Cerebral palsy is a disorder that results from problems within the brain and the way the brain controls muscles. There are many different causes of cerebral palsy. Although ?CP,? as it is abbreviated is often related to problems before or during birth, it can also occur because of problems after birth.
CP is actually quite common and it is important to realize that symptoms can be very mild or very severe. Every child is different and each child requires individualized attention.
Symptoms of CP may include involuntary or spastic movements, decreased mobility, epilepsy, abnormal gait (i.e. walking pattern), mental retardation, impaired sensory sensation, including sight or hearing, and problems with speech. The damage to the brain is a one-time event and does not get worse, but the effects of CP change as the child matures. CP cannot be totally cured, but a child with this condition can benefit from certain types of therapy, including physical therapy, bracing, and other orthopaedic treatments. Many people with CP live absolutely normal lives.
- Cerebral palsy was first described around 1850 by an orthopaedic surgeon named Little.
- There are three major types of cerebral palsy:
- Spastic CP ? characterized by stiffness of the body parts and decreased mobility
- Athetoid CP ? marked by uncontrolled, involuntary movements
- Ataxic CP ? primary symptoms include unsteady gait, with decreased balance and depth perception
- While most patients are diagnosed with one of the three major types of CP, many children have features of more than one type.
- Athetoid and ataxic CP are relatively rare types of the condition. Spastic CP, the most common type, has several subtypes:
- Spastic hemiplegia ? affected children show decreased movement on one side of the body, usually worse in the hands and arm than the legs
- Spastic diplegia ? generally characterized by spasticity in both legs
- Spastic quadriplegia ? the most severe type of CP, with affected movements in all four limbs and a high association with seizures and mental retardation
- Cerebral palsy begins at birth or in childhood, but most patients live well into adulthood with the condition. There are many high-functioning adults in the U.S. and throughout the world with CP.
- 5 out of 2,000 babies in the U.S. are diagnosed with CP. Around 10,000 new cases occur each year, and between 500,000 and 700,000 people have CP in this country.
- Because the birth rate in the U.S. has decreased in recent years, the overall number of children born with CP has decreased slightly. However, the percentage of babies with CP has actually increased to a degree, primarily because of the higher number of newborns that survive prematurity or other perinatal events compared to previous decades, due to improved neonatal medical management.
- The condition is more common among premature infants.
- Siblings of children with CP are not at increased risk for being born with the condition.
There are a number of different causes of cerebral palsy, but in most children, the exact cause is not identified. Vascular insufficiency or asphyxia, in which the brain does not receive enough blood or oxygen for a period of time, is the most common known cause and can relate to obstetrical problems during and around the time of birth. CP may result from abnormal development of the brain, or the brain may undergo an injury during development. Such an injury may result from a traumatic event, such as a fall or a motor vehicle accident, or the brain may be subject to a toxic substance or chemical. Certain genetically determined metabolic disorders can lead to deficient or excessive amounts of normal biological molecules, which can produce CP through damage to brain tissue. Infections in the child or the mother can also infiltrate the fetal brain and lead to cerebral palsy. Other conditions that are not causes of, but are associated with, cerebral palsy include premature birth and low birth weight.
Cerebral palsy may be diagnosed at different ages in different children, depending on the type of CP an affected child has. Spastic hemiplegia usually manifests itself in infancy or young childhood, marked by decreased growth of the affected extremity, delayed walking (usually after 18 months of age) or manual function, deformity of the foot, and/or a seizure disorder arising during the first two years of life. Symptoms of spastic diplegia are often first noticed around the time an infant begins crawling because children will often drag their legs, while using their arms normally. Spastic quadriplegia is frequently diagnosed in the newborn period because of the array of significant symptoms that can become evident. Spasticity or decreased movement in the extremities may be the first sign. Neonates may also have problems with vision or swallowing, which can lead to aspiration pneumonia and warrant a diagnostic workup by a neurologist or head and neck specialist. Children with athetoid CP may be taken to a pediatrician because the tongue may be thrust forward, there may be problems feeding, excessive drooling, poor head control or generalized hypotonia (i.e. poor muscle tone and weakness). Diagnosis of ataxic CP generally results from clinical investigation of an unsteady gait, seen in young childhood after a child begins walking. Diagnostic workup for cerebral palsy requires a thorough physical examination by a pediatrician, and often involves a referral to a neurologist. Other neurological conditions need to be ruled out, such as muscular dystrophy, spinal cord disorder, or brain or spinal cord tumor. An orthopaedic surgeon should also be involved to perform a thorough physical exam of the extremities and to investigate muscular abnormalities, such as contractures , in which one or muscles become abnormally contracted and stiff, or skeletal problems. In general, a collaborative, team approach by physicians from several specialties should ensure an optimal diagnostic and therapeutic strategy for a child with cerebral palsy.
In general, children with CP can benefit from treatment from a variety of medical specialists, as well as educators, psychologists, physical therapists, occupational therapists, speech therapists, and social workers. Orthopaedic surgeons should also play a prominent role in the management of CP patients, depending on what types of musculoskeletal problems or deformities exist. The following is a summary of the common conditions and treatments that are frequently utilized by orthopaedic surgeons.
Most of these problems, especially if caught early, can be treated non-operatively with a brace, physical therapy, or even use of Botox (discussed below). In fact, we believe that physical therapy and bracing should be the mainstay of treatment in young children with CP. In some cases, however, children can benefit from surgical intervention. There are a number of types of surgery that are commonly performed, depending on the type of problem.
Foot Problems :
Foot problems are common in children with CP.
Equinus foot deformity ? Children with one of the various types of spastic CP may develop an equinus foot, in which the foot points downwards due to the spasticity or permanent tightening of the calf muscles. In other, more advanced cases, a surgical procedure known as a gastrocnemius and/or Achilles lengthening may be performed, in which the Achilles tendon and one of the calf muscles is cut and extended, so as to release the foot from its abnormal position and to leave it pointing straight forward. A plaster cast is applied to the lower leg and ankle, and allows weight-bearing for the 6 week period that the cast is worn.
Equinovarus foot deformity ? Most common in patients with spastic diplegia, this condition causes the foot to point downward and inward, so that the soles of the foot face each other. The most appropriate surgical treatment is usually lengthening of the posterior tibial muscle , a different calf muscle than described for simply equinus deformity, with perhaps a tibial tendon-transfer as well, which, together, should provide better control of the foot in a more normal position. A below-the-knee weight-bearing cast is worn for 6 weeks following surgery.
Valgus foot deformity ? This condition is the opposite of a varus deformity and is marked by the sole of the foot pointed laterally, to the side, causing the ankle to cave inwards slightly over time. For this deformity, either a subtalar arthrodesis or lateral opening wedge osteotomy is performed. The arthrodesis involves fixing a bone in the middle portion of the foot with a screw and a piece of bone, usually taken from the tibia , or shinbone. The osteotomy entails changing the position of a piece of bone in the heel to straighten the foot. Both procedures require casting to maintain the corrections, which create a flatter foot with decreased pain and more stable walking.
Leg Problems :
Crouched gait ? This condition, in which a child with CP appears crouched down somewhat, can be caused by spasticity of iliopsoas muscle in the hip, spasticity of the hamstring muscles (on the back of the leg), a deformity in the calcaneus (heel) bone, or a combination of the above. One common cause is hamstring tightness, which is treated with a surgical lengthening procedure, followed by casting or splinting.
Stiff-Knee Gait Pattern ? Difficulty walking can arise in this condition, in which spasticity of the rectus femoris muscle , one of the quadriceps muscles of the thigh, prevents full flexion of the knee. A tendon transfer operation, which essentially loosens the connection of the muscle, is a successful procedure for correction of gait, when followed by conscientious physical therapy to improve the flexibility and strength of the muscle.
Adduction contracture ? The muscles on the inside of the thigh that normally move the leg towards the other leg and the middle of the body can become abnormally tight and the leg relatively immobile. To treat this condition, an adductor tenotomy and tendon transfer may be performed, in which the tight muscles are released from the thighbone and may also be attached to a part of the pelvis, without the need for casting.
Flexion contracture ? When the upper leg is abnormally flexed upwards, toward the abdomen, in walking children and those in wheelchairs, the hip flexor muscles, such as the iliopsoas and rectus femorus muscles , can be lengthened or released operatively. In cases of severe contracture or persistent hip pain, an osteotomy of the femur or pelvis may be appropriate instead.
Hip Problems :
Hip Subluxation/Dislocation ? Many children with CP have problems with their hips, whether they can walk or are wheelchair-bound. The hip can become subluxated , which means that the ?ball’ does not sit properly in its ?socket,’ or dislocated , which means that the ball is completely out of the socket. Both conditions are associated with pain and instability. Many times, neither condition is present, but there is a high risk of the development of either. However, these can be prevented with early orthopaedic care. Several different conditions can cause these and other hip problems, and each is treated differently.
Femoral anteversion ? In this condition, spasticity of the muscles around the hip and thigh creates an imbalance of forces that gradually rotates the thighbone inward to an excessive degree. This creates a pigeon-toed appearance and an abnormal gait, and puts the hip at risk for subluxing/dislocating posteriorly, (i.e. backwards). A corrective osteotomy is a surgical treatment sometimes employed to realign and stabilize the hip.
Shallow/Oblique acetabulum ? Sometimes the portion of the pelvis that represents the socket of the hip joint, called the acetabulum , has structural variations that puts the hip at risk of subluxation or dislocation. Children with spastic quadriplegia are at a higher risk of developing instability with this condition and operative treatment may be necessary as a preventative measure, such as performing an osteotomy of the acetabulum with placement of a bone graft.
Spine Problems :
Spinal Kyphosis ? Deformities of the spine are frequent among children with cerebral palsy. Kyphosis develops in children because of weakness in the muscles surrounding the spine that normally extend the back upright, causing an affected child to hunch forward slightly in the middle or lower spine. This condition is especially not uncommon in children with spastic quadriplegia. Treatment usually consists of non-operative measures, such as a back brace or straps placed on a wheelchair that allow a child to sit upright more comfortably. Occasionally, contractures in the hamstrings can tilt the pelvis backwards, creating kyphosis in the lowest portion of the spine. Correction can be achieved operatively with a hamstring release procedure, which normalizes the pelvic tilt.
Spinal Lordosis ? This condition describes a spine with a backward tilt, causing the abdomen to protrude forward slightly, and arises when the hip flexor muscles develop contractures. Repetitive stretching or surgically lengthening these muscles allows for more stable sitting.
Scoliosis ? Scoliosis occurs when weakness on one side of the spine leads to a lateral C-shaped or S-shaped curve, and is quite common in children with cerebral palsy, particularly in more severe cases. Symptoms that can develop include pain, problems with sitting upright, and even difficulty breathing in severe instances. For mild curves (under 40 degrees), observation may be the only treatment necessary, which entails regular visits to an orthopaedic surgeon, who will monitor the progression of the curve. Use of an orthosis , or back brace, worn around the trunk, may be necessary for more severe curves, but usually only slows and does not stop curve progression in CP patients. It is useful to help maintain an upright posture, sometimes only to delay the period before which surgery is pursued. When curves are severe (over 40 degrees) and warrant operative intervention, orthopaedic surgeons may use any one of a number of effective procedures, but most commonly perform posterior spinal arthrodesis with instrumentation . In such an operation, multiple vertebrae, or bony segments of the spine, are fused together from the backside and the upright position of the spine is maintained by the placement of many small rods on either side, secured with screws. Sometimes, an anterior fusion is necessary, which is a slightly more complicated procedure because the spine is approached from the front side with an opening in the chest. Both procedures have minimal in-patient hospital days and excellent post-operative results, such as better overall stability, balance, and mobility, which facilitates improved feeding and general care.
Dorsal Rhizotomy ? Occasionally, this procedure is undertaken to reduce spasticity in certain areas of the body. It involves cutting some of the nerve fibers in the dorsal roots, which emerge from the spinal cord, out of the bony spinal column and run into the nerves that stimulate muscular movement. Because the nervous stimuli in patients with CP are abnormal, the procedure often enhances stability, but should be followed by diligent physical therapy.
Botulinum Toxin (Botox®) Therapy :
A final type of therapy that has been shown to be extremely effective in treating the muscular conditions that arise from CP is the use of botulinum toxin, or Botox®. Botox® is actually a neurotoxin produced in nature by a bacteria, known as Clostridium botulinum . Scientists have genetically constructed, purified, and diluted this chemical and it is now an extremely commonly used drug for a variety of purposes. Botox® injections have been proven to be overwhelmingly safe and hundreds of thousands of injections have been performed in the U.S. You have probably heard of Botox® used as a cosmetic treatment to decrease facial wrinkles. It does this by slightly weakening the facial muscles so that the normal frown, smile and grimace do not create wrinkle lines in the face.
Botox® is also very commonly used in patients with cerebral palsy in an effort to delay or even avoid surgery and as a way to improve walking and physical function in some children. By blocking the signal that muscles usually receive from nerves to contract, Botox® can temporarily weaken and paralyze muscles that are receiving inconsistent and unwanted signals from the brain with cerebral palsy. The effects of Botox® are reversible and will wear off over time. Intra-muscular injections are safe, easy, and well-tolerated, and can be repeated if necessary. We have also found that, sometimes, one or two injection of Botox® are enough to allow a child to get back into a brace or to achieve an increased benefit from physical therapy, leading to a long-lasting improvement.
A large number of CP patients are candidates for the positive effects of Botox®, which can be injected into virtually any spastic muscle. For non-ambulatory patients with severe spastic or mixed quadriplegia, injections into the paraspinal muscles have been shown to reduce pain, improve balance and control of sitting, allow for more comfortable resting, and enhance wheelchair positioning. Patients who are non-ambulatory because of lower extremity spasticity (diplegia and quadriplegia, with or without athetosis) can have their spasticity reduced and improve their positioning following Botox® injections as well. This can aid in the prevention of hip dislocation and other problems and may allow beneficial bracing, not otherwise possible. Children who are ambulatory but have foot deformities (hemiplegia or diplegia) can also have marked improvement in their gait and comfort level as a result of the injections.
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