CEREBRAL PALSY

C. ADNAMS

Child Health Unit and Division of

Child Development and Neurosciences
UCT
2006
Cerebral Palsy

a
health paradigm for
developmental disability
CP: Paradigm for disability

– Complex, multisystem condition

– Motor, cognitive, sensory, behavioural

aspects

– Includes entire spectrum of developmental

disabilities
 Where are we with
classification?
Old Definition (Bax 1964)
 Disorder of movement and posture due to
a defect or lesion of the immature brain

 Placed entire focus on motor aspects

 Classification by topography
 What are the cerebral palsies?
Another ‘old’ (but good) definition
An umbrella term covering a group of non-progressive but often changing,
motor impairment syndromes 2° to lesions or anomalies of the brain
arising in the early stages of its development (Mutch et al 1992)

Brain lesion neither resolves nor progresses

 What are the cerebral palsies?
 Term defined by clinical description only
 No definitive test
 Poor comparability
 Imprecise - clinical picture changes (time)
 If clinical symptoms disappear or become progressive, review Dx
 Proposed Definition and
Classification
 July 2004 – group of international experts
 Availability of new knowledge about neurobiology,
dev. brain injury from epidemiology and modern
brain imaging, precise measuring instruments,
increased studies in therapies
 Need to change present definition and
classification
 Increase communication between clinicians ,
investigators and public health workers
 Proposed revised definition and
classification 2005 (DMCN)
 “C.P. describes a group of 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 are often accompanied by disturbances of
sensation, cognition, communication, perception,
and/or behaviour, and/or by a seizure”. !!!
Proposed classification
Motor abnormalities:
a) nature and type
b) functional motor abilities (extent of limitation
/severity measure by objective functional scales)
Associated impairments:
(neurodevelopmental or sensory e.g. seizures,
visual, attentional, behavioural etc)
Anatomic distribution and radiological findings:
Causation and timing:
Advances in epidemiology:
How common is cerebral palsy?

Ofall conceptions:
- > 15% will miscarry

Ofall births
- 5% birth impairment
- 6% preterm (< 37w)
- 0.2% cerebral palsy by
age 5yrs (2/1000)
- 0.4% intellectual disability
Stanley et al
 Epidemiology:
Changing paradigms of causation
 In the past
- single factors e.g. birth hypoxia
- explained small % etiology
 Recent
- end result of several causal pathways of
interdependent factors
- often no recognised antecedents
Causal pathways of cerebral palsy
 identification of prenatal causes (imaging, MRI, U/S)
 contribution of prematurity to birth events
 increased survival of preterms
 multiple births (infertility)
Causes of cerebral palsy:
Prenatal (early)
 Maternal / Fetal-placental
 Fetal malformation syndromes (cerebral
dysgenesis, neuronal migration defects)
 Genetic Syndromes (30% ataxic CP; inborn errors
of organic, amino acids)
 Chromosomal abnormalities
 Intra -uterine infections (cmv, rubella)
 Intrauterine vascular events
 Teratogens
Causes of cerebral palsy:
Perinatal
 Hypoxic ischaemic encephalopathy
 Labour / delivery problems
 Infection / sepsis
 Biochemical (kernicterus)
 Prematurity (*complications of)
 Low Birth Weight (*association with)
Causes of cerebral palsy:
Postnatal / Childhood
 Meningitis / encephalitis
 Traumatic Brain Injury
 Toxins

Idiopathic
Motor Classification of cerebral
palsies
 Spastic (80%)
 Dyskinesia/Extrapyramidal (20%) (mixed / associated with spasticity)
– dystonic, athetoid, ataxic
 Ataxia, hypotonia - seldom alone
 Isolated hypotonia associated with intellectual disability not classified as C.P
 All co-exist
 Most classified by predominant feature
 Topograpy

 Monoplegia
 Hemiplegia
 Diplegia
 Triplegia
 Quadriplegia
 Paraplegia (spinal cord
injury)
 Health management of children
with cerebral palsy
Associated problems
- special needs

- almost all individuals

have at least one other
developmental
disability
 Approach to health management of CP

Optimal assessment
and management

- comprehensive

- multidiciplinary
Diagnosis and Assessment in CP
Clinical
 Neurological problems routinely sought in children with delay / deviance in development and growth

Examination
 Dysphmorphism and congenital anomalies
 Supine and prone
 Nb: head, hips, spine
 Neurodevelopmental: focus on tone (DTR’s, primitive reflexes), postures, body movements, joint mobility
 Functional skills and developmental assessment
When to diagnose cerebral palsy
in a child
 Motor Impairment
– neurologically detectable involuntary resistance to passive stretch
– abnormal reflexes
– clumsiness alone not sufficient
Investigations
 Guided by history and examination
 Neuroimaging (correlate clinical/ neuroanatomy)
 Chromosomes, specific genetic or diagnostic tests
 Metabolic :
– Amino, organic acids, thyroid functions
– Ig’s /AFP- (ataxia)
 X-rays - hips, (spine)
 U/S (GOR)
 Electrophysiological studies ( EEG, VER, ERG, BAER)
Management of children with
cerebral palsy
Associated problems
- special needs
- almost all at least one other disability
most common:
- cognitive (> 60%)
- more limbs, more spastic, worse outcome
- hemiplegia, best outcome
- perceptual impairments, LD common
Cerebral palsy
Associated problems
Sensory deficits
 Vision
- strabismus (>50%)
- cortical visual impairment
- refractive errors
- amblyopia
- visual perceptual impairment
Cerebral palsy
Associated problems
Sensory deficits
 Hearing (10% esp. post CNS infection)
 Communication disorders
– expressive, receptive
– motor and/or central processing
 Seizures
 Nutrition/Feeding problems (also dental)
 Orthopaedic
- contractures, subluxed/dislocated hips
 Behavioural and/or emotional
- self injurious behaviour (SIB)
- sleep disturbance
- attentional deficits
 2º disability
- mental health, behaviour, social
adaptation , ↑in adolescence
Seizures in Cerebral palsy

 30 %
 manifest by 2 years
 usually tonic-clonic, partial complex
Rx: Seizures in Cerebral palsy
 Phenobarbitone daily (severe/profound I.D.)
 Na Valproate (Epilim) tds, tonic-clonic
 Carbemazepine (Tegretol), tds, focal
 Valium prn
 (Phenytoin) (Epanutin)
 Other newer generation antiepileptics for difficult to control seizures
 Nutrition related problems in
cerebral palsy
 Special health needs
 At risk for nutritional, growth, developmental problems
 Food intolerance, allergies
 GOR, vomiting, oral motor dysfunction
 Constipation
 Management of growth
 Regular nutritional
assessment
 Anthropometric
assessment
 Regular follow-up
 Adequate management of
other health
problems
 Feeding problems
 90% clinical signs of oro-motor dysfunction (Reilly, UK 1996)
 Sucking and swallowing problems, RTI common
 NB predictor of low growth outcome
 Failure to thrive (severe CP > at risk)
 NB referral to feeding therapist (speech / dietician)
 Percutaneous Endoscopic
Gastrostomy (PEG)

 Effective, simple, few risks

 Significant improvement in growth and
development
 50% paediatric insertions for CP
Interventions
 Mutisystemic Ξ Mutidiciplinary

 Understand prognosis and establish

priorities
– Picture of child’s future

– How body will grow and how children learn

to move
– How changes in CP will lead to deformity
– How we can influence this process

– What environmental factors influence natural

history of CP
Choice of Interventions
 Explosion evolving interventions

 Wide range – conventional to crazy

 Includes general care, oral meds, intrathecal meds,

surgery (orthopaedics, rhizotomy, gastrostomy),
ganglion pacing, Botox, muscle strengthening, seating,
positioning, orthotics, casting, lifestyles, broad range
physio’s, O.T, speech and oral therapy
Best Interventions
 Most important :
correct aims of
treatment

 Better to achieve
small part of important
gain than all of a less
important one.
 Physical (Re)habilitation
Aim:- -Improve function by increasing
day to day competence -
(better patterns of movement,
posture, increased joint R.O.M)

-Reduce activity limitations

-Resolve participation restrictions (WHO)

Conventional physical therapy
 Physiotherapy (mainstay)
- mobility, co-ordination, positioning
 Occupational therapy
- co-ordination, fine motor activities
 Speech and language therapy
- oral co-ordination, drooling
- alternative augmented communication
 Feeding therapy
Orthopaedic management
 Orthopaedic Clinical Examination

joint flexion contractures (hips, knees,

achille’s tendons)
spine (scoliosis)

subluxation, dislocation of hips
 Orthopaedic Surgery
 Enables mobility (releases contractures)
 Prevents/reduces hip subluxation
/dislocation
 Soft tissue release hip (adductors, psoas mm’s,
neurectomy, tenotomy) hamstrings, achille’s
tendons
 Reconstructive surgery
 Neurosurgery - rhizotomy (selected cases)
 Neurosurgery

Rhizotomy
-selected circumstances
 Selected Therapies

Botulinum A Toxin
 Effective adjuvant to other therapies
 Increases stretch in relaxed muscle
 Reduces dynamic spastic deformity
 Selected indications
Assistive Devices
Cerebral palsy: School Placement
Cerebral palsy: School Placement for

Associated cognitive problems

 Learning Disorder (>15 verbal, non verbal IQ)
 - LSEN School
 Intellectual Disability

- Mainstream or LSEN School (mild ID)

- LSEN (Training Centre) (moderate I.D)
- Special care + Care Grant (severe I.D.)
 Motor Disability only

- Mainstream or LSEN
 Social Assistance
 Child Support Grant
 Poverty alleviation
 All children 0 - 14 years (2006)
 Means test (income) - about R1000 per mth
 R190 per mth (April 2006)
 Social Assistance
 Care Dependency Grant
 Severe physical or intellectual
disability, 24-hour care
 Public service employed MO’s
certificate
 R840 per mth April 2006 (means test)
Transitional care in cerebral palsy
 Role of paediatrician, family practitioner
 Continuity of care, liaison
 Ongoing management of common and complex health problems
 Advocacy for child and family as child approaches adulthood
Counseling in cerebral palsy
 What to say

Address family coping
 Life Expectancy
– depends on severity of I.D., level of care, other health problems, mobility, ability to feed
– normal IQ has normal life expectancy