Cerebral palsy

Cerebral palsy
Specialty	Neurology, pediatrics
Frequency	0.305—0.315% (United States of America)

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

The incidence in developed countries is approximately 2.12–2.45 per 1000 live births. Incidence has not declined over the last 60 years despite medical advances (such as electro-fetal monitoring) because these advances allow extremely low birth weight and premature babies to survive. Cerebral refers to the affected area of the brain, the cerebrum (however the centres 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. Eighty percent of causes are unknown; for the small number where cause is known this can include infection, malnutrition, and/or head trauma in very early childhood.

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. Overall, CP ranks among the most costly congenital conditions to manage effectively.

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”.

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 hemiplegia and injury to the right side a left hemiplegia. Childhood hemiplegia is a relatively common condition, affecting up to one child in 1,000.

• 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 characterized 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 suffer from hemiparetic tremors; an uncontrollable shaking that affects the limbs on one side of the body and impairs normal movement. A common problem with children suffering from 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) is damage to the cerebellum which results in problems with balance, especially while walking. It is the rarest type, 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 and it is common for these individuals to have difficulty with visual or auditory processing of objects and instability in balance and relation to gravity.

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 ~20% of all cases.

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 CP each year in the United States. One recent study found a CP prevalence of 2.4 out of 1000 children.

Each year, around 1,500 preschoolers are diagnosed with the disorder in the USA. One recent study found mental retardation in 31% of children diagnosed with CP. The actual rate may be difficult to determine, as the physical and communicational limitations of people with CP lowers their IQ scores if not given a correctly modified test.

Overall, advances in care of pregnant mothers and their babies has not resulted in a noticeable decrease in CP. 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.

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

Despite medical advances, the incidence and severity of CP 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).

Signs and symptoms

All types of CP are characterized 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 "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. 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.

Causes

Doctors aren't sure what causes CP. This matter has been debated over the years with no obvious answers or conclusions.

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, and placenta abruptio.

Between 40% and 50% of all children who develop cerebral palsy were born prematurely. Premature babies have a higher risk in part 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 CP. Periventricular leukomalacia is an important cause of CP.

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.

The risk of a baby having CP increases as the birth weight decreases. A baby who is born prematurely usually has a low birth weight, less than 5.5 lb, but full-term babies can also have low birth weights. Multiple-birth babies are more likely than single-birth babies to be born early or with a low birth weight.

After birth, the condition may be caused by 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 suffer from 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.) 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. Many children go on to enjoy near-normal adult lives if their disabilities are properly managed. 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 physical therapy, occupational therapy, speech therapy, drugs to control seizures, alleviate pain, relax muscle spasms (e.g. benzodiazepienes, baclofen and intrathecal phenol/baclofen), 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.

Occupational therapy helps adults and children maximise their function, adapt to their limitations and live as independently as possible. Often young children will wear an Ankle-foot orthosis to minimize gait.

Conductive education developed in Hungary from 1945 based on the work of Andras 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 improves mobility, self-esteem, stamina and independence as well as daily living skills and social skills. The conductor is the professional who delivers conductive education (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 various centres in the UK and elsewhere.

Speech therapy helps control the mouth muscles. This therapy can be of great benefit to children with speech or eating problems. 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 breath, talk clearly, bite, chew and swallow food. Speech therapy often starts before the child begins school and continues throughout the school years.

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

Biofeedback may be useful as part of physical therapy or on its own. During a biofeedback session, 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.

Both massage therapy and hatha yoga 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.

Physiotherapy programs designed to encourage the patient to build a strength base for improved gait and volitional movement, together with stretching programs to limit contractures. Often in conjunction with Occupational Therapy and Speech Therapy. Usually structured towards improving independence and activities of daily living. Many experts believe that life-long physiotherapy is crucial to maintain muscle tone, bone structure, and prevent dislocation of the joints.

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.

• 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.

• 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.

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.

Cultural aspects

Use of terms when referring to people with CP

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:

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. 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. In the United States the word spaz has the same usage as an insult, but is not generally associated with CP.

Misconceptions

As misconceptions about Cerebral Palsy are frequent, one should be aware of what the exact definition of Cerebral Palsy actually is: Cerebral Palsy is damage to the part of the brain that controls movement. CP by definition has absolutely nothing to do with intelligence. It is the case that many children with CP can also suffer damage to the 'thinking parts' of their brains- but this mental retardation should be separated from the Cerebral Palsy. One should assume that a child with CP is perfectly intelligent.

The most common type of CP is Spastic. Spastic Cerebral Palsy causes the muscles to be tense, rigid and movements are slow and difficult. One of the reasons people with severe CP especially are mistaken for being congnitively delayed is because their facial expressions and vocalisations are different and difficult to understand. You must not forget that CP can affect all muscles- not just those of the legs or arms, but also those of the face- even the eyes and mouth are affected. Therefore, people with CP are unable to vocalise normally because the CP has affected the muscles of their throat and mouth. They do not 'gargle' because they lack the intelligence to speak- but because they lack the ability to use the muscles required for speaking effectively. The same can be said for what can often seem like 'out of context facial expressions.' Many children with severe CP will display what can be described as a 'constant smile'- which looks like just that.- The muscles of the mouth are tense so that the mouth opens widely and appears to be a large 'smile'. People often mistake this as the child being 'constantly happy'- as many cognitively delayed children are because they are 'easy to please'- This is not the case.

Cultural dimensions

See also: United Cerebral Palsy

Public perception

Those with CP 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, 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

• In the film The Usual Suspects, Kevin Spacey plays Verbal Kint, a criminal who appears to have CP.
• The film Inside I'm Dancing focuses on a quadriplegic youth in Dublin who befriends someone who has CP and acts as his translator.
• The South Korean film Oasis (film) follows the unconventional romance between two social outcasts, a marginalized ex-con and a young woman with CP.
• In the film Storytelling, a love interest of Selma Blair's character has CP.
• 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) but then the character corrects her and he goes on to sympathise with her.
• In the William Horwood novel Skallagrigg, the central character Esther Marquand, has CP.

Notable cases

• Christy Brown, the famous 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, 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."
• 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.
• 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.
• 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 athelete 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.

See also

• Boccia
• Chorioamnionitis
• Rhizotomy

External links

• Cerebral palsy at Curlie