NeuromyelitisopticaversusMS.pptVIP

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 Demographic and historic features predict relapses Relapse prevention requires broad-spectrum or B-cell-specific immunosuppression 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 Key clinical features 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 The broadening spectrum of NMO ‘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 NMO – disease or syndrome? Differential diagnosis Multiple sclerosis Acute disseminated encephalomyelitis (ADEM) Lupus Sjogren’s syndrome Parainfectious How does NMO compare with MS? Similarities Female predilection Age of onset Relapse rate Differences Geography Brain symptoms Prognosis MRI appearance Cerebrospinal fluid findings Response to treatment 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 ‘The NMO antibody’ IgG autoantibody localizes to glia at blood-brain-barrier Binds to aquaporin-4, the main water c