1. Cerebral Palsy Paralytic Disorders ARTHROGRYPOSIS MULTIPLEX CONGENITA
POLIOMYELITIS
MYELOMENINGOCELE
ARTHROGRYPOSIS MULTIPLEX CONGENITA
BRACHIAL PLEXUS PALSY

2. Cerebral Palsy

3. ETIOLOGY (1) Some degree of motor impairment is present
(2) an insult to the developing brain has occurred
(3) a neurological deficit is present that is nonprogressive

4. The insult to the brain is believed to occur between the time of conception and age 2 years, at which time a significant amount of motor development has already occurred.

5. neurological deficit is
Permanent
Nonprogressive .

6. amount and quality of prenatal care,
PREVALENCE:
amount and quality of prenatal care,
the socioeconomic condition of the parents,
the environment,
obstetrical and pediatric care the mother and child receive.
prevalence = 0.6 to 7cases per 1000 live births
Children with cerebral palsy constitute the largest group of pediatric patients with neuromuscular disorders in the United States. The prevalence of cerebral palsy varies around the world according to the amount and quality of prenatal care, the socioeconomic condition of the parents, the environment, and the type of obstetrical and pediatric care the mother and child receive. The determination of the true prevalence also is difficult because many children are not diagnosed until age 2 or 3 years; this most often occurs in socioeconomic groups that have decreased access to medical care. In the United States, the occurrence is approximately two per 1000 live births; there are approximately 25,000 new patients with cerebral palsy each year, and approximately 400,000 children with cerebral palsy at any given time. The United States experienced an initial decrease in the number of affected children in the 1950s and 1960s as a result of better understanding and treatment of maternal-fetal Rh incompatibility and improvements in obstetrical techniques. More recently, the prevalence of cerebral palsy was thought to be increasing because of the increased survival of premature and low-birth-weight infants; however, two large population-based studies by Winter et al. and O'Shea showed that the improved survival of these infants has not contributed to the increase in prevalence of cerebral palsy in the United States. Worldwide, the prevalence ranges from 0.6 to seven cases per 1000 live births

7. prenatal, perinatal, postnatal.
Contrary to popular belief, fewer than 10% of injuries that result in cerebral palsy occur during the birth process.
Injury to the developing brain can occur anytime from gestation to early childhood and typically is categorized as prenatal, perinatal, or postnatal. Contrary to popular belief, fewer than 10% of injuries that result in cerebral palsy occur during the birth process, with most occurring in the prenatal period..

8. prenatal fetus (most commonly genetic disorders),
mother (seizure disorders, mental retardation, and previous pregnancy loss),
pregnancy itself (Rh incompatibility, polyhydramnios, placental rupture, and drug or alcohol exposure).
External factors, such as TORCH (toxoplasmosis, other agents, rubella, cytomegalovirus, herpes simplex).
A wide variety of risk factors for cerebral palsy have been identified in the prenatal period, including risk factors inherent to the fetus (most commonly genetic disorders), factors inherent to the mother (seizure disorders, mental retardation, and previous pregnancy loss), and factors inherent to the pregnancy itself (Rh incompatibility, polyhydramnios, placental rupture, and drug or alcohol exposure). External factors, such as TORCH (toxoplasmosis, other agents, rubella, cytomegalovirus, herpes simplex), also can lead to cerebral palsy in the prenatal period. Occurences in the absence of any known risk factors may be due to some yet unknown factor during this critical time in brain development. Several more recent studies have suggested a possible role of chorioamnionitis as one of these factors

9. perinatal Pregnancies involving multiple births Oxytocin augmentation,
asphyxia or trauma that occurs during labor.
Oxytocin augmentation,
umbilical cord prolapse
breech presentation.
Low-birth-weight infants (<1500 g) incidence of 60 per 1000 births compared with two per 1000 births in normal-weight infants.
periventricular blood vessels.
Pregnancies involving multiple births 
Cerebral palsy in the perinatal period, from birth until a few days after birth, typically is associated with asphyxia or trauma that occurs during labor. Oxytocin augmentation, umbilical cord prolapse, and breech presentation all have been associated with an increased occurrence of cerebral palsy. Nelson reported that only 10% of cases of cerebral palsy occur during this time period, and most patients with cerebral palsy have no history of asphyxia. Although cerebral palsy often is associated with low Apgar scores during this period, many neonates have low scores because of other conditions, such as genetic disorders, that are completely unrelated to asphyxia. Low-birth-weight infants (<1500 g) are at dramatically increased risk of cerebral palsy, with an incidence of 60 per 1000 births compared with two per 1000 births in normal-weight infants. This increased incidence is believed to be due to the fragility of the periventricular blood vessels, which are highly susceptible to physiological fluctuations during pregnancy ( Fig ). These fluctuations, which include hypoxic episodes, placental pathology, maternal diabetes, and infection, can injure these vessels and lead to subsequent intraventricular hemorrhages. These injuries are graded on a scale from I to IV ( Table 30-1 ), with an increased incidence of neurological consequences such as hydrocephalus and cerebral palsy in grade III (bleeding into ventricles with dilation) and grade IV (bleeding into brain substance). In addition, the periventricular area, which is important for motor control, is especially susceptible from the 26th to the 32nd week of pregnancy. If injured, diplegia usually results. Often, a synergistic combination of events leads to brain injury and the subsequent development of cerebral palsy. Pregnancies involving multiple births also are at increased risk for cerebral palsy, primarily because of their association with premature delivery

10. white matter
Periventricular leukomalacia. Cross-sectional view shows blood vessels that supply brain with blood (left) and brain structures (right). Area surrounding ventricles contains “white matter” that includes descending neuronal pathways of motor control system. This area, especially farther forward in brain, is susceptible to damage in premature infants because of relative paucity of blood vessels. Fluctuations in blood flow, blood oxygen, or blood glucose levels can cause damage in this area, resulting in disturbance of motor control system and subsequent (usually spastic) cerebral palsy

11. postnatal Hypoxic-ischemic encephalopathy,
persistent fetal circulation with true ischemia
meconium aspiration
which is characterized by hypotonia, decreased movement, and seizures
Infections such as encephalitis and meningitis
Traumatic brain injury
Although most children born with cerebral palsy are full-term, full-term infants are at a much lower risk of developing cerebral palsy than are premature infants. Hypoxic-ischemic encephalopathy, which is characterized by hypotonia, decreased movement, and seizures, is a common cause of cerebral palsy during the postnatal period. Meconium aspiration and persistent fetal circulation with true ischemia are the most common causes of hypoxic-ischemic encephalopathy. Infections such as encephalitis and meningitis, most commonly caused by group B streptococcus and herpes, can lead to cerebral palsy during this period. Traumatic brain injury from accidents or child abuse also accounts for a significant number of cases of cerebral palsy that develop in the postnatal period. Improvements in obstetrical care have dramatically decreased the frequency of iatrogenic brain injury

13. Geographical Classification

14. Monoplegia very rare usually occurs after meningitis.
Most patients diagnosed with monoplegia actually have hemiplegia

15. Diplegia most common 50% premature infants intelligence normal
Most children with diplegia walk eventually
although walking is delayed usually until around age 4 years

16. Hemiplegia 30%, typically have sensory changes
Hemiplegic patients also may have a
leg-length discrepancy

17. Quadriplegia significant cognitive deficiencies
that make care more difficult.
Head and neck control which helps with communication, education, and seating.
Treatment goals
straight spine level pelvis,
located mobile hips, plantigrade feet that..
Quadriplegia

18. Total Body profound cognitive deficits head and neck control.
full-time assistance
for activities of daily living and specialized seating systems to assist with head positioning.
Drooling, dysarthria, and dysphagia

19. Paraplegia is very rare triplegia

21. Physiological Classification

23. athetoid cerebral palsy,
different pathways of the brain are myelinated at different times,
spastic diplegia
8 to 10 months of age;
hemiplegia,
20 months of age
athetoid cerebral palsy,
after 24 months of age.
Synaptic connections and myelination begin during the third trimester and continue through adolescence in a highly organized fashion. As these synapses develop, and myelinization continues, primitive reflexes disappear, and more mature motor patterns arise. Because of this continued development after birth, many injuries to the newborn nervous system go unrecognized until the absence of expected patterns can be detected. Because different pathways of the brain are myelinated at different times, spastic diplegia usually is not detected until 8 to 10 months of age; hemiplegia, 20 months of age; and athetoid cerebral palsy, after 24 months of age. It is important to keep this in mind because a child's pattern may change over time

25. DIAGNOSIS History and physical examination are the primary…
spastic paraparesis and congenital ataxia.
Ancillary studies, such as radiographs, hematological studies, .
Diagnosis of cerebral palsy before age 2 years can be very difficult.
Transient dystonia of prematurity is a condition characterized by increased tone in the lower extremities between 4 and 14 months
old and often is confused with cerebral palsy.
History and physical examination are the primary tools in making the diagnosis of cerebral palsy. The history should include a thorough investigation of the pregnancy and delivery. With the exception of several rare conditions, such as familial spastic paraparesis and congenital ataxia, there is no known genetic component to cerebral palsy. Ancillary studies, such as radiographs, hematological studies, chromosomal analysis, CT, MRI, and positron emission tomography, rarely are needed to make the diagnosis, but may be helpful in determining the type and extent of cerebral palsy present. Diagnosis of cerebral palsy before age 2 years can be very difficult. Nelson and Ellenberg found that 55% of children diagnosed with cerebral palsy by 1 year of age did not meet the criteria by age 7 years. Transient dystonia of prematurity is a condition characterized by increased tone in the lower extremities between 4 and 14 months old and often is confused with cerebral palsy. This is a self-limiting condition and resolves without treatment. In addition, African American children tend to have higher muscle tone than other ethnic groups, which also can lead to a misdiagnosis of cerebral palsy.
Knowledge of normal motor developmental milestones and primitive reflexes allows identification of children who are delayed in their motor development. Motor development usually occurs in a cephalad-to-caudal pattern, starting with swallowing and sucking, which are present at birth, and proceeding to sphincter control, which occurs at 24 to 36 months of age ( Table 30-4 ). Primitive reflex patterns of motor activity that are outgrown as part of the normal maturation process persist longer than normal and in some cases permanently in children with cerebral palsy ( Fig ). Other, more mature motor patterns, which are essential for normal ambulation, may be significantly delayed or never appear. By determining which reflexes are present or absent, the child's neurological age can be determined. Comparing the neurological age with chronological age, a neurological quotient can be determined, which is useful in determining prognosis and treatment. The presence of these primitive reflexes also can contribute to further deformity.

26. DIAGNOSIS
Knowledge of normal motor developmental milestones and primitive reflexes allows identification of children who are delayed in their motor development.
Motor development usually occurs in a cephalad-to-caudal pattern, starting with swallowing and sucking, which are present at birth, and proceeding to sphincter control, which occurs at 24 to 36 months of age.
Primitive reflex patterns of motor activity that are outgrown as part of the normal maturation process persist longer than normal and in some cases permanently in children with cerebral palsy.
Neurological quotient can be determined, which is useful in determining prognosis and treatment.
History and physical examination are the primary tools in making the diagnosis of cerebral palsy. The history should include a thorough investigation of the pregnancy and delivery. With the exception of several rare conditions, such as familial spastic paraparesis and congenital ataxia, there is no known genetic component to cerebral palsy. Ancillary studies, such as radiographs, hematological studies, chromosomal analysis, CT, MRI, and positron emission tomography, rarely are needed to make the diagnosis, but may be helpful in determining the type and extent of cerebral palsy present. Diagnosis of cerebral palsy before age 2 years can be very difficult. Nelson and Ellenberg found that 55% of children diagnosed with cerebral palsy by 1 year of age did not meet the criteria by age 7 years. Transient dystonia of prematurity is a condition characterized by increased tone in the lower extremities between 4 and 14 months old and often is confused with cerebral palsy. This is a self-limiting condition and resolves without treatment. In addition, African American children tend to have higher muscle tone than other ethnic groups, which also can lead to a misdiagnosis of cerebral palsy.
Knowledge of normal motor developmental milestones and primitive reflexes allows identification of children who are delayed in their motor development. Motor development usually occurs in a cephalad-to-caudal pattern, starting with swallowing and sucking, which are present at birth, and proceeding to sphincter control, which occurs at 24 to 36 months of age ( Table 30-4 ). Primitive reflex patterns of motor activity that are outgrown as part of the normal maturation process persist longer than normal and in some cases permanently in children with cerebral palsy ( Fig ). Other, more mature motor patterns, which are essential for normal ambulation, may be significantly delayed or never appear. By determining which reflexes are present or absent, the child's neurological age can be determined. Comparing the neurological age with chronological age, a neurological quotient can be determined, which is useful in determining prognosis and treatment. The presence of these primitive reflexes also can contribute to further deformity.

27. Prognostic Factors
If a child has not learned to walk by age 8 years, and he or she is not limited by severe contractures, it is unlikely he or she will ever walk at all.
sitting independently before age 2 years was not a good predictor of ultimate ambulatory ability, but the inability to sit independently by 4 years predicted nonambulation
Bleck used the presence or absence of primitive reflexes to determine prognosis for ambulation for children with cerebral palsy
Considerable work has been done investigating prognostic factors for function, including ambulation, in patients with cerebral palsy. Paine noted that the presence of tonic neck reflexes usually is incompatible with independent standing balance and the ability to perform alternating movements of the lower extremities necessary for walking. In his experience, sitting independently by 2 years old was a good predictor of independent ambulation. He found that approximately half of children who can sit independently by 2 to 4 years old eventually walk, and if a child cannot sit independently by 4 years, it is unlikely he or she will ever walk without assistance.
Finally, if a child has not learned to walk by age 8 years, and he or she is not limited by severe contractures, it is unlikely he or she will ever walk at all. Molnar and Gordon found that sitting independently before age 2 years was not a good predictor of ultimate ambulatory ability, but the inability to sit independently by 4 years predicted nonambulation.

29. ASSOCIATED CONDITIONS
In one study, adults with cerebral palsy ranked what was most important to them:
education and communication
activities of daily living
Mobility
Ambulation
Because of the often complex nature of these conditions, a multidisciplinary team approach to patients with cerebral palsy is essential.

30. ASSOCIATED CONDITIONS
mental impairment or learning disability (40%);
seizures (30%);
complex movement disorders (20%);
visual impairment (16%);
malnutrition and related conditions (15%)
hydrocephalus (14%).
Bulbar involvement can lead to drooling, dysphagia, and speech difficulties, which can limit cognitive and social development further.
Many children with cerebral palsy (50% in some series) have significant visual difficulties, with 7% having a severe visual defect. Common visual disturbances include myopia, amblyopia, strabismus, visual field defects, and cortical blindness.
Hearing loss has been reported to occur in 10% to 25% of children with cerebral palsy, which can exacerbate communication and learning difficulties further.
Hearing screenings, similar to visual screenings, should be part of the routine evaluation of patients with cerebral palsy.
The most common associated conditions in patients with cerebral palsy are mental impairment or learning disability (40%); seizures (30%); complex movement disorders (20%); visual impairment (16%); malnutrition and related conditions, such as gastroesophageal reflux, obesity, and undernutrition (15%); and hydrocephalus (14%). Mental impairment and learning disability can range from very mild deficits to severe impairment and inability to live independently. Mental retardation, as defined as an IQ less than 50, occurs in 30% to 65% of children with cerebral palsy, most commonly in quadriplegics. Learning disabilities are worsened by seizure disorders, various medications with central nervous system side effects, and communication difficulties. Bulbar involvement can lead to drooling, dysphagia, and speech difficulties, which can limit cognitive and social development further.
Many children with cerebral palsy (50% in some series) have significant visual difficulties, with 7% having a severe visual defect. Common visual disturbances include myopia, amblyopia, strabismus, visual field defects, and cortical blindness. Visual screening is indicated in all children with cerebral palsy. Hearing loss has been reported to occur in 10% to 25% of children with cerebral palsy, which can exacerbate communication and learning difficulties further. Hearing screenings, similar to visual screenings, should be part of the routine evaluation of patients with cerebral palsy.

31. ASSOCIATED CONDITIONS
Seizures(30%),
most commonly patients with :
hemiplegia,
quadriplegia,
postnatally acquired syndromes.
Osteopenia with increased risk of fracture also is common in children with cerebral palsy, especially children who are more severely affected. The nonoperative and operative treatment of these fractures
Bisphosphonates have been shown in small studies
Approximately 30% of patients with cerebral palsy also have seizures, most commonly patients with hemiplegia, quadriplegia, or postnatally acquired syndromes. Seizures and the medications used in their management can have profound effects on learning, communication, and ambulation. This has led to renewed interest in alternative medication delivery systems, such as intrathecal baclofen and intramuscular botulinum toxin injections.
Osteopenia with increased risk of fracture also is common in children with cerebral palsy, especially children who are more severely affected. The nonoperative and operative treatment of these fractures has a high complication rate and usually interferes with the child's social and school activities and can make it difficult for caretakers. Henderson found significant femoral osteopenia (bone mineral density Z-score of < -2) in 77% of children with cerebral palsy and 97% of nonstanders in his study of moderately to severely affected children; fractures had occurred in 26% of patients older than 10 years of age. Bisphosphonates have been shown in small studies to be safe and effective in increasing bone mineral density in children with cerebral palsy, but large multicenter trials are lacking.

32. ASSOCIATED CONDITIONS
Severe medical problems, such as aspiration pneumonia and profound feeding problems, can lead to malnutrition, immune suppression, and metabolic abnormalities.
Gastroesophageal reflux often can be managed medically and with positioning, but fundoplication may be necessary. Enteral feeding augmentation often is necessary because of swallowing dysfunction and the risk of aspiration pneumonia. This can be done with a gastrostomy or jejunostomy tube.
Emotional problems add to these associated conditions. The child's self-image plays an important role, especially in adolescence, when the differences between the affected child and peers become more apparent.
Communication difficulties also may affect self-image at this stage.
As young adulthood is reached, concerns about employment, self-care, sexual function, marriage, childbearing, and caring for aging parents may become emotional stressors.
Severe medical problems, such as aspiration pneumonia and profound feeding problems, can lead to malnutri-tion, immune suppression, and metabolic abnormalities. Gastroesophageal reflux often can be managed medically and with positioning, but fundoplication may be necessary. Enteral feeding augmentation often is necessary because of swallowing dysfunction and the risk of aspiration pneumonia. This can be done with a gastrostomy or jejunostomy tube. Patients with protein malnutrition have been shown to be at increased risk of postoperative infection.
Emotional problems add to these associated conditions. The child's self-image plays an important role, especially in adolescence, when the differences between the affected child and peers become more apparent. Communication difficulties also may affect self-image at this stage. The attitudes of the parents, siblings, treatment team, and community are important to help the child or young adult maximize his or her independence and function. As young adulthood is reached, concerns about employment, self-care, sexual function, marriage, childbearing, and caring for aging parents may become emotional stressors.

33. TREATMENT a multidisciplinary team approach
Four basic treatment principles exist.
1- cp is nonprogressive, the deformities are progressive.
2-treatments currently available correct the secondary deformities only and not the primary problem.
3-deformities typically become worse during times of rapid growth.
4-operative or nonoperative treatment should be done to minimize the impact it has on the patient's socialization and education.
Because of the heterogeneous nature of cerebral palsy, it is difficult to make generalized statements regarding treatment, and it is best to have an individualized approach to each patient and his or her needs. In some centers, a multidisciplinary team approach—including physical, occupational, and speech therapy; orthotics; nutrition; social work; orthopaedics; and general pediatrics—has been successful. Four basic treatment principles exist. The first is that although the central nervous system injury, by definition, is nonprogressive, the deformities caused by abnormal muscle forces and contractures are progressive. The second, which can be a source of frustration, is that the treatments currently available correct the secondary deformities only and not the primary problem, which is the brain injury. The third is that the deformities typically become worse during times of rapid growth. For some patients, it may be beneficial to delay surgery until after a significant growth spurt to decrease the risk of recurrence. The fourth is that operative or nonoperative treatment should be done to minimize the impact it has on the patient's socialization and education. It is important to be aware of these timing issues when considering any form of treatment in this patient population. It also is important to recognize that for most patients a combined approach using nonoperative and operative methods is more beneficial rather than one form of treatment alone

34. TREATMENT Nonoperative
splinting and bracing
physical therapy,
most common agents =diazepam and baclofen, which act centrally,
dantrolene, which acts at the level of skeletal muscle.
Baclofen
mimics the action of g-aminobutyric acid, a powerful inhibitory neurotransmitter centrally and peripherally, whereas diazepam potentiates the activity of g-aminobutyric acid.
Dantrolene acts at the level of skeletal muscle
develop profound weakness, hepatotoxicity
New trend intrathecal baclofen and intramuscular botulinum toxin injections
Nonoperative modalities, such as medication, splinting and bracing, and physical therapy, commonly are used as primary treatment or in conjunction with other forms of treatment such as surgery. A wide variety of medications have been used to treat cerebral palsy. The three most common agents are diazepam and baclofen, which act centrally, and dantrolene, which acts at the level of skeletal muscle. Baclofen mimics the action of g-aminobutyric acid, a powerful inhibitory neurotransmitter centrally and peripherally, whereas diazepam potentiates the activity of g-aminobutyric acid. These medications can be difficult to use because of wide variability in effectiveness among children and a narrow therapeutic window. Because these drugs increase inhibitory neurotransmitter activity, common systemic side effects include sedation, balance difficulties, and cognitive dysfunction, which can have a dramatic detrimental effect on ambulation, education, and communication.
Dantrolene acts at the level of skeletal muscle and decreases muscle calcium ion release. It has an affinity for fast twitch muscle fibers and selectively decreases abnormal muscle stretch reflexes and tone. Dantrolene is used less frequently than other medications because some patients taking it develop profound weakness, and there is a risk of hepatotoxicity with long-term use. Because of the systemic side effects of these medications, there is a renewed interest in alternative drug delivery systems, such as intrathecal baclofen and intramuscular botulinum toxin injections.

35. TREATMENT
Baclofen, in addition to inhibiting abnormal monosynaptic extensor activity and polysynaptic flexor activity, has been shown to decrease substance P levels, which limits nociception. Baclofen has been shown to penetrate the blood-brain barrier poorly, and it has a short half-life (3 to 4 hours). This requires gradual titration of medication and the use of extremely high systemic levels to obtain a central effect of spasticity reduction. Intrathecal injection of baclofen requires 1/30 the dose of oral baclofen to achieve a similar or better response. Injecting baclofen intrathecally with an implantable programmable pump dramatically decreases the dose required to affect spasticity and decreases some of the side effects such as sedation. This pump typically is implanted subcutaneously in the abdominal wall and requires refilling approximately every 2 to 3 months ( Fig ). A meta-analysis of 14 studies of intrathecal baclofen management found that it reduced lower extremity spasticity, seemed to improve function and ease of care, and had manageable complications. Baclofen also works at the level of the spinal cord to slow abnormal spinal reflexes and decrease motoneuron drive, which can reduce spasticity further. Careful monitoring is required to prevent overdosage, which can cause a decrease in trunk tone, weakness, and sedation. Complications from intrathecal baclofen include catheter and pump infection or malfunction, spinal fluid leak, respiratory depression, drug reactions, and oversedation. Approximately 10% to 20% of patients require further surgery or pump removal. There also have been concerns about the progression of scoliosis in patients who receive intrathecal baclofen therapy. Until longer term studies can be done, this treatment method is indicated for patients whose spasticity significantly interferes with self-care and quality of life and in whom other modalities have failed

36. TREATMENT Botulinum toxin Contraindications
a potent neurotoxin, of which there are seven serotypes, produced by Clostridium botulinum..
Contraindications
BTX-A therapy include known resistance or antibodies, fixed deformity or contracture, concurrent use of aminoglycoside antibiotics, failure of previous response, and certain neurological conditions such as myasthenia gravis
Botulinum toxin is a potent neurotoxin, of which there are seven serotypes, produced by Clostridium botulinum. Botulinum toxin type A (BTX-A) (Botox, Dysport) has been used to weaken muscles selectively in patients with cerebral palsy. BTX-A injected directly into the muscle acts at the level of the motor end plate, blocking the release of the neurotransmitter acetylcholine and inhibiting muscle contraction ( Fig ). Because it can diffuse 2 to 3 cm in the tissues, it is easier to achieve the desired effect with BTX-A than with other agents, such as phenol or alcohol, which require more accurate injection. It also is safer than these other agents because it binds selectively to the neuromuscular junction and not to other surrounding tissues. This effect begins approximately 24 hours after injection and lasts 2 to 6 months. Care must be taken to prevent systemic injection of this toxin, which in large enough doses can cause respiratory depression and death. The maximal safe dose of BTX-A based on primate data is 36 to 50 U/kg body weight; however, most studies report doses of less than 20 U/kg body weight. BTX-A has been shown to be effective when used in conjunction with other modalities, such as physical therapy or serial casting. The most common side effects are local pain and irritation from the injection. The most common use of BTX-A is as an adjuvant to a bracing, casting, or physical therapy treatment program over a finite period. It is beneficial in young patients in whom there is a need to delay surgery. It also has been used to predict the results of tendon-lengthening surgery; however, this is controversial. BTX-A also has been shown to improve energy expenditure with walking and has been reported to improve upper extremity function and self-care, but the results are highly variable. With long-term use, efficacy may decrease because of the production of antibodies to the toxin; it is recommended that injections be done 3 to 4 months apart and only when other methods have failed. Contraindications to BTX-A therapy include known resistance or antibodies, fixed deformity or contracture, concurrent use of aminoglycoside antibiotics, failure of previous response, and certain neurological conditions such as myasthenia gravis.

37. TREATMENT Physical therapy Bracing,
The therapist plays a crucial role in all aspects of care, including identifying children who may have cerebral palsy, treating their spasticity and contractures, fabricating splints and simple braces, providing family education and follow-up, acting as a liaison with the school and other health care providers, and implementing home stretching and exercise programs with the patients and their families.
Bracing,
The most commonly used braces for the treatment of cerebral palsy include ankle-foot orthoses, hip abduction braces, hand and wrist splints, and spinal braces or jackets. A patient-centered approach should be used.
The goals of bracing for an ambulatory child differ from the goals for a child with severe involvement. Bracing of the lower extremities, most commonly with ankle-foot orthoses, is common in patients with cerebral palsy.
Physical therapy is an essential component in the treatment of patients with cerebral palsy. Physical therapy typically is used as a primary treatment modality and in conjunction with other modalities, such as casting, bracing, BTX-A, and surgery. The therapist plays a crucial role in all aspects of care, including identifying children who may have cerebral palsy, treating their spasticity and contractures, fabricating splints and simple braces, providing family education and follow-up, acting as a liaison with the school and other health care providers, and implementing home stretching and exercise programs with the patients and their families. Because of the variability in patients with cerebral palsy, an individualized approach to therapy is necessary. Goals for ambulatory patients include strengthening of weakened muscles, contracture prevention, and gait and balance training; for severely affected individuals, goals are improvements in sitting balance, hygiene, and ease of care for caregivers. The parents should be encouraged from the beginning to take an active role in the child's therapy program.
Objective data in the literature supporting or disputing the use of physical therapy in patients with cerebral palsy are few because most studies involve small groups of heterogeneous patients who are not randomized. Unanswered questions include what types of therapeutic modalities should be used, by whom, and for how long. There are no clear data to support lifelong physical therapy, although many parents request this. Lifelong physical therapy may be detrimental to the child and the family financially, developmentally, socially, and emotionally.
Bracing, as with physical therapy and medication, typically is used in conjunction with other modalities. Bracing in patients with cerebral palsy most commonly is used to prevent or slow progression of deformity. The most commonly used braces for the treatment of cerebral palsy include ankle-foot orthoses, hip abduction braces, hand and wrist splints, and spinal braces or jackets. A patient-centered approach should be used. The goals of bracing for an ambulatory child differ from the goals for a child with severe involvement. Bracing of the lower extremities, most commonly with ankle-foot orthoses, is common in patients with cerebral palsy. These have been shown to improve gait function and decrease crouch during walking, even in the absence of surgery in ambulatory children. The goals of bracing in a severely affected child include facilitating shoe wear, preventing further progression of contractures, improving wheelchair positioning, and assisting standing programs. The use of floor-reaction ankle-foot orthoses, which use a plantar flexion–knee extension couple to help eliminate crouched-knee gait and improve stance phase knee extension, has dramatically decreased the need for bracing above the knee with knee-ankle-foot orthoses.

38. TREATMENT Operative (1) correct static or dynamic deformity,
(2) balance muscle power across a joint
(3) reduce spasticity (neurectomy), and
(4) stabilize uncontrollable joints.
Often, procedures can be combined—an adductor tendon release can be done at the time of pelvic osteotomy for hip subluxation .
Operative treatment of deformities related to cerebral palsy can be divided into several groups, including procedures to
(1) correct static or dynamic deformity,
(2) balance muscle power across a joint
, (3) reduce spasticity (neurectomy), and
(4) stabilize uncontrollable joints. Often, procedures can be combined—an adductor tendon release can be done at the time of pelvic osteotomy for hip subluxation.
Flexible static and dynamic deformities typically are corrected with a muscle-tendon lengthening procedure; capsulotomies and osteotomies are reserved for more severe or rigid deformities. Over time, spasticity causes a relative shortening of the musculotendinous unit, leading to abnormal joint motion and loading and, if left untreated, degenerative changes. Operative lengthening of the musculotendinous unit causes a relative weakening of the muscle with restoration of more normal forces and motion across the joint. Lengthening can be done using a recession or release of the muscular aponeurosis at the musculotendinous junction, a Z-plasty within the substance of the tendon itself, or a complete tenotomy depending on the circumstances. Recessions tend to avoid complications that can occur with overlengthening and subsequent weakness that can occur with tenotomy or Z-plasty. More severe deformities usually cannot be corrected with soft-tissue release alone and typically require osteotomy.
Balancing muscle forces across any joint can be difficult and is even more difficult in patients with cerebral palsy because of the decreased control of voluntary muscle function, lowered threshold of stretch reflexes, increased frequency of cocontraction of antagonistic muscle groups, and inability to learn to use the transferred muscle in an altered location or function. In addition, muscles that are spastic throughout the gait cycle typically remain spastic after transfer. Often, the goal of tendon transfer in this patient population is either to remove a deforming or out-of-phase muscle force away from a joint or to act as a passive tendon sling.
Neurectomy, by a variety of mechanical and chemical methods, has been proposed as a way to decrease the muscle forces acting across a joint. A primary concern about neurectomy is the overweakening of the affected muscle, leading to uncontrolled antagonistic function and development of a secondary opposite deformity. If neurectomy is considered, a trial can be conducted by temporarily disrupting nerve function mechanically or pharmacologically before the definitive procedure. A short-acting anesthetic agent, such as lidocaine, can be injected to determine if an ablative procedure such as neurectomy would have the desired effect. If this is beneficial, the nerve can be injected with a longer acting or permanent agent. Injection of alcohol directly into the muscle also has been tried to inhibit neurological transmission, with limited success. This requires general anesthesia, and the duration of effect varies. The irreversible and often variable responses to neurectomy and intramuscular injection have caused them to fall out of favor.
With continued abnormal muscle forces applied across a joint, pathological changes to the joint can occur, including subluxation, dislocation, and cartilaginous degeneration. Joint stabilization procedures, such as osteotomies, usually combined with soft-tissue releases, have produced good long-term results. For severe joint destruction, procedures such as arthrodesis, especially in the foot, and resection arthroplasty, especially in the hip, have been shown to be beneficial. Joint replacement, which was initially contraindicated in patients with neuromuscular diseases such as cerebral palsy, also has been used in patients with end-stage arthritis with good functional improvement and pain relief. Joint replacement should be done only in carefully selected patients and in a center with experience with this type of procedure.

39. TREATMENT
Operative treatment typically is indicated when contractures or deformities decrease function, cause pain, or interfere with activities of daily living. It is the only effective treatment when significant fixed contractures exist. Because many patients with cerebral palsy have significant comorbidities, operative treatment carries with it an increased risk of complications compared with the general population. Surgical procedures should be scheduled to minimize the number of hospitalizations and interference with school and social activities. “Birthday surgery” or multiple procedures performed at different times, as described by Rang, should be avoided whenever possible ( Fig ). Preoperative consultation with the patient's pediatrician, pulmonologist, and other members of the care team can help optimize the patient's condition before surgery and decrease risk. Thirty percent of patients with cerebral palsy have been shown to be malnourished, which increases the risk of postoperative wound healing problems and infection. Jevsevar and Karlin showed that a serum albumin level less than 35 g/L and a blood lymphocyte count of less than 1.5 g/L were associated with a significant increase in the risk of postoperative infection. Determination of a patient's nutritional status and improving it before surgery may decrease the overall complication rate.

40. TREATMENT
HIP
In patients with cerebral palsy, all hips should be considered abnormal until proved otherwise
Deformities of the hip in patients with cerebral palsy range from mild painless subluxation to complete dislocation with joint destruction, pain, and impaired mobility.
prevalence of hip pain in 234 patients (mean age 28 years old) was 47%
It has been estimated that progressive hip instability occurs in approximately 15%.
commonly severely affected patients and patients with spastic quadriplegia
HIP
Deformities of the hip in patients with cerebral palsy range from mild painless subluxation to complete dislocation with joint destruction, pain, and impaired mobility. When a hip begins to subluxate, it rarely improves without treatment. Hip pain is one of the main complaints of young adults with cerebral palsy. Hodgkinson et al., in a cross-sectional multicenter study, showed that the prevalence of hip pain in 234 patients (mean age 28 years old) was 47%. It has been estimated that progressive hip instability occurs in approximately 15% of patients with cerebral palsy, most commonly severely affected patients and patients with spastic quadriplegia. Sharrard found that if nonspastic types of cerebral palsy and hemiplegia were excluded, 92% of patients with cerebral palsy had some degree of hip deformity. Lonstein and Beck found that hip subluxation and dislocation occurred in 7% of ambulators and 60% of dependent sitters. In patients with cerebral palsy, all hips should be considered abnormal until proved otherwise.

41. Subluxated left hip joint
Subluxated left hip joint. Migration index (MI) is calculated by dividing width of uncovered femoral head A by total width of femoral head B. Acetabulum is dysplastic (type 2 sourcil), with lateral corner of acetabulum above weight bearing dome. Normal hip (left side) with acetabular index (AI) indicated. There is normal (type 1) sourcil; lateral corner is sharp and below weight bearing dome. H, horizontal axis.

42. TREATMENT
KNEE
Hip and Knee Relationships
Pelvic, hip, knee, ankle, and foot deformities are interrelate
because of the muscles that cross both joints, the “two-joint muscles.”
Flexion is the most common knee deformity in patients with cerebral palsy and frequently occurs in ambulatory children.
Hip and Knee Relationships
Deformities of the knee in patients with cerebral palsy are difficult to evaluate and treat and rarely occur in isolation. Pelvic, hip, knee, ankle, and foot deformities are interrelated. The hip and the knee are tightly coupled because of the muscles that cross both joints, the “two-joint muscles.” These muscles include the rectus femoris anteriorly, gracilis medially, and semimembranosus, semitendinosus, and biceps femoris posteriorly. Pathological conditions that affect these muscles, such as spasticity or contracture, and surgical changes affect the function of both joints. A similar relationship exists between the knee and ankle with the gastrocnemius muscle, which crosses both joints. A patient with cerebral palsy who ambulates with his or her knees flexed may not have hamstrings that are tight or spastic. A patient with a hip flexion contracture ambulates with increased knee flexion to help maintain sagittal balance. A careful physical examination of the entire lower extremity is essential when evaluating the knee in patients with cerebral palsy.
Flexion Deformity
Flexion is the most common knee deformity in patients with cerebral palsy and frequently occurs in ambulatory children. Knee flexion deformities keep the knee from fully extending at the end of the swing phase of gait. This causes the knee to be flexed during stance phase, leading to decreased stride length and increased energy expenditure. Spastic hamstrings, weak quadriceps, or a combination of both can cause isolated knee flexion. It also can result from hip or ankle pathology. Patients with spastic hip flexors or weak hip extensors or both develop compensatory knee flexion that results in a “jump gait,” in which the hips, knees, and ankles are flexed ( Fig ). Patients with weakened gastrocnemius-soleus muscles, from cerebral palsy or from Achilles tendon lengthenings, ambulate with knee flexion to accommodate for the relative overpull of the ankle dorsiflexors. Prolonged spasticity and crouched knee gait can lead to true contracture of the knee itself.

45. POLIOMYELITIS

46. POLIOMYELITIS
POLIOMYELITIS

47. a viral infection localized in the
POLIOMYELITIS
a viral infection localized in the
anterior horn cells of the spinal cord and certain brainstem motor nuclei.
One of three types of poliomyelitis viruses
One of three types of poliomyelitis viruses usually is the cause of infection, but other members of the enteroviral group can cause a condition clinically and pathologically indistinguishable from poliomyelitis.
Initial invasion by the virus occurs through the gastrointestinal and respiratory tracts and spreads to the central nervous system through a hematogenous route.

48. poliomyelitis vaccine,
the incidence of acute anterior poliomyelitis in the Western world has decreased dramatically.
Live oral poliovirus vaccine (OPV)
Inactivated poliovirus vaccine (IPV)
OPV remains the vaccine of choice for global eradication in many parts of the world, specifically

49. Pathological Findings
When the poliomyelitis virus invades the body through the oropharyngeal route, it multiplies….
The incubation period is 6 to 20 days. .
Pathological Findings
When the poliomyelitis virus invades the body through the oropharyngeal route, it multiplies in the alimentary tract lymph nodes and spreads through the blood, acutely attacking the anterior horn ganglion cells of the spinal cord, especially in the lumbar and cervical enlargements. The incubation period is 6 to 20 days. The anterior horn motor cells may be damaged directly by viral multiplication or toxic by-products of the virus or indirectly by ischemia, edema, and hemorrhage in the glial tissues surrounding them. Destruction of the spinal cord occurs focally, and within 3 days, wallerian degeneration is evident throughout the length of the individual nerve fiber. Macrophages and neutrophils surround and partially remove necrotic ganglion cells, and the inflammatory response gradually subsides. After 4 months, residual areas of gliosis and lymphocytic cells fill the area of destroyed motor cells in the spine. Reparative neuroglial cells proliferate. Continuous disease activity has been reported in spinal cord segments 20 years after onset of the disease.
The number of individual muscles affected by the resultant flaccid paralysis and the severity of paralysis vary; the clinical weakness is proportional to the number of lost motor units. According to Sharrard, weakness is clinically detectable only when more than 60% of the nerve cells supplying the muscle have been destroyed. Muscles innervated by the cervical and lumbar spinal segments are most often affected, and paralysis occurs twice as often in the lower extremity muscles as in upper extremity muscles. In the lower extremity, the most commonly affected muscles are the quadriceps, glutei, anterior tibial, medial hamstrings, and hip flexors; in the upper extremity, the deltoid, triceps, and pectoralis major are most often affected.
The potential for recovery of muscle function depends on the recovery of damaged, but not destroyed, anterior horn cells. Most clinical recovery occurs during the first month after the acute illness and is almost complete within 6 months, although limited recovery may occur for about 2 years. According to Sharrard, a muscle paralyzed at 6 months remains paralyzed.

50. The anterior horn motor cells may be damaged
directly by viral multiplication or toxic by-products of the virus or
indirectly by ischemia, edema, and hemorrhage in the glial tissues surrounding them.

52. Destruction of the spinal cord occurs focally, and within 3 days,
wallerian degeneration is evident throughout the length of the individual nerve fiber.
Macrophages and neutrophils surround and partially remove necrotic ganglion cells, and the inflammatory response gradually subsides.
Destruction of the spinal cord occurs focally, and within 3 days, wallerian degeneration is evident throughout the length of the individual nerve fiber.
Macrophages and neutrophils surround and partially remove necrotic ganglion cells, and the inflammatory response gradually subsides.

53. Reparative neuroglial cells proliferate.
After 4 months,
residual areas of gliosis and lymphocytic cells fill the area of destroyed motor cells in the spine.
Reparative neuroglial cells proliferate.

54. Continuous disease activity has been reported in spinal cord segments 20 years after onset of the disease

55. The number of individual muscles affected by the resultant flaccid paralysis and the severity of paralysis vary;
the clinical weakness is proportional to the number of lost motor units.
weakness is clinically detectable only when more than 60% of the nerve cells supplying the muscle have been destroyed.

56. Muscles innervated by the cervical and lumbar spinal segments are most often affected
paralysis occurs twice as often in the lower extremity muscles as in upper extremity muscles.

57. the deltoid, triceps, and pectoralis major
lower extremity
quadriceps, glutei, anterior tibial, medial hamstrings, and hip flexors
upper extremity
the deltoid, triceps, and pectoralis major

59. recovery of muscle function
depends on the recovery of damaged, but not destroyed, anterior horn cells.
first month
almost complete within 6 months,
although limited recovery may occur for about 2 years

60. Clinical Course and Treatment
acute
convalescent
chronic
Clinical Course and Treatment
The course of poliomyelitis can be divided into three stages: acute, convalescent, and chronic. General guidelines for treatment are described here. Specific indications and techniques for operative procedures are discussed in specific sections.
Acute Stage
The acute stage generally lasts 7 to 10 days. Symptoms range from mild malaise to generalized encephalomyelitis with widespread paralysis. In younger children, systemic symptoms include listlessness, sore throat, and a slight temperature elevation; these may resolve, but recurrent symptoms, including hyperesthesia or paresthesia in the extremities, severe headache, sore throat, vomiting, nuchal rigidity, back pain, and limitation of straight leg raising, culminate in characteristically asymmetrical paralysis. In older children and adults, symptoms include slight temperature elevation, marked flushing of the skin, and apprehension; muscular pain is common. Muscles are tender even to gentle palpation. Superficial reflexes usually are absent first, and deep tendon reflexes disappear when the muscle group is paralyzed. Differential diagnoses include Guillain-Barré syndrome and other forms of encephalomyelitis.
Treatment of poliomyelitis in the acute stage generally consists of bed rest, analgesics, hot packs, and anatomical positioning of the limbs to prevent flexion posturing and contractures. Padded foot boards, pillows, sandbags, and slings can help maintain position. Gentle, passive range-of-motion exercises of all joints should be carried out several times each day

61. Acute Stage lasts 7 to 10 days
Symptoms range from mild malaise to generalized encephalomyelitis with widespread paralysis.
In younger children,
systemic symptoms include listlessness, sore throat, and a slight temperature elevation; these may resolve, but recurrent symptoms, including hyperesthesia or paresthesia in the extremities, severe headache, sore throat, vomiting, nuchal rigidity, back pain, and limitation of straight leg raising, culminate in
characteristically asymmetrical paralysis.

62. Acute Stage symptoms slight temperature elevation
In older children and adults,
symptoms
slight temperature elevation
marked flushing of the skin
apprehension
muscular pain is common. Muscles are tender even to gentle palpation.
Superficial reflexes usually are absent first, and deep tendon reflexes disappear when the muscle group is paralyzed.
In older children and adults,
symptoms include slight temperature elevation, marked flushing of the skin, and apprehension; muscular pain is common. Muscles are tender even to gentle palpation. Superficial reflexes usually are absent first, and deep tendon reflexes disappear when the muscle group is paralyzed.

63. Acute Stage Differential diagnoses include Guillain-Barré syndrome
other forms of encephalomyelitis.

64. Acute Stage Treatment
bed rest, analgesics, hot packs, and anatomical positioning of the limbs to prevent flexion posturing and contractures.
Padded foot boards, pillows, sandbags, and slings can help maintain position.
Gentle, passive range-of-motion exercises of all joints should be carried out several times each day

65. Convalescent Stage
begins 2 days after the temperature returns to normal and continues for 2 years.
Muscle power improves spontaneously during this stage, especially during the first 4 months and more gradually thereafter.

66. Convalescent Stage Treatment
is similar to that during the acute stage.
Muscle strength should be assessed monthly for 6 months and then every 3 months.
Physical therapy
Muscles with more than 80% return of strength recover spontaneously without specific therapy.

67. Convalescent Stage
Vigorous passive stretching exercises and wedging casts can be used for mild or moderate contractures.
Surgical release of tight fascia and muscle aponeuroses and lengthening of tendons may be necessary for contractures persisting longer than 6 months. Orthoses should be used until no further recovery is anticipated.

68. Chronic Stage
The chronic stage of poliomyelitis usually begins 24 months after the acute illness.

69. Chronic Stage treatment goals
correcting any significant muscle imbalances and preventing or correcting soft-tissue or bony deformities.
Static joint instability
usually can be controlled indefinitely by orthoses.
Dynamic joint instability
eventually results in a fixed deformity that cannot be controlled with orthoses.

70. Chronic Stage
Young children are more prone to develop bony deformity than are adults because of their growth potential.
Soft-tissue surgery, such as tendon transfers, should be done in young children before the development of any fixed bony changes;
bony procedures for correcting a deformity usually can be delayed until skeletal growth is near completion.