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What is Cerebral Palsy? is a general term for a group of permanent, non-progressive movement disorders that cause physical disability, mainly in the areas of body movement. There may also be problems with sensation, depth perception, and communication ability. Difficulty with cognition and epilepsy are found in about one-third of cases. There are subtypes including a type characterized by spasticity, a type characterized by poor coordination, and types which feature both symptoms or neither. 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. About 2% of all cerebral palsy cases are believed to be due to a genetic cause. Cerebral palsy is not an infectious disease and is not contagious. Most cases are diagnosed at a young age rather than during adolescence or adulthood Improvements in the care of newborns has helped reduce the number of babies who develop cerebral palsy and increased the survival of those with very low birth weights. There is no cure, with efforts attempting to treat and prevent complications. It occurs in about 2.1 per 1,000 live births. Cerebral refers to the cerebrum, which is the affected area of the brain. The disorder may often involve connections between the cortex and other parts of the brain such as the cerebellum. The root word "palsy" means "paralysis". In medicine, this is often used in reference to the paralysis or weakness that often accompanies nerve damage, loss of sensation or muscle disorders involving uncontrollable movements such as trembling or shaking. Signs and symptoms Cerebral palsy is defined as "a group of permanent disorders of the development of movement and posture, causing activity limitation, that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain." While the central feature of CP is a disorder with movement, difficulties with thinking, learning, feeling, communication and behavior often occur along with cerebral palsy. Of those with CP, 28% have epilepsy, 58% have difficulties with communication, at least 42% have problems with their vision, and 23–56% have learning disabilities. Cerebral palsy is characterized by abnormal muscle tone, 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 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 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 61⁄2 to 9 months and is starting to mobilise, where preferential use of limbs, asymmetry, or gross motor developmental delay is seen. Resulting conditions can include seizures, epilepsy, apraxia, dysarthria or other communication disorders, eating problems, sensory impairments, intellectual disability, learning disabilities, urinary incontinence, fecal incontinence, and/or behavioural disorders. Language 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, and oralarticulation 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. Overall language delay is associated with problems of intellectual disability, hearing impairment, and learned helplessness. Children with cerebral palsy are at risk of learned helplessness and becoming passive communicators, initiating little communication. Early intervention with this clientele, and their parents, 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. 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 centres (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 Pain is common and may result from the inherent deficits associated with the condition, along with the numerous procedures children typically face. Pain is associated with tight or shortened muscles, abnormal posture, stiff joints, unsuitable orthosis, etc. There is also a high likelihood of chronic sleep disorders secondary to both physical and environmental factors. Eating Those with CP may have difficulty preparing food, holding utensils, or chewing and swallowing due to sensory and motor impairments. An infant with CP may not be able to suck, swallow or chew. Children with CP may have too little or too much sensitivity around and in the mouth. Fine finger dexterity, like that needed for picking up a utensil, is more frequently impaired than gross manual dexterity, like that needed for spooning food onto a plate.[non-primary source needed] Grip strength impairments are less common.[non-primary source needed] Causes Micrograph showing a fetal (placental) vein thrombosis, in a case of fetal thrombotic vasculopathy. This is associated with cerebral palsy and is suggestive of a hypercoagulable stateas the underlying cause. Cerebral palsy is due to damage occurring to the developing brain. This damage can occur during pregnancy, delivery, the first month of life, or less commonly in early childhood. Structural problems in the brain are seen in 80% of cases, most commonly within the white matter. More than three quarters of cases are believed to result from issues that occur during pregnancy. While in certain cases there is no identifiable cause, typical causes include problems in intrauterine development (e.g. exposure to radiation, infection), hypoxia of the brain, and birth trauma during labour and delivery, and complications around birth or during childhood. Prematurity Between 40% and 50% of all children who develop cerebral palsy were born prematurely. Most of these cases (75-90%) are believed to be due to issues that occur around the time of birth, often just after birth. Multiple-birth infants are also more likely than single-birth infants to have CP. They are also more likely to be born with a low birth weight. In those who are born with a weight between 1 kg and 1.5 kg CP occurs in 6%. Among those born before 28 weeks of gestation it occurs in 11%. Genetic factors are believed to play an important role in prematurity and cerebral palsy generally. While in those who are born between 34 and 37 weeks the risk is 0.4% (three times normal). Term infantsIn babies that are born at term risk factors include: problems with the placenta, birth defects, low birthweight, breathing meconium into the lungs, a delivery requiring either the use of instruments or an emergency Caesarean section,birth asphyxia, seizures just after birth, respiratory distress syndrome, low blood sugar, and infections in the baby. It is unclear how much of a role birth asphyxia plays as a cause. It is believed, however, that only a small number of cases are caused by lack of oxygen during birth. It is unclear if the size of the placenta plays a role. Genetics About 2% of all CP cases are inherited, with glutamate decarboxylase-1 being one of the possible enzymes involved. Most inherited cases are autosomal recessive, meaning both parents must be carriers for the disorder in order to have a child with the disease. Early childhood 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. Other Infections in the mother, even infections that are not easily detected, may triple the risk of the child developing the disorder. Infections of the fetal membranes known as chorioamnionitis increases the risk. Intrauterine and neonatal insults (many of which are infectious in nature) increase the risk. It has been hypothesised that many cases of cerebral palsy are caused by the death in very early pregnancy of an identical twin. Diagnosis The diagnosis of cerebral palsy has historically rested on the person's history and physical examination. A general movements assessment, which involves measuring movements that occur spontaneously among those less than four months of age, appears to be most accurate. It is a type of developmental delay. Once diagnosed with cerebral palsy, further diagnostic tests are optional. Neuroimaging with CT or MRI is warranted when the cause of a person'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 tumour (which a few studies suggest are present 5–22% of the time). Furthermore, an abnormal neuroimaging study indicates a high likelihood of associated conditions, such as epilepsy and intellectual disability. The age at which CP is diagnosed is important, but there is disagreement over what is the best age to make the diagnosis. The earlier CP is diagnosed correctly, the better the opportunities are to provide the child with physical and educational help, but there also might be a greater chance that CP will be confused with another problem, especially if the child is 18 months of age or younger. Infants may have temporary problems with muscle tone or control that can be confused with CP, which is permanent. A metabolism disorder or tumors in the nervous system may also appear to be CP, and metabolic disorders in particular can produce brain problems that look like CP on an MRI. Disorders that deteriorate the white matter in the brain, and certain kinds of problems that cause spasms and weakness in the legs, may also be mistaken for CP when they first appear early in life. However, these disorders get worse over time, and CP does not (although it may change in character); in infancy it may not be possible to tell the difference between them.Fragile X syndrome (a cause of autism) and general intellectual disability must also be ruled out. Cerebral palsy specialist John McLaughlin recommends waiting until the child is 36 months of age before making a diagnosis. Classification CP is classified by the types of motor impairment of the limbs or organs, and by restrictions to the activities an affected person may perform. There are three main CP classifications by motor impairment: spastic, ataxic, and athetoid/dyskinetic. Additionally there is a mixed type that shows a combination of features of the other types. These classifications also reflect the areas of the brain that are damaged. Spastic Main article: Spastic cerebral palsy Spastic cerebral palsy, or cerebral palsy where spasticity (muscle tightness) is the exclusive or almost-exclusive impairment present, is by far the most common type of overall cerebral palsy, occurring in upwards of 70% of all cases. People with this type of CP arehypertonic 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 receive gamma-Aminobutyric acid properly, 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. 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. Occupational therapy and physical 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 Main article: Ataxic cerebral palsy Ataxia-type symptoms can be caused by damage to the cerebellum. Ataxia is a less common type of cerebral palsy, occurring between 5% and 10% of all cases. 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. They usually have an awkward gait and as well with some dysarthria. AthetoidMain article: Athetoid cerebral palsy Athetoid cerebral palsy or dyskinetic cerebral palsy is mixed muscle tone – both hypertonia and hypotonia mixed with involuntary motions. 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. 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 the basal ganglia (kernicterus), which can lead to dyskinetic cerebral palsy. Mixed Main article: Mixed cerebral palsy Mixed cerebral palsy is symptoms of athetoid, ataxic and spastic CP appearing simultaneously, each to varying degrees, and both with and without certain symptoms of each. Mixed CP is the most difficult to treat as it is extremely heterogeneous and sometimes unpredictable in its symptoms and development over the lifespan. Prevention In those at risk of an early delivery magnesium sulphate appears to decrease the risk of cerebral palsy. It is unclear if it helps those who are born at term. Cooling high-risk full-term babies shortly after birth may reduce disability. Management Over time, the approach to CP management has shifted away from narrow attempts to fix individual physical problems–such as spasticity in a particular limb–to making such treatments part of a larger goal of maximizing the person's independence and community engagement.:886 However, the evidence base for the effectiveness of intervention programs reflecting this philososphy has not yet caught up: effective interventions for body structures and functions have a strong evidence base, but evidence is lacking for effective interventions targeted toward participation, environment, or personal factors. There is also no good evidence to show that an intervention that is effective at the body-specific level will result in an improvement at the activity level, or vice versa. Although such cross-over benefit might happen, not enough high-quality studies have been done to demonstrate it. Treatment of cerebral palsy is a lifelong process focused on the management of associated conditions. It tries to allow healthy development on all levels. The brain, up to about the age of 8, is not set and has the ability to reroute 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. The treatments with the best evidence are medications (anticonvulsants, botulinum toxin, bisphosphonates, diazepam), therapy (bimanual training, casting, constraint-induced movement therapy, context-focused therapy, fitness training, goal-directed training, hip surveillance, home programmes, occupational therapy after botulinum toxin, pressure care) and surgery (selective dorsal rhizotomy). Various forms of therapy are available to people living with cerebral palsy as well as caregivers and parents. Treatment may include one or more of the following: physical therapy; occupational therapy; speech therapy; water therapy; drugs to control seizures, alleviate pain, or relax muscle spasms (e.g. benzodiazepines,); 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 synthesisers. However, 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. Therapy See also: Occupational therapy in the management of cerebral palsy 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 lifelong physiotherapy is crucial to maintain muscle tone, bone structure, and prevent dislocation of the joints. 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. Conductive education (CE) 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 specialised centres. Biofeedback is a therapy in which people learn how to control their affected muscles. Biofeedback therapy has been found to significantly improve gait in children with cerebral palsy.[42 Massage therapy 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 helps adults and children maximise their function, adapt to their limitations and live as independently as possible. 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. CP commonly causes hemiplegia. Those with hemiplegia have limited use of the limbs on one side of the body, and have normal use of the limbs on the other side. Hemiplegics often adapt by ignoring the limited limbs, and performing nearly all activities with the unaffected limbs, which can lead to increased problems with muscle tone, motor control and range of motion. An emerging technique called constraint-induced movement therapy (CIMT) is designed to address this. In CIMT, the unaffected limbs are constrained, forcing the individual to learn to use the affected limbs. There is limited, preliminary evidence that CIMT is effective, but more study is needed before it can be recommended with confidence. MedicationBotulinum toxin 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. Botulinum toxin is focal treatment, meaning that a limited number of muscles can be injected at the same time. The effect of the toxin is reversible and a reinjection is needed every 4–6 months. Surgery 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. Selective Percutaneous Myofascial Lengthening (SPML) is one example. 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 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. 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. Other surgical procedures are available to try to help with other problems. Those who have serious difficulties with eating may undergo a procedure called a gastrostomy: a hole is cut through the belly skin and into the stomach to allow for a feeding tube. There is no good evidence about the effectiveness or safety of gastrostomy. Orthotics Orthotic devices such as ankle-foot orthoses (AFOs) are often prescribed to achieve the following objectives: correct and/or prevent deformity, provide a base of support, facilitate training in skills, and improve the efficiency of gait. The available evidence suggests that orthoses can have positive effects on all temporal and spatial parameters of gait, i.e. velocity, cadence, step length, stride length, single and double support. AFOs have also been found to reduce energy expenditure. Other Hyperbaric oxygen therapy (HBOT), in which pressurised 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. However, HBOT results in no significant difference from that of pressurised room air, and some children undergoing HBOT may experience adverse events such as seizures and the need for ear pressure equalisation tubes. 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 organisation, but has been criticised by the American Academy of Pediatrics. Prognosis CP is not a progressive disorder (meaning the brain damage does not worsen), but the symptoms can become more severe over time. A person with the disorder may improve somewhat during childhood if he or she receives extensive care, 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 the capabilities of a person with CP and to give them every opportunity to learn. The life expectancy of those with CP is less than that of the general population but has improved with the utilization of modern medicine. The ability to live independently with CP varies widely, depending partly on the severity of each person's impairment and partly on the capability of each person to self-manage the logistics of life. Some individuals with CP require personal assistant services for all activities of daily living. Others only need assistance with certain activities, and still others do not require any physical assistance. But regardless of the severity of a person's physical impairment, a person's ability to live independently often depends primarily on the person's capacity to manage the physical realities of his or her life autonomously. In some cases, people with CP recruit, hire, and manage a staff of personal care assistants (PCAs). PCAs facilitate the independence of their employers by assisting them with their daily personal needs in a way that allows them to maintain control over their lives. People with CP can usually 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 affect reproductive function, people with CP can have children and parent successfully. Self-careSelf-care is any activity people 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, can be difficult for children with CP as self-care depends primarily on use of the upper limbs. 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. As the hands are used for many self-care tasks, sensory and motor impairments of the hands make daily self-care more difficult.[non-primary source needed][non-primary source needed] Motor impairments cause more problems than sensory impairments.[non-primary source needed] The most common impairment is that of finger dexterity, which is the ability to manipulate small objects with the fingers.[non-primary source needed]
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