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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
CEREBRAL PALSY: An Integrated Approach
Michael J. Ward, MDAssociate Professor, CHS
Orthopedics and Rehabilitation MedicineUniversity of Wisconsin Medical School
March 1, 2014
Cerebral Palsy: An Integrated Approach
Integrated Whole Person PerspectiveIntegrated Treatment Team PerspectiveIntegrated Medical Approach to Problems
MULTIDISCIPLINARY TEAMMD: Rehabilitation,
Developmental PediatricsNursing
PT, OT , Speech Evaluations
Community resources:Family, School,
Equipment vendor
AFCH specialists:Orthopedic Surgery,Neuropsychology,
Neurology
Neurosurgery, Audiology, Feeding,
Social Work, Psychology, CASC
WHAT IS CEREBRAL PALSY?
?
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
WHAT IS CEREBRAL PALSY?
Diagnosis of Cerebral Palsy has 4 requirements:1. Non-progressive impairment 2. Immature or developing3. Brain (cerebral)
4. Abnormal motor development (palsy)
DIAGNOSIS: Non-progressive
Excludes conditions which cause ongoing brain injury over time
Also excludes conditions which resolveHowever, symptoms can transform through the life
span even when the primary brain injury remains the same
CP is non-progressive, but not unchanging
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
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
DIAGNOSIS: Brain impairment
Excludes diseases of spinal cord or muscles, etc.
Includes many different types of brain injuries
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
DIAGNOSIS: Brain injury
Prematurity and low birth weight often associated with a brain change called PVL:
Periventricular leukomalacia
Peri = aroundVentricular = deep brain fluid spacesLeuko = white matterMalacia = thinning
DIAGNOSIS:MRI with Periventricular leukomalacia
Normal brain PVL
DIAGNOSIS: Etiology
Includes a range of other types of brain injury:
Birth hypoxia Brain malformationPrenatal stroke EncephalitisHyperbilirubinemia Other
Can be caused by a combination of factorsOccasionally the factors are not known
Cerebral Palsy: Cranial imaging findings
PVLGray matterBasal gangliaMalformationMiscellaneousNormal
Bax JAMA 2006
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
DIAGNOSIS: Disturbance of motor developmentCP 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
DIAGNOSIS Disturbance of motor developmentCP is usually described by type of motor problem
Other types:– Dystonic– Dyskinetic (choreoathetosis)– Ataxic
DIAGNOSIS: Types by motor pattern
ExtrapyramidalOtherDiplegicQuadriplegicHemiplegic
DIAGNOSIS: Disturbance of motor developmentThere is partial correlation between etiology and type
of motor problem:
MRI abnormality Motor problemPVL DiplegiaBirth Hypoxia Quadriplegia and dystoniaPrenatal stroke Hemiplegia
DIAGNOSIS:MRI with Periventricular leukomalacia
Normal brain PVL
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
CEREBRAL PALSY Associated concerns
Cognitive– Cognitive impairment 40-60%– Learning disabilities common– Attention deficit disorder– Other behavioral disturbances– Language disorders
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
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
CEREBRAL PALSY Associated concerns
Secondary problems: Gastrointestinal– Malnutrition– Growth delays– Gastric reflux– Constipation– Swallowing difficulties– Drooling– Dental changes
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
PROGNOSIS
WILL MY CHILD ?????????
Related to underlying etiologyRelated to motor, cognitive and sensory abilities
Risks v absolutes in early periodRequires serial discussions
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
MOTOR DELAYS:
GMFCS: Gross Motor Classification Systemfor mobility
MACS: Manual Abilities Classification Systemfor hand function
CFCS: Functional Communication Scalefor speech
All describe 5 functional ability levels
MOTOR DELAYS: GMFCS
Level I: Walks without limitationsLevel II: Walks with limitationsLevel III: Ambulation with device onlyLevel IV: Limited mobility, power wheelchairLevel V: Dependent manual wheelchair
GMFCS
MOTOR DELAYS:REHABILITATION INTERVENTIONS
Physical therapyOrthopedic surgerySpasticity reductionCasting/splintingBracingMobility aids
Help but do not change the GMFCS level (usually)
Combining all of this provides a more complete description of CP:
Type: SpasticDistribution: QuadriplegicEtiology: VLBW and prematurityMRI Imaging: Periventricular leukomalaciaFunctioning: GMFCS V, MACS IV, CFCS IIIAssociated: Cognitive, visual, orthopedic,
etc.
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
WHAT IS THE MOST COMMON MEDICAL PROBLEM
ADDRESSED WITH CHILDREN WHO HAVE CEREBRAL PALSY?
?
CONSTIPATION:Contributing factors
Poor hydration, poor hydration, poor hydrationPoor dietary fiber intakeImpaired GI motilityBehavioral/developmental levelPhysical access to toilet, safe sitting positionSensory processing
CONSTIPATION:Treatment approaches
Fluids, fluids, fluidsIncreased dietary fiberSwallow abilities and feeding behaviors importantOral or rectal medicationsBathroom access and support on the toiletBehavioral approaches to toileting based on cognitive abilities and developmental level
MULTIDISCIPLINARY TEAMMD: Rehabilitation,
Developmental PediatricsNursing
PT, OT , Speech Evaluations
Community resources:Family, School,
Equipment vendor
AFCH specialists:Orthopedic Surgery,Neuropsychology,
Neurology
Neurosurgery, Audiology, Feeding,
Social Work, Psychology, CASC
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
TEAM SUPPORT ACROSS THE LIFESPAN
Newborn Follow-up Clinic: child at riskFeeding Clinic, Audiology, Resource center
Neuromotor Development Clinic: child with delayOrthopedic Surgery, Neurology, Genetic Evaluations
Cerebral Palsy Clinic: child with disabilitySpasticity and Movement Disorder clinic, CASC
Transition to adult providersDVR, Guardianship, Independent Living
Cerebral Palsy: An Integrated Approach
Integrated Whole Person PerspectiveIntegrated Treatment Team PerspectiveIntegrated Medical Approach to Problems
