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CEREBRAL PALSY: INTRODUCTION

Dott.ssa Sarah Bompard

Dott.ssa Alessandra Lacopo

Sarah.bompard@opbg.net

Alessandra.lacopo@opbg.net

Unit of Neurorehabilitation, Bambino Gesù Children’s Hospital

CEREBRAL PALSY

Cerebral palsy is a group of permanent disorders affecting the development of movement and posture, causing activity limitations, attributed to non-progressive disturbances occurring in the developing fetal or infant brain. (Bax,1964)

CEREBRAL PALSY

CEREBRAL PALSY: etiopathogenesis

Paul S et al. A Review on Recent Advances of Cerebral Palsy. Oxidative Medicine and Cellular Longevity 2022 Jul

AND POSSIBLE!!!

Term infant born healthy

Preterm infant

Upto24months,the milestonesof psychomotor developmentshould beassessed consideringthe correctedage (chronologicalage minustheperiodof prematurity).

Preterm infant

Pretermbirthisoneof themostsignificant riskfactorsforthe developmentof cerebralpalsy.

Preterm Infant: Key Considerations and Monitoring

Inthefirstmonths,itisessentialtopromptlyidentifymajordisabilities.

- ModeratetosevereCerebralPalsy(CP)withGrossMotorFunction ClassificationSystem(GMFCS)≥2. - Cognitivescorebelow70ontheBayleyScaleseGMFCS≥2 - Bilateralvisionimpairmentwithvisualacuitylessthan1/10

- Hearingimpairmentlimitingcommunicationdespitehearingaidsor cochlearimplant

How to Identify Neonates at Risk for Developing Cerebral Palsy?

Preconceptional Risk Factors:

•Previousspontaneousabortions

•Medicallyassistedreproduction

•Lowsocioeconomicstatus

•Parentscarryingchromosomal abnormalities

Surveillance of Cerebral Palsy Europe and Australian Cerebral Palsy Register inclusion criteria

Risk Factors During Pregnancy:

•Genetic defects

•Complications during delivery

•Multiple pregnancy (twinning)

•Male sex

•Maternal thyroid dysfunction

•Pre-eclampsia

•Infections

•Intrauterine growth restriction (IUGR)

•Prematurity

•Substance abuse

Surveillance of Cerebral Palsy Europe and Australian Cerebral Palsy Register

How to Identify Neonates at Risk for Developing Cerebral Palsy?

Perinatal Risk Factors:

•Acute intrapartum hypoxia

•Seizures

•Hypoglycemia

•Infections

Surveillance of Cerebral Palsy Europe and Australian Cerebral Palsy Register inclusion criteria

How to Identify Neonates at Risk for Developing Cerebral Palsy?

Postnatal Risk Factors:

•Stroke

•Neurological damage

•Infections

Surveillance of Cerebral Palsy Europe and Australian Cerebral Palsy Register inclusion criteria

Neonates at Risk for Cerebral Palsy: Referral to Paediatric Neurologist

International Guidelines for the Early Diagnosis of Cerebral Palsy

Before 5 months of corrected age:

Abnormal brain MRI, absent fidgety movements on General Movements

Assessment (GMA), and an abnormal Hammersmith Infant Neurological Examination (HINE) demonstrate a sensitivity of 97.86% and specificity of 99.22% for diagnosing CP.

International Guidelines for the Early Diagnosis of Cerebral Palsy

After 5 months of corrected age:

Abnormal brain MRI and a Hammersmith

Infant Neurological Examination (HINE) score below 73 predict CP in 90% of cases.

Cerebral MRI

Hammersmith Neurological Examination

HINEisastandardized neurologicalassessment tooldesignedtoevaluate motorandneurological functionininfantsaged2to 24months.Itassesses cranialnervefunction, posture,movements,tone, andreflexes,providinga scorethathelpsinearly detectionofneurological impairments,including cerebralpalsy.

Hammersmith Neurological Examination

Abnormal movementsof theeyes

Hammersmith Neurological Examination

Followstargetin anincomplete arc

Doubtfulreaction tostimuli

Noswallowing, enteralfeeding

Hammersmith Neurological Examination

Handswith persistent adductedthumb

Hammersmith Neurological Examination

Feetwith tendencyto standontiptoes

Trunkinsitting veryrounded

Hammersmith Neurological Examination

Increasedmuscle tonethatlimited alltherangeof movements

Hammersmith Neurological Examination

Armprotectionabsent

Tendonreflexbrisk

General Movements

Refer to spontaneous, complex, and variable movements involving the whole body, observed in fetuses and young infants. These movements are characterized by their fluency, variability, and complexity and are an important indicator of the integrity of the developing nervous system.

General Movements: abnormalities

Writhing Movements: frompretermageuntilabout 6–9weekspost-term.

Fidgety Movements: 9weekspost-termuntil20 weekspost-term

-poorrepertoire:monotonous movements

-crampedsynchronized:infant’s limbsandtrunkmusclescontract andrelaxalmostsimultaneously, producingstiffandrigidmovements withalackofsmoothness

-chaotic:movementsarelarge, abrupt,andlacksmooth coordination.

-abnormal

-absent

The Importance of Early Rehabilitation

"is the brain's ability to reorganize and form new neural connections in response to learning, experience, or injury. It allows the brain to adapt, recover from damage, and optimize function.".

The Importance of Early Rehabilitation

Earlyrehabilitation maximizes neuroplasticity.

The Importance of Early Rehabilitation

Earlyrehabilitationprevents secondarycomplications:

Hipdislocation

Jointcontractures

Scoliosis

The Importance of Early Rehabilitation

PREVENTION OF HIP DISLOCATION

The Importance of Early Rehabilitation

PREVENTION OF SPINE DEFORMITY

•High Prevalence & Risk Factors

Scoliosisoccursin20–25%ofCPpatients;riskincreaseswithseverity ofmotorimpairmentandreducedambulation.

•Functional Impact

Trunkimbalanceandpelvicobliquityaffectsitting,standing,and walking;severecurvescancauserespiratoryandskincomplications.

•Curve Progression Patterns

Curvesworsenrapidlyduringadolescenceandcontinuepost-skeletal maturityif>50°,especiallyinnon-ambulatorypatients.

•Curve Types

Group1:ambulatory,idiopathic-likecurves.

Group2:non-ambulatory,longcollapsingcurveswithpelvicobliquity.

•Conservative Management

Includesseatingadaptationsandspinalbracestoimprovepostureand delaysurgery,thoughtheydon’thaltprogression.

•Surgical Indications & Outcomes

Indicatedforcurves>45–50°,rapidprogression,orfunctionaldecline.

Goal:stable,balancedspinetoenhancequalityoflife.

The Importance of Early Rehabilitation

•Spasticity and dystonia often coexist inCPbutrequiredistinct treatment approaches; spasticity may respond to baclofen, while dystoniamayneeddeepbrainstimulation.

•Children with CP have shorter, weaker muscles with reduced fibre diameter, contributing to decreased strength and motor function.

•Physiotherapy should be goal-oriented and engaging, focusing on task-specific training and individualized strengthening,ratherthanpassiveorgeneralizedapproaches.

•Contractures develop progressively as muscles fail to keep pacewithbonegrowth;earlydynamiccontracturesmayrespond tocastingorBoNT-A,butfixedonesmayrequiresurgery.

•Preventive strategies like orthoses, bracing, and positioning arecommonlyused,thoughevidenceislimited;long-termfollowupandgaitanalysisareessentialforoptimizingoutcomes.

PREVENTION OF DEFORMITIES AND CONTRACTURES

From birth to 48 months:

Early intervention – Play and exploration

POSTURAL SUPPORT SYSTEMS

•Head–trunk–pelvis axis alignment

•Pelvic positioning and stability

•Lower limb positioning

•Upper limb freedom to support tabletop activities

•Proper posture during feeding

Greater pelvic containment with abducted sitting and asymmetry

Possibility of tilt-in-space and lumbar support

Reclining backrest with lateral supports

Straps with customized fastenings

Headrest integration

Height-adjustable footrest

High customization options for headrest selection

 Greater pelvic containment

 Reclining backrest with curved supports (pelottes)

 Custom belts with specialized fasteners

 Adjustable headrest

 Elevating footplate

 Rigid shell for support and tilt function

 More stability and comfort than semi-postural and commercial strollers

SYSTEMS FOR STANDING ASSISTANCE

•A multiposition system that allows continuous transition from supine to prone position without any adjustment or change in configuration.

 Standing frames support upright posture and promote weight-bearing in children with cerebral palsy.

 Some models include adjustable hip abduction to accommodate hip subluxation.

 Optional trays enable children to participate in play and therapeutic activities while standing.

GAIT TRAINER

•Provide postural support and promote upright mobility.

•Encourage independent movement and improve gait patterns.

•Can be anterior or posterior based on child’s needs and control.

•Aid in functional mobility, participation, and physical development.

SPINAL ORTHOSIS

BALANCED STATIC BRACE

• Orthosis made of elastic material

• Support and stabilization of the trunk and proximal segments in all three planes of space

• Freedom of movement for the limbs

ELASTO-COMPRESSIVE SUIT

• Properly designed and customized orthoses can realign and stabilize the body.

• They help correct or limit the worsening of mild dysmorphisms and paramorphisms.

• Their effectiveness relies on dynamic elastic actions of deflection and detorsion.

UPPER LIMB ORTHOSES

•Mainly made of elastic material

•Wrist and finger extension

•Wrist stabilization

•Thumb opposition/abduction

•Forearm supination/pronation

•Elbow extension/flexion

•Shoulder elevation/external rotation

•Stabilization of the scapulohumeral girdle

AFO

LOWER LIMB ORTHOSES

Carbon Orthosis

•Supports the ankle and foot, enhancing gait and preventing deformities

•Hybrid carbon orthosis: Lightweight, energyefficient, and preferred by children with cerebral palsy for improved gait and mobility

Hybrid

The Importance of Early Rehabilitation

There is no evidence supporting a change in the Gross Motor Function Classification System (GMFCS)level,BUTearlyinterventionallowsto:

Developfunctionalskills(e.g.,CIMT,containmentofmaladaptivepostures,AAC)

Preventsecondarydeformities

Improvequalityoflife.

The Importance of Early Rehabilitation

Patients with cerebral palsy (CP) who receive Goals-Activity-Motor Enrichment (GAME), an early and intensive motor intervention combined with parent education and environmental enrichment, demonstrate better motor and cognitive abilities at 1 year compared to those receivingusualcare.

The Importance of Early Rehabilitation

InfantswithhemiparesiswhoreceiveearlyConstraint-InducedMovement

Therapy(CIMT)showintheshorttermimprovedhandfunctioncompared tocontrols.

The Importance of Early Rehabilitation

Earlyinterventionoptimizesthemotorandcognitiveplasticityof theinfant,preventssecondarycomplications,andimproves caregiverwell-being.

The Importance of Early Rehabilitation

Regular follow-up, including pelvic Xrays from an early age, and early rehabilitative treatment are essential to prevent hip dislocation in children withseverecerebralpalsy.

The Role of Adapted Physical Activity (APA)

Adapted Physical Activity leads to an improvement in PHYSICAL and MENTAL FUNCTIONING.

• Improvement in pain

Rehabilitation + Adapted Physical Activity:

Improvement in physical functioning

• Improvement in motivation

• Improvement in self-sufficiency

The Role of Adapted Physical Activity (APA)

• BENEFITS OF PARTICIPATING IN APA

Increased well-being

Greater integration into the community

Improved muscle strength

Enhanced fundamental movement skills

• BARRIERS TO APA PARTICIPATION

Misconception: No programs available for this population

Misconception: Participation is unsafe or too risky

Misconception: Sports rules are too difficult to learn or cannot be adapted to include children with disabilities (CWD)

• FACILITATORS TO APA PARTICIPATION

Preliminary assessments to maximize safety with appropriate settings

Organizing sports focused on fun rather than competition

Healthcare professionals, PE teachers, and coaches who prescribe/suggest physical activity practices while recognizing individual needs

Adaptations such as longer rest periods, closer coach-to-athlete ratios, frequent positive feedback, and careful monitoring of fatigue or injury symptoms

The Role of Adapted Physical Activity (APA)

Vite che aiutano la Vita

Il presente documento è stato elaborato da Ospedale Pediatrico Bambino Gesù.

I contenuti sono strettamente riservati; è vietata la riproduzione e la divulgazione, anche solo parziale, senza il benestare scritto di Ospedale Pediatrico Bambino Gesù.

Grazie!