Peter Špalek1, Peter Kosoň1,2
Neuromyelitis optica (NMO) or Devic´s disease typically involves optic nerves and the spinal cord and is most often relapsing. The pathogenesis is one of an acute autoimmune, inflammatory process targeting astrocytes and resulting in demyelination, as well as axonal injury. Recent studies have identified an elevation of serum anti-aquaporin-4 antibody as a hallmark of NMO. Typical cases of NMO significantly differ from multiple sclerosis and establishment of diagnosis is no major issue. At the beginning of the disease, an isolated manifestation of either recurrent optic neuritis or myelitis can lead to incorrect diagnosis of multiple sclerosis. Wingerchuk´s criteria (2006) and EFNS criteria (2010) are very helpful for diagnosis of NMO, with IgG positivity to aquaporine-4 as one of supportive criteria. Whenever a reasonable degree of NMO suspicion exists, therapies directed at limiting acute injury and at preventing further subsequent injury mediated by humoral mechanisms should be instituted immediately. For an acute attack, corticosteroids are the first line treatment starting with high-dose methylprednisolone intravenously and oral prednisone is given sequentially. If clinical improvement is not satisfactory, plasmapheresis is considered as the second line treatment. For prevention of further attacks immunosuppressive therapy directed at humoral mechanism is preferred. Agents recommended are oral azathioprine (or mycophenolate mofetil) with or without low-dose prednisone. Therapy should be planned to continue up to 5 years in all patients, including those with a single attack who are at high risk of further relapse.