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CEREBRAL PALSY: An Integrated Approach
Michael J. Ward, MD Associate Professor, CHS Orthopedics and Rehabilitation Medicine University of Wisconsin Medical School March 1, 2014
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Cerebral Palsy: An Integrated Approach
Integrated Whole Person Perspective Integrated Treatment Team Perspective Integrated Medical Approach to Problems
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MULTIDISCIPLINARY TEAM
MD: Rehabilitation, Developmental Pediatrics Nursing PT, OT , Speech Evaluations Community resources: Family, School, Equipment vendor AFCH specialists: Orthopedic Surgery, Neuropsychology, Neurology Neurosurgery, Audiology, Feeding, Social Work, Psychology, CASC
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WHAT IS CEREBRAL PALSY? ?
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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
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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)
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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
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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
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DIAGNOSIS: Immature brain
Presentation of symptoms in CP: Typically by 6-12 months Mild cases may not be noticed until months -Early abnormal motor signs in infants can disappear and would not be called CP
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DIAGNOSIS: Brain impairment
Excludes diseases of spinal cord or muscles, etc. Includes many different types of brain injuries
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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
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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
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DIAGNOSIS: MRI with Periventricular leukomalacia
Normal brain PVL
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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
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Cerebral Palsy: Cranial imaging findings
Bax JAMA 2006
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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
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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
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DIAGNOSIS Disturbance of motor development
CP is usually described by type of motor problem Other types: Dystonic Dyskinetic (choreoathetosis) Ataxic
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DIAGNOSIS: Types by motor pattern
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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
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DIAGNOSIS: MRI with Periventricular leukomalacia
Normal brain PVL
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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
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CEREBRAL PALSY Associated concerns
Cognitive Cognitive impairment 40-60% Learning disabilities common Attention deficit disorder Other behavioral disturbances Language disorders
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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
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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
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CEREBRAL PALSY Associated concerns
Secondary problems: Gastrointestinal Malnutrition Growth delays Gastric reflux Constipation Swallowing difficulties Drooling Dental changes
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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
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PROGNOSIS WILL MY CHILD ????????? Related to underlying etiology
Related to motor, cognitive and sensory abilities Risks v absolutes in early period Requires serial discussions
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MOTOR DELAYS: GMFCS Gross 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
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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
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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
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GMFCS
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MOTOR DELAYS: REHABILITATION INTERVENTIONS
Physical therapy Orthopedic surgery Spasticity reduction Casting/splinting Bracing Mobility aids Help but do not change the GMFCS level (usually)
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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.
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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
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WHAT IS THE MOST COMMON MEDICAL PROBLEM ADDRESSED WITH CHILDREN WHO HAVE CEREBRAL PALSY?
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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
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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
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MULTIDISCIPLINARY TEAM
MD: Rehabilitation, Developmental Pediatrics Nursing PT, OT , Speech Evaluations Community resources: Family, School, Equipment vendor AFCH specialists: Orthopedic Surgery, Neuropsychology, Neurology Neurosurgery, Audiology, Feeding, Social Work, Psychology, CASC
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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
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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
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Cerebral Palsy: An Integrated Approach
Integrated Whole Person Perspective Integrated Treatment Team Perspective Integrated Medical Approach to Problems
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