Sunteți pe pagina 1din 73

CEREBRAL PALSY

DR SNEHA..
CEREBRAL PALSY

CEREBRAL PALSY

CEREBRAL PALSY
ABERRANT CONTROL OF MOVEMENT & POSTURE
APPEARS EARLY IN LIFE
SECONDARY TO CNS LESION /
DYSFUNCTION
R/O PROGRESSIVE OR DEGENER
-ATIVE BRAIN DISEASE
CEREBRAL PALSY

CEREBRAL PALSY
GROUP OF HETEROGENOUS CLINICAL STATES
VARIABLE ETIOLOGY
SEVERITY MINOR INCAPACITATION TO TOTAL
HANDICAP
INCIDENCE 2 - 2.5 PER 1000 LIVE BIRTHS
USUAL PRESENTING COMPLAINT SLOW
PROGRESS IN REACHING MOTOR MILESTONES
AT APPROPRIATE CHRONOLOGICAL AGE
CEREBRAL PALSY

DEVELOPMENTAL DELAY
STATIC OR PROGRESSIVE
ENCEPHALOPATHY
RESTRICTED TO SPECIFIC AREAS OR
GLOBAL
DEVELOPMENT DELAYED OR
REGRESSING
CEREBRAL PALSY

KEY DEVELOPMENTAL
MILESTONES : GROSS MOTOR

3 MONTHS NECK HOLDING


5-8 MONTHS SITTING
9-12 MONTHS STANDING
10-13 MONTHS - WALKING
11 MONTHS CRAWLING
18 MONTHS RUNNING
24 MONTHS WALKS UPSTAIRS
36 MONTHS RIDES TRICYCLE
CEREBRAL PALSY

KEY DEVELOPMENTAL
MILESTONES : FINE MOTOR
4 MONTHS GRASPS WHEN
PLACED IN HAND
5 MONTHS INTENTIONAL
REACHING WITH BIDEXTROUS
GRASP
7 MONTHS PALMAR GRASP
9 MONTHS PINCER GRASP
CEREBRAL PALSY

KEY DEVELOPMENT
MILESTONES : LANGUAGES
1 month Turns head to sound
3 months Cooing
6 months Monosyllables
9 months Bisyllables
12 months 2 words with
meaning
18 months 10 words with
meaning
24 months Simple sentence
36 months Telling a story
CEREBRAL PALSY

DEVELOPMENTAL
MILESTONES : PERSONAL
SOCIAL

2 MONTHS SOCIAL SMILE


3 MONTHS RECOGNISES
MOTHER
6 MONTHS SMILES AT
MIRROR IMAGE
9 MONTHS WAVES BYE
BYE
12 MONTHS - PLAYS SIMPLE
BALL GAME
36 MONTHS KNOWS GENDER

CEREBRAL PALSY

DIAGNOSIS OF DEVELOPMENT
DELAY : NO REGRESSION
Predominant Speech Delay
bilateral hippocampal sclerosis
congenital bilateral perisylvian syndrome
hearing impairment
infantile autism

CEREBRAL PALSY

DIAGNOSIS OF DEVELOPMENT
DELAY: NO REGRESSION
Predominant motor delay
ataxia
hemiplegia
hypotonia
neuromuscular disorders
paraplegia

CEREBRAL PALSY

DIAGNOSIS OF DEVELOPMENT
DELAY : NO REGRESSION
Global developmental delay
cerebral malformations
chromosomal disturbances
intrauterine infections
perinatal disorders
progressive encephalopathies

CEREBRAL PALSY

PROGRESSIVE
ENCEPHALOPATHY : ONSET < 2
Acquired immunodeficiency syndrome
encephalopathy
Disorders of amino acid metabolism
Disorders of lysosomal enzymes
Niemann Pick disease type A
Sulfatase deficiency disorders
Carbohydrate-deficient glycoprotein
syndrome
CEREBRAL PALSY

PROGRESSIVE
ENCEPHALOPATHY : ONSET < 2

Mitochondrial disorders
Neurocutaneous syndromes
Other disorders of gray matter
Other disorders of white matter
Progressive hydrocephalus

CEREBRAL PALSY

Causes of Apparent Regression in


Static Encephalopathy

Increasing spasticity (1st year)


New onset movement disorders (2 nd year)
New onset seizures
Parental misperception of attained
milestones
Progressive hydrocephalus

CEREBRAL PALSY

HYPOTHYROIDISM
Congenital hypothyroidism : 1:4000
livebirths
Early diagnosis & treatment imperative
Gestation > 42 weeks
Birth weight > 4 kg
Wide-open posterior
fontanelle,constipation,jaundice,poor
temperature control,umblical hernia
CEREBRAL PALSY

HYPOTHYROIDISM
Edema of eyes,hand,feet
Large tongue
Diagnosis low serum thyroxine & high
TSH levels
Management thyroxine 24-50 g/d

CEREBRAL PALSY

ETIOPATHOGENESIS OF CP

Cerebral malformations
Perinatal hypoxia
Birth trauma
Metabolic disturbances
Infections - intrauterine / acquired
Stroke
Intracerebral haemorrhage
Arterioveinous malformations
Hydrocephalus
Periventricular leukomalacia
Genetic disorders
CEREBRAL PALSY

CEREBRAL PALSY - FORMS


Spastic CP
Spastic quadriparesis
Spastic diplegia
Spastic hemiplegia
Hypotonic CP
Extrapyramidal CP

CEREBRAL PALSY

Differential Diagnosis of Infantile


Hypotonia
Cerbral hypotonia
Benign congenital hypotonia
Chromosome disorders
Prader Willi syndrome
Trisomy
Chronic non progressive encephalopathy
Cerebral malformation
Perinatal distress
Postnatal disorders
CEREBRAL PALSY

INFANTILE HYPOTONIA

Spinal cord disorders


Spinal muscular atrophies
Polyneuropathies
Neuromuscular transmission disorders
Fiber-type disproportion myopathies
Metabolic myopathies
Muscular dystrophies
CEREBRAL PALSY

NEUROLOGIC EXAMINATION
Goals assess development & integrity of
the nervous system
- determine location & cause of
suspected dysfunction
Impediments patients age &
willingness to cooperate
- complexity of complete neuro. exam.
- fluctuating nature of neuro. exam.
CEREBRAL PALSY

NEUROLOGIC EXAMINATION
Children v/s adult - more observation,
considerable flexibility,greater
patience,less reliance on instructions that
children easily misunderstand
Repeated examination
Thoughtful examination

CEREBRAL PALSY

Neurologic Exam. : Mental Status


methodologic approach
- comprehension responses to who,
what, when, where
- memory,organisation & language skills
story telling
- naming identify colours / handy objects
- reading / writing
CEREBRAL PALSY

Neuro Exam. : Cranial Nerves


IInd Cranial nerve
visual acuity blink to bright light
- snellens chart
visual field finger counting by
confrontation
fundoscopy

CEREBRAL PALSY

Neuro Exam. : Cranial Nerves


IIIrd,IVth &VI th position of eyes at rest
- abnormal eye movements at rest &
during fixation
- nystagmus
- pupils , light reflex
- ptosis
- cover - uncover test
- dolls eye movement
CEREBRAL PALSY

Neuro. Exam. : Cranial Nerves


Vth & VIIth facial sensation
- facial weakness
- lacrimation , salivation
- blink reflex
- jaw jerk

CEREBRAL PALSY

Neuro. Exam. : Cranial Nerves


VIIIth hearing assessed behaviorally or
by standardised measures
- audiometry low threshold
IXth, Xth & XIIth phonation,
articulation, swallowing
- palatal movements
- tongue atrophy / fasciculations
CEREBRAL PALSY

Neurologic Exam. : Motor


Identify handedness/dominance
- appears around 1 year age
- early appearance unilateral weakness
Posture,Tone Abnormal movements chorea, dystonia, tics,
stereotypies
Muscle bulk atrophy,hypertrophy, fasciculations
Strength
Gait
Coordination
CEREBRAL PALSY

Neurologic Exam. : Reflexes


Deep tendon reflexes

sluggish or absent peripheral nerve, muscle or


cerebellar involvement
brisk brain or spinal cord involvement
Developmental reflexes
rooting reflex disappears by 3 months
moros reflex disappears by 4 months
asymmetric tonic neck reflex 4-6 months
tonic labyrinthine reflex 6 months
grasp reflex 6 months
plantar grasp reflex 10 months
CEREBRAL PALSY

Neurologic Examination
Neurocutaneous markers hypopigmented
spots, caf-au-lait spots, angiomas,
dorsal midline dimple, angioma, tuft of hairs
Head growth birth(35 cm), 3 months(40 cm),
9 months(45 cm),3 years (50 cm)
- macrocephaly familial, megalencephaly,
hydrocephalus
- microcephaly perinatal insult to brain,
IUGR, genetic syndrome
CEREBRAL PALSY

Neurologic Examination

Spine
Autonomic abnormality
Abdomen hepatosplenomegaly
Heart congenital/valvular defects

CEREBRAL PALSY

DIAGNOSTIC ASSESSMENT
Presenting complaint motor
milestones not attained at
appropriate chronological age
Brain abnormality pre- peri- or
postnatally
Worldwide incidence of CP 22.5/1000 livebirths
CP 33% hemiplegic, 44% diplegic
, 6% quadriplegic
CP a syndrome with many
etiologies
CEREBRAL PALSY

DIAGNOSTIC ASSESSMENT
Common etiologies with prenatal
onset intrauterine infection, stroke,
toxaemia, placental abruption

Perinatal onset hypoxic ischemic


encephalopathy, kenicterus, trauma

Postnatal onset infection, trauma,


hydrocephalus
CEREBRAL PALSY

DIAGNOSTIC ASSESSMENT
Accurate determination of etiology of
CP - specific implications regarding
treatment,prognosis & management of associated
conditions.
- assessment of recurrence risk, counseling of
families & implementation of prevention programs
& in MLCs
Common associated problems epilepsy,
mental retardation, speech & language disorders,
and ophthalmologic & hearing impairments

CEREBRAL PALSY

DIAGNOSTIC ASSESSMENT
Neuroimaging recommended - if
etiology not established

MRI preferred to CT scan eitology and


timing of insult leading to CP
Potentially treatable lesions identified
hydrocephalus, porencephaly,
AVmalformations, subdural haematomas
& hygromas, vermian tumor
CEREBRAL PALSY

DIAGNOSTIC ASSESSMENT
Metabolic & genetic testing certain

congenital malformations ( lissencephaly,


schizencephaly,pachygyria) are ever
increasingly associated with specific genetic
disorders.
Neurometabolic disorders may masquerade as
CP.
Metabolic or genetic causes of CP occur
infrequently

CEREBRAL PALSY

DIAGNOSTIC ASSESSMENT
Metabolic and genetic studies should not
be routinely obtained in evaluation of child with
CP

Metabolic or genetic testing should be


considered If clinical history or findings do not determine
a specific structural abnormality or if there are
additional and atypical features

Detection of brain malformation in a child with


CP

CEREBRAL PALSY

Clinical Indications for


Chromosome Analysis
Genitourinary
ambigious genitalia
Head and Neck
high nasal bridge
microphthalmia
occipital scalp defect
small/fish mouth
upward slant of eyes

polycystic kidney
hyper/hypotelorism
mongoloid slant
small mandible
small/low - set ears
webbed neck
CEREBRAL PALSY

Clinical Indications for


Chromosome Analysis
Limbs

abnormal dermatoglyphics
low set thumb
overlapping fingers
polydactyly
radial hypoplasia
rocker-bottom feet
CEREBRAL PALSY

DIAGNOSTIC ASESSMENT
Coagulopathies Hemiplegic CP
prenatal or perinatal cerebral infarction
Stroke etiology in children coagulopathy,
congenital heart disease,infections
Factor V Leiden deficiency,
anticardiolipin/antiphospholipid antibodies,
Protein C or Protein S deficiency
CEREBRAL PALSY

DIAGNOSTIC ASSESSMENT
Associated conditions

mental retardation (52%)


epilepsy (45%)
ophthalmologic defects (28%)
speech & language disorders (38%)
hearing impairment (12%)

CEREBRAL PALSY

Diagnostic Assessment :
Ophthlmologic Defects
strabismus
amblyopia
nystagmus
optic atrophy
refractory errors

CEREBRAL PALSY

DIAGNOSTIC ASSESSMENT

EEG should not be done to determine the

etiology of CP
- should be done if history & physical exam.
of the child with CP suggest presence of
epilepsy or epileptic syndrome

All CP children should be screened for


MR,ophthlmologic &hearing impairments and
speech & language disorders

Nutrition, growth ,swallowing - monitored


CEREBRAL PALSY

MANAGEMENT
Rationale & strategies for a
comprehensive & goal directed approach
to caring for CP patients focus on 4 main
areas
1. Communication & education
2. Mobility
3. Physical fitness
4. Independence
CEREBRAL PALSY

Management : Communication &


Education
Most important & most neglected priorities
Irreversible brain injury (periventricular
leukomalacia, cerebral haemorrhage,
hypoxic ischemic encephalopathy )
threatens CP childs education & intellect
Telling parents Bad News badly has
profound & long lasting negative impact on
the childs outcome that extends beyond
the structural damage to childs brain
CEREBRAL PALSY

Management : Communication &


Education
It is not possible to make bad news sound
good
Goal for first visit Establish that the
childs development is not normal & Share
the parents concern
Further discussion during next visit
Parents want to know will he walk, need
special schools, live alone? What can I do
to help my child ?
CEREBRAL PALSY

Management : optimizing communication

It allows us relay our most basic needs


Communication is essential to our very quality of life
To learn effectively one must have opportunity &
to communicate
means
Communication
Communication requires ability to gather information
from ones environment through visual, auditory &
tactile sensation
Every CP child should be evaluated by trained
pediatric ophthalmologist

CEREBRAL PALSY

Management : optimizing
communication
Delayed speech evaluation by trained
paediatric audiologist & otolaryngologist
( to correct treatable causes of hearing
loss)
Alternative means of communication augmented communication devices
Children with speech abnormality
enrolled for early speech & language
therapies
CEREBRAL PALSY

Management : Optimizing Education

Accesible classroom & computer technology


Coordinated efforts of parents, school officials &
physicians
Exchanging stairs for ramps/elevators, widening of doors,
improving seating systems, providing aides to assist children
who need them
Computer technology level playfield for children with
motor & speech impairment
CEREBRAL PALSY

Management : Mobility
Major determinant of ones independence
CP individuals tend to become less mobile
as they reach adulthood
Cost of immobility progressive deformity,
contracture, chronic pain, severe bone
loss, fracture, scoliosis, hip dislocation,
reduced cardiovascular fitness, obesity,
depression
CEREBRAL PALSY

Management : Mobility
Preventing immobility requires
concerted effort
-Optimise Biomechanics, Tone
management,Strength,Balance,
and Physical fitness
-Assisted mobility greatly
augments persons mobility &
independence( Crutches,
Canes, Walker,Normal &
Powered Wheelchairs)
CEREBRAL PALSY

Management : Mobility
Biomechanics Stretching,
weightbearing, orthotics ,
bracing, serial casting,
Btx , orthopedic surgery

CEREBRAL PALSY

Management : Mobility Tone


management
Management of Spasticity primary focus for
many physicians
Tone reduction alone should not be the goal :
improve & maximise function
Determine whether positive
symptoms(hyperreflexia, clonus, increased tone)
are the source of functional impairment
Negative symptoms (underlying weakness &
loss of agility ) most disabling of motor
symptoms associated with CP
Spasticity may assist in function
CEREBRAL PALSY

Management : Mobility - Tone


management
Initial & periodic evaluation by
Interdisciplinary team
Appropriateness of a modality depends on
multiple factors Age
- Complicating medical issues
( seizures, pain, rapid development of
contracture, prior response to therapy)
- Familial & societal factors including
compliance & cost factors
CEREBRAL PALSY

Management : Mobility Tone


management
Oral medication
Baclofen,Tizanidine,
Benzodiazepines
- tone reduction modest &
rarely sufficient
- best for spasms, sleep
disturbances, adjunctive in
seizure disorder
CEREBRAL PALSY

Management : Mobility Tone


management
Botulin toxin ( BTX ) Neurotoxins that
prevent depolarisation at neuromuscular
junctions
Therapeutic goal of btx injections in spastic CP
complete or partial paralysis of specific target
(agonist ) muscles affecting a spastic joint while
leaving antagonist muscles unaffected
Joint forces become more balanced & functional
passive range of motion restored
CEREBRAL PALSY

Management : Mobility Tone


management - BTX
BTX inj. should be accompanied by aggressive
physiotherapy to increase muscle strength &
dexterity
Safe & well tolerated
Response seen in 3-4 days & maintained for 3-4
months or longer
Primary criteria presence of persistent or
dynamic hypertonia in the absence of significant
fixed deformity regardless of anatomic site or
distribution of palsy
CEREBRAL PALSY

Management : Mobility Tone


management - BTX
Indications for BTX
Upper limbs
- persistent thumb in palm or thumb adduction
- wrist posture preventing effective hand use
- tight elbow flexion
- tone in shoulder extensors or external rotators
significantly limit functional mobility

CEREBRAL PALSY

Management : Mobility Tone


management - BTX
Lower limbs dynamic equinus
persisting through out the gait cycle
- knee hyperextension secondary to
overactive plantar flexors
- significant scissoring or adduction at hips
In either UL or LL relative muscle
weakness may be a contraindication
for BTX therapy & should be
thoroughly assessed
CEREBRAL PALSY

Management : Strength & Balance


Goal directed physical
therapy + daily home
exercise programme
Help the patient to develop wide
variety of exercises & activities to
strengthen specific muscle group
, improve balance(sitting, standing
,static & dynamic)&maintain &
improve muscle or
tendon length.
CEREBRAL PALSY

Management : Mobility Strength


& Balance
Sports & aerobic exercises
should be included as part of
their daily routine
Weight training & resistive
exercises build muscle mass
& with proper supervision be
safely performed without
worsening their spasticity
Electrical stimulation of muscles
CEREBRAL PALSY

For CP individuals having


the stamina to move can
mean the difference between
dependence & independence
CEREBRAL PALSY

Management : Physical Fitness


Cardiovascular fitness is vital to health & well
being
Wide variety of sports & athletic activities
( swimming, water aerobics, basketball, soccer,
snow & waterskiing, dance & martial arts)
promote overall strength & improve balance
Regular aerobic conditioning protects ones
health, increases energy levels & endurance

For CP individuals having the stamina


to move can mean the difference
between dependence & independence
CEREBRAL PALSY

Management : Independence
Ability to make ones own choices in life
and pursue ones own dreams , is the
goal for any child ,including persons
with disabilities

CEREBRAL PALSY

CP is a group of heterogenous clinical states with


variable (pre-,peri-,or postnatal) etiology
-Aberrant control of movement & posture
-Static encephalopathy
-Presents early in life
Neuro. Exam Assess development & integrity
of nervous system
-Determine location & cause of suspected
dysfunction

CEREBRAL PALSY

Diagnostic assessment Accurate determination


of etiology
- Look for associated problems epilepsy, MR, speech& language
defects,and visual & hearing impairments

Neuroimaging if etiology not established


- MRI preferred to CT scan where possible
- Metabolic & genetic testing, Coagulopathy tests - in select group of
patients
- EEG if epilepsy or epileptic syndrome suspected
- All CP patients should be evaluated by trained paediatric
ophthalmologist
-All CP children with speech delay be evaluated by trained audiologist
and paediatric otolaryngologist

CEREBRAL PALSY

CEREBRAL PALSY

However none of these interventions will make a truly significant impact


Unless we work to create a world in which all children are permitted to
thrive to their Greatest potential ,a world in which accessibility applies
to buildings doorways,& sidewalks as well as to all opportunities life
has to offer.

CEREBRAL PALSY

DO YOU NEED HELP?


DESIGN a home programme for
your
Child based upon what you have
learnt

START your programme


BE CONSISTENT in whatever you
do

KEEP A SIMPLE DIARY of the


changes you observe and the
questions you have

LEARN MORE about how you can


help your child

CEREBRAL PALSY

CEREBRAL PALSY

CEREBRAL PALSY

CEREBRAL PALSY

S-ar putea să vă placă și