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Devic’s neuromyelitis optica: its distinctive features and treatment

Devic’s neuromyelitis optica: its distinctive features and treatment. Mark Morrow, MD Providence Multiple Sclerosis Center Portland, Oregon. Conclusions. Neuromyelitis optica is a distinct demyelinating disease with accurate diagnostic criteria

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Devic’s neuromyelitis optica: its distinctive features and treatment

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  1. Devic’s neuromyelitis optica: its distinctive features and treatment Mark Morrow, MD Providence Multiple Sclerosis Center Portland, Oregon

  2. Conclusions • Neuromyelitis optica is a distinct demyelinating disease with accurate diagnostic criteria • Demographic and historic features predict relapses • Relapse prevention requires broad-spectrum or B-cell-specific immunosuppression

  3. Age 4: Severe visual loss OU and generalized burning sensation Age 7: Quadriparesis and progression to no light perception OU Age 28-33: Progressive weakness, neuropathic pain Exam: no light perception, sheet-white optic atrophy OU; severe spastic quadriparesis

  4. NMO-IgG antibody: >1:60,000

  5. Neuromyelitis optica (NMO) • Acute/subacute demyelination, necrosis of optic nerves, spinal cord • Often preceded by viral illness, associated with systemic autoimmune disease • Significant residua common • Partial responses to steroids, other immunosuppressants

  6. Optic neuritis Acute/subacute neuropathic visual loss Typically painful Mild, if any, disc edema Myelitis Acute/subacute weakness, numbness Bowel/bladder problems L’hermitte’s sign Key clinical features

  7. ‘Textbook’ form’ Monophasic Simultaneous ON and SC disease Bilateral ON involvement No disease outside SC, ONs No brain MRI lesions Current description >70% recurrent ON and SC attacks may be years apart ON disease may be unilateral Brain disease occurs (ca. 10%) Brain MRI changes may occasionally resemble multiple sclerosis The broadening spectrum of NMO ON – optic nerve SC – spinal cord

  8. NMO – disease or syndrome? Differential diagnosis Multiple sclerosis Acute disseminated encephalomyelitis (ADEM) Lupus Sjogren’s syndrome Parainfectious

  9. Similarities Female predilection Age of onset Relapse rate Differences Geography Brain symptoms Prognosis MRI appearance Cerebrospinal fluid findings Response to treatment How does NMO compare with MS?

  10. Ancillary tests in NMO MRI • Elongated, expansile, enhancing spinal cord lesions • Brain MRI usually normal; occasional multiple-sclerosis-like plaques or confluent/symmetrical lesions CSF • >50 white blood cells/mm3 or >5 polymorphonuclear leucocytes/mm3 common • Oligoclonal bands, ↑IgG synthesis less common

  11. ‘The NMO antibody’ • IgG autoantibody localizes to glia at blood-brain-barrier • Binds to aquaporin-4, the main water channel in the central nervous system • About 90% specific, 75% sensitive for NMO • Often + in brain MRI- negative relapsing myelitis/optic neuritis • Available commercially

  12. The epidemiology of MS and NMO differs in Japan and the West • Lower prevalence of MS in Japan • Higher ratio of classic, monophasic NMO to MS, likely true throughout Asia • More Japanese ‘MS’ patients present with bilateral optic neuropathy and severe ON or SC disease (ca. 25%) • Up to 60% of ‘Asian optospinal’ MS may be + for NMO-IgG, implying that this condition represents recurrent NMO

  13. Laotian woman Age 47-52: 4 bouts unilateral optic neuritis Age 54: transverse myelitis Exam: no light perception OD, 20/20 OS, spastic paraparesis NMO-IgG positive

  14. NMO: the latest criteria • History of optic neuritis • History of acute myelitis • Two of three of: • MRI spinal cord lesion > 3 segments • + NMO-IgG antibody • Brain MRI not consistent with multiple sclerosis Wingerchuk et al. Revised diagnostic criteria for NMO. Neurology 2006;66:1485-9 (99% sensitive, 90% specific)

  15. Who will relapse? • Older patients with more common, sequential optic neuritis/myelopathic disease • Less severe disease at onset • High-titer + NMO-IgG antibodies • Step-wise progression portends worse prognosis than monophasic disease

  16. 45 year old man 1.5 yrs ago: subacute myelopathy preceded by flu-like illness Since then: several mild myelopathic relapses, occasional blurring Exams: spastic paraparesis, normal optic nerves and vision Normal CSF, - NMO-IgG

  17. Treatment for NMO* • Relapses/acute disease • IV methylprednisolone 1000 mg/day, 3-5 days • Plasmapheresis • Prevention / stabilization • Consensus: ABCR drugs not helpful • Azathioprine 2.5-3 mg/kg/day • Concurrent prednisone 1 mg/kg/day, tapering slowly after azathioprine takes effect • Mycophenolate mofetil, Mitoxantrone, Rituximab, IVIg, Plasmapheresis possible second liners * No class I or II data

  18. From Ransohoff R. J Clin Invest. 2006 September 1; 116(9): 2313–2316.

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