Cerebral palsy

Cerebral palsy (CP) is an umbrella term encompassing a group of non-progressive,^[1] non-contagious diseases that cause physical disability in human development.

Cerebral refers to the affected area of the brain, the cerebrum (however the centers have not been perfectly localised and the disease most likely involves connections between the cortex and other parts of the brain such as the cerebellum) and palsy refers to disorder of movement. CP is caused by damage to the motor control centers of the young developing brain and can occur during pregnancy (about 75 percent), during childbirth (about 5 percent) or after birth (about 15 percent) up to about age three.^{[citation needed]}

It is a non-progressive disorder, meaning the brain damage does not worsen, but secondary orthopedic deformities are common. There is no known cure for CP. Medical intervention is limited to the treatment and prevention of complications possible from CP's consequences.

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. CP's resultant motor disorder(s) are sometimes, though not always, accompanied by "disturbances of sensation, cognition, communication, perception, and/or behavior, and/or by a seizure disorder".^[2][3]

CP is the second-most expensive developmental disability to manage over the course of a person's lifetime (second to mental disabilities), with an average lifetime cost per person of USD$921,000 (in 2003 dollars).^[4] The incidence in the six countries surveyed is approximately an average of 2.12–2.45 per 1000 live births;^[5] there has been a slight increase in recent years. Although improvements in neonatal nursing help reduce the number of babies who develop cerebral palsy, they also mean that babies with very low birth weights survive, and these babies are more likely to have cerebral palsy.^[6][7]

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Classification

CP is divided into four major classifications to describe the different movement impairments. These classifications reflect the area of brain damaged. The four major classifications are:

• Spastic
• Athetoid/Dyskinetic
• Ataxic
• Mixed

In 30 percent of all cases of CP, the spastic form is found along with one of the other types. There are a number of other, less prevalent types of CP, but these are the most common.

A general classification is as follows:

Spastic

Spastic (ICD-10 G80.0-G80.1) cerebral palsy is by far the most common type, occurring in 70% to 80% of all cases. People with this type are hypertonic and have a neuromuscular condition stemming from damage to the corticospinal tract, motor cortex, or pyramidal tract that affects the nervous system's ability to receive gamma amino butyric acid in the area(s) affected by the spasticity. Spastic CP is further classified by topography dependent on the region of the body affected; these include:

• spastic hemiplegia (one side being affected). Generally, injury to the left side of the brain will cause a right sided deficit.
• spastic diplegia (whole body affected, but the lower extremities affected more than the upper extremities). Most people with spastic diplegia do eventually walk. The gait of a person with spastic diplegia is typically characterised by a crouched gait. Toe walking and flexed knees are common. Hip problems, dislocations, and side effects like strabismus (crossed eyes) are common. Strabismus affects three quarters of people with spastic diplegia. This is due to weakness of the muscles that control eye movement. In addition, these individuals are often nearsighted. In many cases the IQ of a person with spastic diplegia is normal.

• spastic quadriplegia (Whole body affected; all four limbs affected equally). Some children with quadriplegia also have hemiparetic tremors; an uncontrollable shaking that affects the limbs on one side of the body and impairs normal movement. A common problem for children with quadriplegia is fluid buildup. Diuretics and steroids are medications administered to decrease any buildup of fluid in the spine that is caused by leakage from dead cells. Hardened feces in a quadriplegia patient are important to monitor because it can cause high blood pressure. Autonomic dysreflexia can be caused by hardened feces, urinary infections, and other problems, resulting in the overreaction of the nervous system and can result in high blood pressure, heart attacks, and strokes. Blockage of tubes inserted into the body to drain or enter fluids also needs to be monitored to prevent autonomic dysreflexia in quadriplegia. The proper functioning of the digestive system needs to be monitored as well.

Occasionally, terms such as monoplegia, paraplegia, triplegia, and pentaplegia may also be used to refer to specific manifestations of the spasticity.

Ataxic

Ataxia (ICD-10 G80.4) type symptoms can be caused by damage to the cerebellum. Forms of ataxia are less common types of Cerebral Palsy, occurring in at most 10% of all cases. Some of these individuals have hypotonia and tremors. Motor skills like writing, typing, or using scissors might be difficult, as well as problems with balance, especially while walking. It is common for individuals to have difficulty with visual and/or auditory processing of objects.

Athetoid/dyskinetic

Athetoid or dyskinetic (ICD-10 G80.3) is mixed muscle tone - sometimes hypertonia and sometimes hypotonia. People with athetoid CP have trouble holding themselves in an upright, steady position for sitting or walking, and often show involuntary motions. For some people with athetoid CP, it takes a lot of work and concentration to get their hand to a certain spot (like scratching their nose or reaching for a cup). Because of their mixed tone and trouble keeping a position, they may not be able to hold onto objects (such as a toothbrush or pencil). About one-fourth of all people with CP have athetoid CP. The damage occurs to the extrapyramidal motor system and/or pyramidal tract and to the basal ganglia. It occurs in 40% of all cases.

Incidence and prevalence

In the industrialised world, the incidence of cerebral palsy is about 2 per 1000 live births.^[8] The incidence is higher in males than in females; the Surveillance of Cerebral Palsy in Europe (SCPE) reports a M:F ratio of 1.33:1.^[9] Variances in reported rates of incidence across different geographical areas in industrialised countries are thought to be caused primarily by discrepancies in the criteria used for inclusion and exclusion. When such discrepancies are taken into account in comparing two or more registers of patients with cerebral palsy (for example, the extent to which children with mild cerebral palsy are included), the incidence rates converge toward the average rate of 2:1000.

In the United States, approximately 10,000 infants and babies are diagnosed with CP each year, and 1200-1500 are diagnosed at preschool age.^[10]

Overall, advances in care of pregnant mothers and their babies has not resulted in a noticeable decrease in CP. This is generally attributed to medical advances in areas related to the care of premature babies (which results in a greater survival rate). Only the introduction of quality medical care to locations with less-than-adequate medical care has shown any decreases. The incidence of CP increases with premature or very low-weight babies regardless of the quality of care.^{[citation needed]}

Prevalence of cerebral palsy is best calculated around the school entry age of about six years, the prevalence in the U.S. is estimated to be 2.3 out of 1000 children^[11]

The SCPE reported the following incidence of comorbidities in children with CP (the data are from 1980-1990 and included over 4,500 children over age 4 whose CP was acquired during the prenatal or neonatal period):

• Mental retardation (IQ < 50): 31%
• Active seizures: 21%
• Mental retardation (IQ < 50) and not walking: 20%
• Blindness: 11%^[12]

The SCPE noted that the incidence of comorbidities is difficult to measure accurately, particularly across centers. For example, the actual rate of mental retardation may be difficult to determine, as the physical and communicational limitations of people with CP would likely lower their scores on an IQ test if they were not given a correctly modified version.

Apgar scores have sometimes been used as one factor to predict whether or not an individual will develop CP.^[13]

Signs and symptoms

All types of CP are characterised by abnormal muscle tone, posture (i.e. slouching over while sitting), reflexes, or motor development and coordination. There can be joint and bone deformities and contractures (permanently fixed, tight muscles and joints). The classical symptoms are spasticity, spasms, other involuntary movements (e.g. facial gestures), unsteady gait, problems with balance, and/or soft tissue findings consisting largely of decreased muscle mass. Scissor walking (where the knees come in and cross) and toe walking are common among people with CP who are able to walk, but taken on the whole, CP symptomatology is very diverse. The effects of cerebral palsy fall on a continuum of motor dysfunction which may range from virtually unnoticeable to"clumsy" and awkward movements on one end of the spectrum to such severe impairments that coordinated movements are almost impossible on the other end of the spectrum.

Babies born with severe CP often have an irregular posture; their bodies may be either very floppy or very stiff. Birth defects, such as spinal curvature, a small jawbone, or a small head sometimes occur along with CP. Symptoms may appear, change, or become more severe as a child gets older. Some babies born with CP do not show obvious signs right away.

Secondary conditions can include seizures, epilepsy, speech or communication disorders, eating problems, sensory impairments, mental retardation, learning disabilities, and/or behavioral disorders.

History

CP, 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.^[14] 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.^[15]

Causes

The causes of CP remain unclear,with debate over the years giving no obvious answers .

Some causes of CP are asphyxia, hypoxia of the brain, birth trauma, premature birth, and certain infections in the mother during and before birth such as strep infections, central nervous system infections, trauma, consecutive hematomas, placenta abruptio and multiple birth.

Between 40% and 50% of all children who develop cerebral palsy were born prematurely. Premature infants are at higher risk in part because their organs are not yet fully developed, increasing the risk of asphyxia and other injury to the brain, which in turn increases the incidence of CP. Periventricular leukomalacia is an important cause of CP. About 10% of cases with CP are caused by malformation of the CNS.

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.^[16] Low birthweight is a risk factor for CP--and premature infants usually have low birth weights, less than 2.0kg, but full-term infants can also have low birth weights. Multiple-birth infants are also more likely than single-birth infants to be born early or with a low birth weight.

After birth, other causes include toxins, severe jaundice, lead poisoning, physical brain injury, shaken baby syndrome, incidents involving hypoxia to the brain (such as near drowning), and encephalitis or meningitis. The three most common causes of asphyxia in the young child are: choking on foreign objects such as toys and pieces of food; poisoning; and near drowning.

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 and CP is not genetic or hereditary.

Presentation: 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. People with CP tend to be shorter in height than the average person because their bones are not allowed to grow to their full potential. Sometimes bones grow at different lengths, so the person may have one leg longer than the other.

Prognosis

CP is not a progressive disorder meaning the actual brain damage does not worsen, but the symptoms can become worse over time due to 'wear and tear'. 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, orthopedic surgery may be required for fundamental improvement. People who have CP tend to develop arthritis at a younger age than normal because of the pressure placed on joints by excessively toned and stiff muscles.

The full intellectual potential of a child born with CP will often not be known until the child starts school. People with CP are more likely to have some type of learning disability, but this is unrelated to a person's intellect or IQ level. Intellectual level among people with CP varies from genius to mentally retarded, as it does in the general population. The important thing is to not under estimate the child's capabilities and to give them every opportunity to learn.

The ability to live independently with CP also 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, some persons with CP have children and parent successfully.

According to OMIM, only 2% of cases of CP are inherited (with glutamate decarboxylate-1 as one known enzyme involved.)^[17] There is no evidence of an increased chance of a person with CP having a child with CP.

Treatment

There is no cure for CP, but various forms of therapy can help a person with the disorder to function and live more effectively. In general, the earlier treatment begins the better chance children have of overcoming developmental disabilities or learning new ways to accomplish the tasks that challenge them. Treatment may include one or more of the following: physical therapy; occupational therapy; speech therapy; drugs to control seizures, alleviate pain, or relax muscle spasms (e.g. benzodiazepienes, baclofen and intrathecal phenol/baclofen); hyperbaric oxygen; the use of Botox to relax contracting muscles; surgery to correct anatomical abnormalities or release tight muscles; braces and other orthotic devices; wheelchairs and rolling walkers; and communication aids such as computers with attached voice synthesizers. 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 CP are often successful availing themselves of augmentative and alternative communication systems such as Blissymbols.

Physical therapy (PT) programs are designed to encourage the patient to build a strength base for improved gait and volitional movement, together with stretching programs to limit contractures. Many experts believe that life-long physical therapy is crucial to maintain muscle tone, bone structure, and prevent dislocation of the joints.

Occupational therapy helps adults and children maximise their function, adapt to their limitations and live as independently as possible.^[18][19]

Orthotic devices such as ankle-foot orthoses (AFOs) are often prescribed to minimise gait irregularities. AFOs have been found to improve several measures of ambulation, including reducing energy expenditure^[20] and increasing speed and stride length.^[21]

Speech therapy helps control the muscles of the mouth and jaw, and helps improve communication. Just as CP can affect the way a person moves their arms and legs, it can also affect the way they move their mouth, face and head. This can make it hard for the person to breathe; talk clearly; and bite, chew and swallow food. Speech therapy often starts before a child begins school and continues throughout the school years.^[22]

Hyperbaric oxygen therapy Recent studies have demonstrated a dramatic improvement in CP symptomology when hyperbaric oxygen therapy is used as a treatment. Researchers in Brazil found a significant alleviation in symptomology and other characteristics in a study involving 218 cerebral palsy patients. Significant enhancements were documented showing improved vision, hearing and speech as well as a reduction of spasticity by 50%, which occurred in 94% of study patients.^[23]

Nutritional counseling may help when dietary needs are not met because of problems with eating certain foods.

Both massage therapy^[24] and hatha yoga^{[citation needed]} are designed to help relax tense muscles, strengthen muscles, and keep joints flexible. Hatha yoga breathing exercises are sometimes used to try to prevent lung infections. More research is needed to determine the health benefits of these therapies for people with CP.

Surgery for people with CP usually involves one or a combination of:

• Loosening tight muscles and releasing fixed joints, most often performed on the hips, knees, hamstrings, and ankles. In rare cases, this surgery may be used for people with stiffness of their elbows, wrists, hands, and fingers.

• Straightening abnormal twists of the leg bones, i.e. femur (termed femoral anteversion or antetorsion) and tibia (tibial torsion). This is a secondary complication caused by the spastic muscles generating abnormal forces on the bones, and often results in intoeing (pigeon-toed gait). The surgery is called derotation osteotomy, in which the bone is broken (cut) and then set in the correct alignment.^[25]

• Cutting nerves on the limbs most affected by movements and spasms. This procedure, called a rhizotomy, "rhizo" meaning root and "tomy" meaning "a cutting of" from the Greek suffix 'tomia' reduces spasms and allows more flexibility and control of the affected limbs and joints.^[26]

• Botulinum Toxin A (Botox) injections into muscles that are either spastic or have contractures, the aim being to relieve the disability and pain produced by the inappropriately contracting muscle.^[27]

Another way is that a new study has found that cooling the bodies and blood of high-risk full-term babies shortly after birth may significantly reduce disability or death.^[28]

Conductive education (CE) was developed in Hungary from 1945 based on the work of András Peto". It is a unified system of rehabilitation for people with neurological disorders including cerebral palsy, Parkinson's disease and multiple sclerosis, amongst other conditions. It is theorised to improve mobility, self-esteem, stamina and independence as well as daily living skills and social skills. The conductor is the professional who delivers CE in partnership with parents and children. Skills learned during CE should be applied to everyday life and can help to develop age-appropriate cognitive, social and emotional skills. It is available at specialised centres.

Biofeedback is an alternative therapy in which people with CP learn how to control their affected muscles. Some people learn ways to reduce muscle tension with this technique. Biofeedback does not help everyone with CP.

Cultural aspects

Use of terms when referring to people with CP

Many people would rather be referred to as a person with a disability instead of handicapped. "Cerebral Palsy: A Guide for Care" at the University of Delaware offers the following guidelines:^[29]

Impairment is the correct term to use to define a deviation from normal, such as not being able to make a muscle move or not being able to control an unwanted movement. Disability is the term used to define a restriction in the ability to perform a normal activity of daily living which someone of the same age is able to perform. For example, a three year old child who is not able to walk has a disability because normal three year old can walk independently. Handicap is the term used to describe a child or adult who, because of the disability, is unable to achieve the normal role in society commensurate with his age and socio-cultural milieu. As an example, a sixteen-year- old who is unable to prepare his own meal or care for his own toileting or hygiene needs is handicapped. On the other hand, a sixteen-year- old who can walk only with the assistance of crutches but who attends a regular school and is fully independent in activities of daily living is disabled but not handicapped. All disabled people are impaired, and all handicapped people are disabled, but a person can be impaired and not necessarily be disabled, and a person can be disabled without being handicapped.

The term "spastic" describes the attribute of spasticity in types of spastic CP. In 1952 a UK charity called The Spastics Society was formed.^[30] The term "spastics" was used by the charity as a term for people with CP. The word "spaz" 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.^[30] In the United States the word spaz has the same usage as an insult, but is not generally associated with CP.^[31]

Misconceptions

A common misconception about those born with Cerebral Palsy is that they are less intelligent than those born without it. Cerebral Palsy is defined as damage to the part of the brain that controls movement; areas of the brain which define a persons intelligence are not affected by CP.

Spastic Cerebral Palsy, the most common form of CP, causes the muscles to be tense, rigid and movements are slow and difficult. This can be misinterpreted as cognitive delay due to difficulty of communication. Individuals with cerebral palsy can have learning difficulties, but sometimes it is the sheer magnitude of problems caused by the underlying brain injury, which prevents the individual from expressing what cognitive abilities they do possess. [1]

Public perception

Those with CP are sometimes stigmatised and shunned. This has lessened since the 1950s thanks to public education and to United Cerebral Palsy in the U.S. and similar organisations in other countries. 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.^{[citation needed]} 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, CP 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 develop mental retardation. While learning difficulties and CP may co-occur, it is common for individuals with CP to lead normal lives.

Cultural references

• (1987) In the William Horwood novel Skallagrigg, the central character Esther Marquand has CP.

• (1991) In the book Gridlock (novel) the main character Geoffrey Peason has Spastic Cerebral Palsy.

• (1995) In the film The Usual Suspects, Kevin Spacey plays Verbal Kint, a criminal who appears to have CP.

• (2001) In the film Storytelling, a love interest of Selma Blair's character has CP.

• (2002) The South Korean film Oasis follows the unconventional romance between two social outcasts, a marginalised ex-con and a young woman with CP.

• (2002) An Alex Pardee character in "The Used" is Sandy, a prostitute with CP.

• (2004) The film Inside I'm Dancing focuses on a quadriplegic youth in Dublin who befriends someone with CP and acts as his translator.

• (2005) In the Ricky Gervais and Stephen Merchant sitcom "Extras", a series one episode featured main protagonist Andy Millman ridiculing a female extra with CP (by mistaking her as a drunk); after the character (played by Francesca Martinez) corrects him, he empathises with her.

Notable cases

• Christy Brown, Irish 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,^[32] an open source developer

• Eric S. Raymond, an open source evangelist
• Christopher Widdows aka Steady Eddy, Australian Comedian
• Francesca Martinez, English stand-up comedienne.
• 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 USA Declaration of Independence, reputed to have stated, "My hand trembles, but my heart does not."^[33]
• 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.^[34]
• Gabriela Brimmer, poet and disability rights activist
• Geri Jewell, actress and comedienne
• Paul Henshall, British TV actor
• Ruben Gallego, writer of White on Black: A Boy's Story, a memoir book on his early life in Russian institutes.
• David Ring, American Christian evangelist and motivational speaker
• Emperor Claudius, the Roman Emperor who was also a stutterer and walked with a limp is believed to have had CP.^[35]
• Susie Maroney, world-record holding marathon swimmer.
• Baruch Kimmerling, Israeli sociologist and political dissident
• Greg Walloch, American writer, actor and comedian
• Joey Deacon, British author and television personality
• Rick Hoyt, American athlete and member of Team Hoyt with his father, Dick Hoyt. The two have competed in a total of 942 events (including 6 Ironman triathalons and 25 Boston marathons), in addition to cycling across the country, a 3,375 mile journey that took them 45 days. When not competing, Rick also works as a teacher at Boston College.
• Lucy Coleman, of Signing Time! fame

See also

• Boccia
• Chorioamnionitis
• Rhizotomy
• United Cerebral Palsy

References