What Specific Treatments Are Available?
Physical therapy, usually begun in the first few years of life or soon after the diagnosis is made, is a cornerstone of cerebral palsy treatment. Physical therapy programs use specific sets of exercises and activities to work toward two important goals: preventing weakening or deterioration in the muscles that aren't being used (disuse atrophy), and keeping muscles from becoming fixed in a rigid, abnormal position (contracture).
Resistive exercise programs (also called strength training) and other types of exercise are often used to increase muscle performance, especially in children and adolescents with mild cerebral palsy. Daily bouts of exercise keep muscles that aren't normally used moving and active and less prone to wasting away. Exercise also reduces the risk of contracture, one of the most common and serious complications of cerebral palsy.
Normally growing children stretch their muscles and tendons as they run, walk, and move through their daily activities. This insures that their muscles grow at the same rate as their bones. But in children with cerebral palsy, spasticity prevents muscles from stretching. As a result, their muscles don't grow fast enough to keep up with their lengthening bones. The muscle contracture that results can set back the gains in function they've made. Physical therapy alone or in combination with special braces (called orthotic devices) helps prevent contracture by stretching spastic muscles.
Occupational therapy. This kind of therapy focuses on optimizing upper body function, improving posture, and making the most of a child's mobility. An occupational therapist helps a child master the basic activities of daily living, such as eating, dressing, and using the bathroom alone. Fostering this kind of independence boosts self-reliance and self-esteem, and also helps reduce demands on parents and caregivers.
Recreational therapies. Recreational therapies, such as therapeutic horseback riding (also called hippotherapy), are sometimes used with mildly impaired children to improve gross motor skills. Parents of children who participate in recreational therapies usually notice an improvement in their child's speech, self-esteem, and emotional well-being.
Controversial physical therapies. "Patterning" is a physical therapy based on the principle that children with cerebral palsy should be taught motor skills in the same sequence in which they develop in normal children. In this controversial approach, the therapist begins by teaching a child elementary movements such as crawling -- regardless of age - before moving on to walking skills. Some experts and organizations, including the American Academy of Pediatrics, have expressed strong reservations about the patterning approach because studies have not documented its value.
Experts have similar reservations about the Bobath technique (which is also called "neurodevelopmental treatment"), named for a husband and wife team who pioneered the approach in England . In this form of physical therapy, instructors inhibit abnormal patterns of movement and encourage more normal movements.
The Bobath technique has had a widespread influence on the core physical therapies of cerebral palsy treatment, but there is no evidence that the technique improves motor control. The American Academy of Cerebral Palsy and Developmental Medicine reviewed studies that measured the impact of neurodevelopmental treatment and concluded that there was no strong evidence supporting its effectiveness for children with cerebral palsy.
Conductive education, developed in Hungary in the 1940s, is another physical therapy that at one time appeared to hold promise. Conductive education instructors attempt to improve a child's motor abilities by combining rhythmic activities, such as singing and clapping, with physical maneuvers on special equipment. The therapy, however, has not been able to produce consistent or significant improvements in study groups.
Speech and language therapy. About 20 percent of children with cerebral palsy are unable to produce intelligible speech. They also experience challenges in other areas of communication, such as hand gestures and facial expressions, and they have difficulty participating in the basic give and take of a normal conversation. These challenges will last throughout their lives.
Speech and language therapists (also known as speech therapists or speech-language pathologists) observe, diagnose, and treat the communication disorders associated with cerebral palsy. They use a program of exercises to teach children how to overcome specific communication difficulties.
For example, if a child has difficulty saying words that begin with "b," the therapist may suggest daily practice with a list of "b" words, increasing their difficulty as each list is mastered. Other kinds of exercises help children master the social skills involved in communicating by teaching them to keep their head up, maintain eye contact, and repeat themselves when they are misunderstood.
Speech therapists can also help children with severe disabilities learn how to use 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.
Speech interventions often use a child's family members and friends to reinforce the lessons learned in a therapeutic setting. This kind of indirect therapy encourages people who are in close daily contact with a child to create opportunities for him or her to use their new skills in conversation.
Treatments for problems with eating and drooling are often necessary when children with cerebral palsy 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. Some children develop gastroesophageal reflux disease (GERD, commonly called heartburn) in which a weak diaphragm can't keep stomach acids from spilling into the esophagus. The irritation of the acid can cause bleeding and pain.
Individuals with cerebral palsy are also at risk for malnutrition, recurrent lung infections, and progressive lung disease. The individuals most at risk for these problems are those with spastic quadriplegia.
Initially, children should be evaluated for their swallowing ability, which is usually done with a modified barium swallow study. Recommendations regarding diet modifications will be derived from the results of this study.
In severe cases where swallowing problems are causing malnutrition, a doctor may recommend tube feeding, in which a tube delivers food and nutrients down the throat and into the stomach, or gastrostomy, in which a surgical opening allows a tube to be placed directly into the stomach.
Although numerous treatments for drooling have been tested over the years, there is no one treatment that helps reliably. Anticholinergic drugs - such as glycopyrolate -- can reduce the flow of saliva but may cause unpleasant side effects, such as dry mouth, constipation, and urinary retention. Surgery, while sometimes effective, carries the risk of complications. Some children benefit from biofeedback techniques that help them recognize more quickly when their mouths fall open and they begin to drool. Intraoral devices (devices that fit into the mouth) that encourage better tongue positioning and swallowing are still being evaluated, but appear to reduce drooling for some children.
Drug Treatments
Oral medications such as diazepam, baclofen, dantrolene sodium, and tizanidine are usually used as the first line of treatment to relax stiff, contracted, or overactive muscles. These drugs are easy to use, except that dosages high enough to be effective often have side effects, among them drowsiness, upset stomach, high blood pressure, and possible liver damage with long-term use. Oral medications are most appropriate for children who need only mild reduction in muscle tone or who have widespread spasticity.
Doctors also sometimes use alcohol "washes" -- injections of alcohol into muscles -- to reduce spasticity. The benefits last from a few months to 2 years or more, but the adverse effects include a significant risk of pain or numbness, and the procedure requires a high degree of skill to target the nerve.
The availability of new and more precise methods to deliver antispasmodic medications is moving treatment for spasticity toward chemodenervation, in which injected drugs are used to target and relax muscles.
Botulinum toxin (BT-A), injected locally, has become a standard treatment for overactive muscles in children with spastic movement disorders such as cerebral palsy. BT-A relaxes contracted muscles by keeping nerve cells from over-activating muscle. Although BT-A is not approved by the Food and Drug Administration (FDA) for treating cerebral palsy, since the 1990s doctors have been using it off-label to relax spastic muscles. A number of studies have shown that it reduces spasticity and increases the range of motion of the muscles it targets.
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.
Because BT-A does not have FDA approval to treat spasticity in children, parents and caregivers should make sure that the doctor giving the injection is trained in the procedure and has experience using it in children.
Intrathecal baclofen therapy uses an implantable pump to deliver baclofen, a muscle relaxant, into the fluid surrounding the spinal cord. Baclofen works by decreasing the excitability of nerve cells in the spinal cord, which then reduces muscle spasticity throughout the body. Because it is delivered directly into the nervous system, the intrathecal dose of baclofen can be as low as one one-hundredth of the oral dose. Studies have shown it reduces spasticity and pain and improves sleep.
The pump is the size of a hockey puck and is implanted in the abdomen. It contains a refillable reservoir connected to an alarm that beeps when the reservoir is low. The pump is programmable with an electronic telemetry wand. The program can be adjusted if muscle tone is worse at certain times of the day or night.
The baclofen pump carries a small but significant risk of serious complications if it fails or is programmed incorrectly, if the catheter becomes twisted or kinked, or if the insertion site becomes infected. Undesirable, but infrequent, side effects include overrelaxation of the muscles, sleepiness, headache, nausea, vomiting, dizziness, and constipation.
As a muscle-relaxing therapy, the baclofen pump is most appropriate for individuals with chronic, severe stiffness or uncontrolled muscle movement throughout the body. Doctors have successfully implanted the pump in children as young as 3 years of age.
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 cerebral palsy, improving the appearance of how they walk - their gait - is also important. A more upright gait with smoother transitions and foot placements is the primary goal for many children and young adults.
In the operating room, surgeons can lengthen muscles and tendons that are proportionately too short. But first, they have to determine the specific muscles responsible for the gait abnormalities. Finding these muscles can be difficult. It takes more than 30 major muscles working at the right time using the right amount of force to walk two strides with a normal gait. A problem with any of those muscles can cause an abnormal gait.
In addition, because the body makes natural adjustments to compensate for muscle imbalances, these adjustments could appear to be the problem, instead of a compensation. In the past, doctors relied on clinical examination, observation of the gait, and the measurement of motion and spasticity to determine the muscles involved. Now, doctors have a diagnostic technique known as gait analysis.
Gait analysis uses cameras that record how an individual walks, force plates that detect when and where feet touch the ground, a special recording technique that detects muscle activity (known as electromyography), and a computer program that gathers and analyzes the data to identify the problem muscles. Using gait analysis, doctors can precisely locate which muscles would benefit from surgery and how much improvement in gait can be expected.
The timing of orthopedic surgery has also changed in recent years. Previously, orthopedic surgeons preferred to perform all of the necessary surgeries a child needed at the same time, usually between the ages of 7 and 10. Because of the length of time spent in recovery, which was generally several months, doing them all at once shortened the amount of time a child spent in bed. Now most of the surgical procedures can be done on an outpatient basis or with a short inpatient stay. Children usually return to their normal lifestyle within a week.
Consequently, doctors think it is much better to stagger surgeries and perform them at times appropriate to a child's age and level of motor development. For example, spasticity in the upper leg muscles (the adductors), which causes a "scissor pattern" walk, is a major obstacle to normal gait. The optimal age to correct this spasticity with adduction release surgery is 2 to 4 years of age. On the other hand, the best time to perform surgery to lengthen the hamstrings or Achilles tendon is 7 to 8 years of age. If adduction release surgery is delayed so that it can be performed at the same time as hamstring lengthening, the child will have learned to compensate for spasticity in the adductors. By the time the hamstring surgery is performed, the child's abnormal gait pattern could be so ingrained that it might not be easily corrected.
With shorter recovery times and new, less invasive surgical techniques, doctors can schedule surgeries at times that take advantage of a child's age and developmental abilities for the best possible result.
Selective dorsal rhizotomy (SDR) is a surgical procedure recommended only 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. In the procedure, a surgeon locates and selectively severs overactivated nerves at the base of the spinal column.
Because it reduces the amount of stimulation that reaches muscles via the nerves, 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.
Even though the use of microsurgery techniques has refined the practice of SDR surgery, there is still controversy about how selective SDR actually is. Some doctors have concerns since it is invasive and irreversible and may only achieve small improvements in function. Although recent research has shown that combining SDR with physical therapy reduces spasticity in some children, particularly those with spastic diplegia, whether or not it improves gait or function has still not been proven. Ongoing research continues to look at this surgery's effectiveness.
Spinal cord stimulation was developed in the 1980s to treat spinal cord injury and other neurological conditions involving motor neurons. An implanted electrode selectively stimulates nerves at the base of the spinal cord to inhibit and decrease nerve activity. The effectiveness of spinal cord stimulation for the treatment of cerebral palsy has yet to be proven in clinical studies. It is considered a treatment alternative only when other conservative or surgical treatments have been unsuccessful at relaxing muscles or relieving pain.
Orthotic Devices
Orthotic devices - such as braces and splints - use external force to correct muscle abnormalities. The technology of orthotics has advanced over the past 30 years from metal rods that hooked up to bulky orthopedic shoes, to appliances that are individually molded from high-temperature plastics for a precise fit. Ankle-foot orthoses are frequently prescribed for children with spastic diplegia to prevent muscle contracture and to improve gait. Splints are also used to correct spasticity in the hand muscles.
Assistive Technology
Devices that help individuals move about more easily and communicate successfully at home, at school, or in the workplace can help a child or adult with cerebral palsy overcome physical and communication limitations. There are a number of devices that help individuals stand straight and walk, such as postural support or seating systems, open-front walkers, quadrapedal canes (lightweight metal canes with four feet), and gait poles. Electric wheelchairs let more severely impaired adults and children move about successfully.
The computer is probably the most dramatic example of a communication device that can make a big difference in the lives of people with cerebral palsy. Equipped with a computer and voice synthesizer, a child or adult with cerebral palsy can communicate successfully with others. For example, a child who is unable to speak or write but can make head movements may be able to control a computer using a special light pointer that attaches to a headband.
Alternative Therapies
Therapeutic (subthreshold) electrical stimulation, also called neuromuscular electrical stimulation (NES), pulses electricity into the motor nerves to stimulate contraction in selective muscle groups. Many studies have demonstrated that NES appears to increase range of motion and muscular strength.
Threshold electrical stimulation, which involves the application of electrical stimulation at an intensity too low to stimulate muscle contraction, is a controversial therapy. Studies have not been able to demonstrate its effectiveness or any significant improvement with its use.
Hyperbaric oxygen therapy. Some children have cerebral palsy as the result of brain damage from oxygen deprivation. Proponents of hyperbaric oxygen therapy propose that the brain tissue surrounding the damaged area can be "awakened" by forcing high concentrations of oxygen into the body under greater than atmospheric pressure.
A recent study compared a group of children who received no hyperbaric treatment to a group that received 40 treatments over 8 weeks. On every measure of function (gross motor, cognitive, communication, and memory) at the end of 2 months of treatment and after a further 3 months of followup, the two groups were identical in outcome. There was no added benefit from hyperbaric oxygen therapy.
Are There Treatments for Other Conditions Associated with Cerebral Palsy?
Epilepsy. Twenty to 40 percent of children with mental retardation and cerebral palsy also have epilepsy. Doctors usually prescribe medications to control seizures. The classic medications for this purpose are phenobarbital, phenytoin, carbamazepine, and valproate. Although these drugs generally are effective in controlling seizures, their use is hampered by harmful or unpleasant side effects.
Treatment for epilepsy has advanced significantly with the development of new medications that have fewer side effects. These drugs include felbamate, gabapentin, lamotrigine, levetiracetam, oxcarbazepine, tiagabine, iramate, vigabatrin, and zonisamide.
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. Specially designed absorbent undergarments can also be used to protect against accidental leaks.
Osteopenia. Children with cerebral palsy who aren't able 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 was recently 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 cerebral palsy 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.
Doctors often prescribe diazepam to reduce the pain associated with muscle spasms, but it's not known exactly how the drug works to interfere with pain signals. The drug gabapentin has been used successfully to decrease the severity and frequency of painful spasms. BT-A injections have also been shown to decrease spasticity and pain, and are commonly given under anesthesia to avoid the pain associated with the injections. Intrathecal baclofen has shown good results in reducing pain, but its delivery is invasive, time intensive, and expensive.
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.
