2. Cerebral Palsy
Presented by: Dr. Naji

3. Cerebral palsy (CP)
Cerebral palsy (CP) is a clinical entity characterized by a three­part definition: a
(1)disorder of movement and posture
(2)caused by a non-progressive injury
(3) to the immature brain
The distinctive characteristic of these syndromes is the change in muscle tone and posture, both at rest and with voluntary activity.
The definition of CP implies that the underlying pathologic process in the brain does not progress, and occurred during early development of the brain.

4. The first year or two of life is included in most definitions, although it is unclear what the upper age limit is of a postneonatal brain insult. The definition of CP is being reviewed at present.
A wide variety of etiologies can result in the injury to the brain that causes CP. The injury results in neurologic sequelae, with similar issues affecting motor function, musculoskeletal, and cognitive development.

5. The diagnosis is made in children who are demonstrating delayed motor development, and is confirmed with MRI.
Where indicated, other metabolic, biochemical, or genetic testing might be necessary.' There is a subset of children for whom no imaging abnormality is seen.128
During growth and development, these children need to be monitored to ensure optimal progress and avoid complications.
Lifelong care issues are now being recognized, as survival is increasing. Careful planning of the transition to adulthood is an integral part of the overall plan of care.

6. EPIDEMIOLOGY
Cerebral palsy is one of the most common disabling conditions affecting children.
The reported incidence varies but is approximately per 1000 live births.203 The Collaborative Perinatal Project measured a prevalence rate of 5.2 per 1000 live births at 1 year of age, but reported resolution in up to half of these children by 7 years of ageZ02
Discrepancies in the rate are due to difficulties in diagnosis for a variety of reasons.
Diagnosis is not made at any specific age, and can 'resolve' in up to 50%of children diagnosed prior to 2 years of age or the brain insult might not occur until later in childhood.

7. Various terms are also used, including neonatal encephalopathy, birth asphyxia, periventricular leukomalacia (PVL) , hypoxic brain injury, stroke, traumatic brain injury, and shaken infant syndrome. There is also a wide variation in normal attainment of motor milestones, making early diagnosis less reliable.
There were hopes that recent improvements in neonatal care would decrease the incidence of CP, but the prevalence in full-term infants has remained relatively constant.
Despite improved neonatal outcomes in general, the increased survival of premature low birth weight «2500 g) to extremely low birth weight ( < 1000 g) infants with higher CP risk has kept the prevalence of CP in childhood relatively constant
Infants born between 32 and 42 weeks' gestation,with a birth weigl below 10th percentile, had four to six times higher risk of CP compared with those between the 25th and 75th percentiles for birth weight.140

8. Maternal mental retardation,
maternal seizure disorder, and
hyperthyroidism;
two or more prior fetal deaths;
a sibling with motor deficit;
third-trimester bleeding or
increased urine protein excretion; and
fetal bradycardia,
chorioamnionitis,
low placental weight,
fetal malformations, and
neonatal seizures all increase CP risk in term or near-term infants.

9. Before pregnancy, increased CP risk is associated with
Multiple birth pregnancies also have a higher risk of CP, and it more often occurs in the second twin.
Before pregnancy, increased CP risk is associated with
long menstrual cycles or
repeated fetal loss during pregnancy,
fetal growth retardation,
congenital malformations,
abnormal fetal presentation, or
low socioeconomic class.
During labor and delivery, only
premature separation of the placenta and
tight nuchal cord pose an associated CP risk.
Despite these many associations, most children with these risk factors do not develop CP.

10. ETIOLOGY
The brain injury that leads to CP can occur in the prenatal, perinatal, or postnatal period.
The causes of these lesions have been attributed to a wide variety of brain injury mechanisms. It is now thought that most causes of CP occur in the prenatal period . Prenatal causes include TORCH (toxoplasmosis, 'rubella, cytomegalovirus, herpes simplex, other) infections, intrauterine stroke, and genetic malformation.
For this reason, some authors advocate using terminology such as 'cerebral palsy due to .. .

11. General Maternal history During gestation Fetal factors
Gestational age <32 weeks
Birth weight <2500 g
Maternal history
Mental retardation
Seizure disorder
hyperthyroidism
Two or more prior fetal deaths
Sibling with motor deficits
During gestation
Twin gestation
Fetal growth retardation
Third-trimester bleeding
Increased urine protein excretion
Chorionitis
Premature placenta separation
Low placenta weight
Fetal factors
Abnormal fetal presentation
Fetal malformations
Fetal bradycardia
Neonatal seizures

12. The most common currently understood causes are related to brain injury occurring in children born prematurely" The combination of immaturity, fragile brain vasculature, and the physical stresses of prematurity predisposes these children to compromised cerebral blood flow.
The blood vessels are particularly vulnerable in the watershed zone next to the lateral ventricles in the capillaries of the germinal matrix.
Bleeding in this area is arterial in origin and can occur in differing degrees:
cerebral intraventricular hemorrhage isolated to germinal matrix (grade 1),
intraventricular hemorrhage223 with normal ventricular size (grade 2),
intraventricuiar hemorrhage with ventricular dilatation (grade 30) or
intraventricular hemorrhage with parenchymal hemorrhage (grade 4

13. This can be detected early on using transfontanelle cerebral high-resolution ultrasound.
PVL is one of the strongest predictors of CP in the premature neonate.
PVL is almost always associated with a history of prematurity.
Extremely low birth weight infants are also at increased risk of CP, especially with a history of resuscitation and prolonged ventilation.
Protective factors were prenatal care and steroids.

14. Almost half of all children with CP were not born prematurely.
For term births that result in CP, the cause of brain injury is often elusive. Although uncommon, most known perinatal injuries that cause CP are due to severe anoxic or ischemic brain injury.
This can occur with mechanical difficulties of the placenta, umbilical cord, or the actual delivery itself.
Intrapartum asphyxia must be severe and prolonged to cause CP. Injuries of this type unfortunately tend to be more global and are more likely to cause a more severe disability.

15. Chorio­amnionitis has recently been found to be associated with a 4,1 times Increased risk for Cp .
Athetoid CP is associated with injury to the basal ganglia.128
This is most often caused by hyperbilirubinemia and is associated with hearing loss.
Incidence has declined since testing and treatment for Rh incompatibility has become routine, and it is now relatively rare.

16. CLASSIFICATION
The most commonly used classification system describes the type of tone abnormality as spastic, dyskinetic (dystonic or athetoid), or mixed, and some systems also include ataxic and hypotonic subtypes .
The distribution of limb involvement is characterized as monoplegia, 'diplegia (both legs affected more than arms), hemiplegia (arm frequently more affected than leg), and quadriplegia.
Triplegia is a combination of diplegia and hemiplegia. It has been suggested that the terms diplegia, quadriplegia, and hemiplegia replaced by bilateral and unilateral involvement.5o

17. Classification of cerebral palsy types
By tone abnormalities
Spastic
Dyskinetic
Athetoid
Choreiform
Ballistic
Ataxic
Hypotonic
'Mixed'
By body parts involved
Diplegia
Quadriplegia
Triplegia
Hemiplegia

20. As the upper limb involvement becomes more severe, it becomes more difficult to determine whether the upper or lower extremity is more involved. This has led to inconsistencies in classifying diplegia and quadriplegia.
For this reason, the Gross Motor Functional Classification System (GMFCS), which stratifies children based on gross motor mobility, is favored by some.219
The GMFCS classification does not describe the limbs affected, so the GMFCS classification is typically used in addition to describing the tone disorder and the limb distribution .

21. Gross motor functional classification
Level 1: walks without restriction, limitations in high level skills
Walks independently by age 2 years without devices
Walks as preferred mobility by age 4 .
Difficulty with speed, coordination, and balance for high-level tasks
Level 2: walks without devices, limitations walking outdoors
Sits with hand support by age 2
Crawls reciprocally or walks with device as preferred mobility by age 4
Uses hands to get up from the floor or a chair by age 6
Walks without devices indoors by age 6

22. Level 3: walks with devices, limitations walking outdoors
Sits with support by age 2.
Cruises age 4, walks with device short distances
Does stairs with help by age 6
Walks indoors with a device by age 12
Level 4: limited mobility, power mobility outdoors
Rolls by age 2 years
Sits with hand support by age 4
.May walk short distances indoors with device, poor balance
Preferred independent mobility is a wheelchair by age 12
Level 5: very limited self-mobility, even with assistive technology
Needs help to roll by age 2
Does not attain independent mobility by age 12
With high-level assistive technology, may learn to use power mobility

23. The areas of brain involvement on MRI can also be helpful to predict areas of difficulty. Generally, the vast majority of children (over 85%) with hemiplegia can eventually walk independently .
For unclear reasons, isolated right hemiplegia is twice as common as left hemiplegia.
The spastic-subtype is the alost common, affecting about 75%'of children with CP.

24. Spasticity is defined as velocity­dependent increased tone, associated with upper motor neuron syndrome findings of increased muscle stretch reflexes, Babinski's response, weakness, and difficulty with coordination.
Dystonia is defined as a dyskinetic tone abnormality with alternating tone or cocontraction in the antagonist and agonist muscle groups, causing varied abnormal postures and often fluctuating tone.
the other dyskinetic forms are athetosis,' choreiform and choreoathetoid.
Athetoid movements are involuntary constant rotatory or writhing movements of the' distal extremities and are associated with basal ganglia involvement. These movement stop during sleep.

25. The incidence of the athethoid type 'has drastically been reduced by the advent of treatment for Rh incompatibility.
The ataxic form is rare, and must be differentiated from degenerative processes of the cerebellum.
Spasticity and dystonia frequently coexist in CP, as the mixed form of tone abnormality.
Studies to differentiate spasticity from dystonia using clinical assessment" or gait analysis'" are important, as some treatments control spasticity or dystonia more effectively.
Surgical outcomes are much less predictable in children with dystonia, as tone fluctuates. form of dystonia that is responsive to dopaminergic medication is well described.2IO

26. A small percentage of patients have the hypotonic type of CP.
These children need to be differentiated from those with identifiable causes of neonatal hypotonia such as muscle disease, metabolic disorders, and genetic syndromes.
Many of these children develop spastic or extrapyramidal-type disorders after the first few months of life.

27. CLINICAL EFFECTS
Hypotonia and motor delay are often seen as early signs of CP. The severity of involvement varies widely, depending on the extent of neurologic involvement.
Upper motor neuron injury features include positive findings of increased tone and reflexes, positive Babinski's reflex, and negative findings of reduced strength, selective motor control, balance, and coordination.
About three-quarters of children with CP have spasticity, although the first several months can be characterized with hypotonia .
Early on, assessment reveals tone in the extremities, and retention of primitive reflexes can be noted.

28. Obligatory primitive reflexes that the child cannot emerge from- are always abnormaI
Examples of these are the asymmetric tonic neck reflex (ATNR), symmetric tonic neck reflex (STNR), and tonic labyrinthine -reflex
Other problems in infancy that suggest CP include irritability, lethargy weak suck with tongue thrust, poor head control, high-pitched cry, oral hypersensitivity ;
adopting a hand preference before the first birth­day .
Trunk or central hypotonia often remains throughout life.

30. Abnormalities of muscle tone are frequently accompanied by muscle weakness.
As tone develops in children with spasticity, abnormal posturing such as 'scissoring', 'guarding' of the upper extremities, extensor posturing, or proximal 'fixing' can be seen.
Scissoring is the simultaneous adduction, knee hyperextension, and plantar flexion of the lower extremities.

32. Flexion synergy patterns of the upper extremities include flexion at the fingers, wrists, and elbows with shoulder abduction. As this upper' extremity pattern becomes stronger, the child hands rise from the waist, producing a low, mid, or high guard position .
The abnormalities of muscle tone are often accompanied by weakness in individual muscles. Applying the traditional methods of measuring muscle strength is problematic in CP, because the tone abnormalities mask the patient's ability to generate force.
Modified sphygmomanometer and hydraulic force measures can provide 'better reliability than Medical Research Council muscle strength grades.

33. Hypersensitivity can interfere with development of hand and lower extremity weight-bearing skills.
The abnormal sensory experience of disordered motor control can contribute to disordered sensory perception, which further interferes with the child's ability to perform high-level motor activities.
Decreased ability to distinguish two-point discrimination has been found in the upper extremities of children with all types of CP.

34. Balance difficulties can be due to a combination of weakness, abnormal tone, and sensory issues, including visual perceptual skills.
The major secondary effects of disordered muscle tone, control, and balance are change in joint alignment, leading to contracture and deformity.
Contracture (passive shortening that can limit joint and soft tissue movement) frequently affects the adductor, hamstring, and plantar flexor muscles of the lower extremities and the flexors of the upper extremities.

35. Bony deformity can occur because the abnormal muscle force of CP act on a growing skeleton.
The integrity of the hips and spine are a principal concern, because of their primary role in weight bearing and positioning.
The femur is affected by muscle and gravity-loading forces during growth. Muscle forces in CP can cause increased anteversion of the femur neck.
The typical increase in hip flexion, adduction, and internal rotation of the femur acts to influence the femoral head in a superoposterolateral direction out of the acetabulum. The result is coxa valgus, deformation of the femoral head, and a shallow acetabulum, which causes the hip to be more prone to subluxation

36. Asymmetric muscle pull and immobility can contribute to significant deformity of the spine, including kyphosis, scoliosis, or rotational deformities. These spinal deformities can significantly affect comfort, tone, sitting and standing alignment, and balance.
When these are severe, respiratory function can be compromised by the mechanical restriction of the chest, combined with decreased efficiency of available respiratory muscle strength. This can have a significant impact on endurance, health, and longevity.
Spondylolisthesis and spondylolysis are not increased in CP.

37. Bony abnormalities of the feet can occur in a variety of patterns
Bony abnormalities of the feet can occur in a variety of patterns. The most common is deformity of the hind foot with exaggerated heel vaIgus or varus.

39. ASSOCIATED MEDICAL AND FUNCTIONAL PROBLEMS
Visual sequelae occur due to the central visual pathways being affected. Strabismus is common in children with diplegia
Hemiplegia can be associated with hemianopsia.
Premature infants can have had retinopathy of prematurlty.
Hearing impairment can be related to ototoxic drug exposure, hyperbilirubinemia, or TORCH infection in utero.

40. A large number, of children with CP have abnormalities, of oral motor function: These deficits include drooling, dysphagia, and dysarthria, due to weakness and incoordination of lips, tongue, and masticatory and facial muscles.
Treatment can include behavioral techniques, speech therapy, anticholinergic medications
botulinum toxin A injections, and surgical redirection of the salivary gland ducts.

41. Feeding difficulties can contribute to substantial undernutrition or malnutrition
Gastrointestinal symptoms are frequent in children with Cp. Gasteroesophageal' reflux, due to weakness of the lower 'esophageal, sphincter, can cause episodic emesis .
Urinary symptoms are not uncommon in CP. Around one­third of patients have symptoms of freqllency, incontinence.

42. Cognitive impairments are not obligatory with CP but can occur to varying degrees.
Seizure disorders can occur in up to one-third of children with CP . Children with hemiplegia have the highest rate
Osteoporosis is present in children with CP due to multiple factors.

43. FUNCTIONAL PROGNOSIS
Children typically develop motor skills craniocaudally. A child will first develop neck control, then upper trunk control, and then lower trunk control.
Sitting balance follows, then standing. The age at which these skills are developed help to predict the eventual outcome.
Seventy-five percent of children with spastic CP eventually ambulate, about 85% with diplegia, and 70% with quadriplegia. Most children with hemiplegic or ataxic CP ambulate independently, the majority by age 3 years."

44. The combination of increased tone and underlying muscle weakness greatly increases the energy expended for motor activity.
Contractures and bone or joint deformities can also greatly limit a child's function.

45. THERAPEUTIC MANAGEMENT
The therapeutic management of the child with CP emphasizes a functional aim- or goal-oriented approach." The two major goals of rehabilitation, according to Molnar. are to decrease complications of the CP and to enhance or improve the acquisition of new skills.
The role of the physician is to provide an accurate assessment of the child's impairments. In the case of the child with CP, this can include learning disabilities, mental retardation, respiratory compromise, fractures, and other comorbidities that can limit function.

46. Therapy approaches
Children with CP who have motor problems that interfere with educational activities have the opportunity to participate in therapy programs in school. The services should be delivered in the least restrictive environment to allow the child to participate in and benefit from the educational experience.
The current philosophic trend in schools is to include disabled children as much as possible into the regular classroom, and few 'pull out' services are stressed.

47. MEDICAL AND SURGICAL MANAGEMENT
Management of hypertonia
Management of hypertonia for children with CP has had increasing medical attention over the decade.
Oral medications are utilized to decrease global hypertonia.
The most commonly used drugs are baclofen (Lioresal), dantrolene (Dantrium), and diazepam.
The response to these drugs is generally unpredictable, and side effects can limit long­term use.
The pediatric doses can be quite variable and depend on the response of patient.

48. Specific medications for dystonia can impact the movement in additional ways. Motor control can be improved in the oral motor and fine motor areas with trihexyphenidyl hydrochloride (Artane) and levodopa-carbidopa (Sinemet) and other dopaminergic medications are drugs that warrant trial in a child with dystonia.
Reappraisal of spasticity or hypertonia in a growing child with CP every 6 months is necessary.
Motor point blocks, and recently botulinum toxin injections, can be used as adjunct management of the spastic muscle.
Botulinum toxin's effect in children with CP has been studied and promoted in the past decade and is widely utilized in the management of CP.

49. There is a large body of literature that supports its use in children with hypertonia, both spasticity and dystonia.
Botulinum toxin Type A (Botox) is effective in improving range of motion and reducing tone, and also potentially effective in improving motor control.

50. current suggested indications for the use of botulinum toxin include:
calf injection for dynamic equinus persistent throughout the gait cycle;
hamstring injection for dynamic knee flexion;
adductor injection for scissoring;
diagnostic measures before surgery;
management of focal limb dystonia;
analgesia for pain and spasm in the perioperative period;
in the upper limb, persistent thumb in palm, wrist posture
preventing effective hand use, and elbow flexion.

51. Phenol is an inexpensive neurolytic agent used for over 30 years
Phenol is an inexpensive neurolytic agent used for over 30 years. The technical difficulties in administration have limited the use for children.
Comparisons of phenol to botulinum toxins have been few. The theoretic advantages of using the combination of phenol are greater control over focal spasticity involving more than three muscle groups, longer duration for the phenol, and lower costs.

52. Neurosurgical interventions
Selective dorsal rhizotomy (SDR) and the intrathecal baclofen (ITB) pump are two current neurosurgical procedures that hold promise in reducing spasticity.
Reduction in tone, as recorded by the modified Ashworth scale, is improved with both procedures
SDR has been utilized since the early 1980s to reduce spasticity by interrupting the sensory input into the dorsal horn.

53. Analysis studies of children undergoing SDR have consistently shown an improved availability of range of motion at the knee and hip, resulting in an increased stride length.
Careful selection is critical, because the subsequent weakness, which is an anticipated part of the postoperative recovery period, can reduce the level of independence of children who depend on their spasticity to transfer or stand.

54. Intrathecal baclofen is another neurosurgical option for hypertonia.
The ideal candidate for SDR is the premature child , with spastic diplegia, good balance, and good selective motor skills, aged 4 or 5 years, with minimal contractures, and able to walk unassisted.
Athetosis is a contraindication for SDR as an intervention. Dystonia can become more problematic post SDR.234
Intrathecal baclofen is another neurosurgical option for hypertonia.
In addition to spasticity reduction, it also has an impact on dystonia.

55. The AACPDM evidence-based medicine review reported benefits of ITB for those who are severely involved.
For those children in GMFCS 4 and 5, improved positioning, decreased pain, and easier caregiving occurred.
The ambulatory child will have decreased energy requirement for walking; however, the balance between weakness and spasticity control has to be carefully managed.258

56. Complications with ITB are as high as 50%.
Improvements in technology with catheters and pumps might reduce the mechanical risk. Experience in management also improves the time required to diagnose catheter complications and related adverse events.
Abrupt withdrawal in children must be treated aggressively.
Deep brain stimulation is a neurosurgery technique that has shown promise in adults with dystonia.z90

57. Orthopedic intervention
The natural history of CP is one of progressive effect of spasticity or hypertonia on the growing child and progressive degeneration.
The argument could be made that maintaining function over time is a satisfactory goal.
Close observation, aggressive bracing, and vigorous PT can temper the effect of dynamic tone and static contractures.Z58

58. Surgery for sitting
Sitting is a realistic functional goal for every child.
necessary postural alignment for sitting includes a level pelvis and a reasonably straight spine.1Z3
The loss of motion associated with hip dislocation can alter seating
Excessive pelvic obliquity reduces the sitting surface area and causes excessive pressure on the bony prominences of the pelviS.166 The manage­ment of the hip is complex.
Early detection of subluxation is possible with sequential radiographs of the pelvis. Physical examination of the hip alone is not sufficient to detect subluxation.3

59. If the hip is subluxed, the surgeon might be able to reduce the overpowering muscle forces by lengthening the iliopsoas and adductor muscles around the hip.
This is a brief procedure that offers several benefits. It not only improves femoral head coverage under the acetabulum, but also allows easier dressing, diapering, cleaning, and positioning. The child treated early is more likely to maintain well-seated hips.

60. Scoliosis or kyphosis can be progressive
Scoliosis or kyphosis can be progressive. Early treatment usually involves using a molded thoracic lumbar orthosis
If a curve progresses beyond 40°, fusion is considered to avoid compromise of the respiratory system.

61. Surgery for standing
Supported standing and transfers are possible when the ankle can be held in the neutral position and the knee has less than 20° of flexion contracture.
The surgical procedures used to improve alignment for these skills are hamstring lengthenings and Achilles tendon lengthenings.
Hip flexion contractures can also hinder standing, and need attention if greater than 20°.

62. COMPLEMENTARY AND ALTERNATIVE MEDICINES
Because CP is a non-curable condition, treatments that are not supported by evidence will often be utilized by patients and their families.206 It is important that an open approach is maintained for discussion.
Evidence reviews are available from organizations such as United Cerebral Palsy and the AACPDM on current treatment approaches that can assist the clinicianin evaluating literature on specific topics
Complementary medicine is an important part of the lives of many families
Up to 56% of families having a child with CP utilize complementary and alternative medicine (CAM) treatments.

63. The American Academy of Pediatrics has developed policy statements on CAM that encourage the physician to become knowledgeable about the various complementary treatments, and to remain non-judgmental as the family is guided through the risks, theo­retic benefits, and any available credible research.l/ Even fami­lies actively involved in traditional treatments can expose their child to alternative treatments. It is at times a fine line that divides the traditional PT, OT, and speech therapy from the treatment considered non-traditional or CAM. An example of this is the use of soft tissue stretching versus techniques such as cranial-sacral, Feldenkrais, or even spinal manipulation. Hyperbaric oxygen is a treatment that has had anecdotal support from families but with no substantiated evidence-based literature.

65. Ashworth Scale
1.      No increase in muscle tone.
2.      Slight increase in tone giving a “catch” when affected part is moved in flexion or extension.
3.      More marked increase in tone but affected part is easily flexed.
4.      Considerable increase in tone; passive movement difficult.
5.      Affected part is rigid in flexion or extension.