HISTORICAL BACKGROUND
There are references in history and literature which crippled from Egypt through the darkness of middle ages, in 1843, the crippling condition being now known as Cerebral Palsy which was first described by the English surgeon, William J Little who believed that deformities in these cases were due to the nervous affections or paralysis. It was also observed that diarrhea and other internal upsets were also responsible for it—even a large proportions were in first pregnancy, and that many patients were immature/premature weighing only 40 ounces at birth. He also noticed a high degree of partial subnormality, fits and uncontrollable behavior for which he suggested massage, baths, gymnastics and manipulation as therapeutic management. The birth palsies of cerebral origin were commonly referred to as Little's disease. His description of difficult labor, premature birth, abnormal parturition and asphyxia neonatorum, on mental and physical condition of the child was in many ways in agreement with modern findings and concepts. He later defined it in the neurological classification of cerebral palsy. Dr Phelps first grasped that CP can be with normal mental status but with motor handicap.
The term cerebral palsy was very carefully designed by the Dr Phelps to dissociate feeble mindedness from motor handicap. Today, universally accepted concept is of its cerebral origin with nonprogressive origin accompanied by feeble mindedness. He also demonstrated that such children could be rehabilitated to lead useful happy life.
OCCUPATIONAL THERAPY IN THE TREATMENT OF CEREBRAL PALSY
Bower published a comprehensive review that traced the history of rehabilitation and cerebral palsy from the early 1900s to the present. She noted that Phelps was one of the first clinicians to treat patients with cerebral palsy in the 1930s, followed by Peto and Bobath, who 2became interested in cerebral palsy a few years later. The major developments in use of physical therapy in the treatment of cerebral palsy, however, did not occur until the 1950s. This was largely due to the decline in the incidence of poliomyelitis after the Salk vaccine was discovered, and the shifting of research dollars and therapeutic techniques to persons with cerebral palsy.
Bower's description of the major therapeutic approaches to the treatment of cerebral palsy over the past 50 years is briefly summarized below.
Phelps, an orthopedic surgeon, recommended 5 years of institutionalized therapy from a team approach (physical, occupational, speech and other therapies). He described five different categories of cerebral palsy. His work included the use of motion pictures to describe children and evaluate the effectiveness of treatment. He also endorsed tenotomies (surgery on individual muscles). Paine researched the work of Phelps in 1962 and reported that children with mild spastic hemiplegia improved with and without treatment, and that Phelps' treatment strategies were not effective for children with athetosis. The treatment proposed by Phelps did not change whether or not a child would need surgery. The work of Rood was not based on afferent sensory stimulation, but no empirical research has ever been carried out on the efficacy of this therapy.
Vojta, a German neurologist, combined the techniques of Fay, Rood and Kabat. In 1981, research was carried out on one subject with cerebral palsy whose subluxed hip was reduced after 3 years of treatment. In 1984, Kanda looked at Vojta's work with 29 subjects with cerebral palsy. Eight were children with spastic diplegia who had gotten treatment early in their lives, and 21 children with spastic diplegia were treated several months later. All walked by the age 3. The children who were treated earlier seemed to have an improved gait pattern compared to the children treated later. Vojta also reported on his own work but there are disputes concerning how many of the 207 infants he wrote about (who supposedly became “normal” in mental and motor performance) were children with cerebral palsy.
In 1991, Mayo studied one of the most widespread therapeutic techniques used to treat children with cerebral palsy, the Bobath or neurodevelopmental approach (NDT). Started in the 1940's by Karel and Berta Bobath, this treatment was based on the view that cerebral palsy results from interference with development of normal posture against gravity. The brain lesion leads to the loss of inhibition of abnormal primitive reflexes. Mayo examined the effectiveness of NDT and did not find this technique to be any better than other forms of physical therapy. Wright and Nicholson researched Bobath's work 3and concluded that there were no differences between children treated with the Bobath method compared to children who received no treatment. The one exception was the ability of children with quadriplegia to roll.
In summarising her findings, Bower noted that although each of the above methods have been used for decades in treating children with cerebral palsy, there is very little scientific evidence to prove that any one therapy is more or less effective in improving the long-term function of children with cerebral palsy, and that often therapy continues when it may no longer be needed. She noted that “research needs to be undertaken by clinical scientists to assess the relative merits of the various approaches of therapy. Research methodologies used in psychology and the social sciences may well prove to be more useful for this purpose than those used in tradititional medical research.”
In 1988, a classic study by Palmer and coworkers entitled, “Effects of Physical Therapy on Cerebral Palsy, “and published in the highly respected New England Journal of Medicine, investigated two early intervention programs in 48 infants with mild to severe spastic diplegia (12 to 19 months of age). One intervention involved neurodevelopmental therapy and the other intervention was a published infant stimulation program called learning games. The investigators found that there was no motor, cognitive, or social advantage for infants receiving physical therapy after six and 12 months of treatment, and that trends favored the infant stimulation program. More frequent contact between therapist and patient may have been necessary to make physical therapy more beneficial to infants with cerebral palsy, but if so, this would require higher costs. The investigators concluded with the following statement:
“This clinical trial offers no support for the idea that neurodevelopmental physical therapy is a preferred intervention in infants with mild to severe spastic diplegia. Although it is possible that there are longer-term benefits of physical therapy or benefits in domains not reported in this study, the goal of improved motor development was not achieved in infants receiving physical therapy as compared with infants receiving infant stimulation. Furthermore, physical therapy applied earlier offered no advantage over physical therapy applied six months later. The findings underscore a fundamental issue in developmental pediatrics and public policy affecting developmentally disabled children: the immediate and long-term effectiveness of traditional interventions must be examined critically. Alternatively, less costly outcomes may improve function.”4
In 1995, Graves reviewed the literature on therapeutic methods for cerebral palsy and came to a similar conclusion:
“In reviewing these methods it becomes clear that while all have their zealous proponents, the methodology of evaluation studies is often flawed and the results are inconclusive. Again the call is often made for more and better studies, but the reality is that these methods have had several decades to provide proof of efficacy and none has been forthcoming. Significantly, there has been little or no attention to the negative effects of therapy.”
Graves went on to say that although “…‥therapy does not lead to dramatic improvements in the neurological status of children with cerebral palsy, therapists have an important role in helping families understand how to work with their children in a physical and social setting.”
In an extensive review of therapeutic techniques for persons with cerebral palsy, Bleck wrote: “After half a century of sincere and intense effort by professionals to ‘treat’ cerebral palsy, most now acknowledge that these remedial efforts have been unsuccessful in achieving function. Perhaps it is time to give up trying to ‘cure’ the neurological deficits by remedial methods, stop looking for positive studies, and get on with the task of helping children and their families.”
Despite periodic calls for scientific inquiry into the effect of physical therapy in the treatment of children with cerebral palsy, there are only a handful of clinical trials in the literature that have examined the efficacy of physical therapy. As a result of small, heterogeneous samples substantial attrition among subjects, and nonrandom assignment of treatment, it is difficult to interpret what are the most effective treatment strategies for improving the motor performance in persons with cerebral palsy. Fetters and Kluzik noted that “this lack of scientific evidence of treatment effectiveness is true for many types of physical therapy for children with cerebral palsy.
This cursory review of the literature on cerebral palsy and therapeutic techniques provides a strong incentive for the Task Force develop new intervention strategies that will prove useful in the overall physical, psychological and social development of persons with cerebral palsy. Clearly, we must establish a more ecological approach to treatment and must develop safe and effective exercise guidelines for persons with cerebral palsy that can be implemented in community-based fitness settings.5
HISTORY OF CEREBRAL PALSY
In the 1860s an English surgeon named William Little wrote the first medical descriptions of a puzzling disorder that struck children in the first years of life, causing stiff, spastic muscles in their legs and, to a lesser degree, their arms. These children had difficulty grasping objects, crawling, and walking. They did not get better as they grew up nor did they become worse. Their condition, which was called Little's disease for many years, is now known as spastic displegia. It is just one of several disorders that affect control of movement and are grouped together under the term cerebral palsy.
Because it seemed that many of these children were born following complicated deliveries, Little suggested their condition resulted from a lack of oxygen during birth. This oxygen shortage damaged sensitive brain tissues controlling movement. But in 1897, the famous psychiatrist Sigmund Freud disagreed. Noting that children with cerebral palsy often had other problems such as mental retardation, visual disturbances, and seizures, Freud suggested that the disorder might sometimes have roots earlier in life, during the brain's development in the womb. “Difficult birth, in certain cases,” he wrote, is merely a symptom of deeper effects that influence the development of the fetus.
Despite Freud's observation, the belief that birth complications cause most cases of cerebral palsy was widespread among physicians, families, and even medical researchers until very recently. In the 1980s, however, scientists analyzed extensive data from a government study of more than 35,000 births. While they found evidence that birth trauma was the cause of thousands of cerebral palsy cases, no cause could be found in the majority of cases. These findings profoundly altered medical theories about cerebral palsy and have spurred today's researchers to explore alternative causes.
At the same time, biomedical research has also led to significant changes in understanding, diagnosing, and treating persons with cerebral palsy. Identification of infants with cerebral palsy very early in life gives youngsters the best opportunity for developing to their full capacity. Biomedical research has led to improved diagnostic techniques-such as advanced brain imaging and modern gait analysis-that are making this easier. Certain conditions known to cause cerebral palsy, such as rubella (German measles) and jaundice, can now be prevented or treated. Physical, psychological, and behavioral therapy that assist with such skills as movement and speech, and foster social and emotional development can help children who have cerebral palsy to achieve and succeed. Medications, surgery, and braces can often 6improve nerve and muscle coordination, help treat associated medical problems, and either prevent or correct deformities.
Contact doctor if you have questions about your child's cerebral palsy, or if you would like to learn more about protecting your child's right to a lifetime of benefits.
DEFINITION OF CEREBRAL PALSY
Cerebral palsy is a broad term used to describe a group of chronic movement or posture disorders. “Cerebral” refers to the brain, while “palsy” refers to a physical disorder, such as a lack of muscle control. Cerebral palsy is not caused by problems with the muscles or nerves, but rather with the brain's ability to adequately control the body. Cerebral palsy can be caused by injury during birth, although sometimes it is the result of later damage to the brain. Symptoms usually appear in the first few years of life and once they appear, they generally do not worsen over time.
It comprises of those motor and other symptom complexes caused by a non-progressive brain lesion/lesions. The various types may be defined as under:
A. Spasticity: It is caused by lower threshold of the stretch reflex, an enlarged reflexogenic area, augmented responses with clonus and abnormal synchronization of discharge rate in various parts of the spastic muscle. The pathologic student reflex must be present to make degree of spasticity. There is a tendency to contracture due to involvement of antigravity muscles.
B. Athetoid: It may also be known as athetosis. It exhibits abnormal amount of involuntary type of movements, reflexes normal, E.M.G. findings are normal uncontrolled and inco-ordinate motions with varying degree of tensions.
C. Rigidity: It is reason of agonist and antagonist disturbances with resistance to slow passive motion of both agonist and antagonist muscles. If resistance to passive motion is continuous, then it is known as “lead-pipe” rigidity and if discontinuous, then “cog-wheel” rigidity. The more resistance is to slow than rapid motion where in spasticity, resistance is to rapid motion.
Antagonists to antigravity muscles are most involved in rigidity. Total motion may be decreased. Main features is hypertonicity, normal or diminished reflexes, no clonus, no stretch reflexes and involuntary motion.7
D. Ataxia: Due to disturbances of the kinesthetic or/and balance areas leading to incordination. Characteristic features are disturbances of balance, equilibrium, dyssynergias, rebound phenomenon with a sterognosia. It involves the depth perception. It may show atonia and hypotonia.
E. Tremor: It may be intentional or nonintentional or constant, uncontrollable, involuntary motions of a rhythmic, alternating or pendular pattern due to alternate agonist and antagonist contractions.
F. Atonia: It is lack of tone and failure of muscle to respond to volitional stimulation. The motor system lacks firmness or turgor of normal relaxed muscles. Weak or increased deep reflexes but no involuntary motion present distinguishing from non-tension athetosis, which is rare but may be initial symptom in any cerebral palsy case. Special mention made because may be striking initial symptom in cerebral palsy which makes an outstanding symptom of a type of cerebral palsy.
G. Mixed: It may not need mention as the predominating symptoms describe class and type of cerebral palsy.
Cerebral Palsy is defined in number of ways by independent workers. It is a general term used to describe any paralysis, weakness and incoordination as functional deviation of the motor system resulting from an intracranial lesions. Brain being center not only of muscle control but also of intelligence, behavior, personality and many other functions. Any brain dysfunction resulting due to injury may lead to paralysis and/or convulsions, mental subnormality, personality disorder or sensory defect like visual, auditory, tactile, etc. depending on location and extent of involvement of general symptoms may be obvious clinically than mental and emotional and other associated handicap.
Cerebral palsy basically is a physical disability arising out of intracranial lesions leading to paralytic disorders, weakness, inco-ordination or functional deviations of the motor system. Cerebral palsy is nonprogressive.
It was also defined as “nonprogressive central nervous system lesion/lesions is present or motor dysfunction results due to this lesion' or ‘sensory, motor, emotional or psychological disturbances can co-exist. It was also known earlier as “cerebral spastic paralysis” with combination of both sensory and motor involvement. While treating such children difficulties were faced only due to developmental handicap.8
According to Perstein, “Cerebral palsy is generally defined as a condition characterized' by paralysis, weakness, inco-ordination or any other aberration of motor function due to pathology of the motor control centers of the brain.”
Denhoff defined, “A condition in which interference with the control of the motor system arises as a result of lesions occurring from birth trauma.”
Cerebral palsy is a neuromotor component of the “brain damage syndrome.” It may accompany any other defects of various kinds as above. It is a nonprogressive clinical entity with a multitude of handicaps.
What is Cerebral Palsy?
Cerebral palsy is a long-term disorder causing impaired control of movement. The symptoms develop over the first few years of life, and generally do not worsen thereafter. The disorder is caused by damage to the motor control areas of the brain which disrupts the brain's ability to control movement and posture. The term cerebral refers to the brain and the term palsy refers to impaired control of body movement.
There is a spectrum of symptoms for cerebral palsy. Someone with cerebral palsy may have difficulty with fine motor tasks, such as writing; or experience trouble with main maintaining balance and walking or be affected by involuntary movements such as uncontrollable writhing motion of the hands or drooling. The symptom of life from medical disorder including epilepsy change over time. Unformtunately people with cerebral palsy are often affected by medical disorderd or mental impairment. Contrary to common belief, however, cerebral palsy does not always cause profound handicap.
Cerebral palsy is not contagious nor is it usually inherited. At this time, cerebral palsy cannot be cured, although scientific research continues to yield improved treatments.
Cerebral palsy is the commonly used name for a group of conditions characterized by motor dysfunction due to nonprogressing brain damage in early life, i.e. before brain matures. This is disorder of movement and posture.
Cerebral palsy takes many forms; in fact, no two spastic people are precisely alike. Some are so lightly affected that they have no obvious disability. Others may be much more seriously disabled. They may be clumsy in their walk, or they have difficulty with their hands or speech. Some are even unable to sit and can do little for themselves.9
Sometimes, the damage involves some parts of the brain as well, leading to deafness and other difficulties of perception. Many spastic children also have special learning difficulties which make them slow to learn. Spastic people have higher than average intelligence, though they are backward because of their handicaps. They can find it hard to control their facial expression and many seem to be mentally disabled but some of those who seem to be severely disturbed are, in fact, the least affected in their intellect.
Cause of injury can be prenatal, natal or postnatal. The birth of a spastic child is the fault or either parent if Rh –ve factor is not considered before planning a child.
Inherited defects do not play a large part in cerebral palsy. The problem is of brain damage. There is no direct injury to the limbs. There are two main classifications of cerebral palsy.
- Neurological
- Orthopedic.
Damage to basal ganglia causes athetosis. The main features is frequent involuntary movement which mask and interfere with the normal movement of the whole body.
Damage to cerebellum interferes with the coordination of movement, posture and balancing. The spastic child with ataxia caused by injury to the cerebellum has an unsteady gain and difficulty in balancing.
The source of problem is not in the affected limbs but in the cells of the brain. Correct treatment given early enough in life can often bring great benefit though not an absolute cure. No way has been found to repair the damage done to the brain cells, themselves. The damage does not spread but without skilled treatment the effect of that damage does become more serious. Thus, early recognition that something is wrong needs to be followed by speedy and skillful assessment of the child's needs by specialists, followed by whatever treatment is needed. Spastic boys and girls deserve the best standard of care and love that modern knowledge can give, and in recent years, there has been wonderful progress in providing the special services which can bring about the greatest possible benefit to spastics and help them to make the very most of what they have.
Although the majority of spastics should be capable of earning their living in the right occupation with the necessary training and guidance, a large proportion do not find work. Hundreds of them are unemployed because there are not enough special work facilities available and too many older spastics who are today in institutions or mental hospitals are only because they lacked the proper early treatment and training that could have helped them to useful living 10spastic people. They do not want to be a life long burden either to their families or to the community but they need expert help at right time.
To establish a department general survey should be made according to which one can know a workable plan. A general survey is conducted to:
- Assess the magnitude of problem,
- To study accessiblities of services,
- To identify problems encountered by people in obtaining services.
The survey can be based on the interview, security or extension type. The survey can be simple, and in the form of a national survey.
PATHOGENESIS
Cerebral palsy forms the major cause of crippling in children, perhaps next to poliomyelitis. This is increasing day by day with increasing magnitude of the problems. This may pose a great challenge to the rehabilitation experts in the rehabilitation of these cases.
ETIOLOGY
There are various factors responsible for the causes of cerebral palsy. Some have indicated that multiple factors are involved in causation of the cerebral palsy. Many studies show that neonatal, prenatal and natal abnormalities may cause it alongwith the abnormal symptoms in the newborn period. Neonatal abnormalities may cause cerebral insult.
Causes of Cerebral Palsy
During Birth
Cerebral palsy is not one disease with a single origin, like chicken-pox or measles. It is a group of disorders that are related but probably stem from a number of different causes. When physicians diagnose cerebral palsy in an individual child, they look at risk factors, the symptoms, the mother's and child's medical history, and the onset of the disorder.
About 10 or 20 person of children who have cerebral palsy acquire the disorder after birth, while many more cases are caused by an incident that occurs during pregnancy or birth. Acquired cerebral palsy is the result of brain damage in the first few months or years of life. Common causes of brain damage are sickness such as bacterial meningitis, viral encephalitis, or even jaundice. Another common cause is head injury such as from a motor vehicle accident, a fall, or 11child abuse. When a baby is “born” with cerebral palsy, the causes may be due to avoidable or unavoidable incidents that occurred during pregnancy or birth.
During Pregnancy
Cerebral palsy can also be caused by things that happen during pregnancy which cause permanent damage to the fetus.
1. Prenatal Infections
Certain bacterial and viral infections can damage the fetus during pregnancy. More commonly, untreated or maltreated maternal infections can induce premature labor and delivery. These newborn babies may suffer the consequences of brain damage from infection directly, or they may be damaged as a direct result of prematurity. Sometimes the mother can be unaware of the source of infection if it goes undetected by her health care provider. Maternal infections such as German measles (or rubella), cytomegalovirus and toxoplasmosis, can cross the placenta and infect the fetus as well, causing damage to the developing nervous system.
2. Improper Dating of the Pregnancy
One of the most important influences in prenatal care is the accurate dating of a pregnancy. Ultrasounds have made the estimation of due dates far more reliable. Failure to properly date the pregnancy may result in either premature delivery of post-mature delivery, both of which may be responsible for brain damage or cerebral palsy to newborn baby.
3. Rh IncompAtibility
In this blood condition, the mother's body produces immune cells called antibodies that destroy the fetus's blood cells, leading to a form of jaundice in the newborn. Severe, untreated jaundice can damage brain cells.
4. Bleeding in the Brain
Bleeding in the brain has several causes, including broken blood vessels in the brain, clogged blood vessels, or abnormal blood cells. Although bleeding in the brain(or stroke) is better known for its effects on older adults, it can also occur in the fetus during pregnancy or the newborn around the time of birth, damaging brain tissue and 12causing neurological problems or cerebral palsy. Ongoing research is testing potential treatments that may one day help prevent stroke in fetuses and newborns.
Birth Injury
Sometimes cerebral palsy is the result of a birth injury that causes the infant to be traumatized or deprived of oxygen (asphyxia). A number of things can cause cerebral palsy trauma of asphyxia, including:
- Difficult forceps delivery,
- Difficult vacuum extraction delivery,
- Exceptionally long labor,
- Low amniotic fluid,
- A twisted or compressed umbilical cord,
- Macrosomia or a baby that is “large for gestational age”,
- Placental abruption and
- Hemorrhage.
Most often when a baby is damaged, she is damaged late in labor from a continuous lack of oxygen and/or trauma. While a newborn/ infant's blood is specially equipped to compensate for low levels of oxygen, and asphyxia (lack of oxygen caused by interruption in breathing or poor oxygen supply) is common in babies during the stresses of labor and delivery, this ability is not infinite. If asphyxia severely lowers the supply of oxygen to the infant's brain for lengthy period, the baby may develop brain damage called hypoxic-ischemic encephalopathy. A significant proportion of babies with this type of brain damage die. Others may develop cerebral palsy, which is then often accompanied by mental impairment and seizures.
During most labors, electronic fetal heart rate monitoring and fetal scalp pH sampling are used to assess fetal wellbeing and to detect fetal distress preventing cerebral palsy. Experts can preview the fetal heart monitoring strips and take appropriate action, often an emergency cesarean section.
Premature Delivery and Neonatal Intensive Care
Another major cause of cerebral palsy/mental retardation is premature birth. Premature labor can often be halted by medication, but it must be diagnosed at the earliest stage. Medication given to the mother can accelerate fetal lung development. Immature lungs at birth are a major factor in subsequent complications, including brain hemorrhage and death.
Contact doctor if you have questions about your child's cerebral palsy, or if you would like to learn more about protecting your child's right to a lifetime of benefits.13
Risk Factors
Scientists have examined thousands of expectant mothers, followed them through childbirth, and monitored their children's early neurological development. As a result, they have uncovered certain characteristics, called risk factors, which increase the possibility a child will later be diagnosed with cerebral palsy.
Knowledge of these risk factors helps doctors keep a closer eye on children who may exhibit later warning signs for cerebral palsy. However, do not become too alarmed if your child has one or more of these factors. Most such children do not have and do not develop cerebral palsy.
Cerebral Palsy Risk Factors Include
- Breech presentation
- Babies with cerebral palsy are more likely to present feet first, instead of head first, at the beginning of labor.
Complicated Labor and Delivery
Problems with the baby during labor, or a long and complicated delivery may sometimes be the first sign that a baby has suffered brain damage during the pregnancy or in the early stages of birth, or that a baby's brain has not developed normally. Additionally, the complications themselves can cause permanent brain damage in an otherwise normal baby.
Birth Defects
Babies with physical birth defects such as malformation of the spinal bones, hernia (a protrusion of organs through an abnormal opening inside the body) in the groin area, or an abnormally small jaw bone-are at an increased risk for cerebral palsy.
Low Apgar Score
The apgar score (named for anesthesiologist Virginia Apgar) is a numbered rating that reflects a newborn's condition. To determine an Apgar score, doctors periodically check the baby's heart rate, breathing, muscle tone, reflexes, and skin color in the first minutes after birth. They then assign point (0–2 for each of the five areas); total scores closer to 10 are desirable. A low score at 10–20 minutes after delivery is often considered an important sign of potential problems.14
Low Birthweight and Premature Birth
The risk of cerebral palsy is higher among babies who weigh less than 2500 grams (5 lbs, 7 1/2 oz) at birth and among babies who are born less than 37 weeks gestation. This risk increases as the less the baby weighs.
Multiple Births
Twins, triplets, and other multiple birth are linked to an increased risk of cerebral palsy.
Nervous System Malformations
Some babies born with cerebral palsy have visible signs of nervous system malformation, such as an abnormally small head (microcephaly). This suggests that problems occurred in the development of the nervous system while the baby was in the womb.
Maternal Bleeding or Severe Proteinuria Late in Pregnancy
Vaginal bleeding during the sixth to ninth months of pregnancy and severe proteinuria (the presence of excess proteins in the urine) are linked to a higher risk of having a baby with cerebral palsy.
Maternal Hyperthyroidism, Mental Retardation or Seizures
Mothers with any of these conditions are slightly more likely to have a child with cerebral palsy.
Seizures in the Newborn
An infant who has seizures at birth is at a higher risk of being diagnosed later in childhood with cerebral palsy.
Contact doctor if you have questions about your child's risk factor, or if you would like to learn more about protecting your child's right to a lifetime of benefits.
Warning Signs
Cerebral palsy is often suspected when a child does not achieve normal growth milestones, such as rolling over, sitting, crawling, smiling, or walking. However, more severe symptoms may also appear at birth. If you are concerned about your baby's development, you should check with your doctor. He or she can help your distinguish between normal developmental variation among children and a more significant developmental disorder.15
Contact doctor if you have questions about your child's cerebral palsy, or if you would like to learn more about protecting your child's right to a lifetime of benefits. Here are some cerebral palsy signs to look for in your child.
At birth
Difficult breathing,
Trouble eating,
Trouble eliminating,
Lack of certain reflexes.
At three Months
Does not respond to you voice,
Does not follow toys with eyes,
Does not use facial expressions,
Has unusually stiff or floppy muscles,
Consistently uses one side of his body more than the other,
Does not enjoy being around people.
At Four to Five Months
Has difficulty in getting objects to his mouth,
Doesn't turn his head to locate sounds,
Doesn't smile spontaneously.
At six Months
Cannot sit without help,
Does not laugh or make sounds,
Does not actively reach for toys.
At Seven to Eight Months
Does not follow toys with both eyes at both near and far ranges,
Does not actively reach for toys,
Does not bear some weight on legs,
Does not try to attract attention through actions,
Does not babble,
Would not play games.
A Broad Spectrum of Etiology
For the purpose of study we divide all the causes of cerebral palsy under three basic groups:
- Perinatal factors and
- Postnatal factors
Prenatal Factors
It comprises causative factors from the time of conception to the onset of labor.
Hereditory
The familial diseases like familial athetosis, congenital tremors, familial spastic paraplegia, familial rigidities due to genetically transmitted developmental defects in the basal neuclei and/or in their tracts. Also certain progressive, hereditary, neurofibromatosis, tuberous sclerosis and other rare neurological conditions, etc. can cause cerebral palsy. These children suffered more of congenital malformations like extradigits, cleft palate, etc. Even premature deliveries could be the cause sometimes.
Acquired During Gestations
Irradiation: The X-ray (therapeutic irradiation of a pregnant mother) of lower abdomen may lead to cerebral damage of the fetus which may be the result of the lowest amount of exposure too. Examine mother for pregnancy before X-rays.
Even those who were within 1200 meters of the center of atomic explosions, the pregnant mothers gave birth to microcephalics and mentally deficient children.
Maternal Infection: Development to the brain in fetus reaches maximum during second and fourth months of gestation. German measles to the mother can cause infections like deafness, cataract, congenital cardiac malformations, and auditory aphasia. The above abnormalities can be developed even when mother suffers from the following:
- Toxoplasmosis,
- Mumps
- Chickenpox
- Herpeszoster
- Influenza and
- Syphilis.
Prenatal anoxia: If any interference in the oxygen exchange between placenta and fetus takes place for sufficiently long time, it can lead to premature/permanent injury to the fetal brain.17
The mechanisms to produce or induce interference are:
- Placental abnormalities: As abruptio placenta, placenta previa, poor development of placenta—in hyperthyroid mothers, multiple infarcts of placenta and bleeding.
- Maternal anoxia: Due to sudden atmospheric changes from low to high altitude, maternal allergies to analgesic drugs during labor, shock of trauma, spinal shock reactions and nitrous oxide anesthesia during labor for long period.
- Cord anomalies: They may collapse, kinking, knotting, compression of the cord leading to anoxia. Cord may wrap around the neck of the fetus tightly causing anoxia/asphyxia which requires immediate procedures to cut cord.
- Maternal hypertension: May be with maternal anoxia.
Hemorrahage during pregnancy: Mothers of cerebral palsy children generally give history of it. The prenatal hemorrhage in mothers resulted into quadriplegia in cerebral palsied child. Even premature separation of placenta leading to bleeding was responsible for anoxia of the fetus.
Fetal cerebral hemorrahage: It may take place within the fetal brain or on the brain surface, or may occur due to prolonged anoxia of cerebral blood vessels, or trauma to brain in utero. The blood vessels also may suffer damage due to toxemia or blood dyscrasias in mothers.
Kernicterus: A permanent damage of basal ganglia of brain due to mismanagement of hemolytic disease of the newborn or hyperbilirubinemia in premature babies is known as kernicterus. It may produce the following:
- Athetosis
- Deafness
- Mental retardation and
- Auditory aphasia.Its incidence is far low nowadays to produce cerebral palsy than in the past.
Prematurity: Birth injury is more susceptible to cerbral palsy. Partial separation of placenta and other types of prenatal hemorrhages may lead to premature labor and fetal anoxemia. It even resulted into intracranial hemorrahage because of great fragility of the blood vessels in the children.
Predisposition to miscarriage: The number of miscarriage is directly proportional to the incidence of cerebral palsy.18
Metabolic disturbances during pregnancy: Though exact mechanism of such children is not clear, the diabetic mothers also produce cerebral palsy who have difficulty of conceiving, overweight during pregnancy, delivering unusually large baby, who have difficulties in being revived and in postnatal management during the first 96-hour after birth.
Maternal toxemia: The pre-eclampsia is the main cause of cerebral palsy; in certain cases, hemiplegia, quardriplegia and athetosis. The toxemias pass through placental barriers and fetus is severely involved. Postnatal care of infant of toxic mother is most difficult. Postnatal asphyxia of infant is extremely difficult to overcome and susceptibility to infection is very high.
Perinatal Factors
Severe cerebral agenesis due to prenatal factors could very well be allied with apnea at birth distress of fetus, drowsiness, and even convulsion. It may be due to instrument delivery or some other procedures or may be due to intracranial complications by anoxemia in utero.
During this period causes can be best studied in two headings as:
- Traumatic and vascular, i.e. mechanical causes, and
- causes producing anoxia.
Mechanical Factors
Prolonged Labor: The increased time in labor is directly proportional to the occurrence of cerebral hemorrahage due to trauma. It is 18 hours in primiparous and 12 hours in multiparous mothers respectively.
Mechanical factors depending upon the type of delivery: It has been observed that birth trauma differs from head injury which occurs later in life due to slow molding and compression of the head more frequently than to violent blows and trauma. Distortion of the fetal head may also result in the tearing of the dural septa.
Thoracic compression may be an important cause of cerebral congestion and venous extravasation. The blood is forced out of the large venous channels of the chest and abdomen into the head and neck. If this compression is severe and prolonged, traumatic asphyxia may be developed. Due to this severe cyanosis of head and neck persists for several days and may be associated with meningeal and retinal hemorrhages. Intracranial hemorrahage in cesarean section results due to fetal head being delivered through a very small incision in the uterus. Even use of forceps cause trauma to fetal head.19
Physical injury may result in cerebral damage by laceration of the cortex by depressed fracture from excessive pressure by obstetrical forceps or from hemorrahage into subdural space on the hemisphere themselves. Subdural space hemorrhage is most of the time cause of death of the child. Traumatic delivery also is the cause of cerebral hemorrhage. Generally injuries like high forceps, breech delivery, mid-forceps, cesarean section, low forceps, spontaneous delivery, low forceps with episiotomy are seen.
Cesarean section is indicated when possibility of intracranial hemorrhage cannot be ruled out. Percentage trauma in CS is less than other types of deliveries. Baby thus delivered by the section is deprived of those stimuli usually received during vaginal delivery, i.e. initiation of normal respiration. Consequently, adequate and immediate resuscitation may be necessary in cesarean section if anoxia is to be prevented.
Such children, even though immediate breathing is given, tend to develop apnea. So they must be observed for next 48 hours after birth. Such children have more amniotic fluid and debris in bronchial tree than the normally delivered child and should have careful bronchial aspiration at birth to avoid hyaline membrane, atelectasis and pneumonia.
Forthcoming head in breech delivery can also produce severe anoxemia. The child is handled at room temperature and change enabling efforts aspirating large amount of debris. Child of hemorrhagic diathesis is prone to cerebral hemorrhage in addition to hemorrhage anywhere else in the body. It may not be associated with birth trauma. It may appear within 48 hours after birth.
Fetal Asphyxia
Perinatal anoxia is very common cause of cerebral palsy. Asphyxia at birth may cause several cerebral lesions found on autopsy in cerebral palsy. Undue suppression of the respiratory centers through excessive premedication of the mother or by prolonged use of the anesthetics, particularly nitrous oxide plays an important role in such patients. Prolonged delivery due to improper dilation of birth canal, deep transverse arrest, uterine inertia, excessive pressure by forceps, or holding the head back for unduly long time, are the predisposing factors of the perinatal asphyxia. They cause damage to basal ganglion and lead to athetosis. Prematurity is due to sudden changes between intrauterine pressure and atmospheric pressure.20
Fetal Anoxia
It can be due to:
Mechanical respiratory obstruction: They cite asphyxia, cyanosis, atelectasis or congenital pneumonia, and hyaline membrane formation as common factor. This occurs more frequently in premature baby and cesarean sections. Frequency is related to the aspirated amniotic fluid and other debris during the delivery. Atelectasis/ bronchitis cause decrease in the functional number of alveoli.
Injudicious use of analgesics and anesthetics: Use of such drugs cause depression of the respiratory centers of the fetus lasting 4 to 36 hours after birth producing anoxia and period of apnea. Narcotised body should be observed for at least 48 hours after birth.
Use of resuscitation and oxygen must have been of considerable help in maintaining adequate cerebral oxygenation. The forced oxygenation of premature babies shall also develop retinal damage along with cerebral damage.
Perinatal causes are the biggest causes of cerebral palsy and this is the period when possibility lies a great deal in prevention of the cerebral palsy. Inadequate development of premature baby may be weak, having easily traumatised vessels, a tendency to anemia and hypoproteinemia, a lower resistance to infection and great tendency to aspirate mucus, amniotic fluid and other debris.
Postnatal Factors
These causes are numerous. The possible role of a prenatal cause for the postnatal development of cerebral palsy must not be overlooked.
These causes have been classified by Russ and Soboloff as under:
- Traumatic injuries: Accidents involving head and skull fracture due to automobile and contact sports producing large percent of brain injuries.
- Infections: Cerebral infections are very common in children. Like encephalitis, meningitis, brain abscess are common ones. Scarlet fever may cause thrombosis of one of ascending frontal branches of the middle cerebral artery resulting into damage of motor cortex, and that otitis media may produce hemiplegic syndrome by a retrograde thrombosis of the regional veins.Rheumatic heart disease may cause an embolism causing a focal lesion resulting in the hemiplegic state of cortical origin.
- Pyogenic meningitis (post infection) and
- Toxic factors: They are very uncommon. Any substance, digested, inhaled or injected into the infant which causes toxic changes in the brain must be the factors precipitating cerebral palsy. A frequent postnatal cause is leading to encephalopathy.
- Vascular accident: More frequent in adults. In children so called “stroke” may occur in congenital aneurysm of brain in the circle of Willis.
- Cerebral anoxia: Insufficient oxygenation of brain due to carbon monoxide poisoning and high altitude anoxia can be the causes in postnatal period.
- Brain tumors: Congenital or acquired are rarely the cause of cerebral palsy. It may also include brain cysts/internal hydrocephalus, etc. The residual sequela of surgical intervention and the tumor may produce the syndrome known as cerebral palsy.
APPLIED ANATOMY AND PATHOLOGICAL FINDINGS
Cerebral palsy is the result of intracranial pathology which falls in three groups:
- Lesions of the motor cortex
- Lesions of the basal ganglion and
- Lesions of the cerebellum.
Motor cortex lesions result into the sapasticity and flaccidity of the muscles, if in the basal ganglion they cause athetosis, tremor and sometimes rigidity and lesions of the cerebellum cause ataxia and incordination and diffuse lesion of the brain produces rigidity.
Limitation of motion due to concomitant involvement of extrapyramidal system/pathways, even further control through interference with cerebellar function complications increase with diminished/loss of vision, abnormal movements due to dysfunction due to striatal and subthalamic system which may modify disability at any attempt to correct it. Gravity may increase due to loss of vision, frequent fits, hyperkinetic activity, impaired intelligence, speech and auditory disturbances, which may arise due to other cortical lesions.
APPLIED ANATOMY AND PHYSIOLOGY
Pyramidal Motor Pathways
Pyramidal system has its origin in the large pyramidal cells of the precentral gyrus as area 4 in the cellular elements of giants cells of Betz. Area 4 is the transmitter for accomplishing voluntary movement where intent purpose of the same origins in cerebral context lying anterior to it. The tracts of the pyramidal system join with the lower 22motor neuron in the nuclear masses of brainstem and in the anterior horn cells of the spinal gray matter which is eventually completed through series of the internuncial elements serving as distributors of the impulses to the parts responsible for motor activity.
The complex arrangement as follows: any effective treatment of spasticity, at present this field appears but in its infancy must take into account the neurologic features as:
- The spinal stretch reflex.
- Central inhibitory influence which reduces it.
- Central facilitatory influence which reduces it, so (i) – (ii) + (iii) = Spasticity.
Spastic paralysis develops due to pyramidal system damage, flaccid paralysis develops when area 4 of motor area is damaged or when pyramidal system/tract is interrupted in the medulla where only motor fibers are present.
Though cerebral palsy involves rarely (cerebral) motor cortex lesion—a flaccid paralysis is very rare. Extrapyramidal influences when modified give rise to spastic paralysis which is an accessory system associated with pyramidal pathways in controlling motor activity.
Cortical representation of this accessory factor is in the postfrontal areas lying just anterior to motor strip. This is area 6. So like area 4 is suppressor area and 6 as the cortical zone in which gross muscle movements of the trunk, upper extremity and lower extremity have their origin.
This shows that spasticity of cerebral origin is due to lesion in this accessory extrapyramidal system tracts from here terminate in corpus striatum and substantia nigra and red nucleus in upper part of the brain. These nuclear centers exert their influence on internuncial fibers of the cord, acting by suppression mechanism within the spinal segments which make for spasticity. There is another mechanism—reticulofacilitatory system exerting influence on the stretch reflexes of cord producing hyper-reflexia of spasticity.
This is removal of suppression exerted by the accessory areas of 4 and 6, result of which is modified by reflex facilitatory mechanism in the brainstem on reflex connections on the spinal cord through the reflex spinal pathways.
When complex syndrome of spasticity of rigidity is seen may not be infrequently involuntary motions—may result due to cellular lesions in basal ganglion and their connecting fiber tracts. Ganglionic elements of the extrapyramidal involuntary movement patterns in the forms of tremor, athetosis, dystonia, etc. Peculiar torsion movements of dystonia may be due to lesion of subthalamic nucleus of Luys.23
Tremors result due to abnormal discharge of impulses descending in the reticulospinal bundle leading to synchrosed motor firing in the spinal cord.
Cerebellar Elements
Ataxia and dysmetria may arise due to cerebellar lesions keeping to the definition of cerebral palsy as paralysis of cerebral origin. Cerebellum exerts a controlling influence on the motor system by maintaining muscle tone, by checking movements which make precise actions possible, by integration of individual movements to have complicated movements without breaking down to separate elements. So cerebellar lesions result into muscle tone alteration, difficulty in limitation of a motion, and decomposition of a movement.
Congenital Deformities
Congenital brain defect may not lead to cerebral palsy. Congenital abnormalities of individual convolution, or focal regions of the cortex (tuberous sclerosis). Vascular defects of congenital origin, may produce motor disability.
Traumatic Lesions
Cranial fractures when compress underlying brain which may often due to depression of the fragments. Laceration of meninges may be accompanied by extradural and subdural hemorrhage which may be in meninges than in brain substance. Brain may be injured due to compression resulting into hematoma.
A linear scar of the brain may result from a curved depressed fracture resulting from excessive compression of obstetrical forceps. Many children do not survive this.
The depressed fracture of cranial vault due to traffic accidents or falls may result in bruising/laceration of the brain of child survives-scar remains with an ingrowth of connective tissue. Residual hemiplegia may be complicated due to conseizures.
SCLEROSING ATROPHY OF THE BRAIN
Generally in cerebral palsy, there is irregular nodular shrinkage of cerebral convolutions. This can be minor affecting only a portion of a gyrus or localized gyri. They may be associated with perinatal anoxia and other causing ischemia of the brain due to vascular spasm, inflammation and embolus.24
Diffuse Degeneration of the Cerebral Cortex and Basal Ganglion
It results into diffuse alteration of the gray matter of cerebral cortex, the basal ganglion, sometimes motor nuclear masses of brainstem and cerebellum. This may be associated with some familial diseases or degenerative disorders.
Subdural Hematoma of Infants
This lesion may be due to subdural (hematoma) bleeding at the time of birth, with symptomless effusion. Hematoma becomes encapsulated-acting as semipermeable membrane permitting the passage of fluid from subarachnoid space into the clot, which enlarges hematoma compressing the cerebral cortex-leading to ischemia.
CLASSIFICATION AND DIAGNOSIS OF CEREBRAL PALSY
Classification of Phelps
There is standardization of the prevalent types of cerebral palsy which can be mentioned (as Phelps described):
- Flaccid paralysis
- Spasticity
- Rigidity
- Tremors
- Athetosis and
- Ataxia.
This is based on clinical manifestations of cerebral palsy which is useful for both the medical and rehabilitation team members. It was even classified as:
- Spastic and
- Extrapyramidal.
It has been common experience that true cerebral monoplegia is extremely rare. In spastic hemiplagia arm involvement is invariably recognised before by and most of the cases supposed of monoplegia turn out of hemiplegia. Large cases of spastic tetraplegia developed extrapyramidal features and became true mixed palsies.
Athetosis
An impairment of motion in which attempted activity results in a succession of slow involuntary movements which are smooth riding in character on close observation involving instability of opposing postures such as flexion and extension or pronation on supination, involving the distal musculature. It was said that athetosis is present during sleep and disappeares if patient is completely secure posturally or otherwise and is not attempting any activity.25
Chorea
It is fully or largely involuntary irregular, spasmodic contracture of individual muscle/muscles or muscle groups of extremities or of the face with muscular weakness or ineffectiveness in attempted activity.
Dystonia
It is a disorder of the movement producing rhythmic and to a large extent, involuntary twisting, distortion and changes of tone mainly of the trunk musculature and proximal parts of the limb.
Ballismus
A coarse, large scale, involuntary jerking or flinging motion of an extremity. They also emphasized that the disorder of movements in patient in the extrapyramidal group usually changing with the advancing age.
Classification of Brenda
Cerebral palsy is divided as under:
- Little's spastic rigidity (decerebrate rigidity).
- Pyramidal Type (mono-, hemi-, di- and paraplegias).
- The mixed extrapyramidal-pyramidal type (paraplegias with athetosis).
- The ataxic atonic-cerebellar type.
Nowadays the clinicians have accepted motor classification of the cerebral palsy in planning their treatment program. It should be recognized that so-called motor classification which is a descriptive or symptomatic classification is based on neurological signs and symptoms which undergo changes as nervous system matures and it cannot be finally diagnosed in the childhood only.
After nervous system matures the symptomatology becomes static and standardised. Therefore, we must follow changing neurological patterns in childhood. Motor changes may be made as symptomatologic changes.
When child is followed through early infancy through school age, we will find neurological symptomatology changes and child shows athetosis or spasticity. Therefore, motor classification of childhood is only tentative classification.26
GENERAL CLASSIFICATION OF CEREBRAL PALSY
- Physiological (motor)
- Spastic
- Athetosis: Tension, non-tension, dystonic, tremor
- Rigidity
- Ataxic
- Tremor
- Atonic (rare)
- Mixed
- Unclassified.
- Topographical
- Monoplegia: One limb affected, rare, close check for eliminating hemi/paraplegia.
- Paraplegia: Both legs, generally spastic or rigidity types.
- Hemiplegia: One-half affected, usually spastic but athetoid hemi, also seen as like rigidity, hemiplegias, sensory involvement in proprioception, aphasias in right sided acquired lesions.
- Triplegia: Three limbs affected (2 legs + 1 arm), usually spastic, may be hemi plus paraplegia or incomplete quadriplegia. In latter both arms, equal or hemi equal but in former involved arm is short.
- Quadriplegia: All four limbs affected, legs greatly affected and are spastic, patients with greatest arm involvements usually ideokinetic including athetoid.
- Diplegia: Spasm used paralysis affecting like parts on either side of the body, bilateral paralysis.
- Double hemiplegia: Arms more involved than legs, usually are spastic.
- Etiological:
- Prenatal
- Hereditary
- Acquired in utero
- Natal and
- Postnatal.
- Supplemental
- Psychological evaluation: For IQ for mental relationship,
- Physical status: Physical growth evaluation, developed levels prone age, contractures,
- Convulsive seizures,
- Eye-hand behavior patterns:
- Eye-dominance,
- Eye movements,
- Eye-postures,
- Fixations,
- Convergence,
- Presensory approach,
- Grasp,
- Manipulation and
- Hand dominance.
- Visual status
- Sensory-amblyopia
- Field defects
- Motor
- Conjugate deviation
- Fixation defect
- Spasmus fixus (1%)
- Esotropia (5%)
- Exotropia
- Hypertropia
- Hypotropia
- Nystagmus
- Pseudopalsy of externi.
- Auditory status– Pitch range loss– Decibel loss
- Speech disturbances.
- Functional capacity (degree of severity)
- Class I: Patient with CP with no practical limitation of activity.
- Class II: Patient with CP with slight to moderate form of limitation of activity.
- Class III: Patients with CP with moderate to great limitation of activity.
- Class IV: Patients with CP unable to carry on any useful physical activity.
- Therapeutic
- Class A: Patients with CP requiring no treatment
- Class B: Patients with CP requiring minimal bracing and terminal therapy.
- Class C: Patient with CP who need bracing and apparatus and series of CP team.
TYPES OF CEREBRAL PALSY
There are four types of cerebral palsy:
- Spastic cerebral palsy (difficult or stiff movement)
- Ataxic cerebral palsy (loss of depth perception and balance)
- Athetoid cerebral palsy (uncontrolled or involuntary movements)
- Mixed cerebral palsy (a mix of two or more of the above).
Spastic Cerebral Palsy
Spastic cerebral palsy affects around 70 percent of children with cerebral palsy. In this form a child's muscles are stiffly and permanently contracted, limiting their range of motion and causing jerky, unpredictable movements. Often a child has trouble holding or letting go of objects or moving from position to position. Based on the particular areas of the body that are affected, spastic cerebral palsy has several typical manifestations. When both legs are affected, they often turn in and cross at the knees, causing an awkward and stiff walk with a characteristic rhythm, known as the scissors gait. Children may also experience uncontrollable shaking (or tremors) of the limbs on one side of their body. This is known as spastic hemiparesis and, if severe, may seriously impair movement.
Ataxic Cerebral Palsy
This rare form of cerebral palsy effects around 5 to 10 percent children. Impacting a child's sense of balance and depth perception, children with this type of cerebral palsy often have poor coordination, an unsteady or wide-based gait (placing their feet unusually far apart), and experience difficulty when attempting quick or precise movements (such as writing or buttoning a shirt). These children may also suffer from intention tremors. This form of tremor begins with a voluntary movement, such as reaching for a book, and causes a trembling that affects the body part being used. The tremor worsens as the individual gets nearer to the desired object.
Athetoid Cerebral Palsy
This form of cerebral palsy is characterized by uncontrolled, slow, writhing movements. These abnormal movements usually affect the hands, feet, arms, or legs and, in some cases, the muscles of the face and tongue, causing grimacing or drooling. The movements often increase during periods of emotional stress and disappear, during sleep. Children may also have problems in coordinating the muscle movements needed for speech, a condition known as dysarthria. Athetoid cerebral palsy affects about 10 to 20 percent of patients.29
Mixed Cerebral Palsy
As many as 10 percent of children with cerebral palsy have symptoms of more than one of the forms of cerebral palsy. The most common mixed form includes spastic and athetoid movements but other combinations are also possible.
SYMPTOMS OF CEREBRAL PALSY
Symptoms of cerebral palsy can range from mild to severe. They differ from person to person, depending on the type of cerebral palsy the person has, and may even change over time as the child grows. Some children with cerebral palsy have difficulty with fine motor tasks, such as writhing or cutting with scissors, while others experience trouble balancing and walking. Still others are affected by involuntary movements, such as uncontrollable writhing motions of the hands or drooling. Other disorders often accompany cerebral palsy, including seizures or mental impairment. Cerebral palsy is not contagious, nor is it inherited from one generation to the next. At this time, it cannot be cured, although scientific research continues to make advances in treatments and methods of prevention.
Contrary to common belief, cerebral palsy does not always cause profound handicaps. While one child with severe cerebral palsy might need extensive, lifelong care, another child with mild cerebral palsy might require no special assistance at all.
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