Classification, Epidemiology and Medical Management of Cerebral Palsy

Dr Jill KislerConsultant in Child Development and Neurodisability

Child Development Centre, RVI

January 2010

History

• CP affects 2-3/1000 children

• Most common cause of physical disability in childhood

• Debate over 150 years – regarding definition and classification – this remains ongoing

• 2005: Multinational consensus group – revised definition + classification (Bax and Rosenbaum)

– Based on: SCPE data, functional ability measures (GMFCS – 1997)

SCPE: Surveillance Cerebral Palsy in Europe

• 1998 – Collaborative group CP Registers

• 14 European Centres

• Classification Motor Disorder

– Spastic

– Dyskinesia

– Ataxia

– (Mixed)

• Guidelines and Educational Tool

SCPE: Surveillance Cerebral Palsy in Europe

• Informs Prevalence Data

– Trends in Subgroups

• Increase (4x) multiple birth – related to gestation

• Decreasing trend infection as post natal aetiology

• Optimal birthweight – lower risk of CP

• Reducing prevalence CP in birthweight 1000-1500g– Impact on bilateral CP prevelance

• Qualitative work on QOL and Participation

– SPARCLE

Definition

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 behaviour, by epilepsy and by secondary musculoskeletal problems.”

Rosenbaum et al, 2006: Definition and classification of CP

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 behaviour, by epilepsy and by secondary musculoskeletal problems.”

Rosenbaum et al, 2006: Definition and classification of CP

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 behaviour, by epilepsy and by secondary musculoskeletal problems.”

Rosenbaum et al, 2006: Definition and classification of CP

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 behaviour, by epilepsy and by secondary musculoskeletal problems.”

Rosenbaum et al, 2006: Definition and classification of CP

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 behaviour, by epilepsy and by secondary musculoskeletal problems.”

Rosenbaum et al, 2006: Definition and classification of CP

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 behaviour, by epilepsy and by secondary musculoskeletal problems.”

Rosenbaum et al, 2006: Definition and classification of CP

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 behaviour, by epilepsy and by secondary musculoskeletal problems.”

Rosenbaum et al, 2006: Definition and classification of CP

Spastic

Classification: Pathophysiology

Prenatal

•Neuronal Migration disorders

•Congenital infection

•Vascular event

Perinatal

•Prematurity – PVL

•Hypoxic Ischaemic

•Encephalopathy

•Vascular Event

Postnatal

•Infection

•Hypoxic Ischaemic event

•Vascular event

•Acquired Brain injury

Ataxic Dyskinetic

Bilateral Unilateral

Classification of Cerebral Palsy

1) Motor abnormalities

NATURE + TYPOLOGY OF MOTOR DISORDER

FUNCTIONAL MOTOR ABILITY

2) Accompanying impairments

3) Anatomical and Neuroimaging findings

ANATOMICAL DISTRIBUTION

NEURO-IMAGING FINDINGS

4) Causation and Timing

Classification: Motor Disorder

Prevalence and Characteristics of Children with Cerebral Palsy in Europe.

SCPE Dev. Med. Child. Neurol. 2002

Ataxic Cerebral Palsy: NECCPS Data

• 1991 -1996

• 16/549 (2.9%) Registrations = Ataxic CP

• Reviewed age >4 years

• 13/16 – revised diagnosis

– 7 alternative CP (4 spastic, 3 dyskinetic)

– 4 DCD

• 3/16 – confirmed ACP

• 2/16 – other metabolic diagnosis

Gibson, Sethumadhavant, Forsyth

Classification

• Anatomical distribution

• Motor Function

– GMFCS

– BFMF

– MACS

• QOL

• Participation

• Associated impairments

– Severe intellectual impairment - 31%

– Severe Visual impairment – 11.1%

– Active Seizures – 20.7%

– Severe intellectual impairment + not walking – 20.2%

• SCPE, DCMN 2002

Hemiplegia

• Infarct internal capsule

• Likely prenatal

• Hemiplegia

• Spasticity

• Dyskinesia

• Large MCA territory infarct

• Seizures/HIE birth

• Spasticity

• Epilepsy

• Learning /language difficulty

• Homonymous Hemianopia

Bilateral Cerebral Palsy

• Hypoxic Ishaemic encephalopathy

• “watershed territory”

• Assymetry

• Associated

– LD

– Epilepsy

– Cortical visual impairment

Bilateral Cerebral Palsy

Hypoxic Ishaemic encephalopathy

• Peri -natal

• Damage to basal ganglia

• Dyskinesia and dystonia

• Dysarthria and oral motor dysfunction

• Often normal cognitive ability

Periventricular Leucomalacia

• Ex premature infants

• Encephalomalacia following IVH

• Assymmetry

• Often cognitively able – may have specific LD/attention/behavioural concerns

• Cortical visual impairment– May be subtle

Why image children with CP?

• Case 1: E.M

– 41/40 2600g (0.4%) OFC 30.7cm ( 1cm < 0.4th)

– Forceps delivery - foetal distress CTG, meconium

– Resuscitation at birth – IPPV only

– No SCBU

– Mild antenatal ventriculomegaly – USS at 6/52

– Presented with motor delay 12 months

• 4 limb spasticity

• Oral motor difficulty

• Weight/ length = 9th centile OFC 41cm (4cm< 0.4thC)

Why image children with CP?

• Case 2: C.B

– 38/40 2.25kg ( 0.4th – 2nd) OFC 32.2cm (2nd C)

– Foetal bradycardia – ventouse delivery

– No resuscitation at birth, no SCBU

– Presented 21 months – delayed walking and dragging L foot

• L hemiplegia

• Speech delay

• OFC 47cm (0.4th)

Why image children with CP?Cases 1 + 2

EM CB

Why image children with CP?Cases 1 + 2 - MRI

• EM

– Extensive bilateralfrontal, temporal perisylvian and parietal polymicrogyria with occiptal and medial frontal sparing

• CB

– Extensive Right hemisphere frontal, temporal perisylvian and parietal polymicrogyria with occiptal and medial frontal sparing

Why image children with CP?Cases 1 + 2

Likely X – linked polymicrogyria

EM CB

? Carrier

When is it not Cerebral Palsy?

Other CNS causes of disordered motor development

• Genetic Disorders– Hereditary Spastic Paraparesis

– Dopa-responsive dystonia – Segawa syndrome

• Neurodegenerative diseases– Leucoencephalopathies

– Mitochondrial Disease

• Visual impairment

• Cognitive development

• Pervasive developmental disorders

• Other complex medical needs

Red Flags

• No significant perinatal history

• MRI

– Unexpected result

• Normal or Abnormal

• Family History

• Clinical signs inconsistent with history

• Progression of neurological signs or deterioration of expected motor function

• Ataxic cerebral palsy

Medical Management of Cerebral Palsy

Medical Management of Cerebral Palsy

POSTURE AND MOBILITY

ASSOCIATED IMPAIRMENTS

– NUTRITION

– EPILEPSY

– SENSORY DISTURBANCE

– COMMUNICATION

– BEHAVIOUR

Child and Family wishes

Health and Education Resources

InhibitionCorticospina

l Tract

UNDERSTANDING

SPASTICITY

Baclofen

• Centrally acting GABA agonist – increases inhibition of descending cortico-spinal tract

• Oral medication:

– Systemic side effects

• Reduced truncal tone including oral motor control

• Lethargy

– Gradual increasing dose = improved tolerance

• Intra-thecal Baclofen

– Direct effect on CNS – reduced systemic side effects

Botulinum Toxin

• Targeted treatment of spasticity

• Toxin inhibits Ach release from NMJ

• Individual muscle vs multi-level

• Evidence – limited to equinus foot deformity

• Goal orientated approach – functional benefit

– Ambulant: gait, range of movement, orthoses

– Non-ambulant: Hygiene, pain reduction

• Dyskinesia and spasticity: regulatory control feedback via gamma motor neurone

Acceptable Accessible

Integrated to lifestyleMinimal adverse effects

Goal OrientatedFunctional outcome

Increase Participation and QOL

Posture and Mobility Management

Functional Therapy and

Orthoses

Intra-thecalBaclofen

BotulinumToxin

Injections

Oral Medication

Selective Dorsal

Rhizotomy

OrthopaedicSurgery

Child and Family

Conclusions

• Classification of Cerebral Palsy and epidemiological evidence can significantly increase our understanding of pathophysiology, appropriate investigation (genetic) and predict outcome / impairment.

• Medical management should be child and family centred and co-ordinated between health and other professionals.

Thank you for your attention!

Any Questions?