Cerebral Palsy

1. Cerebral Palsy One Size Does Not Fit All Mary Catherine Brake Turner, MD, FACP, FAAP brakem@ecu.edu

2. Objectives • Define cerebral palsy • List systems often affected by cerebral palsy • List three non-surgical treatments for spasticity • Name common causes of pain in cerebral palsy • List three main roles of the primary care provider

3. Agenda • Review cerebral palsy and the complexities that accompany this diagnosis • Highlight special considerations for patients with cerebral palsy • Review the role of the medical home • Discuss important transition issues as patients with cerebral palsy become adults

5. Definition of CP • A group of permanent disorders of movement and posture that limit activity • Non-progressive • Insult to the developing brain • Disturbances of sensation, perception, cognition, communication, and behavior • Epilepsy and secondary MSK problems common

6. Assessment for Intervention • Diagnosis is suspected by PCP • Classify based on localization and type • Assessment of associated impairments • Overall severity

7. Type of Motor Disorder • Spasticity • Dyskinesia (dystonia and choreoathetosis) • Ataxia • Hypotonia

8. Localization • Diplegia: Lower extremities >> upper extremities • Quadriplegia: Upper and lower extremities are affected equally • Hemiplegia: 1 side more involved than its opposite counterpart

9. Functional Motor Abilities • Gross motor – ambulation • Fine motor – self-help skills • Oromotor and speech – communication, eating and drinking

11. Gross Motor Function Classification System for Cerebral Palsy (GMFCS) • Level I – Speed, balance and coordination are limited • Level II- Minimal ability to perform gross motor skills such as running and jumping • Level III – May ambulate with assistive devices • Level IV – Children may achieve self-mobility using a power wheelchair • Level V – All areas of motor function are limited, no means of independent mobility

12. Risk Factors for Development of CP • Chorioamnionitis • Birth weight <2000 gm • Intracranial hemorrhage • Newborn encephalopathy • Periventricularleukomalacia • Hydrocephalus • Congenital malformations

14. Relevance to Us • All PCPs will encounter children with cerebral palsy in their practice • Prevalence of 3.6 per 1000 • More than 100,000 children in the US are affected • More than 90% of children with severe disabilities survive to adulthood • We will see them for health maintenance, care coordination, and acute visits

15. Case • 30 yoM, former 26 week preemie, with CP, GMFCS Level V, mental retardation, seizure disorder, VP shunt, feed formula by a bottle • His PCP is a pediatrician, they live 1 hour away • This pediatrician has referred the patient to see me due to weight loss.

16. What nutritional issues may arise in patients with cerebral palsy? • Malnutrition • Obesity • Vitamin D deficiency • Gastro-esophageal reflux • All of the above

17. Growth/Nutrition • Affected by dysphagia, GERD, delayed gastric motility, constipation • May have to rely on gastrostomy or jejunostomy tubes • +/- fundoplication

18. Growth Charts for CP • Special growth charts are available for CP • Limitation is charts are not standards for ALL pts • Recommend WHO birth - 2 yrsand CDC 2 yrsup • Objective of plotting is to monitor trends • Z-scores: variation from the reference and from each child’s own growth pattern

19. Growth/Nutrition • Protein (grams/kg) • Based on actual weight, DRI • Hydration • Obviously essential, helps reduce constipation • Holliday-Segar method: 100, 50, 20; based on wt • Calories • Calculated per the BMR

20. Nutrition • WHO (basal needs: BMR)[W = weight (kg)]Age (yrs) Gender Equation0-3 Male 60.9W-54 Female 61W-513-10 Male 22.7W+495 Female 22.5W+49910-18 Male 17.5W+651 Female 12.2W+746Gevena, 1985

21. Height based method • 14.7 cal/cm in children without motor dysfunction • 13.9 cal/cm in ambulatory patients with motor dysfunction • 11.1 cal/cm in non-ambulatory patients • Use arm span to estimate height

22. Growth/Nutrition • Micronutrients • If formula is <1L/day for adolescents/adults, will need to add MVI • Consider monitoring vitamin D status

23. What nutritional issues may arise in patients with cerebral palsy? • Malnutrition • Obesity • Vitamin D deficiency • Gastro-esophageal reflux • All of the above

24. What nutritional issues may arise in patients with cerebral palsy? • Malnutrition • Obesity • Vitamin D deficiency • Gastro-esophageal reflux • All of the above

25. Nutrition Explanation • Malnutrition due to decreased ability to take in adequate calories • Obesity can also be an issue due to poor mobility and overfeeding via gastric tube. • Poor exposure to sunlight • GERD common in CP

26. Drooling • Treatment options include: • Decorative scarves and bibs • Glycopyrrolate – risk for mucous plugs • Atropine Drops – local effect • Scopolamine patch • Botulinum toxin injections – expensive procedure • Removal of salivary glands – permanent, not recommended

27. Swallowing • Children with CP often struggle with oral and/or pharyngeal dysphagia • Diagnose formally with a swallow study with radiology and speech pathology • Treatment may include use of Thick-It or oatmeal thickener, or reliance solely on gastrostomy tube

28. Case • 3 yoF with spastic quadriplegic CP is admitted with fever and increased WOB, no increased seizures, tolerating feeds well by g-tube, her mother has been feeding her stage III foods by mouth, she has history of a Nissenfundoplication.

30. What diagnostic procedure will likely help determine cause of her respiratory distress? • Video Swallow study • CT scan of the chest • Sputum for AFB • Gastric emptying study

31. Respiratory • Aspiration (primary or secondary) • Upper airway obstruction • Infections (poor pulmonary clearance) • Restrictive lung disease (scoliosis)

32. Respiratory • Pulmonary clearance techniques may include chest percussion, cough assist, VEST therapy all with the use of bronchodilator therapy • May develop OSA or central sleep apnea • Over time may progress to need for trach and vent if severe chronic lung disease

33. What diagnostic procedure will likely help determine cause of her respiratory distress? • Video Swallow study • CT scan of the chest • Sputum for AFB • Gastric emptying study

34. What diagnostic procedure will likely help determine cause of her infection? • Video Swallow study • CT scan of the chest • Sputum for AFB • Gastric emptying study

36. Case • 5 yoM with history of failure to thrive, had g-tube placed one year ago, no fundoplication, no PPI therapy, minimal weight gain since then, transferred to Vidant Medical Center from a regional hospital for intolerance of bolus G-tube feeds and intermittent coffee ground emesis. MGM reports he has intermittent emesis for past year.

37. What work up would you pursue next to evaluate this patient’s failure to thrive and feeding intolerance? • Dental evaluation • Reflux and gastric emptying study • Plain abdominal films • Plot him on the CP growth chart, determine he is still on the curve, reassure parents • All of the above • None of the above • B and C

38. GI • Reflux • Positioning upright • H2 or PPI therapy • Fundoplication • Constipation • Hydration and fiber • Scheduled miralax • Suppositories

39. GI • Delayed gastric motility • Slow rate of feeds • EES • Reglan • Pyloroplasty

40. What work up would you pursue next to evaluate this patient’s failure to thrive and feeding intolerance? • Dental evaluation • Reflux and gastric emptying study • Plain abdominal films • Plot him on the CP growth chart, determine he is still on the curve, reassure parents • All of the above • None of the above • B and C

41. What work up would you pursue next to evaluate this patient’s failure to thrive and feeding intolerance? • Dental evaluation • Reflux and gastric emptying study • Plain abdominal films • Plot him on the CP growth chart, determine he is still on the curve, reassure parents • All of the above • None of the above • B and C

42. When considering treatment for spasticity, which of the following is not considered a treatment goal? • Reduce muscle spasms • Improve functional ability • Reduce pain • Improve hygiene • Prevent tissue injury • Prevent hip migration • Improve cognitive functioning

43. Modified Ashworth Scale. Blackburn M et al. PHYS THER 2002;82:25-34 Physical Therapy

44. Spasticity • PT, ROM exercises • Enhance skill development, delay contractures • Time required to perform • Orthotics • To improve function, prevent contractures • Possibility of pressure sores or muscle wasting • Systemic medications • Diazepam, baclofen, tizanidine, dantrolene • Decrease pain and muscle spasms • Sedation is adverse side effect

45. Spasticity • Botulinum toxin • Improve pain, improve function, help with hygiene • 2-3 primary muscle groups • Wanes after 3 months • Intrathecalbaclofen pump • No central effect of sedation • Device complication • Dorsal Rhizotomy • Permanent • Improves ambulation for spastic diplegics

46. Hip Dysplasia • Pain arising from the hip • Clinically important leg length difference • Deterioration in ROM of hip • Increasing hip muscle tone • Deterioration in sitting or standing • Increasing difficulty with perineal care or hygiene

47. MSK issues requiring Orthopedics • Contractures • Tendon clipping • Hip dislocation • Surgical stabilization • Scoliosis • Surgical repair

48. When considering treatment for spasticity, which of the following is not considered a treatment goal? • Reduce muscle spasms • Improve functional ability • Reduce pain • Improve hygiene • Prevent tissue injury • Prevent hip migration • Improve cognitive functioning

49. When considering treatment for spasticity, which of the following is not considered a treatment goal? • Reduce muscle spasms • Improve functional ability • Reduce pain • Improve hygiene • Prevent tissue injury • Prevent hip migration • Improve cognitive functioning