1. Cerebral Palsy
Describes a group of disorders of movement and posture, limiting activity, attributed to non-progressive underlying brain pathology.
The motor disorders of CP are often accompanied by disturbances of sensation, cognition, communication, perception, and/or behavior, or by a seizure disorder.

2. Cerebral Palsy
Brain lesions of CP occur from the fetal or neonatal period to up to age 3 years
The etiology of CP is not well understood
Brain lesions are thought to be associated with prenatal, perinatal, or postnatal events of varying causes.

3. Cerebral Palsy Risk factors for CP are multifactorial.
Birth, multiple gestation, intrauterine growth restriction, male sex, low APGAR scores, intrauterine infections, maternal thyroid abnormalities, prenatal strokes, birth asphyxia, maternal methyl mercury exposure, and maternal iodine deficiency

4. Cerebral Palsy Prevalence
In developed countries: about cases per 1000 live births
In developing countries: about cases per 1000 live births.

5. Clinical Presentations of Cerebral Palsy

6. General Clinical Presentations of Cerebral Palsy
Failure to meet expected developmental milestones or failing to suppress obligatory primitive reflexes.
Abnormalities in muscle tone. Presents as either hypotonic or hypertonic with either decreased or increased resistance to passive movements, respectively.
Definite hand preference before age 1 year is a red flag for possible hemiplegia.
Asymmetric crawling or failure to crawl also may suggest cerebral palsy.

7. General Clinical Presentations of Cerebral Palsy
Joint contractures secondary to spastic muscles
Hypotonic to spastic tone
Growth delay
Persistent primitive reflexes
Gait pattern abnormalities
Hip - Excessive flexion, adduction, and femoral anteversion 
Knee - Flexion and extension with valgus or varus stress occur.
Foot - Equinus, or toe walking, and varus or valgus of the hindfoot

8. Types of Cerebral Palsy
Spastic (70-80%) – Increased deep tendon reflexes, sustained clonus, hypertonia, and the clasp-knife response
Spastic diplegia (30-40%) – lower extremity involvement
Spastic hemiplegia (20-30%) – 1 side of the body involved
Spastic quadriplegia (10-15%) – total body involvement
Spastic monoplegia (rare) – 1 limb involved

9. Types of Cerebral Palsy
Dyskinetic (10-15%) – Fluctuating tone, rigid total body involvement by definition. Persistent primitive reflex patterns (asymmetric tonic neck reflex, labyrinthine)
Athetoid – slow writhing movements
Dystonic – posturing of the head, trunk, and extremities

10. Types of Cerebral Palsy
Ataxic (<5%) – characterized by cerebellar signs (ataxia, dysmetria, past pointing, tremor, nystagmus) and abnormalities of voluntary movement.

11. Types of Cerebral Palsy
Mixed - no single specific tonal quality predominating; mixture of spastic and dyskinetic components
Hypotonic - truncal and extremity hypotonia with hyperreflexia and persistent primitive reflexes; thought to be rare

12. Associated Conditions
Sensory:
Sensorineural and conductive hearing loss
Impaired visual acuity
Oculomotor dysfunction
Strabismus
Cortical visual impairment
Somatosensory impairments

13. Associated Conditions
Cognitive and linguistic:
Mental retardation
High incidence of language and learning disabilities
Dysarthria
Attention deficit hyperactivity disorder
Sleep and behavioral disturbances

14. Associated Conditions
Neurologic:
Seizures
Hydrocephalus
Musculoskeletal:
Contractures
Hip dislocation
scoliosis

15. Associated Conditions
Cardiorespiratory:
Upper airway obstruction
Aspiration pneumonitis
GI/Nutritional:
Poor growth
Gastroesophageal reflux
Constipation
Dysphagia

16. Spastic Hemiplegic CP One-sided upper motor neuron deficit
Arm generally affected more than leg; possible early hand preference or relative weakness on one side
Gait characterized by circumduction of lower extremity on affected side
Specific learning disabilities
Oromotor dysfunction
Possible unilateral sensory deficits
Visual-field deficits (eg, homonymous hemianopsia) and strabismus
Seizures

17. Spastic Diplegic CP
Upper motor neuron findings in the legs more than the arms. Little or no functional limitation of the upper extremities.
Scissoring gait pattern with hips flexed and adducted, knees flexed with valgus, and ankles in equinus, resulting in toe walking
Delay in developing gross motor skills.

18. Spastic Quadriplegic CP
All limbs affected, either full-body hypertonia or truncal hypotonia with extremity hypertonia
Oromotor dysfunction
Increased risk of cognitive difficulties
Multiple medical complications
Seizures
Legs generally affected equally or more than arms

19. Dyskinetic CP
Early hypotonia with movement disorder emerging at age 1-3 years
Arms more affected than legs
Deep tendon reflexes usually normal to slightly increased
Some spasticity
Oromotor dysfunction
Gait difficulties
Truncal instability
Risk of deafness in those affected by kernicterus

20. Ataxic CP Hypotonic Tremors
Motor skills might be affected( i.e. writing, typing, or using scissors)
Difficulty in balance esp. while walking
Difficulty with visual and/or auditory processing

21. Pathophysiology of Cerebral Palsy

22. Pathophysiology
<10% children with CP : evidence of intrapartum asphyxia
Associated with increased risk of CP in normal birthweight infants: intrauterine exposure to maternal infection
Chorioamnionitis
Inflammation of the placental membranes and umbilical cord
Foul smelling amniotic fluid
Maternal sepsis
Maternal temp greater than 38⁰C during labor
UTI

23. Pathophysiology
Prevalence of CP is increased among low birthweight infants, particularly those weighing <1,000 g at birth : because of intracerebral haemorrhage and periventricular leukomalacia (PVL)
PVL: appears to reflect the enhanced vulnerability of immature oligodendroglia in premature infants to oxidative stress caused by ischemia or infectious/inflammatory insults

24. Pathophysiology
Believed to be caused by nonprogressive disturbances in the immature and still developing fetal or infant brain
Disturbances affect the development of movement and posture but patients are also frequently seen to have epilepsy, secondary musculoskeletal problems and sensation, perception, cognition, communication and behavior disturbances.

25. Pathophysiology
Insult to immature brain (before birth to postnatal period)  if immediately after postnatal period may be due to hypoxic-ischemic encephalopathy  cerebral insult  altered muscle tone, muscle stretch reflexes, primitive reflexes, postural ractions
Cerebral insults may be vascular, hypoxic-ischemic, metabolic, infectious, toxic, teratogenic, traumatic, and genetic in nature

26. Classification of Cerebral Palsy and Major Causes (Nelsons 17th Ed.)
MOTOR SYNDROME
NEUROPATHY
MAJOR CAUSES
Spastic Diplegia
Periventricular
Leukomalacia (periventricular leukomlacic [PVL])
Prematurity
Ischemia
Infection
Endocrine/metabolic (e.g., thyroid)
Spastic Quadriplegia
PVL
Multicystic encephalomalacia
Malformations
Endocrine/metabolic
Genetic/developmental
Hemiplegia
Stoke: in utero or neonatal
Thrombophilic disorders
Periventricular hemorrhagic infearction
Extrapyramidal (athetoid, dyskenetic)
Basal ganglia
Pathology: putamen, globus pallidus, thalamus
Asphyxia
Kernicterus
Mitochondrial
Genetic/metabolic

27. Pathophysiology Spastic Hemiplegia
focal cerebral infarction secondary to intrauterine or perinatal thromboembolism related to thrombophilic disorders, especially anticardiolipin antibodies, is an important cause
family histories suggesting thrombosis and inherited clotting disorders may be present

28. Pathophysiology Spastic Diplegia Spastic Quadriplegia
the most common neuropathologic finding is periventricular leukomalacia, particularly in the area where fibers innervating the legs course through the internal capsule
Spastic Quadriplegia
most severe form of CP; swallowing difficulties are common as a result of supranuclear bulbar palsies, often leading to aspiration
the most common lesions seen are severe PVL and multicystic cortical encephalomalacia

29. Pathophysiology Athetoid/Chorioathetoid/Extraoyramidal CP
if secondary to acute intrapartum near-total asphyxia is associated with bilateral symmetric lesions in the posterior putamen and ventrolateral thalamus
can also be caused by kernicterus secondary to high levels of bilirubin
can also be associated with lesions in the basal ganglia and thalamus caused by metabolic genetic disorders such as mitochondrial disorders and glutaric aciduria

30. Treatment

31. Therapy Goal: to maximize the functional use of limbs and ambulation
to reduce the risk of contractures
to help the patient in attaining his greatest potential physically, mentally and socially

32. Rehabilitation
Physical therapy – to develop muscle strength, flexibility and strength
Occupational therapy – to help learn physical skills needed to function in everyday life
Recreational therapy
Orthotic devices such as ankle foot orthoses
Speech therapy – to overcome speech problems
Psychotherapy

33. Parent education
Teach the parents how to work with their child in daily activities such as feeding, dressing, bathing, and playing in ways that limit the effects of abnormal muscle tone.
Instruct the parents in the supervision of a series of exercises designed to prevent the development of contractures, especially a tight Achilles tendon.

34. For children with Spastic Diplegia
Use walkers, poles, and standing frames
Surgery may be considered to reduce muscle spasm around the hip girdle (adductor tenotomy or psoas transfer and release)
Rhizotomy procedure – roots of the spinal nerves are divided, produces considerable improvement in some patients
Consider surgery if pt has marked spasticity of the lower extremities or evidence of hip dislocation
Tenotomy is the cutting of a tendon. It is performed in order to lengthen a muscle that has developed improperly, or become shortened and is resistant to stretch. Read more: Tenotomy - procedure, pain, time, infection, operation, medication, risk, children, rate, Definition, Purpose, Demographics, Description, Diagnosis/Preparation, Aftercare, Risks, Normal results 34

35. For children with Spastic Hemiplegia
A tight heel cord may be treated by tenotomy of the Achilles tendon
Constraints can be applied to the unaffected side – this induces improved hand and arm functioning on the affected side. This is effective in patients of all ages.

36. For children with Spastic Quadriplegia
Use motorized wheelchairs, special feeding devices, modified typewriters, and customized seating arrangements

37. Surgical To correct anatomical abnormalities or release tight muscles
To help repair dislocated hips and scoliosis (curvature of the spine)

38. Surgery Dorsal rhizotomy (for severe spastic diplegia)
Cut specific nerves at their roots to reduce spasticity
Stereotactic surgery
To improve rigidity, athetosis and tremors
Reconstructive surgery to an arm
to restore muscle balance, release contractures, and stabilize joints

39. Medical Therapy
Goal of pharmacotherapy is to reduce symptoms (e.g. spasticity) and prevent complications (e.g. contractures)
2 types of medications
For spasticity and abnormal movement
For seizures

40. Medications for spasticity
Dopaminergic drugs
increase dopamine levels to decrease rigidity and abnormal movements
E.g. levodopa/carbidopa
Botulinum toxin
Injected into specific muscle groups
Shows very positive response
May also be used to reduce the severity of drooling when injected into the salivary glands

41. Medications for spasticity
Muscle relaxants
Baclofen: controls muscle contractions and relaxes tight muscles, but lowers seizure threshold
Botulinium toxin A: causes mild muscle paralysis and reduce contractions
Benzodiazepines (valium) – sedation is a side-effect
Oral dantrolene sodium
Need constant follow-up

42. Medications for seizures
Anticonvulsants
Used to terminate clinical and electrical seizure activity as rapidly as possible
Prevent seizure recurrence
Phenobarbital or phenytoin
Effective against partial seizures
Benzodiazepines
Used in acute management of seizures

43. Other Problems
Important to identify and manage behavioral problems early - work with a psychologist or psychiatrist
Learning and attention deficit disorders, and mental retardation – assessed and managed by a psychologist and educator
Strabismus, nystagmus, and optic atrophy are common – consult an ophthalmologist
Promptly assess and treat lower urinary tract dysfunction
Communication - Use Blissymbolics, talking typewriters, and specially adapted computers
Blissymbols or Blissymbolics were conceived of as an ideographic writing system consisting of several hundred basic symbols, each representing a concept, which can be composed together to generate new symbols that represent new concepts. Blissymbols differ from all the world's major writing systems in that the characters do not correspond at all to the sounds of any spoken language.
However, since the 1960s, Blissymbols have become popular as a method of augmentative and alternative communication (AAC) for non-speaking people with cerebral palsy or other disorders, for whom it can be impossible to otherwise communicate with spoken language. Practitioners of Blissymbolics (that is, speech and language therapists and users) maintain that some users who have learned to communicate with Blissymbolics find it easier to learn to read and write traditional orthography in the local spoken language than do users who did not know Blissymbolics. 43

44. Reference Nelson’s Textbook of Pediatrics 18th ed.