Cerebral Palsy

Dr Surya Kumar

Cerebral Palsy = Brain ParalysisDefinitionPrevalenceEtiologyClassificationsClinical PresentationTreatmentsSubstantially Disabling

Cerebral Palsy: DefinitionCerebral palsy is a static encephalopathyEncephalopathy = Brain Injury that is non-

progressive disorder of posture and movement

Variable etiologiesOften associated with epilepsy, speech

problems, vision compromise, & cognitive dysfunction

LATEST DEFINITION OF CEREBRAL PALSY

“Cerebral palsy describes a group of permanent disorders of the development of movement and posture causing activity limitation that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain. The motor disorders of cerebral palsy are often accompanied by disturbances of sensation, perception, cognition, communication and behavior, by epilepsy, and by secondary musculoskeletal disorders”

Rosenbaum P et al: Dev Med Child Neurol (Suppl.) 2007;109:8-14

Cerebral Palsy: ClassificationVarious classifications of Cerebral PalsyPhysiologicTopographicEtiologic

Cerebral Palsy: PhysiologicAthetoidAtaxicRigid-SpasticAtonicMixed

Cerebral palsy

ClassificationAccording to Pattern of involvement

Monoplegia : one limb / rare

Diplegia : both LL >> UL / good intelligence / prematurity

Hemiplegia : unilateral usually UL > LL / 33 % seizures

50 % mentally retarded

Triplegia : rare / usually both LL + one UL

Quadriplegia : total body / often mentally retarded /

with seizures / severe hypoxia

Double hemiplegia : bilateral UL > LL

Cerebral palsy

ClassificationAccording to Type of motor dysfunction

Spastic 65 % Athetoid 10 % Ataxic 5 % Mixed 12 % Hypo tonic 1 %

Gross motor functional classification system

Level Function

I Ambulatory in all settingsII Walks without aides but has limitations in community settingsIII Walks with aidesIV Mobility requires wheelchair or adult assistV Dependent for mobility

Etiology

Prenatal – 70 to 80 %Natal - Upto 10 %Rest postnatal Upto 5 year ? ( Veena kalra AIIMS)

“ASPHYXIA” AND CP IN THE NCPP STUDY

23 CHILDRENWITH OTHER REASONS FOR CP

12 < 2KG, 14 NON-CNS ANOM ALY1 M ICROCEPHALY, 7 PRENATAL RISK

17 CHILDREN"PURE" ASPHYXIAL DAM AGE

<10% OF ALL CP1 PER 2,700 BIRTHS

40 CHILDRENWITH ANY ASPHYXIA INDICATOR

149 CHILDRENWITH NO ASPHYXIA INDICATOR

189 CHILDRENWITH CP

45, 449CHILDREN

All four criteria must be met:

Evidence of metabolic acidosis: umbilical artery pH<7 and base deficit ≥12 mmol/L at delivery

Early onset of severe or moderate neonatal encephalopathy in infants ≥34 weeks of gestation

Cerebral palsy of the spastic quadriplegic or dyskinetic type

Exclusion of other identifiable etiologies (eg, trauma, coagulation disorders, infection, genetic disorders)

Task force on neonatal encephalopathy and cerebral palsy criteria for acute intrapartum events sufficient to cause cerebral palsyAdapted from: Neonatal Encephalopathy and Cerebral Palsy: Executive Summary. Obstet Gynecol 2004; 103:780

A sentinel hypoxic event occurring immediately before or during labor

A sudden and sustained fetal bradycardia or absence of fetal heart rate variability in the presence of persistent late or variable decelerations. This usually occurs after a hypoxic sentinel event with a normal fetal heart rate pattern prior to the event.

Apgar score of 0 to 5 after five minutes

Onset of multisystem involvement within 72 hours of birth

Early imaging studies showing evidence of an acute nonfocal cerebral abnormality

Peripartum events that may be related to development of cerebral palsy but which are not specifically asphyxial insultsAdapted from: Neonatal Encephalopathy and Cerebral Palsy: Executive Summary. Obstet Gynecol 2004; 103:780.

Table 1Mimics of cerebral palsy disorder Clue

Familial spastic paraplegia Family historyTransient toe walking Normal deep tendon reflexesMuscular dystrophy Calf hypertrophy, positive Gower’s signMetabolic disorders Regression, lethargy, unusual vomitingSjogren-Larrson IchthyosisLesch-Nyhan Severe self-mutilationMitochondrial disorders Recurrent stroke, cardiomyopathy, hypoglycemiaGenetic disorders Multiple anomaliesMiller-Dieker LissencephalyRett Syndrome microcephaly, hand wringing

Impaired movements• 65% speech defects• 50% are mentally retarded• 50% ocular defects• 25% hearing impairment• 40% seizure disorders• 20% seriously disabled• 1.5 to 2.5 per 1,000 birthswill result in severe tomoderately severe

Static Vs slowly progressive neurological disorder

Global devlopmental delay/ differential devlopmental delay

Disorder Gross motor

Fine motor Social language

Mental retardation

Delay + + to + + + ++ t+++ +++

Cerebral palsy

Delay +++ ++ + +

CP with MR +++ +++ +++ +++

Hearing impairment

No No No +++

Impaired vision

++ +

Spinal muscular atrophy

++ + + to ++ No Expressive may be delayed

Levine ( poster) criteria P- Posturing/ abnormal movement

O- oropharyngeal problems (normality

tongue thrust and swallowing abnormality

S- strabismus

T – tone ( hyper to hypo)

E- Evolutional maldevlopment ( persistent primitive

reflexex or protective / equilibrium reflexes fail to devlop ( parachute reflex)

R – reflexes ( increased deep tendon/ persistent babinski

( Four out of 6 strongly points to CP)

(

Difficulty to diagnose CP during the 1st year of life1. Hypotonia more common then hypertonia

in 1st yr2. early abundance of primitive reflexes may

confuse 3. limited variety of volitional movement for

evolution4 subtantioal myelination takes months to

evovle5 most instace of CP doesn’t have

substancial risk fac

What behaviour symptoms during 1st year arouse suspicion of CP1. excessive irritablity, crying , sleep difficulties2. early feeding difficulties ( Co-ordination of

sucking and swallowing)3. Jitter or jerky behavoiur4. easily startle behaviour5. Stiffness during dressing , diaper, hand washimg6. paradoxical precocious devlopment a , early rolling ( actually sudden reflex roll rathe

then volitionalStiff leg standing

Feature suggestive of progressive rather then CP1. Abnorma increase in heaad circumferenceEye abnormalitiesSkin abnormaltyHepatomegaly and / or spleenomegaly Decrease or absent deep tendon reflexSensory abnormalitiesDevlopmental regression ( Rett syndrome )

Head and Neck Findings

• 24% inability to chew• 20% inability to swallow easily• 20% frequent dental caries• High rate of temporo-mandibular disorders

Positive signs of spastic CP include:

Spastic hypertoniaHyperreflexia caused by hyperexcitability of the stretch reflexExtensor plantar responsesClonus

Negative signs of spastic CP include:

Slow effortful voluntary movementsImpaired fine-motor functionDifficulty in isolating individual movementsFatiguability

Athetoid CP Findings (con’t)• Grimacing• Drooling• Speech defects• Continuous mouth breathers• Excessive head movements• Tongue protrusion• Primitive reflexes of varying severity

ASSOCIATED DISORDERS 

Intellectual disability 

Children with spastic quadriplegia are typically the most severely affected, while cognitive function usually is better with dyskinetic CP that is mainly athetoid

Psychiatric disorders 

including emotional lability, poor attention and vigilance, and obsessive-compulsive traits

Epilepsymost common in patients with spastic quadriplegia and acquired hemiplegia, and less common in mild symmetric spastic diplegia and CP that is mainly athetoid

Visual disorders 

strabismus and clinically significant refractive errors each occurred in 50 percent, and amblyopia and visual field defects each

Speech impairment 

including aphasia and dysarthria, occur in about 38 percent of children with CP

Hearing impairment most common in those with very low birthweight or severe hypoxic-ischemic insults

Pulmonary disease 

a leading cause of death among patients with severe CP

Growth failure  Urinary disorders  Orthopedic disorders 

Osteopenia 

DIAGNOSIS  The diagnosis of CP depends upon a combination of findings, including motor delay, neurologic signs, persistence of primitive reflexes, and abnormal postural reactions

Neurobehavioral signs 

Motor abnormalities 

Developmental reflexes

Laboratory studies 

serum concentrations of glucose, thyroid, ammonia, lactate and pyruvate, plasma amino acid analysis, urine organic acid analysis, and arterial acid-base status, should be obtained to exclude a metabolic disorder

NEUROIMAGING FOR CP [Bax et al JAMA 2006;296:1602]

Emerging imaging modalities will likely provide further insight into the etiology of CP by making imaging easier in children (PROPELLAR) and by mapping white matter tracts (DTI).

The American Academy of Neurology now recommends that all cases of cerebral palsy of unknown origin undergo neuroimaging

Most children with cerebral palsy have abnormal neuroradiological findings, white matter damage being the most common.

lgorithm for the evaluation of the child with cerebral palsy (CP)

Cerebral palsy

Clinical AssessmentGoals of Physical Examination

Determine grades of muscle strength and selective control.

Evaluate muscle tone and determine type.Evaluate degree of deformity / contracture at

each joint.Assess linear, angular and torsional deformities

of spine, long bones, hands and feet.Appraise balance, equilibrium and standing /

walking posture.

Cerebral palsy

Goals of Management (Treatment)

Turn focus of parents from the disease to the goal-oriented approach

needs time and a lot of discussion

Physician and Physiotherapist must have the same perspective

Cerebral palsy

Types of Management (Treatment)

Physical therapyOrthoticsControl of spasticityOrthopedic surgery

Cerebral palsy

Spasticity

Approaches : Selective dorsal rhizotomy Intrathecal baclofen Botulinum-A toxin

Cerebral palsy

Selective Dorsal RhizotomyCut 30 – 50 % of abnormal dorsal rootlets L2 - S1Followed by intensive physiotherapyResults encouragingMay cause hyperlordosis / hip subluxationBest for : spastic diplegia, 4-8 yrs, no previous

surgery, no contractures, no extra pyramidal signs

? Not enough aloneOrthopedic procedures obtain similar results

Cerebral palsy

BaclofenGABA agonist – inhibits release of excitatory

neurotransmitter at level of spinal cordOral : mixed reports/ side effects/ not selectiveContinuous intrathecal – implantable pumpGood results in releasing spasticity, and

improving functionComplications of pump and catheterNeeds specialized centers

Cerebral palsy

Botulinum-A toxinActs at myo-neural junctions

Inhibits exocytosis of AcetylcholineInject selected muscles at multiple sitesSpasticity reduction may last up to 6 monthsReversible , painless , minimal side effectsMost patients still require lengthening for

permanent correctionRole : - Facilitates physiotherapy and

mobilization - Delays surgical management - Trial to determine effects of

specific proposed surgical treatment

Cerebral palsy

Physical Therapy

Involve parents as much as possible (even if they resist)

Do not raise false hopeswhich could increase frustration

Cerebral palsy

Physical TherapyThere is no evidence that any type of physical therapy can have a beneficial lasting effect on

motor function beyond early to middle childhood (age 4-8 years).

Thomas S. Renshaw ( Lovell & Winter’s Pediatric

Orthop.)

Cerebral palsy

OrthoticsImmobilization may cause atrophyNight splints : - Do not prevent nor reduce deformity - may cause irritation, pain or stimulate

reflexes in spastic muscles and relaxes the weaker apponents – thus may increase deformity rather than reduce it !

May be useful only in Athetoid

Cerebral palsy

Prerequisites for effective surgery

Type : spasticExtent : hemiplegics / diplegics : good results quadriplegics : minimal

improvementAge : 3- 12 yearsIQ : goodGood upper limb function : for walkingUnderlying muscle power : not weakWalker / non-walker : surgery hardly changes state but improves gait

Cerebral palsy

Prerequisites for effective surgery

Type : spasticExtent : hemiplegics / diplegics : good results quadriplegics : minimal

improvementAge : 3- 12 yearsIQ : goodGood upper limb function : for walkingUnderlying muscle power : not weakWalker / non-walker : surgery hardly changes state but improves gait

Cerebral palsy

Timing For Orthop SurgeryFor structural changes : Early e.g. Hip subluxation , usually <5 yearsTo improve function ( gait ) : defer until walking ( independently / with aids ) until gait pattern develops and could be

assessed walking : 18 – 21 months in hemiplegia 3 – 4 years in spastic diplegiaOptimum time of lower extremity surgery 5 – 7 years: can analyze and observe gait

pattern

The ‘‘Birthday Syndrome’’

One group of complications related to a chain of operations over the years is socialisolation, loss of motivation, frustration, and psychosocial problems termed the birthdaysyndrome.31

SEMLARASSSingle Event Multilevel Lever Arm

Restoration Anti Spasticity surgery

Thanks