Cerebral palsy

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

Cerebral palsy (CP) (IPA pronunciation: [ˈsɛ.ɹə.bɹəl 'pɔl.ziː]) 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 2.12–2.45 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.

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. 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).

General classification

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.

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 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 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, 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.

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.

Each year, around 1,500 preschoolers are diagnosed with the disorder in the USA. There is mental retardation in an estimated 60% of cases, due to brain damage outside the parietal, occipital, temporal or basal ganglia. The actual rate may be lower than 60%, as the physical and communicational limitations of people with CP lowers their IQ scores if not given a correctly modified test. 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 with 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. 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).

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, hearing or vision 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 or premature birth.

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. 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." [1]
• 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. [2]
• 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

References

• Hansen, Ruth A.; Atchison, Ben (2000). Conditions in occupational therapy: effect on occupational performance. Hagerstwon, MD: Lippincott Williams & Wilkins. ISBN 0-683-30417-8.{{cite book}}: CS1 maint: multiple names: authors list (link)

• "Cerebral Palsy." (National Center on Birth Defects and Developmental Disabilities, October 3, 2002), www.cdc.gov

• Crepeau, Elizabeth Blesedell; Willard, Helen S.; Spackman, Clare S.; Neistadt, Maureen E. (1998). Willard and Spackman's occupational therapy. Philadelphia: Lippincott-Raven Publishers. ISBN 0-397-55192-4.{{cite book}}: CS1 maint: multiple names: authors list (link)

• Bax M, Goldstein M, Rosenbaum P; et al. (2005). "Proposed definition and classification of cerebral palsy, April 2005". Developmental medicine and child neurology. 47 (8): 571–6. PMID 16108461. {{cite journal}}: Explicit use of et al. in: |author= (help)CS1 maint: multiple names: authors list (link)

See also

• Boccia
• Chorioamnionitis
• Rhizotomy

External links

Associations

International

• Cerebral Palsy International Sports & Recreation Association
• International Boccia Committee

Australia

• CP Australia CP Australia is a peak organisation of the major service providers for people with cerebral palsy in Australia
• The Cerebral Palsy Association - Western Australia
• The Spastic Centre - Sydney, Australia

Canada

• Canadian Cerebral Palsy Sports Association
• Cererbal Palsy Association in Alberta
• Cerebral Palsy Association of BC
• SportAbility (Cerebral Palsy Sports Association of B.C.)
• The Cerebral Palsy Association of Manitoba
• Ontario Federation for Cerebral Palsy
• Ontario Cerebral Palsy Sports Association
• Saskatchewan Cerebral Palsy Association
• Association de Paralysie Cerebrale du Quebec
• Association québécoise de sports pour paralytiques cérébraux

United Kingdom

• Scope UK disability organization that focuses on people with cerebral palsy
• Special Kids in the Uk Website providing parent to parent support relating to chidren with all disabilities including Cerebral Palsy.

USA

• Cerebral Palsy Help in the US

Events

• CPISRA 2007 Boccia World Cup

Further information

• Cerebral Palsy
• Cerebral Palsy at the NINDS
• Cerebral Palsy
• United Cerebral Palsy
• About Cerebral Palsy
• Cerebral Palsy Care Providers