arms, sitting up alone, or crawling
• Favoring one side of the body, such as reaching with only one hand or dragging a leg while crawling
• Difficulty walking, such as walking on toes, a crouched gait, a scissors-like gait with knees crossing, a wide gait, or an asymmetrical gait
• Excessive drooling or problems with swallowing
• Difficulty with sucking or eating
• Delays in speech development or difficulty speaking
• Difficulty with precise motions, such as picking up a crayon or spoon
• Seizures

The disability associated with cerebral palsy may be limited primarily to one limb or one side of the body, or it may affect the whole body. The brain disorder causing cerebral palsy doesn’t change with time, so the symptoms usually don’t worsen with age. However, muscle shortening and muscle rigidity may worsen if not treated aggressively.

Brain abnormalities associated with cerebral palsy also may contribute to other neurological problems. People with cerebral palsy may also have:

• Difficulty with vision and hearing
• Intellectual disabilities
• Seizures
• Abnormal touch or pain perceptions
• Oral diseases
• Mental health (psychiatric) conditions
• Urinary incontinence

Treatment  of Cerebral Palsy 

• Cerebral palsy can’t be cured  – but treatment will often improve a child’s capabilities.  Many children go on to enjoy near-normal adult lives if their disabilities are properly managed. In general, the earlier treatment begins, the better chance children have of overcoming developmental disabilities or learning new ways to accomplish the tasks that challenge them.
• There is no standard therapy  – that works for every individual with cerebral palsy. Once the diagnosis is made, and the type of CP is determined, a team of healthcare professionals will work with a child and his or her parents to identify specific impairments and needs, and then develop an appropriate plan to tackle the core disabilities that affect the child’s quality of life.
• Occupational therapy  – focuses on optimizing upper body function, improving posture, and making the most of a child’s mobility. Occupational therapists help individuals address new ways to meet everyday activities such as dressing, going to school, and participating in day-to-day activities.
• Recreation therapy – encourages participation in art and cultural programs, sports, and other events that help an individual expand physical and cognitive skills and abilities. Parents of children who participate in recreational therapies usually notice an improvement in their child’s speech, self-esteem, and emotional well-being.
• Speech and language therapy – can improve a child’s ability to speak, more clearly, help with swallowing disorders, and learn new ways to communicate—using sign language and/or special communication devices such as a computer with a voice synthesizer, or a special board covered with symbols of everyday objects and activities to which a child can point to indicate his or her wishes.
• Treatments for problems with eating and drooling – are often necessary when children with CP have difficulty eating and drinking because they have little control over the muscles that move their mouth, jaw, and tongue. They are also at risk for breathing food or fluid into the lungs, as well as for malnutrition, recurrent lung infections, and progressive lung disease.

Drug Treatments

Nonoperative modalities, such as medication, splinting and bracing, and physical therapy, are commonly used as primary treatment or in conjunction with other forms of treatment such as surgery.^[rx]–[rx]

Oral medications such as

• Diazepam,
• Baclofen
• Tolperisone and Eperisone relax muscles to increase muscle tone
• Phenobarbitone
• Gabapentin/Pregabalin inhibits the pain receptor in the brain.
• Mecobalamin /vitamin B12 regenerates the myelin sheet in the nervous system.
• Steroids suppress brain inflammation.
• Clonazepam drops to control salivation & proper sleep.
• Tizanidine – is usually used as the first line of treatment to relax stiff, contracted, or overactive muscles. Some drugs have some risky side effects such as drowsiness, changes in blood pressure, and the risk of liver damage that require continuous monitoring. Oral medications are most appropriate for children who need only a mild reduction in muscle tone or who have widespread spasticity.
• Botulinum toxin (BT-A) – injected locally, has become a standard treatment for overactive muscles in children with spastic movement disorders such as CP. BT-A relaxes contracted muscles by keeping nerve cells from over-activating muscle. The relaxing effect of a BT-A injection lasts approximately 3 months. Undesirable side effects are mild and short-lived, consisting of pain upon injection and occasionally mild flu-like symptoms. BT-A injections are most effective when followed by a stretching program including physical therapy and splinting. BT-A injections work best for children who have some control over their motor movements and have a limited number of muscles to treat, none of which is fixed or rigid.
• Intrathecal baclofen- therapy uses an implantable pump to deliver baclofen, a muscle relaxant, into the fluid surrounding the spinal cord. Baclofen decreases the excitability of nerve cells in the spinal cord, which then reduces muscle spasticity throughout the body. The pump can be adjusted if muscle tone is worse at certain times of the day or night. The baclofen pump is most appropriate for individuals with chronic, severe stiffness or uncontrolled muscle movement throughout the body
• Dantrolene –  a peripheral-acting drug, acts at the level of skeletal muscle and selectively decreases abnormal muscle stretch reflexes and tone. Dantrolene is used less frequently than other medications because some patients taking it to develop profound weakness, and there is a risk of hepatotoxicity with its long-term use.

Common medications used to treat cerebral palsy

Each child with cerebral palsy is likely to have a unique combination of medications to treat their specific condition. Some medications are short-term, while others are long-term. Medications and dosages are often used in combination and will likely be adjusted over time.

Common classes of medications for children with cerebral palsy include:

• Anticonvulsants
• Antidepressants
• Anti-inflammatories
• Muscle relaxants
• Benzodiazepines
• Nerve blocks
• Botox
• Baclofen
• Anticholinergics
• Stool Softeners

Medications for spasticity

Spastic cerebral palsy causes spasms and stiff muscles. causes spasms and stiff muscles. This can be painful. Medications to control these symptoms may be given orally or through an injection. Some can be continuously infused through a small pump similarly to how Insulin can be given to diabetics.

Generalized spasticity is typically treated with muscle relaxants such as Baclofen or Botox and benzodiazepines such as Valium. Other medications can also be effective, and the healthcare team can help weigh the risks with the benefits

If spasticity is localized in a specific area, medication may be injected into those muscles. Botox is often used to reduce localized spasticity.

Common side effects from medications that reduce spasticity include:

• Fatigue
• Weakness
• Loss of coordination
• Dizziness
• Headaches
• Blurred vision
• Nausea

There are also more serious short and long-term side effects and some medications are not recommended for children.

Medications for involuntary movement

Athetoid cerebral palsy (also known as dyskinetic cerebral palsy) is characterized by low muscle tone and involuntary movements. abnormal muscle tone and involuntary movements. Children with dyskinetic CP may be prescribed medications to keep uncontrollable movements to a minimum.

Anticholinergic drugs ease symptoms by blocking nerve impulses that cause uncontrollable movements. They may also help control drooling, a common symptom of dyskinetic cerebral palsy.

Common side effects include:

• Incontinence
• Dry mouth
• Dizziness
• Constipation

Medications for seizures

Injuries to the brain increase the likelihood of developing seizures. Therefore, it isn’t surprising that many children with cerebral palsy develop some form of epilepsy. A report by the Centers for Disease Control in 2008 found that 41 percent of children with CP across multiple states had co-occurring epilepsy. Several medications reduce the frequency of seizures.

Two common types of medications used to treat seizures are barbiturates and benzodiazepines. Both slow the activity of the brain and nervous system which helps prevent seizures. Phenobarbital is a common barbiturate medication given to newborns with seizure activity. Benzodiazepines that are often prescribed include Valium, Ativan, Dilantin, and Versed. These drugs are useful anticonvulsants, muscle relaxants, and anti-anxiety drugs.

Anticonvulsant medications, such as Keppra, are commonly used in children with CP to prevent seizure activity.

Some of the common side effects of these drugs include:

• Dizziness
• Weakness
• Fatigue
• Confusion
• Hypotension

Secondary conditions treated with medications

Secondary conditions are common in children with cerebral palsy and other birth injuries because of abnormal muscle movement and brain activity. Some examples of co-occurring conditions are acid reflux, constipation or incontinence, respiratory issues, and difficulty with feeding. Additionally, the brain damage that causes CP may also cause separate issues, such as intellectual disorders that require medication.

Incontinence

The inability to control urinary muscles often leads to incontinence. Tofranil is an antidepressant that can also be used to contract the bladder and neck muscles and control incontinence. DDAVP (desmopressin acetate) mimics natural hormones to decrease urine production and can be used as a nasal spray.

Acid reflux

A secondary issue caused by a lack of muscle control is gastroesophageal reflux. In children with CP, the muscle that prevents stomach acid from traveling up the esophagus may not be strong enough to prevent acid reflux. Over-the-counter medications that decrease acid production in the stomach, such as Zantac, are often beneficial.

Behavioral disorders

Some children with cerebral palsy develop behavioral or intellectual disorders. Attention deficit hyperactivity disorder (ADHD) and autism are common in children with CP. Adderall and Ritalin are some of the most common medications used to treat ADHD. Antipsychotic medications, such as Risperidone, are used to help with Autism symptoms.

Constipation

Lactulose is a synthetic sugar used to treat constipation. MiraLax is often used to treat children with constipation. It works by drawing water into the bowels to help soften stool and move it through the GI tract. This is considered to have low side effects.

Increased oral secretions

Children with CP often have limited or no motor control leading to excessive drooling. Anticholinergic medications, such as Rubino, are effective in controlling the increased oral secretions.

Is medication necessary?

Many parents are understandably concerned about placing their children on medications. Fear of side effects and dependence on these medications often lead parents to explore alternatives.

Activities such as yoga, chiropractic care, music, aquatic therapy, acupuncture, and occupational, and physical therapy can be effective in treating the symptoms related to CP and are often used in conjunction with medications.

Doctors and members of the care team can help decide what is best for your loved one. Please consult your child’s doctor before changing or discontinuing any medications. Doctors strive to tailor medications to each child’s specific needs. Discussing your goals and quality of life expectations with your child’s care providers can help your family find the best strategies to balance CP and its challenges.

Surgery

• Surgery to cut nerves – Selective dorsal rhizotomy (SDR) is a surgical procedure recommended for cases of severe spasticity when all of the more conservative treatments – physical therapy, oral medications, and intrathecal baclofen — have failed to reduce spasticity or chronic pain. A surgeon locates and selectively severs overactivated nerves at the base of the spinal column. SDR is most commonly used to relax muscles and decrease chronic pain in one or both of the lower or upper limbs. It is also sometimes used to correct an overactive bladder. Potential side effects include sensory loss, numbness, or uncomfortable sensations in limb areas once supplied by the severed nerve.
• Orthopedic surgery
  • Orthopedic surgery is often recommended when spasticity and stiffness are severe enough to make walking and moving about difficult or painful. For many people with CP, improving the appearance of how they walk – their gait – is also important. Surgeons can lengthen muscles and tendons that are proportionately too short, which can improve mobility and lessen pain. Tendon surgery may help the symptoms of some children with CP but could also have negative long-term consequences. Orthopedic surgeries may be staggered at times appropriate to a child’s age and level of motor development. Surgery can also correct or greatly improve spinal deformities in people with CP. Surgery may not be indicated for all gait abnormalities and the surgeon may request a quantitative gait analysis before surgery.

  The 6 main types of orthopedic procedures are:

  • Muscle lengthening – Surgical lengthening of the muscles used to relieve tightness in the hands and fingers. Lengthening the arm muscles can help children grasp objects more easily and improve fine motor skills.
  • Tendon lengthening – Lengthening tendons can reduce painful contractures, which is a permanent tightening of the tendons or muscles. This surgery is often used to improve a child’s ability to walk and sit upright.
  • Tendon transfer – Tendon transfers typically involve the cutting and replacement of a tendon. The goal of tendon transfers is to ensure that the muscles in the body are properly aligned. This surgery also helps reduce any pain or walking problems that children with CP may be experiencing, as well as improves the extension and flexibility of the wrists.
  • Tenotomy/myotomy – Tenotomy describes the cutting of the tendon, whereas myotomy involves cutting the muscle. This procedure is used to improve muscle function, increase control of the upper limbs and enhance the ability to grasp objects with hands and feet.
  • Osteotomy – This procedure is used to realign joints for better posture and mobility. It involves repositioning bones at angles more conducive to healthy alignments and is commonly used to correct hip dislocations in children with CP.
  • Arthrodesis – In severe cases of spasticity, when splints and casts aren’t enough, arthrodesis may be used to permanently fuse bones. Fusing the bones in the ankle and foot can make it easier for a child to walk.

  The type of orthopedic surgery that would be best for your child depends mostly on whether they are considered to be ambulatory or not. An ambulatory child can walk around independently or with the use of mobility aids such as crutches or walkers. A nonambulatory child is unable to walk or requires the use of a wheelchair to move about.

• AmbulatoryAmbulatory children with cerebral palsy may tend to step mostly on their toes, cross their hips while walking or bend their knees when taking strides. These issues can be caused by tight muscles, weakness, or misalignment of the joints or bones.

  The purpose of surgery for ambulatory children is to improve their ability to walk, which is essential to improving self-reliance. For example, a child who “toe-walks” may have their stride corrected with a tenotomy or tendon lengthening procedure. Orthopedic surgery can also correct issues caused by poor alignment of the hips or knees.

• Non-ambulatoryNon-ambulatory children are unable to walk, which is why the goal of orthopedic surgery is more centered around increasing comfort and avoiding any further mobility complications.

  Orthopedic surgery is used to stop certain complications from getting worse. Surgeries correcting spinal curvature, hip dislocations, and contractures alleviate pain and promote a comfortable, well-aligned sitting position. Osteotomy may be used to fuse bones in the spine to correct severe scoliosis.

  The benefits of orthopedic surgery aren’t immediate. Children often require physical therapy to ensure a successful recovery and to keep muscles strong and flexible.

• Selective dorsal rhizotomySelective dorsal rhizotomy (SDR) is an aggressive procedure that helps reduce pain and spasticity. SDR is typically recommended in severe cases of spasticity when other treatments have failed to make an impact. Children with spasticity in the legs (spastic diplegia) tend to benefit more than children with spasticity in other locations (quadriplegia, hemiplegia).

  Selective dorsal rhizotomy gets to the root of spasticity issues by cutting the nerves in the spinal column responsible for muscle stiffness. This procedure requires navigating the complex network of nerves to pinpoint which nerves are causing movement issues. SDR can be used to relax muscles in the upper and lower limbs.

  Selective dorsal rhizotomy improves:

  • Walking
  • Sitting
  • Standing
  • Balance
  • Deformities in tendons, muscles, feet, and hips
  • Voluntary movement

  Recovery from selective dorsal rhizotomy requires intensive physical therapy to relearn muscle control and movement.

  Other conditions

  Cerebral palsy affects every child differently, and most children who have it experience secondary conditions, such as hearing loss or acid reflux. These secondary conditions can be a result of limited movement caused by cerebral palsy, or they may be a direct result of the brain injury experienced at birth.

Assistive devices

• Assistive devices such devices as computers, computer software, voice synthesizers, and picture books can greatly help some individuals with CP improve their communication skills. Other devices around the home or workplace make it easier for people with CP to adapt to activities of daily living.
• Orthotic devices help to compensate for muscle imbalance and increase independent mobility. Braces and splints use external force to correct muscle abnormalities and improve functions such as sitting or walking. Other orthotics help stretch muscles or the position of a joint. Braces, wedges, special chairs, and other devices can help people sit more comfortably and make it easier to perform daily functions. Wheelchairs, rolling walkers, and powered scooters can help individuals who are not independently mobile. Vision aids include glasses, magnifiers, large-print books, and computer typefaces. Some individuals with CP may need surgery to correct vision problems. Hearing aids and telephone amplifiers may help people hear more clearly.

Complementary and Alternative Therapies

• Many children and adolescents with CP use some form of complementary or alternative medicine. Controlled clinical trials involving some of the therapies have been inconclusive or showed no benefit and the therapies have not been accepted in mainstream clinical practice.
• Although there are anecdotal reports of some benefits in some children with CP, these therapies have not been approved by the U.S. Food and Drug Administration for the treatment of CP.
• Such therapies include hyperbaric oxygen therapy, special clothing worn during resistance exercise training, certain forms of electrical stimulation, assisting children in completing certain motions several times a day, and specialized learning strategies. Also, dietary supplements, including herbal products, may interact with other products or medications a child with CP may be taking or have unwanted side effects on their own. Families of children with CP should discuss all therapies with their doctor.
• Stem cell therapy – is being investigated as a treatment for cerebral palsy, but research is in the early stages and large-scale clinical trials are needed to learn if stem cell therapy is safe and effective in humans. Stem cells are capable of becoming other cell types in the body. Scientists are hopeful that stem cells may be able to repair damaged nerves and brain tissues. Studies in the U.S. are examining the safety and tolerability of umbilical cord blood stem cell infusion in children with CP.

How surgery can help

Surgery is one of the many options available to help children with cerebral palsy improve mobility, and posture and ensure healthy growth. Most doctors recommend physical therapy and medication before surgery.

Surgery can correct or improve movement and alignment in the legs, ankles, feet, hips, wrists, and arms. These operations are performed on the muscles, tendons, bones, and nerves.

As is the goal with all treatment of cerebral palsy, surgery aims to give children the greatest chance of living as independently as possible. Movement problems caused by cerebral palsy can improve over time, but they also carry the risk of getting worse.

Surgery is used to:

• Relieve stiff muscles
• Correct spinal curvatures (scoliosis)
• Reduce tremors
• Correct foot deformities
• Improve posture
• Improve balance and coordination
• Relieve pain
• Correct fixed joints and tendons
• Prevent spinal deformities
• Free permanently tightened muscles (contractures)
• Prevent hip dislocation
• Treat co-occurring conditions

Cochlear implants

Many children with cerebral palsy have a hearing impairment. Those with severe hearing loss may benefit from cochlear implants—a surgically implanted hearing devices. Congenital hearing impairment (loss of hearing present at birth) can be caused by the same factors that cause cerebral palsy, such as oxygen deprivation during birth. As many as 15 percent of children with CP have a hearing impairment.

Gastrostomy

Children with low muscle tone often have issues with feeding. Their lack of muscle control can make it hard for them to swallow and chew. They may also have severe acid reflux or experience aspiration (food and drink entering the lungs) as a result of low muscle tone. Children can avoid these complications by having a feeding tube surgically implanted. For children with severe cerebral palsy, this may be the only way they can stay healthy. It also makes feeding time less demanding for parents. For children with less severe diagnoses, speech therapists can help with chewing and swallowing.

Hydrocephalus

This is a rare condition that can require surgical treatment. Hydrocephalus is a complication involving the buildup of cerebrospinal fluid in the skull, causing an abnormal increase in the size of the head. Untreated, this condition can be fatal. Treatment can include the implantation of valves to drain off excess fluid. Few children with cerebral palsy have hydrocephalus.

When is surgery appropriate?

Every surgery has its risks. Collaboration between parents and doctors can help families weigh the possible benefits with the risks of surgery. It takes careful consideration to determine when surgery is appropriate because every child is different.

Doctors have differing opinions about the best time in a child’s life to suggest surgery. Some doctors think surgery offers the greatest benefit when children are young; others think it’s best to wait until children are older, recommending physical therapy and medication in the meantime. Generally, children with cerebral palsy are treated between the ages of 3 and 10, but the exact age depends on the individual.

Risk factors

Several factors are associated with an increased risk of cerebral palsy.

• Maternal health – Certain infections or health problems during pregnancy can significantly increase the cerebral palsy risk to the baby. Infections of particular concern include:
• German measles (rubella) – Rubella is a viral infection that can cause serious birth defects. It can be prevented with a vaccine.
• Chickenpox (varicella) – Chickenpox is a contagious viral infection that causes itching and rashes, and it can cause pregnancy complications. It too can be prevented with a vaccine.
• Cytomegalovirus – Cytomegalovirus is a common virus that causes flu-like symptoms and may lead to birth defects if a mother experiences her first active infection during pregnancy.
• Herpes – Herpes infection can be passed from mother to child during pregnancy, affecting the womb and placenta. Inflammation triggered by infection may then damage the unborn baby’s developing nervous system.
• Toxoplasmosis – Toxoplasmosis is an infection caused by a parasite found in contaminated food, soil, and the feces of infected cats.
• Syphilis – Syphilis is a sexually transmitted bacterial infection.
• Exposure to toxins – Exposure to toxins, such as methyl mercury, can increase the risk of birth defects.
• Zika virus infection – Infants for whom maternal Zika infection causes microcephaly can develop cerebral palsy.
• Other conditions. Other conditions may increase the risk of cerebral palsy, such as thyroid problems, intellectual disabilities, or seizures.

Infant illness

Illnesses in a newborn baby that can greatly increase the risk of cerebral palsy include:

• Bacterial meningitis –This bacterial infection causes inflammation in the membranes surrounding the brain and spinal cord.
• Viral encephalitis – This viral infection similarly causes inflammation in the membranes surrounding the brain and spinal cord.
• Severe or untreated jaundice – Jaundice appears as a yellowing of the skin. The condition occurs when certain byproducts of “used” blood cells aren’t filtered from the bloodstream.

Other factors of pregnancy and birth

While the potential contribution from each is limited, additional pregnancy or birth factors associated with increased cerebral palsy risk include:

• Breech births – Babies with cerebral palsy are more likely to be in a feet-first position (breech presentation) at the beginning of labor rather than headfirst.
• Complicated labor and delivery – Babies who exhibit vascular or respiratory problems during labor and delivery may have existing brain damage or abnormalities.
• Low birth weight – Babies who weigh less than 5.5 pounds (2.5 kilograms) are at higher risk of developing cerebral palsy. This risk increases as birth weight drop.
• Multiple babies – Cerebral palsy risk increases with the number of babies sharing the uterus. If one or more of the babies die, the chance that the survivors may have cerebral palsy increases.
• Premature birth – A normal pregnancy lasts 40 weeks. Babies born fewer than 37 weeks into pregnancy are at higher risk of cerebral palsy. The earlier a baby is born, the greater the cerebral palsy risk.
• Rh blood type incompatibility between mother and child – If a mother’s Rh blood type doesn’t match her baby’s, her immune system may not tolerate the developing baby’s blood type and her body may begin to produce antibodies to attack and kill her baby’s blood cells, which can cause brain damage.

Complications

Muscle weakness, muscle spasticity, and coordination problems can contribute to several complications either during childhood or later during adulthood, including:

• Contracture – Contracture is muscle tissue shortening due to severe muscle tightening (spasticity). Contracture can inhibit bone growth, cause bones to bend, and result in joint deformities, dislocation, or partial dislocation.
• Malnutrition – Swallowing or feeding problems can make it difficult for someone who has cerebral palsy, particularly an infant, to get enough nutrition. This may cause impaired growth and weaker bones. Some children may need a feeding tube for adequate nutrition.
• Mental health conditions – People with cerebral palsy may have mental health (psychiatric) conditions, such as depression. Social isolation and the challenges of coping with disabilities can contribute to depression.
• Lung disease – People with cerebral palsy may develop lung disease and breathing disorders.
• Neurological conditions –  People with cerebral palsy may be more likely to develop movement disorders or worsened neurological symptoms over time.
• Osteoarthritis – Pressure on joints or abnormal alignment of joints from muscle spasticity may lead to the early onset of painful degenerative bone disease (osteoarthritis).
• Osteopenia – Fractures due to low bone density (osteopenia) can stem from several common factors such as lack of mobility, nutritional shortcomings, and antiepileptic drug use.
• Eye muscle imbalance – This can affect visual fixation and tracking; an eye specialist should evaluate suspected imbalances.
• Low birth weight and premature birth – Premature babies (born less than 37 weeks into pregnancy) and babies weighing less than 5 ½ pounds at birth have a much higher risk of developing cerebral palsy than full-term, heavier-weight babies.  Tiny babies born at very early gestational ages are especially at risk.
• Multiple births –  Twins, triplets, and other multiple births — even those born at term — are linked to an increased risk of cerebral palsy. The death of a baby’s twin or triplet further increases the risk.
• Infections during pregnancy –  Infections such as toxoplasmosis, rubella (German measles), cytomegalovirus, and herpes, can infect the womb and placenta. Inflammation triggered by infection may then go on to damage the developing nervous system in an unborn baby. Maternal fever during pregnancy or delivery can also set off this kind of inflammatory response.
• Blood type incompatibility between mother and child –   Rh incompatibility is a condition that develops when a mother’s Rh blood type (either positive or negative) is different from the blood type of her baby. The mother’s system doesn’t tolerate the baby’s different blood type and her body will begin to make antibodies that will attack and kill her baby’s blood cells, which can cause brain damage.
• Exposure to toxic substances –  Mothers who have been exposed to toxic substances during pregnancy, such as methyl mercury, are at a heightened risk of having a baby with cerebral palsy.
• Mothers with thyroid abnormalities, intellectual disability, excess protein in the urine, or seizures –  Mothers with any of these conditions are slightly more likely to have a child with CP. There are also medical conditions during labor and delivery, and immediately after delivery that act as warning signs for an increased risk of CP. However, most of these children will not develop CP. Warning signs include:
• Breech presentation –  Babies with cerebral palsy are more likely to be in a breech position (feet first) instead of head first at the beginning of labor. Babies who are unusually floppy as fetuses are more likely to be born in the breech position.
• Complicated labor and delivery –  A baby who has vascular or respiratory problems during labor and delivery may already have suffered brain damage or abnormalities.
• Small for gestational age –  Babies born smaller than normal for their gestational age are at risk for cerebral palsy because of factors that kept them from growing naturally in the womb.
• Low Apgar score –  The Apgar score is a numbered rating that reflects a newborn’s physical health. Doctors periodically score a baby’s heart rate, breathing, muscle tone, reflexes, and skin color during the first minutes after birth. A low score 10-20 minutes after delivery is often considered an important sign of potential problems such as CP.
• Jaundice – More than 50 percent of newborns develop jaundice (yellowing of the skin or whites of the eyes) after birth when bilirubin, a substance normally found in bile, builds up faster than their livers can break it down and pass it from the body. Severe, untreated jaundice can kill brain cells and can cause deafness and CP.

Are there treatments for other conditions associated with cerebral palsy?

Epilepsy

• Many children with intellectual disabilities and CP also have epilepsy. In general, drugs are prescribed based on the type of seizures an individual experiences, since no one drug controls all types. Some individuals may need a combination of two or more drugs to achieve good seizure control.

Incontinence

• Medical treatments for incontinence include special exercises, biofeedback, prescription drugs, surgery, or surgically implanted devices to replace or aid muscles.

Osteopenia

• Children with CP who are unable to walk risk developing poor bone density (osteopenia), which makes them more likely to break bones. In a study of older Americans funded by the National Institutes of Health (NIH), a family of drugs called bisphosphonates, which has been approved by the FDA to treat mineral loss in elderly patients, also appeared to increase bone mineral density Doctors may choose to selectively prescribe the drug off-label to children to prevent osteopenia.

Pain

• Pain can be a problem for people with CP due to spastic muscles and the stress and strain on parts of the body that are compensating for muscle abnormalities. Some individuals may also have frequent and irregular muscle spasms that can’t be predicted or medicated in advance.
• Diazepam can reduce the pain associated with muscle spasms and gabapentin has been used successfully to decrease the severity and frequency of painful spasms. Botulinum toxin injections have also been shown to decrease spasticity and pain.
• Intrathecal baclofen has shown good results in reducing pain. Some children and adults have been able to decrease pain by using noninvasive and drug-free interventions such as distraction, relaxation training, biofeedback, and therapeutic massage.

Premature aging

• The majority of individuals with CP will experience some form of premature aging by the time they reach their 40s because of the extra stress and strain the disease puts upon their bodies.
• The developmental delays that often accompany CP keep some organ systems from developing to their full capacity and level of performance. As a consequence, organ systems such as the cardiovascular system (the heart, veins, and arteries) and pulmonary system (lungs) have to work harder and they age prematurely.

Functional issues at work

• The day-to-day challenges of the workplace are likely to increase as an employed individual with CP reaches middle age.  Some individuals will be able to continue working with accommodations such as an adjusted work schedule, assistive equipment, or frequent rest periods.

Depression

• Mental health issues can also be of concern as someone with cerebral palsy grows older.  The rate of depression is three to four times higher in people with disabilities such as cerebral palsy.
• It appears to be related not so much to the severity of their disabilities, but to how well they cope with them.  The amount of emotional support someone has, how successful they are at coping with disappointment and stress, and whether or not they have an optimistic outlook about the future all have a significant impact on mental health.

Post-impairment syndrome

• This syndrome is marked by a combination of pain, fatigue, and weakness due to muscle abnormalities, bone deformities, overuse syndromes (sometimes also called repetitive motion injuries), and arthritis.   Fatigue is often a challenge, since individuals with CP may use up to three to five times the amount of energy that able-bodied people use when they walk and move about.

Osteoarthritis and degenerative arthritis

• Musculoskeletal abnormalities that may not produce discomfort during childhood can cause pain in adulthood.  For example, the abnormal relationships between joint surfaces and excessive joint compression can lead to the early development of painful osteoarthritis and degenerative arthritis.
• Individuals with CP also may have limited strength and restricted patterns of movement, which puts them at risk for overuse syndromes and nerve entrapments.

Other medical conditions

• Adults have higher than normal rates of other medical conditions secondary to their cerebral palsy, such as hypertension, incontinence, bladder dysfunction, and swallowing difficulties.
• Scoliosis is likely to progress after puberty when bones have matured into their final shape and size. People with CP also have a higher incidence of bone fractures, occurring most frequently during physical therapy sessions.
• Genetic defects are sometimes responsible for brain malformations and abnormalities that cause cerebral palsy.  Scientists are searching for the genes responsible for these abnormalities by collecting DNA samples from people with cerebral palsy and their families and using genetic screening techniques to discover linkages between individual genes and specific types of abnormality – primarily those associated with the process in the developing brain in which neurons migrate from where they are born to where they settle into neural circuits (called neural migration).
• Scientists are scrutinizing events in newborn babies’ brains, such as bleeding, epileptic seizures, and breathing and circulation problems, which can cause the abnormal release of chemicals that triggers the kind of damage that causes cerebral palsy.  For example, research has shown that bleeding in the brain unleashes dangerously high amounts of glutamate, a chemical that helps neurons communicate.  However, too much glutamate overexcites and kills neurons.  By learning how brain chemicals that are normally helpful become dangerously toxic, scientists will have opportunities to develop new drugs to block their harmful effects.
• Researchers are using imaging techniques and neurobehavioral tests to predict those preterm infants who will develop cerebral palsy. If these screening techniques are successful, doctors will be able to identify infants at risk for cerebral palsy before they are born.
• Periventricular white matter damage—the most common cause of CP—is characterized by the death of the white matter around the fluid-filled ventricles in the brain. The periventricular area contains nerve fibers that carry messages from the brain to the body’s muscles. NINDS-sponsored researchers are hoping to develop preventative strategies for white matter damage. For example, researchers are examining the role brain chemicals play in white matter development in the brain.

NIH-funded scientists continue to look at new therapies and novel ways to use existing options to treat individuals with CP, including:

• Constraint-induced therapy (CIT) –  is a promising therapy for CP. CIT typically involves restraining the stronger limb (such as the “good” arm in a person who has been affected by a stroke on one side of the body) in a cast and forcing the weaker arm to perform intensive activities every day for weeks.  A clinical study sponsored by the NICHD is examining the use of different dosage levels of daily training using either full-time cast immobilization vs. part-time splint restraint in improving upper body extremity skills in children with weakness on both sides of their body. Study findings will establish evidence-based practice standards to improve lifelong neuromotor capacity in individuals with CP.
• Functional electrical stimulation (FES)—the therapeutic use of low-level electrical current to stimulate muscle movement and restore useful movements such as standing or stepping—is an effective way to target and strengthen spastic muscles. Researchers are evaluating how FES-assisted stationary cycling can improve physical conditioning and general lower extremity muscle strength in adolescents.
• Robotic therapy – which applies controlled force to the leg during the swing phase of gait is may improve the efficacy of body weight-supported treadmill training in children with CP. The results from this NICHD study will lead to an innovative clinical therapy aimed at improving locomotor function in children with CP.
• Botulinum toxin (Botox) – injected locally, has become a standard treatment in children with spastic movement disorders such as CP. Recent animal studies suggest Botox degrades bone but there are no studies of its skeletal consequences in humans. Other research shows a low-intensity vibration treatment can improve bone structure in the lower extremity leg bones of children with CP. In a novel clinical study being conducted by NICHD, researchers are determining the effect of Botox treatment in conjunction with a daily vibration treatment on bone mass and bone structure in children with spastic CP.
• Systemic hypothermia—the controlled medical cooling of the body’s core temperature—appears to protect the brain and decrease the rate of death and disability from certain disorders and brain injuries. Previous studies have shown that hypothermia is effective in treating neurologic symptoms in term or late preterm babies less than the one-month-old that is attributed to hypoxic-ischemia (HIE, brain injury due to a severe decrease in the oxygen supply to the body), which can cause quadriplegic CP, with or without movement disorder. To determine the most effective cooling strategies, NICHD-funded researchers are studying different cooling treatments to improve the chance of survival and neurodevelopment outcomes 18-22 months post-treatment in infants with neurologic symptoms attributed to HIE. Other researchers are examining if combined therapy using hypothermia and recombinant erythropoietin (a hormone that promotes the growth of new red blood cells and increases oxygen levels in the blood) is more effective than either therapy alone in treating neurodevelopmental handicaps in an animal model involving a lack of oxygen before, during, or just after birth.

References

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