1. CEREBRAL PALSY
CATALINA RUIZ, MD
12/06/12

2. DEFINITION
ETIOLOGY
CLASSIFICATION AND CLINICAL FEATURES
ASSOCIATED SYNDROMES
DIAGNOSIS
TREATMENT
PROGNOSIS

3. DEFINITION Difficulty in motor control Non-progressive brain lesion
Injury to the brain before it was fully mature (prenatally, perinatally, postnatally)
Abnormalities to motor activity and posture

4. Voluntary movements are supposed to be complex, coordinated and varied.
In CP they are uncoordinated, stereotypic and limited (is a spectrum of symptoms, mild to severe)
Diagnosis is difficult before 6 months of age, most of the movements are reflex in origin and don’t involve the voluntary motor cortex

5. ETIOLOGY Prematurity Intrauterine growth restriction
Intrauterine infection
Antepartum hemorrhage
Severe placental pathology
Multiple pregnancy
Perinatal asphyxia
Intracranial hemorrhage
Kernicterus
Near drowning
Trauma
Stroke

6. CLASSIFICATION
Based upon the type and distribution of motor abnormalities.
Spastic (diplegia, hemiplegia, quadriplegia)
Dyskinetic (dystonic, athetoid)
Ataxia (simply ataxia, ataxic diplegia)

7. TO REMEMBER…
TONE: is the continuous and passive partial contraction of the muscles.
Maneuvers to test muscle tone and normal responses include:
Holding the infant under the arms – he/she will assume a sitting position; truncal resistance allows the infant to be easily supported without slipping through the examiner's hands

8. Passive extension of the legs at the knees – resistance is met at approximately 90º.
Pulling the infant from the supine to sitting position – only a slight head lag is expected; the head should wobble in the midline for a few seconds when the sitting position is reached.
Holding the infant in horizontal suspension – he/she flexes the limbs, straightens the back, and maintains the head in the midline for a few seconds.

9. CLASSIFICATION

10. 1. SPASTIC SYNDROMES Spastic hypertonia (clasp-knife)
Hyperreflexia caused by hyperexcitability of the stretch reflex
Extensor plantar responses
Clonus
Slow effortful voluntary movements
Impaired fine-motor function
Difficulty in isolating individual movements
Fatigability

11. SPASTIC

12. SPASTIC DIPLEGIA Affects both term and preterm infants
In preterm infants often is associated with periventricular leukomalacia
The lower limbs are affected predominantly
Atrophy below the waist occurs in many patients

13. SPASTIC HEMIPLEGIA
Typically affects term infants of normal birth weight
The presentation of congenital spastic hemiplegia is variable
It is usually caused by a cortical lesion.
The arm typically is more affected than the leg

14. SPASTIC HEMIPLEGIA
Suspect when: early hand dominance, reduced movement, or abnormal posturing on one side
movement and tone on the affected side typically decrease before tone and tendon reflexes abnormally increase.

15. SPASTIC QUADRAPLEGIA Most severe form of spastic CP.
It usually affects term infants who are small for gestational age.
Affected infants typically are severely handicapped. In addition to spasticity, they may have dystonia and feeding and respiratory difficulties because of pseudobulbar palsy

16. SPASTIC QUADRAPLEGIA
Associated findings hip subluxation, contractures, and scoliosis, intellectual disabilities, visual impairment.

17. 2. DYSKINETIC SYNDROMES
Neuronal necrosis in the hippocampus, thalamus, basal ganglia, reticular formation, and Purkinje cells of the cerebellum.
The neonatal presentation includes encephalopathy characterized by lethargy, decreased spontaneous movement, hypotonia, and suppressed primitive reflexes.

18. DYSKINETIC SYNDROMES

20. DYSKINETIC SYNDROMES There are 2 types:
Athetoid: movements are athetoid, choreiform, or a combination of both.
Dystonic

21. DYSKINETIC SYNDROMES
ATHETOID MOVEMENTS: slow, smooth, writhing movements that involve distal muscles athetosis – YouTube
Athetosis is most apparent during reaching, as the fingers extend and abduct.

22. DYSKINETIC SYNDROMES
CHOREA: rapid, irregular, unpredictable contractions of individual muscles or small muscle groups that involve the face, bulbar muscles, proximal extremities, and fingers and toes.
Stress, excitement, or fever may exacerbate the chorea CHOREA

23. DYSKINETIC SYNDROMES
DYSTONIC: repetitive, patterned, twisting, and sustained movements of the trunk and limbs that may be either slow or rapid. DYSTONIA
The muscle tone typically is normal or hypotonic, especially in early childhood.
Infants tend to have retention of primitive reflexes, involuntary grimacing, a tendency to drool, and delayed psychomotor development.

24. DYSKINETIC SYNDROMES
The involuntary movements evolve with time and may not be apparent until the child is two to three years of age. Unlike spastic CP, contractures usually do not develop, unless they are positional.
Patients often have more than one form of involuntary movement, and the types may overlap in some cases. The extent of dysarthria and motor and intellectual disability are variable.

26. 3. ATAXIA
Although ataxia indicates an incoordination of cerebellar or sensory origin, ataxic CP represents a widespread disorder of motor function. Ataxia
The etiology of ataxic CP is heterogeneous. Most cases are caused by early prenatal events.

27. ATAXIA Most patients have congenital hypotonia.
Motor milestones and language skills typically are delayed. Ataxia usually improves with time.
Speech, which is related to intellectual ability, typically is slow, jerky, and explosive.
The diagnosis of ataxic CP is made by exclusion.

28. ASSOCIATED CONDITIONS
INTELLECTUAL DISSABILITY: 52% of patients. Severity correlates with the extent of motor handicap. Children with spastic quadriplegia are typically the most severely affected PSYCHIATRIC DISORDERS: emotional lability, poor attention and vigilance, and obsessive-compulsive traits

29. ASSOCIATED CONDITIONS
EPILEPSY: 45% of patients. Most common in patients with spastic quadriplegia and acquired hemiplegia. The onset of seizures is typically during the first two years of life. Partial seizures with secondary generalization are the most common type. Intellectual disability is more common in CP patients with seizures than in those without seizures

30. ASSOCIATED CONDITIONS
VISUAL DISORDERS: strabismus and refraction errors are the most common ones. Ambliopia and visual fields defects can also be present. Low visual acuity may be due to cortical impairment.

31. ASSOCIATED CONDITIONS
SPEECH IMPAIRMENT : aphasia and dysarthria (38%). Abnormal function of oropharyngeal muscles and lack of coordination of breathing patterns contribute.

32. ASSOCIATED CONDITIONS
HEARING IMPAIRMENT: 10-20%. It is most common in those with very low birthweight or severe hypoxic-ischemic insults. Sensorineural hearing loss is also a feature of CP caused by kernicterus.

33. ASSOCIATED CONDITIONS
GROWTH FAILURE: mostly due to poor nutrition.
Poor nutritional status is caused by both inadequate intake and
gastrointestinal abnormalities

34. ASSOCIATED CONDITIONS
PULMONARY DISEASE: leading cause of death. Poor coordination of respiratory muscles and chest deformity. Recurrent aspiration (reflux), restrictive disease (scoliosis).

35. ASSOCIATED CONDITIONS
ORTHOPEDIC DISORDERS: subluxation, dislocation, and progressive dysplasia of the hip, foot deformities, and scoliosis. Osteopenia.

36. ASSOCIATED CONDITIONS
URINARY DISORDERS: dysfunctional voiding symptoms, including enuresis, frequency, urgency, and stress incontinence.
Neurogenic bladder and incontinence.

37. DIAGNOSIS
Combination of findings, including motor delay, neurologic signs, persistence of primitive reflexes, and abnormal postural reactions.
Infants with an abnormal obstetric or perinatal history may be at increased risk to develop CP, and should be monitored closely.

38. DIAGNOSIS CLUES: Psychomotor delay
Abnormal oromotor or oculomotor patterns
Hypotonia/hypertonia
Hyperreflexia, clonus

39. DIAGNOSIS

40. DIFERENTIAL DIAGNOSIS
CP is a diagnosis of exclusion.
Neurodegenerative diseases
Inborn errors of metabolism
Neuromuscular or movement disorders
Neoplasm

41. TREATMENT Multidisciplinary treatment
Psychological development, communication, and education are priorities in the management .
Interventions should be directed to maximize the quality of life.

42. TREATMENT PT/OT Botulism toxin
Antispastic drugs (Dantrolene, benzos, Baclofen)
Surgery (muscle-tendon, hip)
Constraint-induced movement therapy (CIMT)
Gastrostomy feeding

43. TREATMENT
Motorized wheelchairs, switching devices used to activate communication systems, voice-activated computers
Braces
Psychological support

44. PROGNOSIS The majority of children with CP survive to adulthood.
Survival of adults with CP also depends upon the degree of disability.
The prognosis for survival and motor function in children with CP is highly variable, and depends on the severity and type.

45. PROGNOSIS
The prognosis for walking is poor in children who do not achieve head balance by 20 months, retain primitive reflexes or have no postural reactions by 24 months, or do not crawl by approximately five years of age. The prognosis for walking is good in children who sit by two years and crawl before 30 months of age.

46. REFERENCES
UP TO DATE
NELSON
PEDS IN REVIEW