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NMO is Rare

NMO is Rare. Prevalence NMO 1/100,000 approx 500 in UK MS 140/100,000 approx 100,000 in UK Female: Male ratio 5:1 Average age diagnosis 39years. History of Devics. Albutt T.1870 described optic neuritis and myelitis

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NMO is Rare

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  1. NMO is Rare Prevalence NMO 1/100,000 approx 500 in UK MS 140/100,000 approx 100,000 in UK Female: Male ratio 5:1 Average age diagnosis 39years

  2. History of Devics Albutt T.1870 described optic neuritis and myelitis Eugene Devic (1894) and Fernand Gault described 17 cases of ‘optic neuritis accompanied by myelitis and occasionally other symptoms or signs not restricted to the cord of ON.’ Devic E. (1894) Myelite subargue compliquee de nevrite optique. Bull. Med 8 1033-34

  3. Diagnostic Criteria of NMO Optic neuritis and Transverse myelitis And at least two of three supportive criteria 1. Contiguous spinal cord MRI lesion extending over 3 vertebral segments 2. Brain MRI not meeting diagnostic criteria for multiple sclerosis 3. Aquaporin-4 antibody positive status

  4. NMO Spectrum Optic neuritis or Longitudinally extensive myelitis And Presence of NMO IgG

  5. Spinal Presentation

  6. Clinical features of NMO Severe ON / Myelitis Disability accrued with attacks Disability: At 5 years - 50% lose functional vision one eye or unable to walk independently1 Secondary progression rare2 Mortality : high (32%) at 5 years1 1.Wingerchuk,Weinshenker 1999.Neurology 53, 1107–1114. 2.Wingerchuk, Neurology 2007:603.

  7. Alternative Diagnosis of NMO Other disorders with optico-spinal presentation MS, ADEM SLE, Sjogren’s Sarcoid Infections Dissemination in time and space equated to MS

  8. NMO v MS Differences

  9. Why is NMO different?

  10. Pathogenesis of NMO Jacob A ,Weinshenker BG et al . Journal of Neuroimmunology 2007:126

  11. NMO Antibody Who to Test? NMO (ON+LETM) Recurrent ON Recurrent LETM Single severe ON Single Severe LETM for no obvious cause Atypical brainstem /hypothalamic changes Giovannoni Neurology 2008: 2192-2193

  12. Who not to test! Any patient with typical MS MRI Partial myelitis – small segment/peripheral (MS more likely) Single ON with full recovery

  13. Treatment of NMO Treating relapses Preventing relapses Symptomatic management Rehabilitation

  14. Treating Acute Relapses Steroids High dose IV methylprednisolone (1g x 5 days) as early as possible. Tailing oral prednisolone over months Plasma exchange Response in 40-60% with NMO (within 3 months of onset of relapse)

  15. Preventing Relapses Azathioprine 2.5mg/kg dly Prednisolone 1mg/kg taper over 6-12 months Rituximab 1gm IV day 1&14 6monthly Methotrexate Oral 7.5-20mg /week Mycophenolate Oral 1-3g/day Cyclophosphamide IV 7-25mg/kg/month for 6m Mitoxantrone IV 12mg/m for 6 m, 3m for 9m ImmunoglobulinIV 2g/Kg over 2 days every 6wks Plasma Exchange IV 5-7 infusions over 9 days

  16. Symptoms of NMO Visual loss Limb weakness/ paralysis of limbs Neuropathic pain Tonic spasms Bladder Bowel Sexual dysfunction

  17. So What’s the Fuss About? Serious disease - life threatening Still being misdiagnosed/treated incorrectly Very much treatable Explosion of new information Unique antibody- 1st specific marker for CNS demyelinating disease

  18. National Specialist Commissioning Group Status for NMO in UK

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