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2009

Regional CP service A vision for a foundation hospital. 2009. 2010. 2011. Dr. G.Vassallo (Paediatric Neurologist job share with Dr.Siobhan West). Ms Anne Foster Paediatric Orthopaedic Surgeon. Introduction-Joint Presentation. Thoughts underlying presentation.

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2009

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  1. Regional CP service A vision for a foundation hospital 2009 2010 2011

  2. Dr. G.Vassallo (Paediatric Neurologist job share with Dr.Siobhan West) Ms Anne Foster Paediatric Orthopaedic Surgeon Introduction-Joint Presentation

  3. Thoughts underlying presentation • Acknowledge the enormous amount of work and initiative of Dr. Anne Ferguson/Wendy Rankin and CP network • Acknowledge that development at tertiary level has been slow and fragmented. Tertiary specialists keen to improve this.

  4. Presentation format • Starting at the basics • CP definition • CP diagnosis • Concept of the upper motor neurone syndrome • Pathogenesis of spasticity • Non invasive oral drugs and mode of action • Invasive management Chemodenervation/neurosurgical and orthopaedic procedures

  5. References • Proposed definition and classification of cerebral palsy April 2005 Dev Med and Child Neurology 2005,47:571-576 • A report: the definition and classification of cerebral palsy April 2006 dev Med and Child Neurology;Feb 2007 • Practice parameter: Diagnositic assessment of the child with cerebral palsy Neurology 2004;62;851-863 S.Ashwal et al • Spasticity Etiology,Evaluation Management and the role of Botulinum Toxin CME module WE MOVE September 2002 • Botulinum Toxin Treatment of Muscle Spasticity Textbook Professor A Magid Bakheit • The Child with Cerebral Palsy: Diagnosis and Beyond Seminars in Paediatric Neurology Volume 13 Issue 4 December 2006 Pages 286-296 • Therapeutic Interventions for Tone Abnormalities in Cerebral Palsy • Ann H. Tilton Neurotreatment Volume 3 Issue 2 April 2006 Pages 217-224 • Developmental - behavioural paediatrics evidence and practiceMark Wolraich, Dennis D. Drotar, Ph.D., Paul H. Dworkin, Ellen C. Perrin, M.D. - Medical - 2007 Text book

  6. Definition Dev Med April 2006 Rosenbaum International workshop Bethesda 2004 Paediatric neurologist ENT/SALT Neurosurgoen Community paediatrician Spinal surgoen Orthopaedic surgeon Cerebral palsy (CP) is 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

  7. Why a regional service? Cerebral Palsy Dev Med April 2006 Rosenbaum International workshop Bethesda 2004 Management not only of a child but a child in the context of a Family as a member of a community

  8. Classification Purposes • Description: • Prediction • Comparison • Evaluation

  9. Components of the classification • Motor abnormalities (a) Nature and typology of motor disorders-Hyper/hypotonic Trunk/limbs Spastic, dyskinetic (Dystonic, choreathetoid) and ataxic. (b) Functional motor abilities GMFS, scales for upper limb, oromotor and speech • Accompanying impairments • Cognitive , vision, hearing etc • Anatomical and neuroimaging findings • The part of the body affected • The neuroimaging finding on CT/MR • Causation and timing • The cause-HIE/meningitis/brain malformation • The timing

  10. History and examination findings suggestive of CP • Ref:current March 2009 • Neurology S.Ashwal et al 2004;62;851-863 • Confirm that history not suggestive of a progressive • Degenerative disorder • 2 Assure features of such not present on examination • 3 Classify the CP • 4 Screen for associated conditions • Learning difficulties • Ophthalmological/Hearing impairments • Speech and language delay • Feeding and swallowing dysfunction • If history of seizures EEG • Did the childhave neuroimaging • that confirms the etiology of CP • Yes • no • Obtain neuroimaging • MR better than CT • No need for further • imaging

  11. Once MR available • MR normal • MR abnormal • Determine if neuroimaging • abnormalities In combination with • history and examination establishes • A specific etiology of CP • 2. If developmental malformation is • present consider genetic evaluation • 3. If previous stroke consider • thrombophilia screen • Consider metabolic or genetic testing if Upon follow up The child has: • Evidence of deterioration or episodes of metabolic decompensation. • No etiology determined by medical evaluation • Family history of childhood disorder associated with CP

  12. Consider metabolic or genetic testingIf MR non specific and atypical clinical picture • Glutaric aciduria type 1 • Lesch-Nyhan Syndrome • 3-methylglutaconic aciduria • PDH deficiency • Argininemia • Cytochrome oxidase deficiency • Succinic semialdehyde dehydrogenase deficiency • Female carriers of Ornitine transcarbamylase deficiency • Dopa responsive dystonia • Hereditary spastic paraparesis • Ataxia telangiectasia

  13. MEMORANDUM BY DR LYNNE TURNER-STOKES, DIRECTOR, REGIONAL REHABILITATION UNIT, NORTHWICK PARK HOSPITAL (H19) The Hub and spoke model Complex CP Tertiary service require Super-specialist complex services Complex specialist CP service Complex multiple commissioning Specialised district CP services Commissioned PCT Local community therapy services (GP practice based) Patients requiring CP service

  14. New neurosciences ward-ideal opportunity • Neurology • Orthopaedics • Neurosurgery • ENT • Spinal surgery

  15. 78? Physicians Surgeons

  16. Hello Orthopaedic surgeon just the person I wanted-delighted to see you!!!!! Ward 78

  17. Non invasive and invasive interventions in CP

  18. The concept of the upper motor neurone syndrome • Upper motor neurone lesions (pyramidal tract/cortico-reticulospinal tract) gives rise to alpha motor neurone hyperexcitability at the segmental level. • Disorders leading to the upper motor neurone syndrome include: • Cerebral palsy • MS/ADEM • Traumatic brain injury • Spinal cord injury • Neuro-degenerative diseases (leukodystrophies)

  19. Upper motor neurone syndrome • UMN are those in any long descending tract that have a more or less direct influence upon the excitability of the lower motor neurone (anterior horn cell) either through a direct synapse (Corticospinal tract) or via an interneuronal network. • The UMN syndrome is caused by lesions that result in a loss of the modulation of the spinal reflexes.

  20. Positive effects (1) Enhanced stretch reflexes (Spacticity) Co-contraction and spastic dystonia most distressing and disabling (2) Released flexor reflexes in lower limbs Eg babinski and mass synergy patterns Negative effects (1) Loss of dexterity (2) Weakness The upper motor neurone syndrome Rheologic changes in the spastic muscle Stiffness Contracture Fibrosis Atrophy

  21. Spacticity • Spacticity is a motor disorder characterised by a velocity dependant increase in the tonic stretch reflex (muscle tone) with exaggerated tendon jerks, resulting from hyperexcitability of the stretch reflex (phasic stretch reflex) as one component of the upper motor neurone syndrome • (Clonus) Repetitive phasic stretch reflex generated by sustained stretch Lance 1980

  22. When a muscle is stretched there is excitation of homonymous and synergistic motorneurons combined with the inhibition of antagonists-reciprocal inhibition. This is disturbed in the upper motor neurone syndrome Muscle spindle physiology 1a afferent Alpha motor neurone Inhibitory

  23. Excitatory Glutamate Aspartate Noradrenaline Serotonin Inhibitory GABA Glycine Opoids Neurotransmitters

  24. Neurotransmitters in the descending pathways DESCENDING PATHWAY Reticulospinal Rubrospinal/vestibulospinal Corticospinal NEUROTRANSMITTERS Adrenaline/noradrenalin/serotonin Dopamine Excitatory amino acids Glutatmate and other excitatory AA Neurotransmitters at spinal level Activity Alpha motor neurone collateral Inhibitory interneurone Presynaptic inhibition Polysynaptic pathways Neurotransmitters Acetylcholine (Nicotinic) Glycine GABA Excitatory amino acids

  25. Oral systemic drugsNon invasive Drugs targeting spacticity • Benzodiazepines (Diazepam and clonazepam) • Baclofen • Dantrolene • Tizanidine Drugs targeting Dystonia • Dopa-Dopa agonist • Trihexyphenidyl-anticholinergic • Bacofen

  26. Mechanism of action of Benzodiazepines • Brain stem and spinal cord • Depresses post synaptic excitatory response to glutamate • Potentiates the presynaptic inhibitory effect of GABA by: • Enhances the affinity of the presynaptic receptor to GABA • Enhances GABA release

  27. Diazepam Start low and go slow Maximum dose 1mg/kg/day • Side effects include somnolence, memory impairment, depression, ataxia, confusion, hypotension and unwanted muscle weakness • Dependency and withdrawal

  28. Baclofen • Gaba b selective agonist • Pre and post synaptic actions • Mono and poly synaptic pathways • Gives considerable relief from flexor spasms and spastic dystonia expecially in the dominantly spinal pathology

  29. Baclofen administration • Slow titration and slow withdrawal • Maximum 2mgs/kd/day • Side effects • Weakness, sedation, confusion, nausea, hypotonia, ataxia and lowered seizure threshold • Sudden withdrawal-rebound spacticity, hallucinations and seizures

  30. Tizanidine • Alpha-2 noradrenergic receptor agonist • Blocks release of excitatory neurotransmitters from spinal interneurons • Inhibits facilitatory spinal pathways • Theoretically does not exacerbate muscle weakness.

  31. Tizanidine • Side effects • Drowsiness, dizzyness and hypotension • Hepatotoxic • LFT must be normal before start • 1/3/6 months and than 6 monthly thereafter

  32. Blocks Calcium release at sarcolemma Uncouples excitation and contraction Greatest effect on low frequency contractions eg Clonus Dantrolene

  33. Dantrolene • Starting dose 0.5mgs/kg/day increasing by the same amount every 7 days to a maximum of 8mgs/kg/day divided in three to four doses • Maximum 400mgs/day • Side effects • Weakness especially in patients with marginal strength • Dizzyness and drowsiness • Hepatotoxicity • 1/3/6/12months LFT

  34. Invasive interventions • Chemodenervation • Botulinum toxin • Phenol • Ethyl alcohol • Surgical intervention • Selective Dorsal Rhizotomy (Oswestry) • Intrathecal baclofen • Orthopaedic Surgery…………

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