Cerebral palsy


Cerebral palsy
Cerebral palsy
Classification and external resources

A child with cerebral palsy being examined.
ICD-10 G80
ICD-9 343
OMIM 603513 605388
DiseasesDB 2232
eMedicine neuro/533 pmr/24
MeSH D002547

Cerebral palsy (CP) is an umbrella term encompassing a group of non-progressive,[1][2] non-contagious motor conditions that cause physical disability in human development, chiefly in the various areas of body movement.[3]

Cerebral refers to the cerebrum, which is the affected area of the brain (although the disorder most likely involves connections between the cortex and other parts of the brain such as the cerebellum), and palsy refers to disorder of movement. However, "paralytic disorders" are not cerebral palsy – the condition of quadriplegia, therefore, should not be confused with spastic quadriplegia, nor Tardive dyskinesia with dyskinetic cerebral palsy, nor diplegia with spastic diplegia, and so on.

Cerebral palsy's nature as an umbrella term means it is defined mostly via several different subtypes, especially spastic, and also mixtures of those subtypes.

Cerebral palsy is caused by damage to the motor control centers of the developing brain and can occur during pregnancy, during childbirth or after birth up to about age three.[4][5] Resulting limits in movement and posture cause activity limitation and are often accompanied by disturbances of sensation, depth perception and other sight-based perceptual problems, communication ability, impairments can also be found in cognition; and epilepsy is found in 1/3. CP, no matter what the type, is often accompanied by secondary musculoskeletal problems that arise as a result of the underlying etiology.[6]

Of the many types and subtypes of CP, none have a known cure. Usually, medical intervention is limited to the treatment and prevention of complications arising from CP's effects. A 2003 study put the economic cost for people with CP in the US at $921,000 per individual, including lost income.[7] In another study, the incidence in six countries surveyed was 2.12–2.45 per 1,000 live births,[8] indicating a slight rise in recent years. Improvements in neonatology, or the medical specialty which is involved with treatment of neonates, have helped reduce the number of babies who develop cerebral palsy, but the survival of babies with very low birth weights has increased, and these babies are more likely to have cerebral palsy.[9][10]

Contents

Classification

Cerebral palsy (CP) is divided into four major classifications to describe different movement impairments. These classifications also reflect the areas of the brain that are damaged. The four major classifications are: spastic, ataxic, athethoid/dyskinetic and mixed.

Spastic

Spastic cerebral palsy is by far the most common type of overall cerebral palsy, occurring in 90% of all cases according to SCPE.[citation needed]

People with this type of CP are hypertonic and have what is essentially a neuromuscular mobility impairment (rather than hypotonia or paralysis) stemming from an upper motor neuron lesion in the brain as well as the corticospinal tract or the motor cortex. This damage impairs the ability of some nerve receptors in the spine to properly receive gamma amino butyric acid, leading to hypertonia in the muscles signaled by those damaged nerves.

As compared to other types of CP, and especially as compared to hypotonic or paralytic mobility disabilities, spastic CP is typically more easily manageable by the person affected, and medical treatment can be pursued on a multitude of orthopedic and neurological fronts throughout life. Spastic CP is classified by topography dependent on the region of the body affected; these include:

  • Spastic hemiplegia is one side being affected. Generally, injury to muscle-nerves controlled by the brain's left side will cause a right body deficit, and vice versa. Typically, people that have spastic hemiplegia are the most ambulatory of all the forms, although they generally have dynamic equinus (a limping instability) on the affected side and are primarily prescribed ankle-foot orthoses to prevent said equinus.[11]
  • Spastic diplegia is the lower extremities affected, with little to no upper-body spasticity. The most common form of the spastic forms (70-80% of known cases), most people with spastic diplegia are fully ambulatory, but are "tight" and have a scissors gait. Flexed knees and hips to varying degrees, and moderate to severe adduction (stemming from tight adductor muscles and comparatively weak abductor muscles), are present. Gait analysis is often done in early life on a semi-regular basis, and assistive devices are often provided like walkers, crutches or canes; any ankle-foot orthotics provided usually go on both legs rather than just one. In addition, these individuals are often nearsighted. The intelligence of a person with spastic diplegia is unaffected by the condition. Over time, the effects of the spasticity sometimes produce hip problems and dislocations (see the main article and spasticity for more on spasticity effects). In three-quarters of spastic diplegics, also strabismus (crossed eyes) can be present as well.
  • Spastic monoplegia is one single limb being affected.
  • Spastic triplegia is three limbs being affected.
  • Spastic quadriplegia is all four limbs more or less equally affected. People with spastic quadriplegia are the least likely to be able to walk, or if they can, to desire to walk, because their muscles are too tight and it is too much of an effort to do so. Some children with spastic quadriplegia also have hemiparetic tremors, an uncontrollable shaking that affects the limbs on one side of the body and impairs normal movement.

In any form of spastic CP, clonus of the affected limb(s) may sometimes result, as well as muscle spasms resulting from the pain and/or stress of the tightness experienced. The spasticity can and usually does also lead to very early onset of muscle-stress symptoms like arthritis and tendinitis, especially in ambulatory individuals in their mid-20s and early-30s. Physical therapy and occupational therapy regimens of assisted stretching, strengthening, functional tasks, and/or targeted physical activity and exercise are usually the chief ways to keep spastic CP well-managed, although if the spasticity is too much for the person to handle, other remedies may be considered, such as various antispasmodic medications, botox, baclofen, or even a neurosurgery known as a selective dorsal rhizotomy (which eliminates the spasticity by eliminating the nerves causing it).

Ataxic

Ataxia type symptoms can be caused by damage to the cerebellum. The forms of ataxia are less common types of cerebral palsy, occurring in at most 10% of all cases.[citation needed] Some of these individuals have hypotonia and tremors. Motor skills such as writing, typing, or using scissors might be affected, as well as balance, especially while walking. It is common for individuals to have difficulty with visual and/or auditory processing.

Athetoid/Dyskinetic

Athetoid or dyskinetic cerebral palsy is mixed muscle tone – both hypertonia and hypotonia. People with Dyskinetic CP have trouble holding themselves in an upright, steady position for sitting or walking, and often show involuntary motions. For some people with dyskinetic 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, especially small ones requiring fine motor control (such as a toothbrush or pencil). About 10% of individuals with CP are classified as dyskinetic CP but some have mixed forms with spasticity and dyskinesia.[12] The damage occurs to the extrapyramidal motor system and/or pyramidal tract and to the basal ganglia. In newborn infants, high bilirubin levels in the blood, if left untreated, can lead to brain damage in in the basal ganglia (kernicterus), which can lead to dyskinetic cerebral palsy.

Signs and symptoms

All types of cerebral palsy are characterized by abnormal muscle tone (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 spasticities, 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 (which can contribute to a gait reminiscent of a marionette) 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 slight clumsiness at the mild end of the spectrum to impairments so severe that they render coordinated movement virtually impossible at the other end 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 or change as a child gets older. Some babies born with CP do not show obvious signs right away. Classically, CP becomes evident when the baby reaches the developmental stage at six and a half to 9 months and is starting to mobilise, where preferential use of limbs, asymmetry or gross motor developmental delay is seen.

Secondary conditions can include seizures, epilepsy, apraxia, dysarthria or other communication disorders, eating problems, sensory impairments, mental retardation, learning disabilities, urinary incontinence, fecal incontinence and/or behavioral disorders.

Speech and language disorders are common in people with cerebral palsy. The incidence of dysarthria is estimated to range from 31% to 88%. Speech problems are associated with poor respiratory control, laryngeal and velopharyngeal dysfunction as well as oral articulation disorders that are due to restricted movement in the oral-facial muscles. There are three major types of dysarthria in cerebral palsy: spastic, dyskinetic (athetosis) and ataxic. Speech impairments in spastic dysarthria involves four major abnormalities of voluntary movement: spasticity, weakness, limited range of motion and slowness of movement. Speech mechanism impairment in athetosis involves a disorder in the regulation of breathing patterns, laryngeal dysfunction (monopitch, low, weak and breathy voice quality). It is also associated with articulatory dysfunction (large range of jaw movements), inappropriate positioning of the tongue, instability of velar elevation. Athetoid dysarthria is caused by disruption of the internal sensorimotor feedback system for appropriate motor commands, which leads to the generation of faulty movements that are perceived by others as involuntary. Ataxic dysarthria is uncommon in cerebral palsy. The speech characteristics are: imprecise consonants, irregular articulatory breakdown, distorted vowels, excess and equal stress, prolonged phonemes, slow rate, monopitch, monoloudness and harsh voice.[13] Overall language delay is associated with problems of mental retardation, hearing impairment and learned helplessness.[3] Children with cerebral palsy are at risk of learned helplessness and becoming passive communicators, initiating little communication.[3] Early intervention with this clientele often targets situations in which children communicate with others, so that they learn that they can control people and objects in their environment through this communication, including making choices, decisions and mistakes.[3]

Skeleton

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) and become thinner during growth. When compared to these thin shafts (diaphyses), the centers (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 to different lengths, so the person may have one leg longer than the other.

Pain and sleep disorders

Pain is common, and may result from the inherent deficits associated with the condition, along with the numerous procedures children typically face.[14] There is also a high likelihood of suffering from chronic sleep disorders associated with both physical and environmental factors.[15] Pain is also associated with tight and/or shortened muscle, abnormal posture, stiff joints, unsuitable orthosis etc.

Causes

File:Fetal thrombotic vasculopathy – intermed mag.jpg
Micrograph showing a fetal (placental) vein thrombosis, in a case of fetal thrombotic vasculopathy (FTV). FTV is associated with cerebral palsy and is suggestive of a hypercoagulable state as an underlying cause. H&E stain.

While in certain cases there is no identifiable cause, typical causes include problems in intrauterine development (e.g. exposure to radiation, infection), asphyxia before birth, hypoxia of the brain, and birth trauma during labor and delivery, and complications in the perinatal period or during childhood.[3] CP is also more common in multiple births.

Between 40% and 50% of all children who develop cerebral palsy were born prematurely. Premature infants are vulnerable, in part because their organs are not fully developed, increasing the risk of hypoxic injury to the brain that may manifest as CP. A problem in interpreting this is the difficulty in differentiating between cerebral palsy caused by damage to the brain that results from inadequate oxygenation and CP that arises from prenatal brain damage that then precipitates premature delivery.

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 2.0 kg, 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 may present with the clinical features 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 people with these anomalies didn't have a normal brain). Often this goes along with rare chromosome disorders and CP is not genetic or hereditary.

It has been hypothetized that many cases of cerebral palsy are caused by the death in very early pregnancy of an identical twin.[17]

Diagnosis

The diagnosis of cerebral palsy has historically rested on the patient's history and physical examination. Once diagnosed with cerebral palsy, further diagnostic tests are optional. The American Academy of Neurology published an article in 2004 reviewing the literature and evidence available on CT and MRI imaging. They suggested that neuroimaging with CT or MRI is warranted when the etiology of a patient's cerebral palsy has not been established – an MRI is preferred over CT due to diagnostic yield and safety. When abnormal, the neuroimaging study can suggest the timing of the initial damage. The CT or MRI is also capable of revealing treatable conditions, such as hydrocephalus, porencephaly, arteriovenous malformation, subdural hematomas and hygromas, and a vermian tumor[18] (which a few studies suggest are present 5 to 22%). Furthermore, an abnormal neuroimaging study indicates a high likelihood of associated conditions, such as epilepsy and mental retardation.[19]

Unusually, cerebral palsy is notable for a glaring overall research deficiency – the fact that it is one of the very few major group of conditions on the planet in human beings for which medical science has not yet (as of 2011) collected wide-ranging empirical data on the development and experiences of young adults, the middle aged and older adults. An especially puzzling aspect of this lies in the fact that cerebral palsy as defined by modern science was first 'discovered' and specifically addressed well over 100 years ago and that it would therefore be reasonable to expect by now that at least some empirical data on the adult populations with these conditions would have long since been collected, especially over the second half of the 20th century when existing treatment technologies rapidly improved and new ones came into being. The vast majority of empirical data on the various forms of cerebral palsy is concerned near-exclusively with children (birth to about 10 years of age) and sometimes pre-teens and early teens (11-13). Some doctors attempt to provide their own personal justifications for keeping their CP specialities purely paediatric, but there is no objectively apparent set of reasons backed by any scientific consensus as to why medical science has made a point of researching adult cases of multiple sclerosis, muscular dystrophy and the various forms of cancer in young and older adults, but has failed to do so with CP. There are a few orthopaedic surgeons and neurosurgeons who claim to be gathering pace with various studies as of the past few years[citation needed], but these claims do not yet seem to have been matched by real-world actualisation in terms of easily-accessible and objectively-verifiable resources available to the general public on the internet and in-person, where many, including medical-science researchers and doctors themselves, would more than likely agree such resources would ideally belong.[original research?]

Treatment

Treatment for cerebral palsy is a lifelong multi-dimensional process focused on the maintenance of associated conditions. In order to be diagnosed with cerebral palsy the damage that occurred to the brain must be non-progressive and not disease like in nature. The manifestation of that damage will change as the brain and body develop, but the actual damage to the brain will not increase. Treatment in the life of cerebral palsy is the constant focus on preventing the damage in the brain from prohibiting healthy development on all levels. The brain, up to about the age of 8, is not concrete in its development. It has the ability to re-organize and re-route many signal paths that may have been affected by the initial trauma; the earlier it has help in doing this the more successful it will be. Various forms of therapy are available to people living with cerebral palsy as well as caregivers and parents caring for someone with this disability. They can all be useful at all stages of this disability and are vital in a person with cerebral palsy's ability 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. The earliest proven intervention occurs during the infant's recovery in the neonatal intensive care unit (NICU). 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. benzodiazepines, 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; 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. Constraint-induced movement therapy (CIMT) has shown promising evidence in helping individuals with neurological disorders that have lost most of the use of an extremity. Research has proven the positive benefits of CIMT for people who have had a stroke and traumatic brain injury. However, later studies have addressed the application of CIMT for children with CP challenged with hemiparesis, that show a significant benefit in constraint induced movement therapy for children with cerebral palsy who are challenged with hemiparesis.[20]

Interpersonal therapy

Physiotherapy 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 physiotherapy 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.[21][22]

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.[23]

Conductive education was developed in Hungary from 1945 based on the work of András Pető. 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 specialized centers.

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.

Patterning is a controversial form of alternative therapy for people with CP. The method is promoted by The Institutes for the Achievement of Human Potential (IAHP), a Philadelphia nonprofit, but has been criticized by the American Academy of Pediatrics.[24] The IAHP's methods have been endorsed by Linus Pauling,[25] as well as some parents of children treated with their methods.[26][27][28]

Massage therapy[29] is designed to help relax tense muscles, strengthen muscles, and keep joints flexible. More research is needed to determine the health benefits of these therapies for people with CP.

Occupational therapy

Occupational Therapy (OT) enables individuals with CP to participate in activities of daily living that are meaningful to them. A family-centred philosophy is used with children who have CP. Occupational therapists work closely with families in order to address their concerns and priorities for their child.[30] Occupational therapists may address issues relating to sensory, cognitive, or motor impairments resulting from CP that affect the child's participation in self-care, productivity, or leisure. Parent counselling is also an important aspect of occupational therapy treatment with regard to optimizing the parent's skills in caring for and playing with their child to support improvement of their child's abilities to do things.[31][32] The occupational therapist typically assesses the child to identify abilities and difficulties, and environmental conditions, such as physical and cultural influences, that affect participation in daily activities.[32] Occupational therapists may also recommend changes to the play space, changes to the structure of the room or building, and seating and positioning techniques to allow the child to play and learn effectively.[32][33]

Medication

Botulinum toxin A injections are given into muscles that are spastic or sometimes dystonic, the aim being to reduce the muscle hypertonus that can be painful. A reduction in muscle tone can also facilitate bracing and the use of orthotics.Most often lower extremity muscles are injected. Botulinumtoxin is focal treatment meaning that a limited amount of muscles can be injected at the same time. The effect of the toxin is reversible and a reinjection is needed every 4-6 month.[34]

Surgery and orthoses

Surgery 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.
  • The insertion of a baclofen pump usually during the stages while a patient is a young adult. This is usually placed in the left abdomen. It is a pump that is connected to the spinal cord, whereby it sends bits of Baclofen alleviating the continuous muscle flexion. Baclofen is a muscle relaxant and is often given PO "per orem" (Latin for "by mouth") to patients to help counter the effects of spasticity.
  • 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.[35]
  • 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.[36]

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[37] and increasing speed and stride length.[38]

Other treatments

Cooling high-risk full-term babies shortly after birth may reduce disability or death.[39]

Early nutritional support: In one cohort study of 490 premature infants discharged from the NICU, the rate of growth during hospital stay was related to neurological function at 18 and 22 months of age. The study found a significant decrease in the incidence of cerebral palsy in the group of premature infants with the highest growth velocity. This study suggests that adequate nutrition and growth play a protective role in the development of cerebral palsy.[40]

Hyperbaric oxygen therapy (HBOT), in which pressurized oxygen is inhaled inside a hyperbaric chamber, has been studied under the theory that improving oxygen availability to damaged brain cells can reactivate some of them to function normally. A 2007 systematic review concluded that treatment with HBOT showed no significant difference from that of pressurized room air, and that some children undergoing HBOT may experience adverse events such as seizures and the need for ear pressure equalization tubes; due to poor quality of data assessment the review also concluded that estimates of the prevalence of adverse events are uncertain.[41]

Prognosis

CP is not a progressive disorder (meaning the brain damage does not worsen), but the symptoms can become more severe over time due to subdural damage. 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 . 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 learning disabilities, although these may be unrelated to IQ, and are more likely to show varying degrees of intellectual disability. Intellectual level among people with CP varies from genius to intellectually impaired, as it does in the general population, and experts have stated that it is important to not underestimate a person with CP's capabilities and to give them every opportunity to learn.[42]

The ability to live independently with CP varies widely depending on the severity of each case. Some individuals with CP will require personal assistant services for all activities of daily living. Others can lead semi-independent lives, needing support only for certain activities. Still others can live in complete independence. The need for personal assistance often changes with increasing age and 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.[43] 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 decarboxylase-1 as one known enzyme involved.)[44] There is no evidence of an increased chance of a person with CP having a child with CP.

The common signs and symptoms associated with CP can have a significant impact on participation in occupations. Occupation is a term used in occupational therapy that refers to all activities a person does throughout their day. These activities may be grouped into the categories of self-care, productivity and leisure activities. Impairments related to CP can impact these activities. For example, children with motor impairments may also experience difficulties moving around their home and community, such as transportation, moving from room to room or transferring from wheelchair to toilet.

Self-care

Self-care is any activity children do to care for themselves. For many children with CP, parents are heavily involved in self-care activities. Self-care activities, such as bathing, dressing, grooming and eating, can be difficult for children with CP as self-care depends primarily on use of the upper limbs.[45] For those living with CP, impaired upper limb function affects almost 50% of children and is considered the main factor contributing to decreased activity and participation.[46] Since the hands are used for many self-care tasks, it is logical that sensory and motor impairments would impact daily self-care. The extent of the hand impairment depends on the location and degree of brain damage.[47] Sensory impairments can make getting dressed and brushing teeth difficult. Along with sensory impairments, motor impairments of the hand are thought to be responsible for difficulties experienced in daily, self-care activities.[48] However, motor impairments are more important than sensory impairments, with the most prevalent impairment being finger dexterity (ability to manipulate small objects).[47] Finger dexterity is essential in fastening buttons, doing up zippers and tying shoelaces. With upper limb spasticity, it may be difficult to get dressed in the morning. If the individual with CP also has cognitive deficits, this may add an additional challenge to dressing and grooming.

Children with CP often have oral sensory disturbances meaning that they have too little or too much sensitivity around and in the mouth.[49] An infant with CP may not be able to suck, swallow or chew and this can result in difficulty eating.[49] As mentioned in the above paragraph, finger dexterity is the most prevalent motor impairment.[47] Finger dexterity is essential for manipulating cutlery or bringing food to the mouth. Fine finger dexterity, like picking up a spoon, is more frequently impaired than gross manual dexterity, like spooning food onto a plate.[47] Grip strength impairments are less common.[47] Overall, children with CP may have difficulty chewing and swallowing food, holding utensils, and preparing food due to sensory and motor impairments.

Productivity

The effects of sensory, motor and cognitive impairments not only affect self-care occupations in children with CP, but also productivity occupations. Productivity can include, but is not limited to: school, work, household chores and contributing to the community.[50] Play is also included as a productive occupation as it is often the primary activity for children.[51]

Play is considered the main occupation for children.[51] If play becomes difficult due to a disability, like CP, this can cause problems for the child.[52] These difficulties can affect a child’s self-esteem.[52] In addition, the sensory and motor problems experienced by children with CP affect how the child interacts with their surroundings, including the environment and other people.[52] Not only do physical limitations affect a child’s ability to play, the limitations perceived by the child’s caregivers and playmates also impact the child’s play activities.[53] Typically, children with disabilities spend more time playing by themselves.[54] When a disability prevents a child from playing, there may be social, emotional and psychological problems[55] which can lead to increased dependence on others, less motivation and poor social skills.[56]

In school, students are asked to complete many tasks and activities, many of which involve handwriting. Many children with CP have the capacity to learn and write in the school environment.[57] However, students with CP may find it difficult to keep up with the handwriting demands of school and their writing may be difficult to read.[57] In addition, writing may take longer and require greater effort on the student’s part.[57] Factors linked to handwriting include: postural stability, sensory and perceptual abilities of the hand and writing tool pressure.[57]

Also, speech impairments may be seen in children with CP depending on the severity of brain damage.[58] Communication in a school setting is quite important because communicating with peers and teachers is very much a part of the “school experience” and enhances social interaction. Problems with language or motor dysfunction can lead to underestimating a student’s intelligence.[59] In summary, children with CP may experience difficulties in school, such as difficulty with handwriting, carrying out school activities, communicating verbally and interacting socially.

Leisure

Leisure occupations are any activities that are done for enjoyment. Enjoyable activities depend on the child’s personality and environment. Leisure activities can have several positive effects on physical health, mental health, life satisfaction and psychological growth for children with physical disabilities like CP.[60] Common benefits identified are stress reduction, development of coping skills, companionship, enjoyment, relaxation and a positive effect on life satisfaction.[61] In addition, for children with CP, leisure appears to enhance adjustment to living with a disability.[61]

Leisure can be divided into structured (formal) and unstructured (informal) activities.[62] Studies show that children with disabilities, like CP, participate mainly in informal activities that are carried out in the family environment and are organized by adults.[63] Typically, children with disabilities carry out leisure activities by themselves or with their parents rather than with friends. Therefore, children may experience limited diversity of activities and social engagements, as well as a more passive lifestyle than their peers.[63] Although leisure is important for children with CP, they may have difficulties carrying out leisure activities due to social and physical barriers.

Participation and barriers

Participation is considered involvement in life situations and everyday activities.[64] Participation includes the domains of self-care, productivity and leisure. In fact, communication, mobility, education, home life, leisure and social relationships require participation and are indicators of the extent to which a child functions in his or her environment.[64] Barriers can exist on three levels: micro, meso and macro.[65] Firstly, the barriers at the micro level involve the person.[65] Barriers at the micro level include the child’s physical limitations (motor, sensory and cognitive impairments) or their subjective feelings regarding their ability to participate.[66] For example, the child may not participate in group activities due to lack of confidence. Secondly, the barriers at the meso level include the family and community.[65] These may include negative attitudes of people towards disability or lack of support within the family or in the community.[67] One of the main reasons for this limited support appears to be the result of a lack of awareness and knowledge regarding the child’s ability to engage in activities despite his or her disability.[67] Thirdly, barriers at the macro level incorporate the systems and policies that are not in place or hinder children with CP. These may be environmental barriers to participation such as architectural barriers, lack of relevant assistive technology and transportation difficulties due to limited wheelchair or public transit that can accommodate the children with CP.[67] For example, a building without an elevator may prevent the child from accessing higher floor levels.

Epidemiology

In the industrialized world, the incidence of cerebral palsy is about 2 per 1000 live births.[68] 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.[69] 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.[70]

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 2.4 out of 1000 children[71]

The SCPE reported the following incidence of comorbidities in children with CP (over 4,500 children over age 4 whose CP was acquired during the prenatal or neonatal period were included):

  • Mental disadvantage (IQ < 50): 31%
  • Active seizures: 21%
  • Mental disadvantage (IQ < 50) and not walking: 20%
  • Blindness: 11%[69]

The SCPE noted that the incidence of comorbidities is difficult to measure accurately, particularly across centers. For example, the actual rate of an intellectual impairment 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.

History

CP, formerly 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.[72] 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.[73]

Society and culture

Economic impact

Access Economics has released a report on the economic impact of cerebral palsy in Australia. Launched by the Hon. Bill Shorten, MP, the report found that, in 2007, the financial cost of cerebral palsy (CP) in Australia was $1.47 billion or 0.14% of GDP. When the value of lost well-being (disability and premature death) was added, the cost rose a further $2.4 billion.[74]

In 2007, the financial cost of CP was $1.47 billion (0.14% of GDP). Of this:

  • 1.03 billion (69.9%) was productivity lost due to lower employment, absenteeism and premature death of Australians with CP;
  • 141 million (9.6%) was the DWL from transfers including welfare payments and taxation forgone;
  • 131 million (9.0%) was other indirect costs such as direct program services, aides and home modifications and the bringing-forward of funeral costs;
  • 129 million (8.8%) was the value of the informal care for people with CP; and
  • 40 million (2.8%) was direct health system expenditure.

Additionally, the value of the lost well-being (disability and premature death) was a further $2.4 billion.

In per capita terms, this amounts to a financial cost of $43,431 per person with CP per annum. Including the value of lost well-being, the cost is over $115,000 per person per annum.

Individuals with CP bear 37% of the financial costs, and their families and friends bear a further 6%. Federal government bears around one third (33%) of the financial costs (mainly through taxation revenues forgone and welfare payments). State governments bear under 1% of the costs, while employers bear 5% and the rest of society bears the remaining 19%. If the burden of disease (lost well-being) is included, individuals bear 76% of the costs.

Use of the term

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:[75]

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 a 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.[76] The term "spastics" was used by the charity as a term for people with CP. The words "spastic" and "spaz" have since been used extensively as a general insult to disabled people, which some see as extremely offensive. They are also frequently used to insult able-bodied people when they seem overly uncoordinated, anxious, or unskilled in sports. The charity changed its name to Scope in 1994.[76] In the United States the word spaz has the same usage as an insult, but is not generally associated with CP.[77]

Misconceptions

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 that prevents the individual from expressing what cognitive abilities they do possess.[78]

Media

Maverick documentary filmmaker Kazuo Hara criticizes the mores and customs of Japanese society in an unsentimental portrait of adults with cerebral palsy in his 1972 film Goodbye CP (Sayonara CP). Focusing on how the CP victims are generally ignored or disregarded in Japan, Hara challenges his society's taboos about physical handicaps. Using a deliberately harsh style, with grainy black-and-white photography and out-of-sync sound, Hara brings a stark realism to his subject.[79]

Notable cases

  • Josh Blue, winner of the fourth season of NBC's Last Comic Standing, whose act revolves around his CP.[80] Blue was also on the 2004 U.S. Paralympic soccer team.[81]
  • Emperor Claudius, the Roman Emperor who was also a stutterer and walked with a limp is believed to have had CP.[82]
  • Abbey Curran, American beauty queen who represented Iowa at Miss USA 2008 and was the first contestant with a disability to compete. She also made an appearance on The Ellen DeGeneres Show and CBS 'The Early Show'.[83][84][85]
  • Jhamak Ghimire, winner of Nepal's most prestigious award Madan Puraskar, was born with cerebral palsy[2][3] and writes with her left foot. She is columnist at the Kantipur newspaper.
  • Stephen Hopkins, signer of USA Declaration of Independence, reputed to have stated, "My hand trembles, but my heart does not."[86]
  • Arun Shourie's son Aditya about whom he has written a book Does He Know a Mother’s Heart[87]
  • Geri Jewell, who was the first person with a disability to have a regular role in prime-time series on The Facts of Life.[88] She has had roles on Sesame Street, 21 Jump Street, The Young and the Restless and Deadwood.[89]
  • Karen Killilea, the subject of the book Karen (1952) and its sequel, With Love from Karen (1963), by her mother Marie Killilea.[90]
  • Gregory Iron, a professional wrestler working in the mid-west.[91] Born with cerebral palsy but still able to perform.
  • Christy Brown, Irish author, painter, and poet. He is the author of the book My Left Foot (book).
  • Rick Hoyt was born with cerebral palsy and today is a member of Team Hoyt with his father, Dick.
  • RJ Mitte, American actor best known for his role as Walter White Jr. on the AMC television series, Breaking Bad.

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Look at other dictionaries:

  • cerebral palsy — cerebral palsied. Pathol. a form of paralysis believed to be caused by a prenatal brain defect or by brain injury during birth, most marked in certain motor areas and characterized by difficulty in control of the voluntary muscles. [1920 25] * *… …   Universalium

  • cerebral palsy — n a disability resulting from damage to the brain before, during, or shortly after birth and outwardly manifested by muscular incoordination and speech disturbances see ataxic cerebral palsy, athetoid cerebral palsy, spastic cerebral palsy… …   Medical dictionary

  • cerebral palsy — [ US .ˌ.. ˈ..] n [U] a disease caused by damage to the brain before or during birth which results in difficulties of movement and speech …   Dictionary of contemporary English

  • cerebral palsy — noun uncount a medical condition affecting someone s control over their movement and speech, usually caused by damage to the brain before or during birth …   Usage of the words and phrases in modern English

  • cerebral palsy — ► NOUN ▪ a condition marked by impaired muscle coordination and spastic paralysis, caused by damage to the brain before or at birth …   English terms dictionary

  • cerebral palsy — n. any of several nonprogressive motor disorders resulting from damage to the central nervous system, esp. before or during birth, and, usually, characterized by spastic paralysis …   English World dictionary

  • cerebral palsy — noun a loss or deficiency of motor control with involuntary spasms caused by permanent brain damage present at birth (Freq. 1) • Syn: ↑spastic paralysis • Derivationally related forms: ↑spastic (for: ↑spastic paralysis) …   Useful english dictionary

  • cerebral palsy — N UNCOUNT Cerebral palsy is a condition caused by damage to a baby s brain before or during its birth, which makes its limbs and muscles permanently weak …   English dictionary

  • cerebral palsy — a disorder of movement and/or posture as a result of nonprogressive but permanent damage to the developing brain. This damage may occur before, during, or immediately after delivery and has many causes, including an inadequate supply of oxygen to …   The new mediacal dictionary

  • cerebral palsy — Synonyms and related words: MS, amyotrophic lateral sclerosis, brain disease, cephalalgia, chorea, emotional disorder, epilepsy, falling sickness, glossopharyngeal neuralgia, headache, herpes zoster, ischialgia, migraine, multiple sclerosis,… …   Moby Thesaurus


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