Cerebral palsy

Cerebral palsy (CP) is an umbrella term encompassing a group of non-progressive neurological physical disabilities in the development of human movement and posture. CP arises from disturbances in the developing fetal or infant brain. The incidence in developed countries is approximately 2-2.5 per 1000 live births, and incidence has not declined over the last 60 years despite medical advances like electro-fetal monitoring.

Often, CP's resultant motor disorder(s) are accompanied by "disturbances of sensation, cognition, communication, perception, and/or behavior, and/or by a seizure disorder” (Rosenbaum et al, 2005).

Cerebral palsy is a non-progressive disorder, but secondary orthopaedic deformities are common, such as hip dislocation and scoliosis of the spine. There is no known cure for CP: medical intervention is limited to the treatment and prevention of complications possible from CP's consequences. Overall, cerebral palsy ranks among the most monetarily costly congenital conditions in the world to manage effectively.

Cerebral palsy is divided into four major classifications to describe the different movement impairments. These classifications reflect the area of brain damaged. 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/or significant head injury in very early childhood.

The four major classifications are:

• Spastic;
• Athetoid;
• Ataxic, and
• Mixed.

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

General Classification

• 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 features (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. 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).

Types of Spastic CP

Based on the group of muscles involved, the below are typically only used to further describe spastic CP.

1. 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.
2. 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 AFOs or with a walking aid such as crutches, a cane (regular to four-pronged) or a walker. In diplegia, arms are unaffected or there are only slight effects.
3. 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.

Occasionally, terms such as monoplegia, paraplegia, triplegia and pentaplegia may be used.

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, and soft tissue findings consist largely of decreased muscle mass, but taken on the whole, 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.

Genetics

Autosomal recessive inheritance pattern.

About 2% of all CP cases are inherited, with glutamate decarboxylase-1 being one of the possible enzymes involved.

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[1]. 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 Americans 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).

History

Cerebral palsy, then known as "Cerebral Paralysis", was first identified by English 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 1980s 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.

Motor difficulties are common in individuals with CP. 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 steep differences between individuals with CP.

Cause

rs 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, probably accounting for no more than 10 percent of all cases; rather, 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.

Bones

In order for bones to attain their normal shape and size, they require the stresses from normal musculature. Osseous findings will therefore mirror the specific muscular deficits in a given person with CP. 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, a person with CP may exhibit a variety of angular joint deformities. Because vertebral bodies need vertical gravitational loading forces to develop properly, spasticity and an abnormal gait can hinder proper and/or full bone and skeletal development.

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, but once bones and musculature become more established, orthaepedic surgery becomes required for fundamental improvement.

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. 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. Non-speaking people with cerebral palsy are often successful availing themselves of Augmentative and Alternative Communication systems such as Blissymbols.

Cultural Dimensions

Public perception

Those with Cerebral Palsy are sometimes stigmatized and shunned. This has lessened since the 1950s thanks to public education and United Cerebral Palsy and its affiliates. Prior to that time the great majority were often sent to asylums or confined to attics. They were perceived to be the products of incest and partial smotherings.

Often parents kept their children away from them in the mistaken belief that the condition was the product of disease or poor sanitary habits.

Thomas Galton believed that there was a correlation between physical disability and aptitude, and this attitude remained prevalent as concerned CP until the 1970s. At this time, cerebral palsy was an overdiagnosed disorder, and a common misunderstanding then and now is that CP causes mental retardation. In fact, only CP individuals with brain damage in the hippocampus or the frontal cerebral cortex suffer from mental retardation. While learning difficulties and CP may co-occur, it is common for individuals with CP to lead normal lives.

Use of the term "spastic"

The term "spastic" describes the attribute of spasticity in types of spastic CP. 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 Claimed as a Learning Disorder

One scholarly point of view makes the claim that the motor disorders of CP can be accurately seen as inherently leading to learning problems (Hari and Tillemans, 1984) wherein the condition, even though it is physical, leads to secondary learning difficulties stemming from the initial trauma. Not all scholars or educated members of the public agree with this analysis, but some of its conclusions do correlate to a lot of the everyday difficulties experienced by adults with certain types of CP.

In this view, while the original brain damage is 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). 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 such as Conductive education 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.
• Although Tom Hanks's title character in "Forrest Gump" is never explicitly diagnosed, he exhibits symptoms consistent with cerebral palsy.
• The film Inside I'm Dancing focuses on a quadriplegic youth in Dublin who befriends someone who has cerebral palsy and acts as his translator.
• The South Korean film Oasis follows the unconventional romance between two social outcasts, a marginalized ex-con and a young woman with cerebral palsy.

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
• Christopher Widdows aka Steady Eddy, Australian Comedian
• Josh Blue, comedian and winner of 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."

• Bill Porter, a salesman for Watkins Incorporated whose life story was told in the TV movie Door to Door.
• Karen Killilea, the subject of the book Karen (1952) and its sequel, With Love from Karen (1963), by her mother Marie Killilea. [2]
• Geri Jewell, actress and comedienne
• Paul Henshall, British TV actor

External links

• CDC's "Learn the Signs. Act Early." campaign - Information for parents on early childhood development and developmental disabilities such as cerebral palsy
• Faults-and-all book marks Scope's 50th anniversary
• Cerebral Palsy Plus, Bristol
• United Cerebral Palsy, including information on physical therapy and exercise
• Cerebral Palsy Association in Alberta
• Cerebral Palsy League of Queensland
• The Spastic Centre
• CP Australia
• Disorders that causes cerebral palsy
• Patient Site giving information on Intrathecal Baclofen Treatment for Spasticity as a result of Cerebral Palsy
• Dame Vera Lynn Trust for Children with Cerebral Palsy
• Infant Cerebral Palsy Resources
• Cerebral Palsy Preventable Causes
• Center for Cerebral Palsy at Gillette Children's Specialty Healthcare
• Help guide to Cerebral and Facial Palsy
• Brianna Research Fund
• Cerebral Palsy Information
• Cerebral Palsy in the Middle East

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