[PDF] - Cerebral palsy Cerebral palsy Cerebral Palsy: Aetiology, Cerebral PDF Document

SLIDE 1

1 Cerebral Palsy: Aetiology, Cerebral Palsy: Aetiology, Associated Problems and Associated Problems and Management Management g

Lecture for FRACP candidates Lecture for FRACP candidates July 2010 July 2010

Cerebral palsy Cerebral palsy

Definitions and

Definitions and prevalence prevalence

Risk factors and

Risk factors and ti l ti l aetiology aetiology

Associated problems

Management options

What is cerebral palsy? What is cerebral palsy? Definition (Bax 1964) Definition (Bax 1964)

Cerebral palsy is Cerebral palsy is a disorder of a disorder of movement and movement and t d t t d t posture due to a posture due to a defect or lesion defect or lesion

f the immature
f the immature

brain brain

Cerebral palsy Cerebral palsy – – an umbrella an umbrella term term

All children are All children are different different The associated The associated problems may be problems may be p y p y more significant than more significant than the motor disorder the motor disorder Permanent, non Permanent, non-

progressive but not

progressive but not unchanging unchanging

New definition of cerebral palsy New definition of cerebral palsy

Cerebral palsy describes a group of Cerebral palsy describes a group of developmental disorders of movement and developmental disorders of movement and posture, causing activity restriction or posture, causing activity restriction or disability, that are attributed to disturbances disability, that are attributed to disturbances

ccurring in the fetal or infant brain. The
ccurring in the fetal or infant brain. The

motor impairment may be accompanied by a motor impairment may be accompanied by a seizure disorder and by impairment of seizure disorder and by impairment of sensation, cognition, communication and/or sensation, cognition, communication and/or behaviour behaviour

(Rosenbaum 2007) (Rosenbaum 2007)

SLIDE 2

2 Storm’s words Storm’s words Classification systems Classification systems

Nature of Nature of movement disorder movement disorder eg spasticity, eg spasticity, dystonia ataxia dystonia ataxia dystonia, ataxia dystonia, ataxia Distribution eg Distribution eg diplegia, diplegia, quadriplegia quadriplegia Severity Severity

Why does classification matter? Why does classification matter?

Descriptors for parents Descriptors for parents Type of treatment needed Type of treatment needed Prognosis Prognosis Associated problems Associated problems Information for service Information for service providers providers Research studies Research studies – – drawing together similar drawing together similar groups groups

Type of motor disorder Type of motor disorder

Spasticity

Dyskinesia

Athetosis Athetosis Dystonia Dystonia Ataxia

Ataxia

Hypotonia

Mixed

Distribution Distribution

Quadriplegia

arms arms legs legs trunk trunk head and neck head and neck Diplegia

Diplegia

Hemiplegia

Severity Severity

Mild

walks independently walks independently Moderate

Moderate

walks with sticks / walks with sticks / frame frame Severe

Severe

wheelchair dependent wheelchair dependent

SLIDE 3

3 Classification of severity Classification of severity Gross Motor Gross Motor Function Function Classification Classification Classification Classification System (The System (The “GMFCS”) “GMFCS”) The GMFCS The GMFCS

Level 1 Level 1 – – walk without walk without restrictions restrictions Level 2 Level 2 – – walk walk independently but more independently but more limitations limitations limitations limitations Level 3 Level 3 – – need mobility need mobility devices eg frames devices eg frames Level 4 Level 4 – – sit on a regular sit on a regular chair, but use wheelchair chair, but use wheelchair Level 5 Level 5 – – no means of no means of independent mobility independent mobility

Measuring motor progress Measuring motor progress

The The Growth Growth Motor Motor Curves Curves

The Growth Motor Curves The Growth Motor Curves

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4

What causes cerebral palsy? What causes cerebral palsy?

Case history of Lisa Case history of Lisa

PMH PMH

– Pregnancy and birth Pregnancy and birth normal normal – Crawled 10 months Crawled 10 months

At 8 years At 8 years

– Presented to RCH Presented to RCH – Falling more Falling more – Motor problems had Motor problems had – Sat 12 months Sat 12 months

At 24 months At 24 months

– Diagnosis of spastic Diagnosis of spastic diplegia diplegia deteriorated deteriorated

History History

Little Little: : poor poor obstetric

bstetric care

care associated associated with with birth birth asphyxia asphyxia responsible responsible for for most most cases cases Freud Freud: adverse adverse fetal fetal events events early early in in Freud Freud: adverse adverse fetal fetal events events early early in in development development may may cause cause both both birth birth complications complications and and spasticity spasticity

Changing views Changing views

1960 1960’s ’s: : mechanism mechanism for for the the prevention prevention of

f

kernicterus kernicterus 1970 1970’s ’s: increased increased resources resources for for obstetric

bstetric

1970 1970 s: increased increased resources resources for for obstetric

bstetric

and and neonatal neonatal care, care, for for example, example, increased increased Caesarean Caesarean Section Section rate rate and and use use

f
f

electronic electronic fetal fetal monitoring monitoring

Results of changes in Results of changes in practice practice

No major change No major change in cerebral in cerebral in cerebral in cerebral palsy rates palsy rates

Prevalence of cerebral palsy Prevalence of cerebral palsy

2 per thousand 2 per thousand live births live births 130 new cases in Victoria 130 new cases in Victoria each year each year

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5

Rates of CP, neonatal deaths and Rates of CP, neonatal deaths and stillbirth rates stillbirth rates

Cerebral palsy, neonatal death and stillbirth rates Victoria, 1973 - 1999

12 14 s Stillbirths 2 4 6 8 10 1973 1976 1979 1982 1985 1988 1991 1994 1997 Year of birth Rate per 1000 birth Neonatal deaths Cerebral palsy

Gender Gender

Males are over

Males are over represented in all represented in all p case series of case series of cerebral palsy cerebral palsy

Risk factors and causes: Risk factors and causes: challenges challenges

Many risk factors, for example, prematurity, but few Many risk factors, for example, prematurity, but few definite causes definite causes

– Rates 25 Rates 25-

30 x higher in infants weighing less than 1500 g

30 x higher in infants weighing less than 1500 g – Babies who weigh <2500g account for 1/3 of children with Babies who weigh <2500g account for 1/3 of children with cerebral palsy cerebral palsy

Causal pathways Causal pathways – – a series of factors leading to the a series of factors leading to the damaging event damaging event

Why do premature infants Why do premature infants develop cerebral palsy? develop cerebral palsy?

1. Are they damaged
1. Are they damaged

before birth and then before birth and then survive with good survive with good neonatal care? neonatal care? neonatal care? neonatal care?

2. Do they develop
2. Do they develop

complications of complications of prematurity such as prematurity such as IVH? IVH?

When does cerebral palsy When does cerebral palsy

ccur?
ccur?

Antenatally

Antenatally 75% 75%

Perinatal

Perinatal 10 10 -

15%

15%

Postnatal

Postnatal10% 10%

Postnatal

Postnatal10% 10%

The cause remains unknown in a substantial

The cause remains unknown in a substantial proportion of cases, and is therefore attributed to proportion of cases, and is therefore attributed to antenatal events antenatal events

Prenatal events Prenatal events

Prenatal (75%)

Brain malformations Brain malformations eg cortical dysplasias eg cortical dysplasias Intrauterine infections Intrauterine infections eg CMV eg CMV

SLIDE 6

6 Prenatal events Prenatal events

Vascular eg infarct Vascular eg infarct in area of middle in area of middle cerebral artery cerebral artery y Metabolic eg iodine Metabolic eg iodine deficiency deficiency Toxic eg lead, Toxic eg lead, mercury mercury

Perinatal events Perinatal events

Perinatal

Perinatal 10 10 -

15%

15% Hypoxic Hypoxic ischemic ischemic encephalopathy encephalopathy Infection Infection

“Birth asphyxia” “Birth asphyxia”

Even in the group where there appears Even in the group where there appears to be evidence of asphyxia, to be evidence of asphyxia, it may not have been preventable it may not have been preventable

Post neonatal causes Post neonatal causes

Postnatal

Postnatal10% 10% injury injury accidental accidental non non-

accidental

accidental infections infections

meningitis

encephalitis

New directions New directions

The Australian The Australian Cerebral Palsy Cerebral Palsy Register Register

– A collaboration A collaboration between all the between all the States and the States and the AIHW AIHW

New directions New directions

The Victorian The Victorian Cerebral Palsy Cerebral Palsy Register Register Collects information Collects information about all children and about all children and young adults with young adults with cerebral palsy born in cerebral palsy born in Victoria since 1970 Victoria since 1970

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7 New directions New directions

Role of thrombophilic Role of thrombophilic mutations mutations – – Factor V Factor V Leiden mutation and Leiden mutation and

ther coagulopathies
ther coagulopathies

Maternal infection Maternal infection – Maternal infection Maternal infection urinary tract infection, urinary tract infection, chorioamnionitis chorioamnionitis Multiple pregnancy Multiple pregnancy Low birth weight Low birth weight More information from More information from MRI MRI

Prevention of cerebral palsy Prevention of cerebral palsy

This will only be

This will only be achieved when more achieved when more information is information is available about the available about the multiple causes multiple causes

Diagnosis Diagnosis

Follow up of “at risk” infants, e.g., those of

Follow up of “at risk” infants, e.g., those of low birth weight low birth weight

Delayed motor development, eg, delay in

Delayed motor development, eg, delay in learning to sit and stand learning to sit and stand

Abnormalities of behaviour

Early signs of cerebral palsy Early signs of cerebral palsy

Assymmetric motor development

Assymmetric motor development

fisting of one hand fisting of one hand early favouring of one hand for reaching early favouring of one hand for reaching early favouring of one hand for reaching early favouring of one hand for reaching and grasping and grasping assymmetrical position in crawling assymmetrical position in crawling “limp” when starts to walk “limp” when starts to walk

Early signs of cerebral palsy Early signs of cerebral palsy

abnormalities of muscle tone

persistence of primitive reflexes

feeding problems

abnormalities of behaviour

Difficulties in diagnosis Difficulties in diagnosis

Different grades of severity

Wide range of intellectual ability

Signs may be late in appearance

Prematurity

SLIDE 8

8 Collaborative perinatal project Collaborative perinatal project

229 children with abnormal signs

229 children with abnormal signs during the first year of life during the first year of life

At 7 years of age, 118 were free of

At 7 years of age, 118 were free of motor handicap motor handicap

Diagnostic approach Diagnostic approach

MRI brain recommended MRI brain recommended (American Academy of (American Academy of Neurology Practice Neurology Practice Parameter) Parameter)

– Genetic counselling Genetic counselling – Establishing timing Establishing timing

The majority of children The majority of children will have abnormalities will have abnormalities

– 80 80-

90 % with MRI

90 % with MRI – 75% with CT 75% with CT

Diagnostic approach Diagnostic approach

Genetic and metabolic causes are unusual Genetic and metabolic causes are unusual but should be considered in the presence but should be considered in the presence

f a normal MRI
f a normal MRI

– the true prevalence of these disorders in CP the true prevalence of these disorders in CP is not known is not known – Some may be treatable eg dopa responsive Some may be treatable eg dopa responsive dystonia, glutaric aciduria, biotidinase dystonia, glutaric aciduria, biotidinase deficiency deficiency – Some may have associated problems that Some may have associated problems that need treatment eg Leisch need treatment eg Leisch-

Nyhan Disease

Nyhan Disease

What can MRI tell us about What can MRI tell us about timing? timing? Brain malformations: 12 Brain malformations: 12 – – 20 20 weeks weeks Periventricular white matter injury: Periventricular white matter injury: Periventricular white matter injury: Periventricular white matter injury: 26 26 – – 34 weeks 34 weeks Cortical and subcortical gliosis: 36 Cortical and subcortical gliosis: 36 – 44 weeks 44 weeks

Confirming the diagnosis Confirming the diagnosis

A period of A period of

bservation may be
bservation may be

necessary necessary

Multidisciplinary

Multidisciplinary assessment may be assessment may be helpful helpful

Issues in management Issues in management

Associated

Associated disabilities disabilities

Health problems

Consequences of

Consequences of the motor disorder the motor disorder

SLIDE 9

9 Associated disabilities Associated disabilities

Epilepsy Epilepsy – Occurs in about 40% of all children with Occurs in about 40% of all children with cerebral palsy cerebral palsy – Most common in spastic quadriplegia (50% Most common in spastic quadriplegia (50% - Most common in spastic quadriplegia (50% Most common in spastic quadriplegia (50% 94%) and hemiplegia (33% 94%) and hemiplegia (33% -

50%)

50%) – Higher incidence of refractory seizures and Higher incidence of refractory seizures and admissions for status epilepticus (Gururaj et admissions for status epilepticus (Gururaj et

al. Seizure 12:2;2003)
al. Seizure 12:2;2003)

– Few population based studies Few population based studies

Associated disabilities Associated disabilities

Hearing problems

Visual deficits

squints squints refractive errors refractive errors field defects field defects cortical visual cortical visual impairment impairment

Associated disabilities Associated disabilities

Cognitive deficits Cognitive deficits

Children may have intellectual, learning

Children may have intellectual, learning and perceptual problems. Assessment can and perceptual problems. Assessment can be difficult in the presence of severe be difficult in the presence of severe physical disability physical disability

Health problems Health problems

Nutritional problems

Under nutrition and failure Under nutrition and failure to thrive to thrive

Diffi

lti ith ki d Diffi lti ith ki d

Difficulties with sucking and

Difficulties with sucking and swallowing swallowing

Tongue thrusting

Decreased tongue

Decreased tongue movements and lip closure movements and lip closure

Hypo or hypersensitive gag

Hypo or hypersensitive gag response response

Meal times become long and

Meal times become long and carers anxious carers anxious

Health problems Health problems -

undernutrition

undernutrition

235 participants with GMFCS 111, 1V and 235 participants with GMFCS 111, 1V and V, average age 9.7 years V, average age 9.7 years

– Indicators of malnutrition common Indicators of malnutrition common

47% of children had weight < 5% for age/gender 47% of children had weight < 5% for age/gender 47% of children had weight < 5% for age/gender 47% of children had weight < 5% for age/gender 68% had short stature 68% had short stature

– Correlated with increased health care Correlated with increased health care utilisation and decreased participation in utilisation and decreased participation in normal activities normal activities (Samson (Samson-

Fang et al J Pediatr 141;5:2002

Fang et al J Pediatr 141;5:2002

Gastrostomy Gastrostomy

Avoids aspiration

Often improves

Often improves nutrition nutrition

Saves many hours of

Saves many hours of meal time assistance meal time assistance which can be devoted which can be devoted to other activities to other activities

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10

Impact of quality of life on carers Impact of quality of life on carers

At 12 months after At 12 months after gastrostomy placement, gastrostomy placement, carers reported carers reported Reduction in feeding Reduction in feeding times, increased ease of times, increased ease of drug administration drug administration g Reduced concern about Reduced concern about their child’s nutritional their child’s nutritional status status Significant measurable Significant measurable improvement in quality of improvement in quality of life of carers life of carers (Sullivan et al Dev Med (Sullivan et al Dev Med Child Neurol 46:2004) Child Neurol 46:2004)