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Wednesday, May 4, 2011

Cerebral palsy

(States Twitter)-Cerebral palsy (CP) is an umbrella term encompassing a group of non-progressive, non-contagious motor conditions that cause physical disability in human development, chiefly in the various areas of body movement.
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. 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, and sometimes even cognition; sometimes a form of CP may be accompanied by epilepsy. CP, no matter what the type, is often accompanied by secondary musculoskeletal problems that arise as a result of the underlying etiology.
Of the many types and subtypes of CP, none of them has 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. In another study, the incidence in six countries surveyed was 2.12–2.45 per 1,000 live births, 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.


Spastic
Spastic cerebral palsy is by far the most common type of overall cerebral palsy, occurring in 70% to 80% of all cases. Moreover, spastic CP accompanies any of the other types of CP in 30% of all cases.
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.


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.


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. There is also a high likelihood of suffering from chronic sleep disorders associated with both physical and environmental factors. Pain is also associated with tight and/or shortened muscle, abnormal posture, stiff joints, unsuitable orthosis etc.


Causes


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. CP is also more common in multiple births.
It has been hypothetized that many cases of cerebral palsy are caused by the death in very early pregnancy of an identical twin. This may occur when twins have a joint circulation through sharing the same placenta. Not all identical twins share the same blood supply (monochorionic twins), but if they do, the suggestion is that perturbations in blood flow between them can cause the death of one and damage to the development of the surviving fetus. It is common knowledge amongst obstetricians and midwives that a small dead fetus (fetus papyraceus) may sometimes be found attached to a placenta following birth. In the past, this has not been considered important and knowledge of the so called ‘vanishing twin’ has been suppressed to avoid triggering feelings of loss, grief, or guilt in mothers and especially the surviving twin.[citation needed] The pathological consequences depend on the severity and the stage of development of the fetus when the imbalances in blood flow between the fetuses occur.
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.
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


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 (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.


Treatment
Treatment for Cerebral Palsy is a life long 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 to prohibit 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. benzodiazepienes, baclofen and intrathecal phenol/baclofen); hyperbaric oxygen; the use of Botox to relax contracting muscles; surgery to correct anatomical abnormalities or release tight muscles; braces and other orthotic devices; 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.


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.
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.
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.
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.
Notable cases
Josh Blue, winner of the fourth season of NBC's Last Comic Standing, whose act revolves around his CP. Blue was also on the 2004 U.S. Paralympic soccer team.
Emperor Claudius, the Roman Emperor who was also a stutterer and walked with a limp is believed to have had CP.
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'.
Nicolas Hamilton, younger brother of 2008 Formula 1 champion Lewis Hamilton and racing driver, competes in the Renault Clio Cup despite suffering from cerebral palsy.
Stephen Hopkins, signer of USA Declaration of Independence, reputed to have stated, "My hand trembles, but my heart does not.
Geri Jewell, who was the first person with a disability to have a regular role in prime-time series on The Facts of Life. She has had roles on Sesame Street, 21 Jump Street, The Young and the Restless and Deadwood.
Karen Killilea, the subject of the book Karen (1952) and its sequel, With Love from Karen (1963), by her mother Marie Killilea.
RJ Mitte, an actor who portrays Walter White Jr. on the television drama Breaking Bad

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