CEREBRAL PALSY

CEREBRAL PALSYCEREBRAL PALSY

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CEREBRAL PALSY

CEREBRAL PALSY

CEREBRAL PALSYCEREBRAL PALSY

• ABERRANT CONTROL OF MOVEM-ABERRANT CONTROL OF MOVEM-ENT & POSTURE ENT & POSTURE

• APPEARS EARLY IN LIFEAPPEARS EARLY IN LIFE

• SECONDARY TO CNS LESION / SECONDARY TO CNS LESION / DYSFUNCTIONDYSFUNCTION

• R/O PROGRESSIVE OR DEGENER -R/O PROGRESSIVE OR DEGENER -ATIVE BRAIN DISEASEATIVE BRAIN DISEASE

CEREBRAL PALSY

CEREBRAL PALSYCEREBRAL PALSY

GROUP OF HETEROGENOUS CLINICAL STATESGROUP OF HETEROGENOUS CLINICAL STATES

VARIABLE ETIOLOGYVARIABLE ETIOLOGY

SEVERITY – MINOR INCAPACITATION TO TOTAL SEVERITY – MINOR INCAPACITATION TO TOTAL HANDICAPHANDICAP

INCIDENCE – 2 - 2.5 PER 1000 LIVE BIRTHSINCIDENCE – 2 - 2.5 PER 1000 LIVE BIRTHS

USUAL PRESENTING COMPLAINT – SLOW USUAL PRESENTING COMPLAINT – SLOW PROGRESS IN REACHING MOTOR MILESTONES PROGRESS IN REACHING MOTOR MILESTONES AT APPROPRIATE CHRONOLOGICAL AGEAT APPROPRIATE CHRONOLOGICAL AGE

CEREBRAL PALSY

DEVELOPMENTAL DELAY

• STATIC OR PROGRESSIVE STATIC OR PROGRESSIVE ENCEPHALOPATHYENCEPHALOPATHY

• RESTRICTED TO SPECIFIC AREAS OR RESTRICTED TO SPECIFIC AREAS OR GLOBALGLOBAL

• DEVELOPMENT DELAYED OR DEVELOPMENT DELAYED OR REGRESSINGREGRESSING

CEREBRAL PALSY

KEY DEVELOPMENTAL KEY DEVELOPMENTAL MILESTONES : GROSS MOTORMILESTONES : GROSS MOTOR

• 3 MONTHS 3 MONTHS – NECK HOLDING– NECK HOLDING• 5-8 MONTHS – SITTING 5-8 MONTHS – SITTING • 9-12 MONTHS – STANDING 9-12 MONTHS – STANDING • 10-13 MONTHS - WALKING10-13 MONTHS - WALKING• 11 MONTHS – CRAWLING11 MONTHS – CRAWLING• 18 MONTHS – RUNNING18 MONTHS – RUNNING• 24 MONTHS – WALKS UPSTAIRS24 MONTHS – WALKS UPSTAIRS• 36 MONTHS – RIDES TRICYCLE36 MONTHS – RIDES TRICYCLE

CEREBRAL PALSY

KEY DEVELOPMENTAL KEY DEVELOPMENTAL MILESTONES : FINE MOTORMILESTONES : FINE MOTOR

• 4 MONTHS – GRASPS WHEN4 MONTHS – GRASPS WHEN PLACED IN HANDPLACED IN HAND• 5 MONTHS – INTENTIONAL 5 MONTHS – INTENTIONAL REACHING WITH BIDEXTROUS REACHING WITH BIDEXTROUS GRASPGRASP• 7 MONTHS – PALMAR GRASP7 MONTHS – PALMAR GRASP• 9 MONTHS – PINCER 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 SMILE2 MONTHS – SOCIAL SMILE• 3 MONTHS – RECOGNISES 3 MONTHS – RECOGNISES

MOTHERMOTHER• 6 MONTHS – SMILES AT 6 MONTHS – SMILES AT

MIRROR IMAGEMIRROR IMAGE• 9 MONTHS – WAVES BYE – 9 MONTHS – WAVES BYE –

BYEBYE• 12 MONTHS - PLAYS SIMPLE 12 MONTHS - PLAYS SIMPLE

BALL GAMEBALL GAME• 36 MONTHS – KNOWS GENDER36 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 (2nd 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 – patient’s 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 - doll’s 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 moro’s 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, café-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 Presenting complaint – motor milestones not attained at milestones not attained at appropriate chronological ageappropriate chronological age• Brain abnormality – pre- peri- or Brain abnormality – pre- peri- or postnatally postnatally • Worldwide incidence of CP – 2-Worldwide incidence of CP – 2-2.5/1000 livebirths2.5/1000 livebirths• CP – 33% hemiplegic, 44% diplegic CP – 33% hemiplegic, 44% diplegic , 6% quadriplegic , 6% quadriplegic• CP – a syndrome with many CP – a syndrome with many etiologiesetiologies

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 polycystic kidney• Head and Neck high nasal bridge hyper/hypotelorism microphthalmia mongoloid slant occipital scalp defect small mandible small/fish mouth small/low - set ears upward slant of eyes 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 child’s education & intellect

• Telling parents Bad News – badly has profound & long lasting negative impact on the child’s outcome that extends beyond the structural damage to child’s brain

CEREBRAL PALSY

Management : Communication & Education

• It is not possible to make bad news sound good

• Goal for first visit – Establish that the child’s 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

• Communication

It allows us relay our most basic needs Communication is essential to our very quality of lifeTo learn effectively – one must have opportunity &means to communicateCommunication requires ability to gather information from one’s environment through visual, auditory & tactile sensationEvery CP child should be evaluated by trained pediatric ophthalmologist

Management : optimizing communication

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 one’s 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 person’s 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 exercisesshould be included as part oftheir daily routine• Weight training & resistive exercises build muscle mass& with proper supervision besafely 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 one’s 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 one’s own choices in life and pursue one’s 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 impactUnless 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 yourChild 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