Cerebral Palsy
3 examples of how pharmacological agents can be useful for children with CP
1.They can be useful for controlling/preventing seizures 2.They can be useful for aiding in digestive problems 3.They can be useful for aiding in breathing difficulties
other disorders associated with CP: Cognitive Impairment
30-50% with CP have it and it is mild in 1/3 of instances. The most significant impairments most often occur with mixed types and severe spastic quadriplegia. Athetoid-type cerebral palsy has the least occurrence. Many children with spastic hemiplegia and diplegia have average intelligence.
The most common medications used to treat Cerebral Palsy include medications within these categories:
Anticholinergics (uncontrolled body movements) Anticonvulsants (seizure medications) Antidepressants (depression medications) Antispastic (muscle relaxers) Anti-inflammatories (pain management) Stool softners
Define Cerebral Palsy (CP)
CP is not one specific condition but a grouping of clinical syndromes that affect movement, muscle tone, and coordination as a result of an injury or lesion of the pediatric brain
Contributing risk factors/causes in Perinatal period/infancy
Endocrine: hypoglycemia, hypothyroidism Hypoxia: respiratory distress Infection meningitis encephalitis Stroke Trauma Abuse
Contributing risk factors/causes in 1st trimester
Endocrine: thyroid malfunction or progesterone insufficiency Malnutrition, vitamin deficiencies, amino acid intolerance Alcohol, drugs, smoking
descriptive statistics on the prevalence of CP in the US.
Estimates in the U.S. range from 1.5 to 4 per 1,000 live births. The United Cerebral Palsy Association estimated in 2001 that 764,000 children and adults in the U.S. show 1+ symptoms of CP. They estimated that each year, 8,000 infants and 1,200 to 1,500 preschool-age children are diagnosed with CP. There is a 400% increase in the probability of CP in twin births than in a single birth (lower birth weight).
Contributing risk factors/causes in 2nd trimester
Infection: Rubella, Toxoplasma, HIV, Syphilis, Chickenpox Placental pathology: fetal malnutrition, chronic hypoxia, vascular occlusion
common medical interventions to manage spasticity including drug therapy and surgical intervention, as outlined by Yamamoto (2012).
Medical management includes both surgical and nonsurgical approaches, with much of the focus on techniques to decrease spasticity. Oral medications such as diazepam (Valium), dantrolene (Dantrium), and baclofen have been used to reduce spasticity in severe cases w/ mixed results. Intrathecal caclofen infusion (ITB) administered through a pump implanted in the abdominal wall to the spinal cord fluid has shown to be more effective than oral medications in reducing severe spasticity and dystonia but has potential for serious side effects and the long-term consequences. Injection of botulinum toxin (Botox) into muscles can reduce spasticity for a period of 3 to 6 months after injection and can increase ROM & reduce deformities with minimal side effects & unknown long term effectiveness on function. Orthotics and splinting are used to improve function and prevent contractures and deformities.
CP Characteristics (child must have all to be considered to have CP)
Onset of symptoms must occur during prenatal, perinatal, or postnatal periods (Age 2-3 = upper age limit of onset) Nonprogressive: one initial brain injury has occurred, there is no further impact/damage to central nervous system Involved sensorimotor development disorder manifested by abnormal muscle tone and patterns of movement Sensorimotor disorder originates in the brain - muscles/nerves/spinal cord are normal Lifelong disability
Generally describe the etiology of CP with attention to the evolution in CP research.
Originally - asphyxia was thought to be the cause of CP, more recent research from US shows only 5-10% of CP cases are a result of birth complications 70-80% of CP cases are cased for prenatal brain injury 10% of CP cases acquired after birth were due to CVA, meningitis, poisoning, abuse/poisoning
surgical management of CP, including a description of common procedures and surgical goals
Orthopedic surgery is often recommended when spasticity and stiffness are severe enough to make walking and moving about difficult or painful. For many people with CP, improving the appearance of how they walk - their gait - is also important. Surgeons can lengthen muscles and tendons that are proportionately too short, which can improve mobility and lessen pain. Tendon surgery may help the symptoms for some children with CP but could also have negative long-term consequences. Orthopedic surgeries may be staggered at times appropriate to a child's age and level of motor development. Surgery can also correct or greatly improve spinal deformities in people with CP. Surgery may not be indicated for all gait abnormalities and the surgeon may request a quantitative gait analysis before surgery. Surgery to cut nerves. Selective dorsal rhizotomy (SDR) is a surgical procedure recommended for cases of severe spasticity when all of the more conservative treatments - physical therapy, oral medications, and intrathecal baclofen -- have failed to reduce spasticity or chronic pain. A surgeon locates and selectively severs overactivated nerves at the base of the spinal column. SDR is most commonly used to relax muscles and decrease chronic pain in one or both of the lower or upper limbs. It is also sometimes used to correct an overactive bladder. Potential side effects include sensory loss, numbness, or uncomfortable sensations in limb areas once supplied by the severed nerve.
factors that provide differential diagnosis for CP.
Physical evidence includes a history of delayed achievement of motor milestones; however, delayed motor development can occur with a host of other developmental disabilities and genetic syndromes The quality of movement is the factor that helps provide a differential diagnosis. However, other causes must be ruled out, such as progressive neurological disorders, mucopolysaccharidosis, muscular dystrophy, or a spinal cord tumor. Many of these disorders can be ruled out by laboratory tests, although some must be differentiated by clinical or pathological criteria.
Contributing risk factors/causes in Intrapartum Events
Premature placental separation uterine rupture maternal hypotension prolapsed umbilical cord
Contributing risk factors/causes in 3rd trimester
Prematurity & low birth weight Jaundice, Rh incompatibility Cytokines: neurological tissue destruction inflammation hypoxia infection: Listeria, meningitis, strep B
other disorders associated with CP: Visual and Hearing Impairments
Strabismus is the most common visual defect, occurring in 20% to 60% of children with CP, with the highest rates in spastic diplegia and quadriplegia. Other visual and ocular abnormalities include nystagmus, homonymous hemianopsia associated with spastic hemiplegia, and difficulties with visual fixation and tracking. Hearing impairments include sensorineural hearing loss, due to congenital nervous system infections. Conductive hearing losses, caused by persistent fluid in the ears and middle ear infections, occur when there is severe motor involvement in children who spend a lot of time lying down.
Use of an interdisciplinary approach for people with CP.
Team member usually include OT, PT, speech, educational psychologist, nurse and social worker--interventions focus on helping the child gain motor control, minimize abnormal muscle tone, techniques to accomplish ADLs, using adaptive equipment to perform desired activities, provide methods for feeding and speech, help reduce parent stress. primary physician treats the primary disorder and helps prevention of health problems physicians with medical specialties may also be involved; neurologist to assist assess neurological status and help control seizures; orthopaedist to prescribe orthotic devices and any necessary surgeries; opthamologist to assist in visual impairments
strategies that can be used to prevent contractures and pressure ulcer development
The best way to increase and maintain joint elasticity and prevent joint contracture is through joint mobilization and stretching. This can best be done with the guidance of a qualified physical or occupational therapist. preventing pressure ulcers: Position changes - change every 15 minutes for wheelchair users and at least once every 2 hours for people in bed; Skin inspections - daily; Diet - maintain good nutrition; Smoking - quit; exercise helps circulation, builds muscle, improves overall health and stimulates a healthy appetite; adhere to healthy sleep routines. Measures should be taken to avoid accidents, irritation and self-inflicted injuries. Prevention measures include: Moisturizing skin - Skin must be washed, moisturized, and kept dry.Maintaining orthotics - Orthotics and adaptive equipment should be properly washed, dried, positioned, and checked for proper fit. Changing incontinence products timely - Diapers, under pads, or incontinence products must be changed often and checked for proper sizing. Freshening bedding - Bedding should be changed regularly. Bed sheets should be periodically checked for bed bugs or dust mites, hair checked for lice, and skin checked, particularly underarms, buttocks, groin, arms and legs for skin irritation. sizing clothing properly - Clothing should not be too tight or too loose. Shoes should be checked for proper sizing. Keep open wounds clean - Scars, bed sores, pressure sores, surgical incisions, and wounds should be properly cared for.
Course of CP and features that appear to have prognostic importance
The course of cerebral palsy varies depending on type, severity, and the presence of associated problems. With mild motor involvement, the child will continue to make motor gains and compensate for motor difficulties. With more severe forms, little progress may be made in attaining developmental milestones and performing functional tasks. As the child grows older, secondary problems such as contractures and deformities will become more common, especially with spasticity. Adults with cerebral palsy experience musculoskeletal difficulties and loss of function at an earlier age than their nondisabled peers. One study found that 75% of individuals with cerebral palsy had stopped walking by age 25 due to fatigue and walking insufficiency (Murphy, Molnar, & Lankasky, 1995). Another study of young adults found clinical evidence of arthritis in 27% of the subjects with cerebral palsy as compared to 4% in the general population (Cathels & Reddihough, 1993). The survival rate for adults with cerebral palsy is good but lower than the general population. A study in Great Britain found there was an 86% survival rate at age 50 for adults with cerebral palsy compared to 96% for the general population. After age 50, the relative risk of death was only slightly higher in women as compared to the general population. The risk for men with cerebral palsy was the same as the general population. Adults with cerebral palsy were more likely to die of respiratory disease than the general population but less likely to die from an accident or injury (Hemming, Hutton, & Pharoah, 2006).
goals of medical management for individuals with CP, as outlined by Yamamoto (2012).
The emphasis of intervention is usually on helping the child gain as much motor control as possible; positioning the child to minimize the effects of abnormal muscle tone; instructing the parents and caregivers on handling techniques and ways to accomplish various activities of daily living (ADLs); recommending adaptive equipment and assistive technology to increase the child's ability to perform desired activities; providing methods to improve feeding and speech if difficulties are present; and helping parents manage behavioral concerns and family stresses.
State the typical age range for onset of CP
The injury/insult occurs when the brain is still developing. It can occur anytime during the prenatal, perinatal, or postnatal periods. Cerebral palsy often is not evident during the first few months of life and is rarely diagnosed that early. Most cases, however, are detected by 12 months and nearly all can be diagnosed by 18 months.
signs and symptoms of CP: delayed motor development
The presence of primitive reflexes and tone abnormalities causes the child to have atypical positions at rest and to demonstrate stereotypical and uncontrollable postural changes during movement. a child with hypertonicity in the lower extremities often lies supine with the hips internally rotated and adducted and the ankles plantar flexed. This posture is caused by a combination of hypertonicity in the affected muscles and the presence of the crossed extension reflex. A child with hypotonicity typically lies with the hips abducted, flexed, and externally rotated because of low muscle tone, weakness in the affected muscles, and the influence of gravity.
signs and symptoms of CP: postural abnormalities (Atypical posture)
The presence of primitive reflexes and tone abnormalities causes the child to have atypical positions at rest and to demonstrate stereotypical and uncontrollable postural changes during movement. a child with hypertonicity in the lower extremities often lies supine with the hips internally rotated and adducted and the ankles plantar flexed. This posture is caused by a combination of hypertonicity in the affected muscles and the presence of the crossed extension reflex. A child with hypotonicity typically lies with the hips abducted, flexed, and externally rotated because of low muscle tone, weakness in the affected muscles, and the influence of gravity.
Differentiate the movement problems presented by children with CP from those presented in other neurological conditions.
The quality of movement is the factor that helps provide a differential diagnosis. The findings of atypical or stereotypical movement patterns and the presence of infantile reflexes and abnormal muscle tone point toward a diagnosis of cerebral palsy. Other disorders can be ruled out by laboratory tests (MRI, CT scan)
signs and symptoms of CP: atypical motor performance
The way in which a child moves when performing skilled motor acts is also a affected. Depending on the type of cerebral palsy, the child may demonstrate a variety of motor abnormalities such as asymmetrical hand use, unusual crawling method or gait, uncoordinated reach, or difficulty sucking, chewing, and swallowing.
incidence of CP among different segments of the US population
There was a 30% higher rate among African American children as compared to non-Hispanic white children. There was a 70% increase in prevalence in middle- and low-income areas as compared with upper-class areas.
signs and symptoms of CP :reflex abnormalities
With hypertonicity, reflex abnormalities are hyperreflexia, clonus, overflow, enhanced stretch reflex, and other signs of upper motor neuron lesions are present. When hypotonia is present, there may be are flexia, or an absence of primitive reflexes. These reflexes should be present during the first several months of life and it is of concern when they are not.
other disorders associated with CP: Seizure Disorder
affects 25-60%. It's most common in spastic hemiplegia and quadriplegia and rare with spastic diplegia and atherosis. Children with cognitive impairment had higher frequency of seizure disorder.
common causes in the perinatal and early childhood periods
cerebrovascular accidents (CVAs), infections such as meningitis or encephalitis, poisoning, trauma such as near-drowning and strangulation, child abuse, and illnesses such as endocrine disorders. The cause remains unknown in 20% to 30% of cases with an early onset of symptoms postnatally.
Types of CP: Ataxia
characterized by a wide-based, staggering, unsteady gait. Children with ataxia often walk quickly to compensate for their lack of stability and control. Controlled movements are clumsy. Intention tremors may be present. The ability to perform refined movements such as handwriting is affected. Hypotonicity is often present
other disorders associated with CP: Gastrointestinal
difficulties occur frequently in CP. Gastroesophageal reflux can create much discomfort and can result in refusal to eat or difficulty transitioning to solid foods. It requires medical intervention such as medication or in more serious cases surgery. Constipation is common due to decreased mobility and exercise as well as inadequate intake of water or unusual diets due to difficulties with oral motor control.
other disorders associated with CP: Oral Motor:
difficulty with speaking and eating. Dysarthria, if it is severe, may affect functional communication resulting in the need for alternative forms of communication. Eating difficulties can result in increased risk of aspiration, limited amount of foods consumed, and difficulty with chewing and swallowing. Drooling in ~10% of cases. Dental problems occur frequently. Motor problems and oral sensitivity can make tooth brushing more difficult. The combination of enamel dysplasia, mouth breathing, and poor hygiene leads to increased tooth decay and periodontal diseases.
signs and symptoms of CP :muscle tone abnormalities
hypertonicity, hypotonicity, and fluctuating tone Most infants with cerebral palsy initially demonstrate hypotonia. Later, the infant may develop hypertonicity, fluctuating tone, or continue to demonstrate hypotonia, depending on the type of cerebral palsy.
Type of CP: Spastic Diplegia
involves both lower extremities, with mild incoordination, tremors, or less severe spasticity in the upper extremities. It is most often attributed to premature birth and low birth weight. Many of these problems result in contractures and deformities, including dorsal spine kyphosis, lumbar spine lordosis, hip subluxation or dislocation, flexor deformities of hips and knees, and equinovarus or equinovalgus deformity of the feet (Bobath). Approximately 80% to 90% of children with diplegia will walk independently, some requiring assistive devices such as crutches or a walker to do so. Walking will be slower and more labored with a crouched gait sometimes developing.
Types of CP Spastic Hemiplegia
involves one entire side of the body, including the head, neck, and trunk. Usually, the upper extremity is most affected. Parietal lobe damage occurs in about 50% of cases and results in impaired sensation, including astereognosis, loss or lack of kinesthesia, diminished two-point discrimination, decreased graphesthesia, and topagnosia
types of CP
neurophysiological and anatomical classification including diplegia, hemiplegia, quadriplegia, athetosis, chorea, spasticity, hypotonia, dyskinesia, ataxia. Spastic: characterized by hypertonicity, retained primitive reflexes in affected areas of the body, and slow, restricted movement. The impact on motor function can range from a mild impairment that does not interfere with functional skills, such as not having isolated finger movement, to a severe impairment, where there is an inability to reach and grasp. Contractures are common. Spastic hemiplegia, spastic diplegia, and spastic quadriplegia are the most common types..
Contributing risk factors/causes in Preconception
parental background Parent(s) age: over 35 years or under 20 years Environmental toxins genetic or metabolic disorders malnutrition radiation damage
typical precautions associated with CP.
precautions mostly relate to preventing falls/accidents
other disorders associated with CP: Pulmonary
scoliosis or other spinal deformities can impact respiration. The respiratory muscles themselves may be affected leading to poor respiration. Prone to frequent upper respiratory infections that can significantly impact health. When difficulty chewing and swallowing is associated with poor breathing and inadequate or decreased ability to cough, this can result in increased aspiration pneumonia.
Types of CP: Spastic Quadriplegia
the entire body is involved. The arms typically demonstrate spasticity in the flexor muscles, with spasticity in the extensor muscles in the lower extremities. Only a small percentage of children with quadriplegia are able to walk independently, and less than 10% ever walk in the community after adolescence. Oral musculature is usually affected, with resulting dysarthria, eating difficulties, and drooling. Individuals are susceptible to contractures and deformities, particularly hip dislocation and scoliosis, and must be closely monitored.
Types of CP: Athetosis
the most common type of dyskinesia or dystonia, characterized by slow, writhing involuntary movements of the face and extremities or the proximal parts of the limbs and trunk. Abrupt, jerky distal movements may also appear. The movements increase with emotional tension and are not present during sleep. Head and trunk control is often affected as is the oral musculature, resulting in drooling, dysarthria and eating difficulties. Whereas spasticity is characterized by hypertonicity in the affected muscle groups and restricted movement, is characterized by fluctuating tone and excessive movement. Contractures are rare, but hypermobility may be present because of fluctuating hypotonicity.
