Cerebral Palsy

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Cerebral Palsy
Margie Ream MD, PhD
Assistant Professor, Pediatric Neurology
Objectives: stuff to know about
cerebral palsy
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Definition, incidence and timing of insult
Diagnosis, exam findings
Classification of CP
Differential Diagnosis and comorbidities
Treatment
Prognosis
Quiz
Cerebral Palsy - definition
“Non-progressive disorder of posture or
movement caused by a lesion in the
developing brain.”
 The diagnosis requires three conditions:
1)
2)
3)
Motor difficulty
Not progressive
Injury to the immature brain (prenatal up to ≈2 yr)
Notice that the definition
does not comment on
intellectual ability.
Incidence and prevalence
 2-5/1,000 births

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At 12 months old 5/1000
At 7 years old 2/1000
 Some kids “grow out of it” and some kids don’t get
diagnosed until 2-3 years old
Timing of insult leading to CP
Term babies
Preterm
Examples
Prenatal
24%
6%
Maternal and
placental factors
Perinatal
20%
50%
HIE, trauma,
cerebral
hemorrhage,
hyperbili
Postnatal
8%
2%
combination
20%
23%
No known cause 30%
17%
<32 weeks,
<2500g
CNS infection,
accident, abuse
Taft 1995
Diagnosis
 Delayed motor development.
 May present as “floppy baby” and later develop
increase tone.
 Increased tendon reflexes
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sustained ankle clonus – always abnormal
any ankle clonus after the first few months
increased sensory input zone for reflexes (percuss over
deltoid and get biceps)
cross adduction at the patellar reflexes.
Normal Developmental Milestones
Age
Gross Motor
Fine Motor & Vision Hearing and speech
Social behavior
Turn head & eye
Sleep most of the time.
Primitive reflex, marked
towards light.
Startled by sudden loud Stop crying when picked
1 mth head lag.
Watches mom's face. noises.
up/spoken to.
Head & body in same
Hands mostly open.
2 mth horizontal plane
Grasp reflex weak. Vocalizes when spoke to.Smile readily (6 wk)
Watches own hands.
Respond with pleasure
Minimal head lag. Supine - Clasps & unclasps Turn to soft sound at ear to friendly handling.
3 mth - lies with head in midline. hands.
level. Squeals in delight. Hold rattle momentarily.
Hands come together Spontaneous vocalizing
No head lag. Rolls from
in midline. Reaches to self, toy and people. Initiates social contact
4 mth front to back.
towards object.
Laugh.
with smile.
Reach with both
Turn to voice and
Grasp feet and put toes
hands to grasp
babbling, tuneful. Laugh in mouth. Smile at mirror
5 mth Rolls from back to front. object.
& squeal in play
& vocalizes.
Sit with support. Prone - Palmar grasp.
lift chest, extend arms.
Transfer objects.
Respond to name. Turn Mouthing. Still friendly
Take weight on legs when Follow adult
toward sound at 40-50 with strangers. Laugh,
6 mth standing.
movements.
cm at ear level.
squeal and chuckle.
Look for toys. Picks Localize sound at 1 m
Look for object fallen out
Sit without support.
up object but can't
above and below ear
of sight. Stranger
Crawling, cruising (10
place down. Pincer level. Say mama and
anxiety. Waves bye bye
9 mth mth).
grasp.
papa.
and peek a boo
Point with index
Drinks from a cup. Put
Walk with 1 hand held.
finger. Pincer grasp. Undestand simple
blocks in and out of cup.
Can rise from lying to
Repeatedly throw
commands. Say 2-3
Knows and turns to own
12 mth sitting.
object.
words with meaning.
name.
Exam findings
Asymmetri
c tonic
neck reflex.
Taft, 1995
 Persistent primitive reflexes:
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Asymmetric tonic neck – should never be
obligatory (held >30 sec) and should
disappear by 6 months.
Crossed extensor reflex – stimulate foot of
extended leg. Contralateral leg will flex then
extend and adduct. Normal up to 4 months.
Crossed extensor reflex.
Intranet.tdmu.edu
Positive supporting reaction – baby looks
like he is stepping, should disappear by 4
months.
Moro should disappear by 6 months.
Moro reflex.
library.med.
utah.edu/
Stepping
reflex.
Artship.org
Classification of CP
 Spastic (70-80%)
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Diplegia (legs) - #1
Hemiparesis
Quadriparesis
 Dyskinetic (10-15%)
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Athetosis, chorea, ballismus, tremor
 Ataxic (1%)
 Mixed (10-15%)
 Rigid (5%)
Di = 2
Hemi = half
Quad = four
Paresis = weak
Plegia = paralyzed
Spastic Diplegia
 Most common form. Affects legs >>> arms.
 Delayed sitting and crawling. May prefer to “army
crawl”
 Leg scissoring with increased tone, increased
reflexes in legs.
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May not be obvious until 1 year old
Risk of hip subluxation or dislocation
Studyblue.com
Spastic diplegia video
Upper body strength/coordination far exceeds that in her lower body.
www.youtube.com/watch?v=mHXNjZS74s
Spastic diplegia video
Child with spastic diplegia undergoing gait analysis using a
walker. Notice toe walking.
www.youtube.com/watch?v=TP37l54UqTE
Spastic Diplegia
 Common cause is IVH in premature babies leading to
periventricular leukomalacia affecting the motor tracks
for legs.
Spastic Quadriplegia
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All limbs affected, legs > arms
Double hemiplegia if arms>legs
Hypotonia early after injury
Profound motor delay
Dysphagia/dysarthria common
Seizures in 50%
Abc.net.au
Hemiplegic Cerebral Palsy
 Affects one side of the body, arm>leg.
 First noticed around 4 months as hand
fisting and contralateral hand preference.
 Increased tone and reflexes noted by 15
months
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tight elbow flexors, wrist pronators,
gastrocnemius
 Causes:
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Stroke
IVH
Cerebral malformations
Physio-pedia.com
Hemiplegic CP video
Listen to narration then turn down volume and play video – we weren’t able to
remove the sound. Notice the upper motor neuron pattern of weakness in
which she keeps right arm flexed and has decreased arm swing. Also she
doesn’t flex at the hip as much on the right when walking.
https://www.youtube.com/watch?v=3w2IhGqIPAU
Less common forms of CP
 Athetoid dystonic CP:
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Prior to Rhogam was caused by
hyperbilirubinemia/kernicterus.
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Basal ganglia and auditory centers are sensitive
to bilirubin toxicity. Often comorbid hearing loss.
Typical course was initial extensor
posturing/opisthotonis, then hypotonia with
obligatory tonic neck reflex. Athetosis and
dystonia at 12-18 months, then rigidity by 2 yrs.
Now most commonly caused by hypoxiaischemia in basal ganglia (HIE)
Quizlet.com
Athetoid CP video
Athetoid movements are slow and writhing, usually distal.
https://www.youtube.com/watch?v=NXuwX7UBOlc
Less common forms of CP
 Ataxic-spastic CP
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Low tone with normal DTR, then develop wide based support and
hyperreflexia.
Can be due to hydrocephalus.
Ataxia can represent early signs of
metabolic disorder so watch for
progression and be suspicious.
Differential diagnosis of CP
Comorbidities of CP
 Intellectual disability
in 50-60%
 Seizures in up to 1/2
 Vision, hearing,
sensory impairments
 Feeding/secretion
difficulty
 Constipation,
incontinence
 Pain and dislocation
from contractures
 Osteopenia
 Communication
difficulties even with
normal IQ
 Spastic bladder
 Depression
 Skin breakdown
Treatment of Spasticity
 Medical management
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Oral meds (Baclofen, Benzodiazepines)
Botulinum toxin injections
Baclofen pumps
 Surgical management
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Dorsal rhizotomy (cut dorsal root from L1-S2)
Bracing or osteotomy for hip dislocation
Tendon release
Rch.org.au
Treatment of comorbidities
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Monitor feeding difficulty and airway protection
Consider g-tube in some patients
Assistive communication devices
Antiepileptics if needed
PT, OT, ST
Educational and
social support
Uakron.edu
Prognosis of CP
 It is difficult to give prognosis, especially based on
<12 mo exam
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20-30% of children with CP at 12 months outgrow the diagnosis.
Some deficits may not be apparent until 2-3 years old.
 Good prognosis for ambulation if sitting
independently by 1.5-2 years
 If can sit by 2-4 years, most can walk.
 Not sitting by 4 years  no ambulation later.
References
 Taft, Lawrence. Cerebral Palsy. Pediatrics in
Review. 1995.
 Krigger, Karen. Cerebral Palsy: An Overview.
American Family Physician. 2006.
 Clinical Pediatric Neurology: A Signs and
Symptoms Approach, Gerald Fenichel
 eMedicine
 Medscape
Bailer and White. Nature Reviews Drug Discovery 9, 68-82
(January 2010)
Thank you!
For questions please contact Dr. Ream at
Margie.Ream@nationwidechildrens.org
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