Dr Jill Kisler

Classification, Epidemiology and Medical Management of Cerebral Palsy Dr Jill Kisler Consultant 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

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

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 • 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

Hemiplegia • Infarct internal capsule • Large MCA territory infarct • Likely prenatal • Seizures/HIE birth • Hemiplegia • Spasticity • Spasticity • Epilepsy • Dyskinesia • 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 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

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 • 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

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

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 Health and Education Resources ASSOCIATED IMPAIRMENTS – NUTRITION Child and – EPILEPSY Family wishes – SENSORY DISTURBANCE – COMMUNICATION – BEHAVIOUR

UNDERSTANDING SPASTICITY Inhibition Corticospina l Tract

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

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

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?

Dr Jill Kisler

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