CEREBRAL PALSY:
Classification and Motor
Manifestations
Michael J. Ward, MD
Michael J. Ward, MD
Associate Professor, CHS
Department of Orthopedics and Rehabilitation Medicine
University of Wisconsin Medical School
I have no relevant financial or
nonfinancial relationships to disclose.
CEREBRAL PALSY: 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
CEREBRAL PALSY: DEFINITION
Includes many types of pathology affecting the
developing brain:
White matter injury associated with prematurity
Congenital malformations
Gray matter injury from vascular events
Basal ganglia injury: toxic etiology
Diffuse injury due to hypoxia or infection
CEREBRAL PALSY: DEFINITION
Full description of Cerebral Palsy includes, when
possible:
Motor impairment pattern
Functional level: gross and fine motor, speech
Etiology
Brain imaging findings
Associated conditions
CEREBRAL PALSY:
Types of motor problem
diplegic
quadriplegic
hemiplegic
Spastic types most common, and described by
distribution of motor control deficits
– Diplegic: Both legs more impaired than both arms
– Quadriplegic: Both arms and both legs
– Hemiplegic: Arm and leg both affected on one side
CEREBRAL PALSY
Types of motor problem
Dyskinetic types defined by movement quality:
Dystonia: Sustained torsional movements
Athetosis: Writhing and variable movments
Chorea: Twitchy, unsustained movments
CEREBRAL PALSY:
Types by motor pattern
Extrapyramidal
Other
Diplegic
Quadriplegic
Hemiplegic
CEREBRAL PALSY
Severity of motor problem: GMFCS
Gross Motor Functional Classification System
Level I:
Level II:
Level III:
Level IV:
Level V:
Walks without limitations
Walks with limitations
Ambulation with device only
Limited mobility, power wheelchair
Dependent manual wheelchair
GMFCS
Cerebral palsy: Etiologies
Incidence of CP is NOT decreasing over time…
but underlying etiologies are shifting.
Therefore, manifestations are different by age group.
CP: Etiology trends
Hyperbilrubinemia (jaundice)
High bilirubin toxic to basal ganglia
Common cause of choreoathetoid CP
in past decades
Caused by rH factor incompatibility
CP: Etiology trends
Hyperbilrubinemia (jaundice)
RhoGAM introduced in 1968
Kernicterus incidence decreased
“My Left Foot”
Many individuals with choreoathetosis are 50-80 yrs old,
and this pattern much less common now
CP: Etiology trends
Emergence of modern NICU 1961
Smaller birth weight survivable.
Correlated with germinal
matrix hemorrhage acutely,
and later white matter disease.
Currently prematurity and VLBW with associated
white (gray) matter disease is currently the
largest single underlying etiology for CP.
MRI with Periventricular leukomalacia
Normal brain
PVL
CP: Etiology trends
Children with prematurity and PVL present with:
46%
33%
14%
6%
spastic diplegia
spastic hemiplegia
spastic quadriplegia
other types
Note: Can have white matter lesion without CP
-Arnfeld Research in Dev Peds 2013
Cerebral Palsy:
Cranial imaging findings
PVL
Gray matter
Basal ganglia
Malformation
Miscellaneous
Normal
Bax JAMA 2006
Combining information for a
multidimensional description of CP:
Example:
Type:
Distribution:
Etiology:
MRI Imaging:
GMFCS:
Associated:
Spastic
Quadriplegic
VLBW and premature twin birth
Periventricular leukomalacia
V
Cognitive, visual, orthopedic, etc.
Congenital spastic hemiplegia
Motor impairment in one arm and one leg
MRI findings divided between prenatal vascular
events (AKA stroke or gray matter lesion) and
periventricular leukomalacia
MRI abnormal in 88% overall
PVL
34%
Focal infarct
27.5%
(Korzenskiewski JCN 2008)
(Bax JAMA 2006)
(Bax JAMA 2006)
Congenital spastic hemiplegia
Usually recognized by asymmetric limb use and
tone, more than by motor delay
Motor level usually GMFCS I
Ambulation typically
independent by 18 months
Variable upper limb dysfunction
Stable long term motor function
Congenital spastic hemiplegia
Frequently use botulinum toxin to improve joint
motion early in life
Upper limb issue with underutilization and potential
use of constraint therapy to “force” use
Frequently have upper limb functional orthopedic and
surgical release for ankle
Congenital spastic hemiplegia
Best example of CNS plasticity
If language areas of the left brain injured, system
moves to mirror areas on the right brain
BUT:
Relative impairment of right hemisphere function and
nonverbal learning disability seen with both right
and left congenital hemiplegia
Congenital spastic hemiplegia
Left hemisphere damage associated with dyspraxia,
often with catch-up in speech by age 5.
Paradoxically, right hemisphere damage can create
more severe language delays.
In either situation, language functions are relatively
preserved at the sacrifice of right hemisphere
function.
Plasticity is not a free lunch
-Aram and Eisele Neuropsychologia 1994
Spastic diplegia
Motor impairment in both legs
Strong association with
prematurity and with
periventricular leukomalacia:
71% on MRI
(Bax JAMA 2006)
Spastic diplegia
Motor delays evident in first year of life
Ambulation related to motor milestones: GMFCS I-III
Pattern of extensor tone and scissor adduction typical
in the lower limbs
Milder upper limb impairment
Minimal oral motor impairment
Spastic diplegia
Children in this group receive a wide range of
interventions for motor systems
Early in life, botulinum toxin and oral medications for
spasticity can be used to improve movement patterns
Spastic diplegia
Ambulatory children with good strength can benefit
from selective dorsal rhizotomy procedure:
Neurosurgical intervention, open spine
Partial cutting of lumbar spinal sensory roots
Permanent reduction in spasticity to LE only
Many have extensive orthopedic surgeries as well
Spastic diplegia
Prematurity, low birth weight and periventricular
leukomalacia also associated with impairment in:
Attention
Visual perceptual skills
Nonverbal learning disability
Spastic quadriplegia
Motor abnormality of all four limbs and trunk
MRI abnormality of PVL is most common 35%
Others have very diffuse brain damage or malformation
Often associated with term birth and perinatal hypoxia
(Bax JAMA 2006)
Spastic quadriplegia
Extensive difficulties with head control and oral
motor control
Commonly also have dystonia
Severe intellectual impairment can be seen with the
hypoxic group, but can also have normal IQ,
NVLD, or ADD with, like the diplegic group
Spastic quadriplegia
Variable long term function with more frequent
declines in motor function as well as general health
as adults
Substantially increased mortality rates through the life
span (especially for those with declines in oral
feeding)
Spastic quadriplegia
Motor interventions are extensive.
Most use a wheelchair: GMFCS IV-V
Orthopedic surgeries common for hips, scoliosis.
Medical treatment for tone with
botulinum toxin,
oral medications,
and intrathecal medication
through pump implantation
Dyskinetic
Underlying injury shifted from hyperbilirubinemia
at term delivery to the complex neonatal case with
multifactorial injury, usually including hypoxic
injury to the basal ganglia.
75% of the MRI studies
with basal ganglia
manifest as dystonic CP
(Bax JAMA 2006)
Dyskinetic
Motor disability changes over time
Children are often hypotonic at birth
Movement disorder becomes more evident over
several years in some cases, as brain myelination
progresses
Movement disorder can also get worse in adults,
estimate about one third of cases
Dyskinetic
Motor symptoms are very difficult to treat
Typically minor benefits from oral medications or
botulinum toxin injections
Intrathecal baclofen pump more helpful
Implanted pump system
Profound reduction in dystonia
ITB can positively impact communication skills
Dyskinetic
Can have preservation of cognitive abilities with
extensive motor disabilities
Stability of language skills, but not necessarily
speech or swallow skills
Dystonia can also be associated with perinatal
hypoxia and severe intellectual impairment