Cerebral PalsyHealth Surveillance

Christopher Ewing R1University of CalgaryPediatrics

What is Cerebral Palsy? Most common cause of chronic

neurological impairment in children Permanent, non-progressive brain injury Motor movement and posture impairments

as well as disturbances in: Sensation and perception Cognition and communication Gastrointestinal function and nutrition Social and behavioural Epilepsy

April 2006

Cerebral palsy (CP) 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 behaviour; by epilepsy, and by secondary musculoskeletal problems.

Epidemiology Most common cause of childhood

disability in Western society. Incidence approximately 2-3/1000 births Incidence has not declined despite

improvements in perinatal and obstetrical care.

However more premature and VLBW children with severe disabilities are surviving.

Etiology Often can only be described in 50% of cases Complex and multifactorial May result from structural abnormalities of

the brain or be acquired Prenatal/perinatal/postnatal injury due to:

Vascular insufficiency Toxins Infections Prematurity Birth asphyxia

Differential Diagnosis Progressive:

Glutaric aciduria type 1 Arginase deficiency Sjogren-Larsson syndrome Metachromic leukodystrophy Lesch-Nyhan syndrome Chiari type 1 malformation Dandy-Walker malformation Angelman syndrome Gillespie syndrome Ataxia-Telangectasia Hexoaminidase A and B

deficiency Serotendinosus

xanthomatosis

Non-progressive Intellectual

disability Deprivation Malnutrition Isolated non-motor

and motor handicaps Spina bifida Myopathies

Risk Factors

Prenatal Prematurity LBW/VLBW Maternal epilepsy Hyp(o/er)thyroidism Infections (TORCH) 3rd TM bleeding Drug abuse Trauma Multiple pregnancies Placental insufficiancy

Perinatal Prolonged labour Premature ROM Presentation anomaly Bleeding prior to labour Bradycardia Hypoxia

Postnatal (0-2 years) CNS infection Hypoxia Seizures Coagulopathies Hyperbilirubinemia Head trauma

CNS Pathology CP lesions often occur in regions sensitive to

blood supply disturbance. Hypoxic-ischemic-encephalopathy (HIE)

Motor Primary impairments:

Tone (spasticity, dystonia) Balance Strength Loss of selectivity Sensation

Secondary impairments Contractures Deformities

Tertiary impairments (adaptive mechanisms)

Contractures & Deformities Contractures

Upper extremity: pronator, wrist and finger flexors, thumb adductor

Lower extremity: hip adductor/flexor, knee flexor, ankle plantar flexors

Deformities Spine: scoliosis, kyphosis Hip: subluxation, dislocation Femur/tibia: internal/external torsion Foot: equinus, valgus, varus

Natural History Brain lesion is incurable but non-progressive Clinical picture changes as child grows and

develops due to growth and maturation of the CNS and MSK.

Primary movement disorder not evident early on in infancy, although some abnormality may be present

Primitive reflexes persist, and advanced postural reactions fail to develop.

Early intervention can minimize functional effects of neurological impairments.

Early Signs Many infants appear normal Abnormal behaviour

Excessive docility/irritability Poor eye contact or sleep

Oromotor problems Frequent vomiting Poor sucking Tongue retraction or thrusting Grimacing

Poor mobility Poor head control Hand preference, fisting prior to age 2 Abnormal tone, asymmetric posture

Major Deficits in CP Loss of selective motor control, and

dependence on primitive reflex patterns for movement

Abnormal muscle tone influenced by posture, position, and movement

Imbalance between agonist and antagonist muscles that may lead to contractures and deformities

Impaired body balance mechanisms Sensory loss

Associated Problems Seizures Visual impairments Intellectual impairment Learning disabilities Hearing Communication Oromotor dysfunction Gastrointestinal Nutrition Dental Respiratory dysfunction Bladder and bowel Social and emotional

Multidisciplinary Care Pediatrician Physiatrist Developmental pediatrician Orthopedic surgeon Neurologist Speech therapy Physical therapy Occupational therapy Orthotist Dietician Ophthalmologist Audiologist Teacher Social Worker Psychologist

Traditional Classifications Spastic

Hemiplegic Diplegic Quadriplegic

Dyskinetic Choreoathetoid Dystonic

Ataxic

Traditional Classifications

Modern Principles of classification

Motor abnormalities Nature and typology of motor disorder Functional motor abilities

Associated impairments Anatomical distribution Radiological findings Causation and timing

Health Surveillance

Regular primary and specialized care required to optimize the various and potentially progressive and debilitating health issues in cerebral palsy

History Developmental milestones Gross motor function:

head control, sitting, crawling, walking, running

Fine motor function and ADL’s: feeding, dressing, toilet care, hand function

Speech and sensory Pain Social and psychological needs Caregiver burden, financial and community

supports

History Associated impairment surveillance

Seizure frequency and management Bowel and bladder function Communication needs Oromotor function drooling Weight gain Respiratory function Pressure sores School function

Physical Examination Neurological

Mental status, cranial nerves vision, hearing, strength, control, reflexes, tone, involuntary movements, sensory

Musculoskeletal Range of motion, contractures,

deformities, posture Cardiorespiratory

Abdominal Skin breakdown

Musculoskeletal Exam Back

Spinal deformities (scoliosis, kyphosis, lordosis) Pelvis

Obliquity, stability and sitting balance Hip

rotation, flexion/adduction contractures Knee

Patella position, popliteal angle, contractures Foot and ankle

Gastroc/soleus, tibial torsion, equinus/varus/valgus Upper extremity

ROM, grasp, pronator contracture/spasticity, function

Functional Examination Communication Sitting Balance Mobility Gait Running, jumping Hand function Ability to perform ADL’s

Gait Assessments Computerized gait

analysis Kinematics Dynamic EMG Kinetics Energetics

Observation Normal gait Balance Propulsion

Video recording

GMFCS 1. Walks without

restrictions 2. Walks without assistive

devices but limitations in community

3. Walks with assistive devices

4. Transported or uses powered mobility

5. Severely limited, dependent on wheelchair

MACS 1. Handles objects easily and

successfully. 2. Handles most objects but with

somewhat reduced quality and/or speed of achievement.

3. Handles objects with difficulty; needs help to prepare and/or modify activities.

4. Handles a limited selection of easily managed objects in adapted situations.

5. Does not handle objects and has severely limited ability to perform even simple actions.

Imaging

X-ray Cranial ultrasound Cranial CT Cranial MRI EEG

Treatment Goals Infancy

Optimize physical development, nutrition, and exercise

Childhood Maximize independent mobility and nutrition

Preschooler Maximize independent mobility, function,

and minimize deformity Adolescence

Education, vocation and community integration

Treatment Principles Communication Activities of daily living Mobility Ambulation Psychosocial

Management Principles Consider the natural history Appreciate significance of sensation/perception Recognize limitation of treatments Focus on function and comfort, not deformity Provide functional mobility Establish appropriate priorities and shift with

age Emphasize child’s assets Maintain family health Avoid management fads Protect the child’s childhood and play

experience

Rehabilitation Physiotherapy Occupational therapy Bracing Assistive devices Adaptive technology Sports and recreation Environment modification

Rehabilitation Infancy

Educate the family, provide optimal sensorineural development with positioning, stimulation, and exercise.

Childhood Achieve independent mobility,

involvement in self care and ADL’s School age/adolescence

School integration, social, and vocational skills

Bracing Increases function Prevents deformity Keeps joint in functional position Stabilize the trunk and extremities Facilitate selective motor control Decrease spasticity Protect extremity from injury in the postoperative phase

AFO Ankle-Foot Orthotic

Most common brace used in CP Crucial in spastic hemi/diplegia

for mobility Maintains foot in plantigrade Provides ground contact and

foot clearance May prevent contractures if

worn at night Hinged, flexible, or solid

Mobility Aids Transfer aids and lifts Standers Walkers Crutches Canes Wheelchairs

Seating system

Spasticity Part of upper motor

neuron syndrome Increase in physiologic

tone as a resistance to passive movement

Velocity dependent Results in difficulty

with movement, posture, and function

May develop into contractures and deformities

Spasticity Treatment Rehabilitation Botox injection Systemic oral medications

Baclofen, benzos, dantrolene, tizanidine, clonidine

Intrathecal baclofen Surgery

Spasticity Problem Patterns Hemiplegic

Rectus femoris – stiff knee gait Gastrocnemius – pes equinus Arm flexor-pronators – forearm pronation Wrist/digit flexors - thumb in palm, wrist/digit flexion

Diplegic Hip adductor-flexors – hip subluxation Hamstring - knee flexion Gastrocnemius - pes equinus

Quadriplegic Hip adductor-flexors – hip subluxation Hamstring – sacral sitting

Hemiplegic 20% of spastic CP Involvement of unilateral arm and leg Upper extremity involvement > lower

extremity Usually few associated problems, may have

seizures, learning or behavioural issues Common MSK problems:

Forearm pronation, wrist flexion and ulnar deviation, thumb in palm, finger flexion, poor grasp, pes equinus, hemiplegic gait (foot drop, intoeing, knee stiff/flexion)

Diplegic 50% of spastic CP Gross motor involvement of lower extremities

and minor fine motor involvement of upper. Usually normal mental function and

communication Primary problem is walking Common MSK problems:

Hip flexion/IR/adduction and scissoring, ankle equinus, jump/crouch gait, risk of hip instability

Quadriplegic

30% of spastic CP Involvement of neck, trunk, and all four

extremities Severe motor impairment and other signs

and symptoms of CNS dysfunction Cognitive, seizures, speech and swallowing

Frequent comorbid medical associations Higher mortality Common MSK problems

Hip dislocation, spinal and postural problems

Dyskinetic Hyperkinetic/Choreoathetoid

Purposeless, often massive involuntary movements

Poor selective motor control/coordination Dystonic

Abnormal shifts in muscle tone in stereotyped pattern

MSK issues Ambulation dependent on severity, clumsy and

unsteady gait, motor planning and execution, scoliosis, degenerative hip disease

Spine Scoliosis

Most common spinal deformity in CP Progressive, causes difficulty with sitting

and respiratory function Related to severity of CP neuromuscular

involvement Hyperlordosis Hyperkyphosis Pelvic obliquity

Hip Surveillance Hip dislocation is a common concern in

cerebral palsy and can be debilitating Second most common MSK deformity in

children (second to equinus) Directly related to GMFCS Common in spastic CP Progressive instability due to:

Muscle imbalance, persistent primitive reflexes, faulty posture, absence of weight bearing.

Hip Surveillance

Surgery Corrective casting Tendon lengthening Split transfer Simple tenotomy Angular osteotomy Hip surgery Rotational osteotomy Arthrodesis Spinal surgery

Chronic Pain Results from:

Hip instability Reflux Back pain Arthritis Capsulitis Spasticity

Issues in Adulthood Pain Fractures Contractures Overuse syndromes Pressure sores Scoliosis Sexuality Nutrition Social and vocational

References The HELP Guide To Cerebral Palsy, Second

Edition. Berker, Yalcın. Merrill Corporation, Washington, USA, 2010

Proposed definition and classification of cerebral palsy, April 2005. Paneth et al, Developmental Medicine & Child Neurology 2005, 47: 571-576

Definition and Classification of Cerebral Palsy. Rosenbaum, Paneth, Leviton, Goldstein, Bax. Developmental Medicine & Child Neurology 2006.

References The updated European Consensus 2009 on

the use of Botulinum toxin for children with cerebral palsy. Heinen et al. European Journal of Pediatric Neurology 2010, 14: 45-66

A classification system for hip disease in cerebral palsy. Robin et al. Developmental Medicine & Child Neurology 2009, 51: 183-192

Consensus statement on Hip Surveillance for Children with Cerebral Palsy: Australian Standards of Care 2008. Wynter, Gibson, Kentish, Love, Thomason, Graham.

Appendices and Pictures

Thomas’ Test