Cerebral palsy

Cerebral palsy or CP is the most common childhood physical disability. It is a permanent physical condition that affects movement. A new international consensus definition has been proposed: "Cerebral palsy (CP) describes a group of disorders of the development of movement and posture, causing activity limitations that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain. The motor disorders of cerebral palsy are often accompanied by disturbances of sensation, cognition, communication, perception, and/or behavior, and/or by a seizure disorder” (Rosenbaum et al, 2005)". The incidence in developed countries is approximately 2-2.5 per 1000 live births. Incidence has not declined over the last 60 years despite medical advances like electro-fetal monitoring. Cerebral palsy is a non-progressive disorder, however secondary orthopaedic deformities are common for example, hip dislocation and scoliosis of the spine. There is no known cure; medical intervention, Conductive Education (w) has been shown to be helpful. These treatments focus on developing the person's participation in everyday life, and not 'fixing' their impairments. While severity varies widely, cerebral palsy ranks among the most costly congenital conditions to manage.

Cerebral palsy is an "umbrella term" in that it refers to a group of different conditions. It has been suggested that no two people with CP are alike even if they have the same diagnosis. Cerebral palsy is divided into four major classifications to describe the different movement impairments. These classifications reflect the area of brain damaged. The four classifications are: (1) Spastic; (2) Athetoid; (3) Ataxic and (4) Mixed. Spastic cerebral palsy is further classified by topography, dependent on the region of the body affected. These typography classifications include: (1) hemiplegia (one side being more affected than the other); (2) diplegia (the lower body being more affected than the upper body); and (3) quadriplegia (All four limbs affected equally).

Cerebral palsy can occur during pregnancy (~75%), at birth (~5%) or after birth (~15%). 80% of causes are unknown. For the small number where cause is known this can include infections, malnutrition, and significant head injury in very early childhood.

Types

Classified by group of muscles involved

Based on the group of muscles involved (typically only used to further describe spastic CP):

Tetraplegia or Quadriplegia : Involvement of the four limbs, the trunk and the head. The great majority of these individuals will not be able to stand up or walk.
Diplegia: Both lower limbs are affected to a degree although most people with diplegia have limited use of their legs. Some diplegic individuals are able to walk either fully independently, with Splints or AFO's or with a walking aid such as crutches or a walker. In diplegia, arms are unaffected or there are only slight effects.
Hemiplegia: Only the right side or the left side of the body is involved. People with hemiplegia are the most likely to walk, even though people with the above two types can often walk without assistance, if severity allows.

NOTE: These are not the only 3 types of spastic CP. Occasionally, terms such as monoplegia, paraplegia, triplegia and pentaplegia may be used.

Classified by presentation

Ataxia (ICD-10 G80.4): Persons with ataxia have damage to their cerebellum which results in problems with balance, especially while walking. It is the most rare type, occurring in at most 10% of all cases. Some of these individuals have hypotonic-like (low-muscle tone). It is common for these individuals to have difficulty with visual or auditory processing of objects and they may have instability in terms of balance or gravity.
Athetoid or dyskinetic (ICD-10 G80.3): Persons with this type generally have involuntary body movements. The damage occurs to the extrapyramidal motor system and/or pyramidal tract and to the basal ganglia. It occurs in ~20% of all cases.
Spastic (ICD-10 G80.0-G80.1): Persons with this type have damage to the corticospinal tract, motor cortex, or pyramidal tract. It occurs in ~70% of all cases.

These three types may be found together. In 30% of all cases of cerebral palsy, the spastic form is found with one of the other types. There are a number of other minor types of cerebral palsy, but these are the most common.

Presentation (signs and symptoms)

All types of cerebral palsy are characterized by abnormal muscle tone, posture, reflexes, or motor development and coordination. The classical symptoms are spasticity, unsteady gait, and dysarthria. CP symptomatology is as diverse as the individuals who have it. Secondary symptoms can include speech or communication disorders, seizures, hearing or vision impairment, cognitive disabilities, learning disabilities, and/or behavioral disorders. Soft tissue findings consist largely of decreased muscle mass.

Bones

In order for bones to attain their normal shape and size, they require the stresses from normal musculature. The osseous findings will therefore mirror the specific muscular deficits in a given patient. The shafts of the bones are often thin (gracile). When compared to these thin shafts (diaphyses) the metaphyses often appear quite enlarged (ballooning). With lack of use, articular cartilage may atrophy, leading to narrowed joint spaces.

Depending on the degree of spasticity in a given patient, they may exhibit a variety of angular deformities about their joints. Vertebral bodies also need vertical gravitational loading forces to develop properly. If a patient with cerebral palsy spends a great deal of time horizontal (in bed) during skeletal maturation, their adults vertebral bodies may be somewhat vertically elongated. Since the horizontal spines of quadrupeds normally appear this way, this finding in humans is sometimes referred to as "caninization". It is suggested to make the patient accustomed to sitting during skeletal maturation (childhood)because it is the easiest way of feeding and also the patient will not have difficulty with sleeping.

History

Cerebral palsy, then known as "Cerebral Paralysis", was first identified by British surgeon William Little in 1860. Little raised the possibility of asphyxia during birth as a chief cause of the disorder. It was not until 1897 that Sigmund Freud, then a neurologist, suggested that a difficult birth was not the cause but rather only a symptom of other effects on fetal development. Research conducted during the 1980's by the National Institute of Neurological Disorders and Stroke (NINDS) suggested that only a small number of cases of CP are caused by lack of oxygen during birth.

Thomas Galton believed that there was consistency between physical disability and aptitude. This attitude remained prevalent until the 1970's when cerebral palsy was itself, an overdiagnosed disorder. Various subtypes, such as hypotonic CP were utilized and when these individuals are taken out of the CP pool, the number drops to 1 in 2,000 individuals. So the number of people with CP depends on what is interpreted to mean. Most scholars acknowledge that ataxic, atheoid, spastic, and mixed are the relevant forms of cerebral palsy; however, various conditions and subtypes may exist. Also, a common misnomer is that CP caused mental retardation when in fact, only individuals whose brain damage is associated with the hippocampus (or frontal cerebral cortex) actually had these difficulties. While learning difficulties and CP may occur, it is common for individuals with CP to have normal lives when their lives are managed coherently and effectively. This means that if the individual can focus on scholastic achievement or improving social skills, rather than when they will get their next treatment, that individual is going to fare better than when the necessary assistive technology is not available.

Motor difficulties are common with individuals who have cerebral palsy. This can vary from paralysis of movement to minor levels of clumsiness. The brain's plasticity at a young age is probably one of the main reasons for the differences between individuals with CP.

Cause

Since cerebral palsy refers to a group of disorders, there is no exact known cause. Some major causes are asphyxia, hypoxia of the brain, birth trauma or premature birth, genetic susceptibility, certain infections in the mother during and before birth, central nervous system infections, trauma, and consecutive hematomas. After birth, the condition may be caused by toxins, physical brain injury, incidents involving hypoxia to the brain (such as drowning), and encephalitis or meningitis. However the cause of most individual cases of cerebral palsy is unknown.

Recent research has demonstrated that intrapartum asphyxia is not the most important cause as it was once considered to be, though it still plays a role, probably accounting for no more than 10 percent of all cases. The research has shown that infections in the mother, even infections that are not easily detected, may triple the risk of the child developing the disorder, mainly as the result of the toxicity to the fetal brain of cytokines that are produced as part of the inflammatory response.

Premature babies have a higher risk because their organs are not yet fully developed. This increases the risk of asphyxia and other injury to the brain, which in turn increases the incidence of cerebral palsy. Periventricular leukomalacia is an important cause of cerebral palsy.

Also, some structural brain anomalies such as lissencephaly cause symptoms of CP, although whether that could be considered CP is a matter of opinion (some people say CP must be due to brain damage, whereas these people never had a normal brain). Often this goes along with rare chromosome disorders.

Incidence and prevalence

Prevalence is best calculated around the school entry age of about six years. In the industrialized world, the incidence is about 2 per 1000 live births^*. In the United States, the rate is thought to vary from between 1.5 to 4 per 1000 live births. This amounts to approximately 5,000-10,000 babies born with cerebral palsy each year in the United States. Each year, around 1,500 preschoolers are diagnosed with the disorder in the USA. There is mental retardation in 60% of the cases, due to brain damage outside the parietal, occipital, temporal or Basal Ganglia. Mental retardation can occur if the child is not given the opportunities to learn; it does not solely occur from brain damage, but from an individual(s)'s ability to 1) communicate with the child and 2) be able to have the child effectively communicate through speech or other means. For example, a child that had CP who suffers from blindness/deafness due to damage that occurred in the occipital and temporal lobes during birth could use tactile sign-language or tulonoma to communicate. Tulonoma is a type of technique where the user puts his/her hands on the speakers mouth and is able to interpret what they say solely based on the lip movement patterns associated with particular word(s). Other disorders paired with CP include disorders of hearing, eyesight, epilepsy, perception of obstacles (such as judging how far away things are when driving a car), speech difficulties, and eating and drinking difficulties. These esimates include individuals who did not have access to an equal opportunity education prior to the American with Disabilities Act of 1990.

Overall, advances in care of pregnant mothers and their babies has not resulted in a noticeable decrease in cerebral palsy. Only the introduction of quality medical care to locations with less than adequate medical care has shown any decreases. The incidence increases with premature or very low-weight babies regardless of the quality of care.

Most recently, Apgar scores have been indicated to not be a reliable method of determining whether or not an individual has CP; it really depends on how quickly oxygen reaches the brain and the body's vital organs that matter, instead.

Despite medical advances, the incidence and severity of cerebral palsy has actually increased over time. This may be attributed to medical advances in areas related to premature babies (which results in a greater survival rate).

Prognosis

Cerebral palsy is not a progressive disorder. A person with the disorder may improve somewhat during childhood, if he or she receives extensive care from specialists. While the brain injury is non-progressive, evidence suggests that functional decline occurs in persons with CP in adulthood. Functional decline can encompass decrease in range of motion, decrease or loss of ambulation, and increased pain. In essence, it appears that adults with CP undergo an accelerated aging process compared to their non-disabled peers. Onset of arthritis and osteoporosis can occur much sooner in adults with CP. Further research is needed on adults with CP, as the current literature body is highly focused on the pediatric patient.

The ability to live independently with cerebral palsy varies widely depending on severity of the disability. Some individuals with CP will require personal assistant services for all activities of daily living. Others can live semi-independently in the community with support for certain activities. Still others can live with complete independence. The need for personal assistance often changes with increasing age and the associated functional decline. However, in most cases, persons with CP can expect to have a normal life expectancy; survival has been shown to be associated with the ability to ambulate, roll and self-feed. As the condition does not directly affect reproductive function, many persons with CP can have children and parent successfully.

Treatment

There is no cure for cerebral palsy, but various forms of therapy can help a person with the disorder to function more effectively. For instance, the use of a standing frame can help reduce spasticity and improve range of motion for people with CP who use wheelchairs. Nevertheless, there is only some benefit from therapy. The treatment is usually symptomatic and focuses on helping the person to develop as many motor skills as possible or to learn how to compensate for the lack of them. The disorder does not affect the expected length of life so treatment focuses on quality of life issues. Non-speaking people with cerebral palsy are often successful availing of Augmentative and Alternative Communication systems such as Blissymbols.

Alternative Treatment

The Institutes for The Achievement of Human Potential (^*) is a non-profit organization dedicated to improving the health and development of children who have some form of brain injury, including children diagnosed with Cerebral Palsy. Established in May of 1955, IAHP has treated and helped thousands of children with neurological problems, including those diagnosed with Cerebral Palsy. In contrast to other treatments, The IAHP claims that with "a home program consisting of a healthy diet, clean air, and respiratory programs many of these children can be well without the need for medication." However, criticism of their program (Doman-Delacato Patterning) is widespread in the medical establishment because studies have not documented its value. One of the most vocal is the American Academy of Pediatrics (AAP).

Usage of the term "spastic"

The term "spastic" describes the attribute of spasticity in one type of cerebral palsy. In 1952 a UK charity called The Spastics Society was formed. The term "spastic" was used by the charity as a term for people with cerebral palsy. The word has since been used extensively as a general insult to disabled people, which some see as extremely offensive. It is also frequently used to insult able-bodied people when they seem overly anxious or unskilled in sports. The charity changed its name to SCOPE in 1994.

Cerebral Palsy as a Learning Disorder, A Different Explanation

Cerebral Palsy can be explained from a different viewpoint. The fundamental principle of this explanation is that motor disorders should be perceived as learning problems (Hari and Tillemans, 1984). Even though the primary problem of the child with Cerebral Palsy is physical, their inability to function could be attributed, first of all, to a psychological problem, i.e., to a learning difficulty that develops on a secondary level. While the original brain damage may be non progressive, its effect upon all areas of development may be constantly changing and can result in, a generalized dysfunction. A motor disordered child, after brain injury, is still actively attempting to solve problems arising from tasks in the environment. At the physical level, it is found that the loss of certain neural tissues does not limit the attempt of the remaining tissues to compensate for the loss.

Experimentation in support of this idea includes Taub’s study (1980) on the deafferentation of a limb in monkeys which started to use the deafferented limb again for functional activities once his intact limb was restrained. This illustrates that non ¬use of the limb does not occur because of the neural deficit but rather because of a learned compensation for the deficit (Tsang,1990).The above can account for the exhibition of various non-functional and stereotyped motor patterns in a child with a motor disorder. Therefore we can not regard dysfunction as a feature of such children, but the product of the interaction between the child and his environment (Hari and Tillemans, 1984). Dysfunction is a change in coordination, which can be viewed separately from any deficiency. Dysfunction is not static or localized and it affects the whole personality of the child with cerebral palsy.

The dysfunction of the child is not the maladaptive movement pattern itself, but the result of an interrupted learning process. The difficulties inherent in adapting to the requirements of an activity leave the child unmotivated to continue the problem-solving skill process. The child then learns to be dependent. One can see that the effect of a lack of motivation is not limited to the physical level, but extends to the psychosocial level, impeding the child’s development as a whole (Kwan 1990). In other words, as individuals, we face greater and greater challenges that require more and more skills. Motor disorder can have a devastating effect upon the ability to meet these challenges and to learn the necessary skills. Dysfunction is a certain organizational characteristic of an individual. It is not a well defined malfunction or symptom or condition. Its manifestation is that the individual wants or should do something but he is unable to do it, not because he is incapable of doing it but because he does not know how to do it (Hari, 1990). While the origin of Cerebral Palsy is medical, the consequences interrupt the general learning ability of the individual. It can be concluded that instead of thinking in therapy and adaptation, by applying an appropriate educational approach the individual may learn to overcome the consequences of the motor disorder.

Pop Culture References

In the film The Usual Suspects, Kevin Spacey plays Verbal Kint, a criminal who appears to have cerebral palsy.
William H. Macy's character in the TV movie "Door to Door" has cerebral palsy.
Although Tom Hanks's title character in "Forrest Gump" is never explicitly diagnosed, he exhibits symptoms consistent with cerebral palsy.
The film Inside I%27m Dancing focuses on a quadraplegic youth in Dublin who befriends someone who has cerebral palsy and acts as his translator.

People with cerebral palsy

Christy Brown, the famous writer and painter who wrote My Left Foot, which was later adapted into an Oscar winning movie of the same name starring Daniel Day Lewis
Bruce Perens, an open source developer
Eric S. Raymond, an open source evangelist
Kaine (Rapper), One half of The Ying Yang Twins
Christopher Widdows aka Steady Eddy, Australian Comedian
Josh Blue, a comedian in the fourth season of Last Comic Standing who often jokes about his condition
Gianna Jessen, singer-songwriter and pro-life activist
Stephen Hopkins, signer of Declaration of Independence (Reputed to have stated, "My hand trembles, but my heart does not.")

Notes

References

"Conditions in Occupational Therapy: effect on occupational performance." ed. Ruth A. Hansen and Ben Atchison (Baltimore: Lippincott Williams & Williams, 2000), 8-21. ISBN 0-683-30417-8
"Cerebral Palsy." (National Center on Birth Defects and Developmental Disabilities, October 3, 2002), www.cdc.gov
"William and Spackman's Occupational Therapy 9th Edition." ed. Maureen E. Neistadt and Elizabeth Blesedell Crepeau (Lippincott-Raven Publishers, 1998), 233, 589-598. ISBN 0-397-55192-4
Bax, M., Goldstein, M., Rosenbaum, P., Leviton, A., Paneth, N., Dan, B., Jacobsson, B., & Damiano, D. (Executive Committee for the Definition of Cerebral Palsy), 2005. Developmental Medicine and Child Neurology, 47(8), 571-6

External links

Disability | Neurological disorders | Congenital disorders

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