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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?
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