Cerebral Palsy (1994)

 

 

 

 

 Submitted to Dr. Roger Weed in Partial Fulfillment of the Requirements for CPS 841

Georgia State University

8 March, 1994

 

Cerebral Palsy is the name of a variety of nonprogressive brain defects which occur during the prenatal, perinatal, or early postnatal periods, and which result in motor impairment (What everyone should know about cerebral palsy, 1977). It is not “a disease in any medical sense” (Crothers & Paine, 1988, p. 1), but can arise for any number of reasons. It causes major disability and is associated with a variety of other disabling conditions, including epilepsy, mental retardation, and sensory deficits (McDonald, 1987).

Ancient drawings and texts describe and portray individuals with cerebral palsy. McDonald (1987), for instance, mentions that early stone carvings from Mexico depict persons with the characteristics of cerebral palsy. Courville (1954) includes reproductions from ancient Egypt and Renaissance Europe in his text, and notes that Hebrew and Greek scriptures frequently mention the lame and halt.

The term palsy has long been a term used for those who shake, and the term cerebrum has long signified the brain, but it was not until the second half of the nineteenth century that the two were combined and a condition which was known from ancient times was finally given a name. The condition was first identified by an English physician, W.J. Little, who wrote mid-century about a condition he termed “spastic rigidity” and others began to call Little’s Disease. Authorities are not sure who coined the term cerebral palsy, as it suddenly began to appear in journal articles in the late 1800s (McDonald, 1987). Kopriva & Taylor (1992) attribute the honor to Dr. Winthrop Phelps in 1937, but Sigmund Freud published a book with the title Infantile cerebral paralysis in 1897, and even before that, in 1888 and 1890, articles were published in medical journals by Osler (“The cerebral palsies of children”) and Sachs & Peterson (“A study of cerebral palsies of early life based on an analysis of one hundred and forty cases”) (McDonald, 1987). The foundation of the American Academy for Cerebral Palsy in 1947 resulted in widespread recognition of and acceptance of the term.

Cerebral palsy is not uncommon. Phelps (1951) estimated an incidence rate of 7 births each year per 100,000 population. Between 500,000 and 700,000 Americans have the condition to some extent. About 3000 American infants are born with CP every year (What everyone should know about cerebral palsy, 1977).

According to Courville (1954), writing only forty years ago, one in seven births of children with cerebral palsy resulted in death. No doubt that rate was much lower than had historically been the case. Recent advances in obstetrics and neonatal treatment have reduced the mortality rate for newborn children. Most children born with cerebral palsy now survive (Sternfeld a, n.d.).

Cerebral Palsy can occur for a variety of reasons. Anything which damages the brain can result in the condition. Causes include, but are certainly not limited to: genetic defects; embryological developmental errors; cerebral hemorrhage or blood clots; brain tumor; maternal substance abuse; maternal diabetes; RH incompatibility factors between mother and fetus; Rubella and other infectious diseases, either in utero or after birth; presence of toxic chemicals in the womb or postnatal environment; premature birth; head trauma; hypoxia or anoxia; and birth difficulties or trauma.

For many years, a major cause, and perhaps the major cause of cerebral palsy was considered to be difficulties during the birth process. Lack of oxygen caused by a constricted umbilical cord and trauma caused by prolonged labor or by use of forceps were considered to cause lesions on the brain. Recently, however, studies have begun to emerge which indicate that most brain damage which results in CP is present before the perinatal period (Skolnick, n.d.). No doubt this is a relief to obstetricians, and particularly to their insurance companies.

The hallmark characteristic of CP is motor disability. This can take a variety of forms, but all interfere significantly with the ability of the individual to do fine and gross motor tasks. In many cases, the individual will be unable to walk, and in some cases, will be unable to propel a manual wheelchair. Even a powered wheelchair requires some voluntary motor ability, and the individual must be able to operate the controls in order to use it.

Cerebral Palsy is classified by motor patterns. The American Academy of Cerebral Palsy classification (Minear, 1956) includes Little’s classic spasticity (caused by a hyperactive stretch reflex, and resulting in jerky movements), athetosis (arrhythmic involuntary movement), rigidity, ataxia (difficulty maintaining balance), tremor (repetitive, rhythmic involuntary movements, and atonia (deficit in muscle tone). (See Table I).

Any or all of the above AACP types can be mixed, and any of the characteristics can be present in mild or severe form. Especially when the high incidence of co-occurring disorders is mixed in, it is clear that cerebral palsy can present an extremely mixed clinical picture.

Spasticity has always been considered to be the most common type of cerebral palsy. In 1981, a sample of more than 2000 cases resulted in the breakdown shown in Table I (O’Reilly & Walentynowicz, 1981).

An alternate way of classification is by topography. Topographical classifications describe the areas of the body which are affected (see Table II).

Cerebral palsy is also classified by severity. The AACP defines the degree of severity by functional capacity, and Rusk (1977) has proposed an alternative classification (Table III).

Obviously, the areas of the brain which control motor movement are damaged in cerebral palsy, but other areas may be affected as well. Even with relatively intact brain structures, the emotional consequences of dealing with a lifelong disability can have tremendous impact. Conditions frequently associated with cerebral palsy include mental retardation, sensory dysfunction, social-emotional complications, educational difficulties, seizure disorders, and physical disability. Crothers and Paine (1988) note “The official diagnosis does not even suggest the variety of problems which arise in the course of management. In the first place, the disorder of motor control is only one, and often the least, of the difficulties which face these children” (p. 1).

The brain damage caused by CP is permanent, but advances in obstetrics can reduce the risk of the condition. Inoculation against Rubella and other diseases, monitoring of blood incompatibilities between mother and fetus, genetic counseling, control of diabetes and other maternal conditions, increased public education of fetal alcohol syndrome and other conditions, ultrasound and other imaging technology, and a variety of other advances help to reduce the incidence of CP. A variety of medical conditions, including hyperbilirubinemia and hyaline membrane disease, which once were leading causes of cerebral palsy, can now be controlled through monitoring and medication (Sternfeld, a, n.d.)

There have also been notable advances in the treatment of CP. Perhaps most significantly, early childhood identification and treatment programs can identify CP at a very early age, and routine screenings at birth and during infancy can result in dietary regimens and medical regiments which can prevent brain damage.

Cerebral palsy is rarely immediately apparent, and is often detected only when the baby fails to meet major developmental milestones (Sternfeld b, n.d.). Increased awareness of normal development, taught to physicians in medical school, and to parents by obstetricians and pediatricians, can result and has resulted in early diagnosis and earlier provision of special equipment and training. Availability of occupational and physical therapy can reduce the probability and severity of contractures and help prevent the deformities which eventually occur. Provision of orthopedic devices and wheelchairs can help the individual to ambulate and increase his or her ability to interact with the environment. Medication can help with seizure conditions and behavioral disorders, and in some instances, surgery can increase functional abilities of limbs with contractures or deformities. Special education services can address mental delays which often co-occur with CP, and provision of sensory correction and amplification devices like eyeglasses and hearing aids can increase learning opportunities by keeping the individual aware of the environment. Speech therapy can address the language deficits which often accompany CP, and augmentative communication devices can help the individual to interact with others. When all these services are provided with the various professionals working together with the family as an interdisciplinary team, the overall needs of the individual can be addressed and the handicapping potential of the disability can be reduced.

It is impossible to overstate the importance of 20-century technology in helping people with physical disabilities to lead more productive lives. Perhaps the most important development of this century has been the microprocessor, which has only begun to live up to its promise in augmentative communication and other devices.

 

Vocational Considerations

Individuals with cerebral palsy are born with the condition. In cases of severe motor involvement, the individual with normal or superior intelligence may have been assumed to be mentally retarded and denied proper educational experiences. Even with the best of treatment and the most advance augmentative devices, the individual may lack social skills because of simple lack of experience. Even with PL 94-142 and the Education of All Handicapped Children Amendments of 1986, there may still be educational limitations.

Many persons with cerebral palsy are capable of entering the work force, given sufficient supporting services. It is important to realize their functional limitations, so they can be considered when setting up the vocational placement. Limitations include deficit in self-help skills; poor fine motor skills; inability to ambulate; presence of other disabling conditions (e.g. epilepsy); limited sensory abilities; inability to operate equipment or drive; inability to produce recognizable speech; mental retardation; limited endurance or strength; limited experience and learning history; and limited experience in the work setting. Of course, all individuals do not have all of these limiting factors, but several are usually present, and it is important to structure a job so that it is within the physical, sensory, and mental limitations of the individual.

Section 504 of the Rehabilitation Act of 1973 and the Americans with Disabilities Act of 1990 increase access and make it illegal for many agencies and work sites to discriminate against Americans with disabilities. Nonetheless, it is often difficult to find productive work which matches the abilities of persons with cerebral palsy, and it is important that vocational rehabilitation counselors be part of the interdisciplinary team so that the individual’s potential for employment can be maximized.

 

Discussion

In my research for this paper, I was initially surprised by the paucity of textbooks and journal articles about cerebral palsy and especially by the lack of community resources for persons with cerebral palsy. I saw little evidence in the library of the “accelerated interest” in cerebral palsy mentioned by Hardy (1983). I was even more surprised to discover the United Way Help Book listed only two sources for information and support for persons with cerebral palsy.

Perhaps this lack of resources is because cerebral palsy is associated with so many other conditions, and help can be found in agencies which deal primarily with persons who are blind and deaf, epileptic, or mentally retarded. Perhaps it is because CP is a relatively rare condition. But then again, perhaps it is because CP is a congenital condition rather than one arising later in life and those with the condition are disregarded and devalued by the rest of us. I hope not, but the fact is that there were only two entries for CP in the United Way Help Book and much more room was devoted to spinal cord injury and brain trauma, conditions which occur later in life.

Percentages are from O’Reilly & Walentynowicz, 1981 and follow the classification suggested by the American Academy of Cerebral Palsy (Minear, 1956).

Rusk (1977) proposed a classification based on competence in carrying out the basic self-help behaviors essential to everyday living:

• Mild: Self-help skills adequate for caring for personal needs, who ambulates without appliances, and has no speech problems
• Moderate: Self-help skills are inadequate for caring for daily personal needs, and special equipment for ambulation may be needed. Speech may be defective
• Severe: Even with treatment and adaptive equipment, the prognosis for developing self-help skills, ambulation, and functional speech is poor.

References

 

Crothers, B., & Paine, R.S. (1988). The natural history of cerebral palsy. London: Mac Keith Press.

Hardy, J.C. (1983). Cerebral palsy. Englewood Cliffs, NJ: Prentice-Hall, Inc.

Kopriva, P., & Taylor, J.R. (1992). Cerebral palsy. In M.G. Brodwin, F. Tellez, & S.K. Brodwin (Eds.), Medical, psychosocial, and vocational aspects of disability. Athens, GA: Elliott & Fitzpatrick, Inc.

McDonald, E.T. (1987). Cerebral palsy: Its nature, pathogenesis, and management. In E.T. McDonald (Ed.), Treating cerebral palsy: For clinicians by clinicians, pp. 1-19. Austin, TX: Pro-Ed.

Minear, W.L. (1956). A classification of cerebral palsy. Developmental Medicine and Child Neurology, 23, 533-538.

Osler, W. (1888). The cerebral palsies of children. Medical News. (Cited by McDonald, 1987).

O’Reilly, D.E., & Walentynowicz, J.E. (1981). Etiological factors in cerebral palsy: An historical review. Developmental Medicine and Child Neurology, 23, 841-852.

Phelps, W.M. (1951). The cerebral palsies. Philadelphia: W.B. Saunders Co.

Rusk, H. (1977). Rehabilitation medicine (4th ed.). St. Louis: C.V. Mosby.

Sachs, B., & Peterson, S. (1890). A study of cerebral palsies in early life based on an analysis of 140 cases. Journal of Nervous Disease. (Cited by McDonald, 1987).

Skolnick, A. (n.d.). New ultrasound evidence appears to link prenatal brain damage, cerebral palsy. Washington, D.C. United Cerebral Palsy.

Sternfeld, L. (a) (n.d.). Research in cerebral palsy. Washington, D.C. United Cerebral Palsy.

Sternfeld, L. (b) (n.d.). How to help a baby with developmental delay. Washington, D.C.: United Cerebral Palsy.

What everyone should know about cerebral palsy. (1977). South Deerfield, MA; Channing L. Bete Co., Inc.