CEREBRAL PALSY

1. CEREBRAL PALSY Reported By: FELVEE M. BASIBAS, PTRP MD

2. CEREBRAL PALSY • Three part definition: • A disorder of movement and posture • Caused by a non-progressive injury • To the immature brain • Change in muscle tone and posture, both at rest and with voluntary activity. • First year or two of life – included in most definitions • Upper age limit of postneonatal brain insult- unclear

3. CP: Epidemiology • One of the most common disabling conditions affecting children. • 1-2.3/1,000 live births • Diagnosis is not made at any specific age • 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.

5. CP: ETIOLOGY • Brain injury can occur in the following periods: • Prenatal • Perinatal • Postnatal • PRENATAL PERIOD- wherein most causes of CP occur. • TORCH infections • Intrauterine stroke • Genetic malformations • The most common currently understood causes are related to brain injury occurring in children born prematurely.

6. CP: ETIOLOGY Immature Fragile brain musculature Physical stresses of prematurity Compromised cerebral blood flow ( blood vessels in the water shed zone next to lateral ventricles in the capillaries of the germinal matrix)

7. Intraventricularhemorrhage • Bleeding is arterial in origin • Transfontanelle cerebral high-resolution UTZ

9. Very LBW infants – increased risk of periventricular hemorrhagic infarction • Periventricular hemorrhagic infarction • Hemorrhagic necrosis lateral to the external angle of the lateral ventricle. • Of venous origin • Usually asymmetric • With healing – Periventricular leukomalacia ( PVL) can develop. • PVL • One of the strongest predictors of CP in the premature neonate. • Almost always associated with a history of prematurity.

10. PVL

11. Extremely LBW infants: • Are also at increased risk of CP. • Especially with a history of resuscitation and prolonged ventilation. • Protective factors: prenatal care and steroids.

12. CP in TERM INFANTS • Almost ½ of children with CP were born term. • Cause of brain injury is often elusive. • Severe anoxic/ischemic brain injury: • Mechanical difficulties of the placenta • Umbilical cord • Actual delivery itself • Intrapartum asphyxia • Must be severe and prolonged to cause CP. • More global and more severe disability.

13. ETIOLOGY: ATHETOID CP • Injury to the basal ganglia. • Hyperbilirubinemia. • Hearing loss. • Incidence has declined since testing and treatment for Rh incompatibility • Now, relatively rare

14. ETIOLOGY: POSTNATAL CEREBRAL INJURY AND CP • Major causes: • CNS infections • Vascular causes • Head injury • Other Causes: • Anoxia • Ischemia • Inflammation

15. CLASSIFICATION of CP

18. SPASTIC SUBTYPE • Most common • 75% of children with CP • Spasticity: • Velocity-dependent increased tone • Associated with UMN syndrome findings • Increased muscle stretch reflexes • Babinski response • Weakness • Difficulty with coordination. • Can be associated with extensor or flexor posturing ( decerebrate and decorticate posturing)

19. DYSTONIA • A dyskinetic tone abnormality • With alternating tone or cocontraction in the antagonist and agonist muscle groups • Causing varied abnormal postures and often fluctuating tone. • Other dyskinetic forms: • Athetosis • Choreiform • Choreoathethoid

20. ATHETOSIS • Involuntary constant rotatory or writhing movements of the distal extremities. • Basal ganglia involvement. • Stop during sleep. • Incidence has been reduced by the advent of treatment for Rh incompatibility.

21. ATAXIC TYPE • Rare • Must be differentiated from degenerative processes of the cerebellum.

22. MIXED TYPE • Spasticity + Dystonia

23. HYPOTONIC TYPE • Needs to be differentiated from those with identifiable causes of neonatal hypotonia: • Muscle disease • Metabolic disorders • Genetic syndromes • Many of these children develop spastic or extrapyramidal-type disorders after the first few months of life.

24. CLINICAL EFFECTS

25. Hypotonia and motor delay- often seen as early signs of CP. • UMN injury features • Positive findings • Increased tone and reflexes • Positive Babinski’s reflex • Negative findings • Reduced strength • Selective motor control • Balance and coordination. • Tone in the extremities • Retention of Primitive Reflexes • Irritability • Lethargy • Weak suck with tongue thrust • Poor head control • High-pitched cry • Oral hypersensitivity • Tonic bite • Asymmetric movements • Unusual posturing

26. Rolling for mobility • Combat crawling • W sitting • Bunny hopping • Adopting a hand preference before the first birthday • Trunk or central hypotonia • Muscle weakness • Abnormal posturing • Sensory deficits • Contractures • Deformities • Kyphosis • Scoliosis • Respiratory compromise • Atlantoaxial instability • Foot deformities

27. ASSOCIATED MEDICAL AND FUNCTIONAL PROBLEMS

28. Visual Sequelae • Strabismus • Exotropia/ Esotropia • Hemianopsia • Retinopathy of prematurity • Hearing impairment • Ototoxic drug exposure • Hyperbilirubinemia • TORCH infections in utero

29. Abnormalities of oral motor function • Due to weakness and incoordination of lips, tongue and masticatory and facial muscles. • Drooling, dysphagia and dysarthria • Treatments: • Behavioral techniques • Speech therapy • Anticholinergic medications • Botulinium toxin A injections • Surgical redirection of the salivary ducts

30. Dysphagia • A problem in the following: • Oral Phase (chewing and bolus preparation) • Laryngeal Phase (propulsion and airway protection problems) • Aspiration - ischemia

31. Undernutrition/ Malnutrition • 1/3 of patients with Hemiplegia and Diplegia (undernourished) • > 2/3 of patients with Quadriplegia (undernourished) • 27% of patients – malnourished • Treatment: • Gastrostomy • Gastrojejunostomy tube

32. GI Symptoms: • Gastroesophageal reflux • Can result to episodic emesis • Interferes with adequate nutrition and growth • Treatment: • Medications • Surgical: Nissenfundoplication or Jejunostomy

33. Chronic constipation • Neuromuscular control of the bowel • Exaggerated by immobility and abnormal diet and fluid intake • Anorectal manometry • Complications: • Large bowel megacolon • Volvulus • Treatment: • Increase activity • Increase fluid and fiber intake medications

34. Urinary Symptoms: • 1/3 of patients – frequency, incontinence or difficulty urinating. • Detrussor instability, vesicoureteric reflux, DSD

35. Cognitive Impairments: • 30% of patients- mental retardation • Risk is directly proportional to severity of motor disability. • 20-30% - have specific learning disabilities • 40-50% of Normal BW CP- cognitive abnormalities

36. Seizure Disorders • 1/3 of children with CP • Hemiplegic> Quadri > Diplegic • Reflects a greater extent of cortical brain injury • Treatment: • Antiseizure techniques • Grid mapping and resection of seizure foci.

37. Osteoporosis • Secondary to the following factors: • Feeding difficulties – deficient Ca and Vit D • Decreased weight bearing/ Immobilization • Muscle stresses • Antiseizure meds • Weight percentile/ Low triceps skinfold • Treatment: • Ca and Vit D supplementation • Bisphosphonates (Pamindronate) • Bone age was not found to be significantly different than chronologic age in children with CP

38. Pain • Can go undiagnosed and untreated in patients who are non-verbal. • Increased in patients in GMFCS Levels 3-5 • Frequency is correlated with the following: • Severity of motor impairment • Presence of gastrostomy • Days of schools missed • Days spent in bed

39. Mortality • Risk factors: • Lack of independent mobility such as rolling • Use of tracheostomy • Lack of any hand function • Prevention: Teach basic life skills (feeding and mobility) • Predictors of survival: • Cognitive level • Ability to speak • Ability to recognize voices • Ability to interact with persons • Physical activity and mobility • Tube feeding • Incontinence • Seizures

40. Morbidity • Injuries, abuse and neglect

41. FUNCTIONAL PROGNOSIS

42. Functional Prognosis • Children typically develop motor skills craniocaudally. • The age at which these skills are developed help to predict the eventual outcome. • 75% of spastic CP eventually ambulate • 85% in diplegics • 70% in quadriplegics

43. PROGNOSIS for AMBULATION GOOD Prognosis POOR Prognosis • Hemiplegics/ Ataxic pxs • Achievement of all motor skills by age of 8. • Independent sitting before 2 years • Persistence of fewer than 3 of the primitive reflexes at age 18 months. • Quadriplegics • Did not attain independent sitting by age 4. • Persistence of primitive reflexes beyond 18 months

44. Character • Inefficient gait • Shorter step length • Decreased ROM of the hips and knees • Increased energy expenditure • Decreased velocity • Decreased endurance

45. OUTCOME MEASURES

47. Ashworth Scale The Modified 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. 0. No increase in muscle tone 1. Slight increase in tone with a catch and release or minimal resistance at end of range 2. As 2 but with minimal resistance through range following catch 3. More marked increase tone through ROM 4. Considerable increase in tone, passive movement difficult. 5. Affected part rigid

50. THERAPEUTIC MANAGEMENT