CEREBRAL PALSY: An Integrated Approach

1. CEREBRAL PALSY: An Integrated Approach Michael J. Ward, MD Associate Professor, CHS Orthopedics and Rehabilitation Medicine University of Wisconsin Medical School March 1, 2014

2. Cerebral Palsy: An Integrated Approach Integrated Whole Person Perspective Integrated Treatment Team Perspective Integrated Medical Approach to Problems

3. MULTIDISCIPLINARY TEAM

4. WHAT IS CEREBRAL PALSY? ?

5. Modern consensus definition: • Group of disorders of movement and posture • Non-progressive etiology • Damage to the fetal or infant brain • Often accompanied by co-occurring problems with sensation, perception, communication, and/or behavior and/or seizure disorder Bax 2005 DMCN

6. WHAT IS CEREBRAL PALSY? Diagnosis of Cerebral Palsy has 4 requirements: 1. Non-progressive impairment 2. Immature or developing 3. Brain (cerebral) 4. Abnormal motor development (palsy)

7. DIAGNOSIS: Non-progressive Excludes conditions which cause ongoing brain injury over time Also excludes conditions which resolve However, symptoms can transform through the life span even when the primary brain injury remains the same CP is non-progressive, but not unchanging

8. DIAGNOSIS: Immature or developing brain When does development end? • Embryonic formation of organs • Birth • 2-3 years: Brain myelination completed • 7-9 years: Maturation of motor skills • 16-18 years: Physical maturity • Social maturity Injury causing CP occurs before or around birth

9. DIAGNOSIS: Immature brain Presentation of symptoms in CP: • Typically by 6-12 months • Mild cases may not be noticed until 12-18 months -Early abnormal motor signs in infants can disappear and would not be called CP

10. DIAGNOSIS: Brain impairment Excludes diseases of spinal cord or muscles, etc. Includes many different types of brain injuries

11. DIAGNOSIS: Brain injury Most common source of injury: Complex series of events in the brain set in motion after birth among newborns with prematurity and very low birth weight Currently largest single etiology of cerebral palsy

12. DIAGNOSIS: Brain injury Prematurity and low birth weight often associated with a brain change called PVL: Periventricular leukomalacia Peri = around Ventricular = deep brain fluid spaces Leuko = white matter Malacia = thinning

13. DIAGNOSIS:MRI with Periventricularleukomalacia Normal brain PVL

14. DIAGNOSIS: Etiology Includes a range of other types of brain injury: Birth hypoxia Brain malformation Prenatal stroke Encephalitis Hyperbilirubinemia Other Can be caused by a combination of factors Occasionally the factors are not known

15. Cerebral Palsy: Cranial imaging findings Bax JAMA 2006

16. DIAGNOSIS: Disturbance of motor development Presenting motor symptoms also vary • Delayed motor milestones: not required • Spasticity: common but not required • Abnormal involuntary movements • Decreased quality of motor control

17. DIAGNOSIS: Disturbance of motor development CP is usually described by type of motor problem Spastic types most common, and described by distribution • Quadriplegic: both arms and both legs • Hemiplegic: Arm and leg on both sides • Diplegic: Both legs more impaired than both arms

18. DIAGNOSIS Disturbance of motor development CP is usually described by type of motor problem Other types: • Dystonic • Dyskinetic (choreoathetosis) • Ataxic

19. DIAGNOSIS: Types by motor pattern

20. DIAGNOSIS: Disturbance of motor development There is partial correlation between etiology and type of motor problem: MRI abnormality Motor problem PVL Diplegia Birth Hypoxia Quadriplegia and dystonia Prenatal stroke Hemiplegia

21. DIAGNOSIS:MRI with Periventricularleukomalacia Normal brain PVL

22. DIAGNOSIS: Disturbance of motor development Required for diagnosis The definition is mute on sensory, cognitive, or behavioral dysfunction, but… CP is not an exclusively motor condition

23. CEREBRAL PALSY Associated concerns Cognitive • Cognitive impairment 40-60% • Learning disabilities common • Attention deficit disorder • Other behavioral disturbances • Language disorders

24. CEREBRAL PALSY Associated concerns Sensory abnormalities: • Hearing loss 7-12% • Abnormal control of eye motions 20-60% • Visual impairment overall 80% • Visuoperceptual abnormality also frequent • Tactile impairment 50-75% • Balance system impairment

25. CEREBRAL PALSY Associated medical concerns Seizures 30-50% Autonomic nervous system also affected: • Abnormal digestive motility • Temperature instability and cold or hot limbs • Bladder dysfunction • Breathing irregularities

26. CEREBRAL PALSY Associated concerns Secondary problems: Gastrointestinal • Malnutrition • Growth delays • Gastric reflux • Constipation • Swallowing difficulties • Drooling • Dental changes

27. CEREBRAL PALSY Associated concerns Many orthopedic complications: • Osteoporosis and fractures (even in children) • Scoliosis • Joint deformities • Musculoskeletal pain Associated skin problems • Skin pressure ulcers • Moisture related skin problems

28. PROGNOSIS WILL MY CHILD ????????? Related to underlying etiology Related to motor, cognitive and sensory abilities Risks v absolutes in early period Requires serial discussions

29. MOTOR DELAYS: GMFCSGross Motor Classification System Track curves of motor development in children with CP from early milestones to adult skills achievement. Predicts general trends at 5 functional levels

30. MOTOR DELAYS: GMFCS: Gross Motor Classification System for mobility MACS: Manual Abilities Classification System for hand function CFCS: Functional Communication Scale for speech All describe 5 functional ability levels

31. MOTOR DELAYS: GMFCS Level I: Walks without limitations Level II: Walks with limitations Level III: Ambulation with device only Level IV: Limited mobility, power wheelchair Level V: Dependent manual wheelchair

32. GMFCS

33. MOTOR DELAYS:REHABILITATION INTERVENTIONS Physical therapy Orthopedic surgery Spasticity reduction Casting/splinting Bracing Mobility aids Help but do not change the GMFCS level (usually)

34. Combining all of this provides a more complete description of CP: Type: Spastic Distribution: Quadriplegic Etiology: VLBW and prematurity MRI Imaging: Periventricular leukomalacia Functioning: GMFCS V, MACS IV, CFCS III Associated: Cognitive, visual, orthopedic, etc.

35. Modern consensus definition: • Group of disorders of movement and posture • Non-progressive etiology • Damage to the fetal or infant brain • Often accompanied by co-occurring problems with sensation, perception, communication, and/or behavior and/or seizure disorder Bax 2005 DMCN

36. WHAT IS THE MOST COMMON MEDICAL PROBLEM ADDRESSED WITH CHILDREN WHO HAVE CEREBRAL PALSY? ?

37. CONSTIPATION:Contributing factors Poor hydration, poor hydration, poor hydration Poor dietary fiber intake Impaired GI motility Behavioral/developmental level Physical access to toilet, safe sitting position Sensory processing

38. CONSTIPATION:Treatment approaches Fluids, fluids, fluids Increased dietary fiber Swallow abilities and feeding behaviors important Oral or rectal medications Bathroom access and support on the toilet Behavioral approaches to toileting based on cognitive abilities and developmental level

39. MULTIDISCIPLINARY TEAM

40. TEAM SUPPORT ACROSS THE LIFESPAN Newborn Follow-up Clinic: child at risk Neuromotor Development Clinic: child with delay Cerebral Palsy Clinic: child with disability Transition to adult providers

41. TEAM SUPPORT ACROSS THE LIFESPAN Newborn Follow-up Clinic: child at risk Feeding Clinic, Audiology, Resource center Neuromotor Development Clinic: child with delay Orthopedic Surgery, Neurology, Genetic Evaluations Cerebral Palsy Clinic: child with disability Spasticity and Movement Disorder clinic, CASC Transition to adult providers DVR, Guardianship, Independent Living

42. Cerebral Palsy: An Integrated Approach Integrated Whole Person Perspective Integrated Treatment Team Perspective Integrated Medical Approach to Problems