Cerebral palsy

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CEREBRAL PALSY
Classification & external resources
ICD-10 G80.
ICD-9 343
DiseasesDB 2232
eMedicine neuro/533  pmr/24
MeSH D002547

Cerebral palsy (CP) is an umbrella term encompassing a group of non-progressive, non-contagious neurological disorders that cause physical disability in human development, specifically the human movement and posture.

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 (such as electro-fetal monitoring). CP is caused by damage to 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, but secondary orthopedic deformities, such as hip dislocation and scoliosis of the spine, 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 in the world to manage effectively.

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
  • 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 minor types of CP, 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. 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” (Rosenbaum et al, 2005).

Contents

  • Spastic (ICD-10 G80.0-G80.1) is by far the most common type of CP, occurring in 70% of all cases. People with this type are hypertonic and have an essentially 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); spastic diplegia (the lower extremities more affected than the upper body); or spastic quadriplegia (All four limbs affected equally). Occasionally, terms such as monoplegia, paraplegia, triplegia, and pentaplegia may also be used to refer to specific manifestations of the spasticity.
  • Ataxia (ICD-10 G80.4) is damage to the 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 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 or dyskinetic (ICD-10 G80.3) is mixed muscle tone - sometimes hypertonia and sometimes hypotonia. Children with athetoid CP have trouble holding themselves in an upright, steady position for sitting or walking, and often show involuntary motions. For some children with athetoid CP, it takes a lot of work and concentration to get their hand to a certain spot (like to scratch their nose or reach for a cup). Because of their mixed tone and trouble keeping a position, they may not be able to hold onto things (like a toothbrush or fork 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.

All types of CP 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 seizures, spasms, and other involuntary movements, speech or communication disorders, hearing or vision impairments, cognitive disabilities, learning disabilities, and/or behavioral disorders.

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

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 CP 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 25% to 30% 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 estimates 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 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.

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 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).[citation needed]

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[citation needed]. 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.[citation needed]

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.

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

Since CP refers to a group of disorders, there is no known precise cause. Some major causes are asphyxia, hypoxia of the brain, birth trauma or premature birth. Between 40% and 50% of all children who develop cerebral palsy are born prematurely. In addition, 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. Genetic susceptibility, certain infections in the mother during and before birth such as strep infections, central nervous system infections, trauma, consecutive hematomas, and placenta abruptio. 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. However the cause of most individual cases of CP 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.[citation needed]

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 CP. Periventricular leukomalacia is an important cause of CP.

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.

The diagnosis of CP requires several things:

  • The presence of symptoms indicating brain damage or dysfunction
  • The presence of motor dysfunction
  • The absence of change in symptoms - CP is by definition a static pathology, which is almost always reflected by static symptomatology

Because of the final requirement, CP may take some time to diagnose; Sometimes it is unclear whether a child's condition is worsening or not. However, most children with CP are diagnosed by about 18 months of age. If a child is born with a severe form of CP, a health professional may be able to diagnose the condition within the first few weeks of life. However, parents and caregivers usually are the first to notice that a baby has developmental delays that may be early signs of CP.

Usually a health professional diagnoses CP based on a baby's medical history (including parents' observations of developmental delays), physical examination, and results of screening tests.

Additional tests, such as developmental questionnaires, computed tomography (CT) scan or magnetic resonance image (MRI) of the head, or an ultrasound of the brain may be done.

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.

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 first questions usually asked by parents after they are told their child has CP are "What will my child be like?" and "Will she/he walk?" Predicting what a young child with CP will be like or what he will or will not do is very difficult. It is generally assumed that if a child is not sitting up by himself by age four or walking by age eight, then he will never be an independent walker.

It is even more difficult to make early predictions of speaking ability or mental ability than it is to predict motor function. Predictions can start being made after the age of two, though the child’s full intellectual potential won’t really be known until the child starts school. People with CP are more likely to have some type of learning disability, but intellectual level varies widely from genius to mentally retarded. The important thing is to not under estimate the child’s capabilities and to give him 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, many persons with CP can have children and parent successfully.

There is no cure for CP, but various forms of therapy can help a person with the disorder to function 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.

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

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.
  • 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.

See also: United Cerebral Palsy

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.[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.

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


  • 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.
  • An episode of Extras (guest-starring Kate Winslet) dealt with the misattribution of mental problems to those with CP.
  • In the film Storytelling, a love interest of Selma Blair's character has CP.

  1. ^ Perens, Bruce (1996). My use of "brain-damage" as a metaphor. Mailing list archive of debian-user. Retrieved on March 16, 2006.

  • "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

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