Ch 19 Cerebral Palsy Flashcards
CP is a
- neurodevelopmental motor condition
- compromised posture, balance, muscle control, movement
- disturbances of sensation, perception, cognition, communication, behavior by epilepsy and musculoskeletal problems
Is CP a permanent disorder?
yes
Is CP progressive or non progressive
non progressive
Where did CP occur?
developing fetus or infant brain
occurs early in the brain development
Onset of CP?
before 2 or 3 years
How is CP diagnosed
formal neurodevelopmental exam
observation
history of activity limitation
MRI
Findings on MRI if CP positive?
White matter damage
cortical and subcortical lesions (basal ganglia)
brain malformation
postnatal injury
Birth asphyxia is the major cause of CP. T or F
False
birth aphyxia accounts for minority (10-20%) of cases
Where does CP originate?
pyramidal and extrapyramidal systems of the brain
what is the pyramidal system?
the system that connects regions of the cortex involved with motor control to muscle via corticospinal tract
initiates and carries signals that allow for volunary skilled movements
Damage to pyramidal system results in…
spastic CP (majority of cases - 70-85%)
Characteristics of spastic CP? (name 3)
abnormally high muscle tone
occurs more frequently in children born preterm than full term
associated with underlying periventricular leukomalacia (PVL), intraventricular hemorrhage (IVH)
Pyramidal motor system CP accounts for what percentage of CP?
70-85%
How many subtypes of CP are there within the pyramidal motor system?
3 subtypes
- spastic hemiplegic CP
- spastic diplegic CP
- spastic quadriplegic CP
Phenotypes/subtypes of CP are described based on what factor?
which limbs are affected
1st subtype of pyramidal motor system CP - Spastic hemiplegic CP
involves arm and leg of one side
usually more arm than leg affected
most common subtype of CP in full term babies
L hemisphere is affected in 2/3 of cases
prognosis of spastic hemiplegic CP
almost ALL can learn to walk
more than half have normal IQ
MRI finding of spastic hemiplegic CP/neuropathology/cause?
unilateral MCA stroke
typical time of injury of spastic hemiplegic CP?
prenatal
Which hemisphere is usually affected in spastic hemiplegic CP
Left hemisphere
2nd subtype of pyramidal motor system CP - Spastic diplegic CP
involves the lower extremities
upper also affected but less (usually clumsy hand movements)
most common subtype of CP in pre term babies
MRI findings/ Neuropathology of spastic diplegic CP/cause?
white matter damage of prematurity, esp PVL
Prognosis of spastic diplegic CP
severity of motor disability correlates with severity of cog impairment
typical time of injury for spastic diplegic CP
prenatal
3rd subtype of pyramidal motor systems CP - spastic quadraplegic CP
involves all 4 extremities, trunk, neck (FULL body)
50% happen prenatally
30% happen perinatally
20% happen posnatally
MRI finding of spastic quadraplegic CP/neuropathology/cause?
anoxia
b/l gray matter injury
Problems with Spastic quadraplegic CP? Prognosis?
most severe type
epilepsy
impaired IQ
What is the extrapyramidal motor system?
comprised of the cerebellum, basal ganglia, brainstem
function: fine tune movements of the pyramidal system by making adjustments to posture and coordination
What happens when extrapyramidal system is damaged?
results in non spastic CP (15-30% of cases)
What is the percentage of non spastic CP?
15-30%, minority of cases
Characteristics of non spastic CP
all extremities affected (more upper than lower)
normal IQ
subtypes of non spastic CP are delineated by what factors?
the kind of abnormal motor movements that occur
Two subtypes of non spastic CP
1) dyskinetic CP
2) Ataxic CP
Characteristic of Dyskinetic CP
variations in muscle tone and involuntary athetoid or dystonic movements
trouble with dyskinetic CP?
abnormal movements make it hard to sit comfortably and coordinate muscles for walking and sleeping
What is Dyskinetic CP caused by?
underlying hypoxic-ischemic injury
usually in full term infants
Characteristic of Ataxic CP
lack of coordination during voluntary gross and fine motor movements
What is ataxic CP caused by
cerebellar dysfunction
Problems with Ataxic CP?
poor balance, unsteadiness, wide based gait, shakiness, tremor
What % does Mixed CP account for?
20% of cases
Characteristics of Mixed CP
abnormalities in pyramidal and extrapyramidal systems
exhibit mixed motor signs
Risk factors for CP
preterm birth - most important RISK FACTOR
Low birth weight
maternal risk factors for CP
IQ
infection, toxic during pregnancy
diseases during pregnancy (epilepsy, thyroid, HTN)
Fetal risk factors for CP
multiple births co twin dead in uterus male IUGR developmental brain malformation thrombophilic disorders
Perinatal risk factors for CP
prematurity, although majority of CP cases are born full term Low Birth weight anoxic damage (10-14%) low apgar scores at 5 minutes abnormal muscle tone perinatal stroke
Postnatal risk factors for CP
infections and brain injury
Other risk factors for CP
SES not well established
black race
Prevalence of CP in developed countries
2-2.5/1000
Has the prevalence of CP changed?
No, it has remained stable because of IVF, improved survival rates
physical morbidity - mobility
33% cannot walk
17% require assistance to walk
50-60% can walk without assistance
physical morbidity - musculoskeletal complication
spasticity
physical morbidity - pain
75% children and 70% adult have chronic pain
epilepsy rate of CP
28-50% incidence
physical morbidity - oral motor impairment
affects chewing, swallowing, saliva
feeding tube for failure to thrive
speech articulation problems, may need augmentative communication
physical morbidity - visual and occulomotor impairment
28% of children of some form of visual/occulomotor impairment
- 10% blindness
- 10-12% hearing impairment
physical morbidity - bladder control
urinary incontinence 25%
physical morbidity - sleep
sleep disorder 20-25%
fatigue
physical morbidity - skin
pressure sores
Cognitive morbidity
30-35% of kids have IQ score < 70
50% of kids have IQ score < 50
What predicts cognitive morbidities in CP?
motor impairments and epilepsy
Which subgroup has the worst cognitive functions?
spastic quadraplegic
Which factor is NOT a reliable indicator of cognitive impairment
motor involvement
People with severe CP can have normal IQ. True or False
True
People with mild CP cannot experience severe cognitive impairment. True or False
False
Social Emotional Morbidity
social difficulties
depression
low self esteem
25% behavioral problems
Mortality - increased or decreased life expectancy?
life expectancy increased
survival rates improved
mild CP comparable with general population
leading cause of death in severe CP
respiratory infection
CP is classified as which types in their severity?
mild, moderate, severe
Standardized measure of CP severity in children
Gross motor function classification system (GMFCS)
Gross motor function classification system (GMFCS)
5 level ordinal rating based on functional mobility and activity limitation
higher number = more severe
GMFCS Level I
Level I = can walk and climb stairs independently and without limitation; some problems with gross motor skills (running, jumping, speed, balance coordination)
GMFCS Level V
severe limitations in voluntary control of movement
impaired mobility even with assistance
wheelchair bound
Can CP be diagnosed in utero?
No, because it’s based on clinically observable motor manifestation
Is it hard to detect CP during infancy (1-12 mos)
Yes, because motor patterns change, early motor abnormalities can be transient
precocious motor signs can be a sign of CP
absence of motor abnormalities in infancy does NOT mean there is no CP (50% of kids with CP may have unremarkable pre and perinatal history)
precocious motor signs (e.g. early standing before 12 months) can mean
hypertonia (stiffness and rigidity) in legs
precocious motor signs (e.g. strong hand preference in 12 - 18 mos) can reflect
weakness in the other hand
most kids exhibit full motor signs of CP at what age?
2 years or beyond
when can a definitive dx of CP be made?
2-3 years old
in mild CP cases, when can definitive diagnosis be made?
4-5 years
can children with the CP dx outgrow the disorder (no longer exhibit signs of CP?)
yes! by age 7
% of children with CP who outgrow the disorder
50%
which types of CP cases are more likely to be outgrown by age 7 in children?
mild
monoparetic (one extremity only)
diplegic
extrapyramidal types
will children who outgrow motor symptoms of CP continue to suffer from other neurological problems?
yes! such as seizures, ID
Is CP progressive?
No, but motor manifestations can worsen (e.g. caused by stress on the body, osteoporosis, arthritis)
NP results for CP
no typical profile BUT
- Verbal skills better than Visuospatial skills
- attention and processing speed affected (white matter and subcortical involvement)
- EF not an issue if motor skills are accounted for
IQ in CP
varies from severe ID to giftedness
75% affected
50% borderline to impaired
IQ correlated with degree of motor impairment
Learning in CP
SLD 25-30%
Attention in CP
affected by periventricular white matter and subcortical damage
epilepsy affects attention
Processing speed in CP
hard to measure, but some slowing
speech and language in CP
oral motor control affects speech, dysarthria
verbal, language skills are not mostly normal
visuospatial in CP
impaired
memory in CP
attention, visual impairment contribute to memory problems
EF in CP
questionable
sensorimotor function in CP
motor abnormalities
28% have visual/ocular-motor impairment
10-12% have hearing impairment
disturbances in sensory, touch, pain, sound…
emotion and personality in CP
more behavioral and social problems
PVT SVT
fatigue and pain interfere with alertness, engagement
boredom
motivation, frustration tolerance
Psychological Treatment of CP
Family centered interventions
medication for CP
more effective at treating SPASTICITY than addressing muscle weakness, incoordination, or movement abnormalities
Medication for spasticity
muscle relaxant
neuromuscular blocker
benzo
How does muscle relaxant work?
e.g. balcofen
works on the brain
inhibits descending excitatory motor pathways that activate reflex
How does neuromuscular blocker work
e.g. botox
works on muscle
inject into muscle and extremities to cause focal muscle paralysis and reduce muscle contraction
How does benzos work
e.g. diazepam
works on the brain
relax muscles by enhancing effect of GABA (inhibitory NT)
Spasticity
muscle tightness and stiffness due to prolonged muscle contraction
increased muscle tone
Apgar scores
performed 1 min and 5 min after birth
1 min - see how well baby tolerated birthing process
5 min - see how well baby is doing outside of the womb
What does APGAR stand for
Appearance Pulse Grimace response (reflex irritability when stimulated) Activity (muscle tone) Respiration
What APGAR scores are associated with risk for CP
lower than 3, but not determining factor
athetosis
slow and writhing involuntary movement
repetitive, rhythmic
dystonia
involuntary sustained or intermittent muscle contraction that cause repetitive and twisting movement
