Peacocks Conference Presentation

1. Classification, Epidemiology and Medical
Management of Cerebral Palsy
Dr Jill Kisler
Consultant in Child Development and Neurodisability
Child Development Centre, RVI
January 2010

2. 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)

3. 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

4. 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

5. Definition

6. 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

7. 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

8. 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

9. 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

10. 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

11. 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

12. 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

13. Classification: Pathophysiology
Prenatal Perinatal Postnatal
•Neuronal Migration disorders •Prematurity – PVL •Infection
•Congenital infection •Hypoxic Ischaemic •Hypoxic Ischaemic event
•Vascular event •Encephalopathy •Vascular event
•Vascular Event •Acquired Brain injury
Spastic
Bilateral Unilateral
Ataxic Dyskinetic

14. 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

15. Classification: Motor Disorder
Prevalence and Characteristics of Children with Cerebral Palsy in Europe.
SCPE Dev. Med. Child. Neurol. 2002

16. 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

17. Classification
• Anatomical distribution • Associated impairments
• Motor Function – Severe intellectual
– GMFCS impairment - 31%
– BFMF – Severe Visual
impairment – 11.1%
– MACS
– Active Seizures – 20.7%
• QOL – Severe intellectual
• Participation impairment + not
walking – 20.2%
• SCPE, DCMN 2002

18. Hemiplegia
• Infarct internal capsule • Large MCA territory infarct
• Likely prenatal • Seizures/HIE birth
• Hemiplegia • Spasticity
• Spasticity • Epilepsy
• Dyskinesia • Learning /language
difficulty
• Homonymous Hemianopia

19. Bilateral Cerebral Palsy
• Hypoxic Ishaemic encephalopathy
• “watershed territory”
• Assymetry
• Associated
– LD
– Epilepsy
– Cortical visual impairment

20. Bilateral Cerebral Palsy
Hypoxic Ishaemic Periventricular
encephalopathy Leucomalacia
• Peri -natal • Ex premature infants
• Damage to basal ganglia • Encephalomalacia following
• Dyskinesia and dystonia IVH
• Dysarthria and oral motor • Assymmetry
dysfunction • Often cognitively able – may
• Often normal cognitive have specific
ability LD/attention/behavioural
concerns
• Cortical visual impairment
– May be subtle

21. 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)

22. 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)

23. Why image children with CP?
Cases 1 + 2
EM CB

24. Why image children with CP?
Cases 1 + 2 - MRI
• EM • CB
– Extensive bilateral – Extensive Right
frontal, temporal hemisphere frontal,
perisylvian and parietal temporal perisylvian and
polymicrogyria with parietal polymicrogyria
occiptal and medial with occiptal and medial
frontal sparing frontal sparing

25. Why image children with CP?
Cases 1 + 2
EM CB
? Carrier
Likely X – linked polymicrogyria

26. When is it not Cerebral Palsy?

27. 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

28. 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

29. Medical Management of
Cerebral Palsy

30. Medical Management of Cerebral Palsy
POSTURE AND MOBILITY Health and Education
Resources
ASSOCIATED IMPAIRMENTS
– NUTRITION Child and
– EPILEPSY Family wishes
– SENSORY DISTURBANCE
– COMMUNICATION
– BEHAVIOUR

31. UNDERSTANDING
SPASTICITY
Inhibition
Corticospina
l Tract

32. 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

33. 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

34. Posture and Mobility Management
Acceptable
Functional
Accessible Orthopaedic
Therapy and
Integrated to lifestyle Surgery
Orthoses
Minimal adverse effects
Selective
Oral Child and Dorsal
Medication Family Rhizotomy
Botulinum Goal Orientated
Intra-thecal
Toxin Functional outcome
Baclofen
Injections Increase Participation and QOL

35. 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.

36. Thank you for your attention!
Any Questions?