Neuromyelitis optica spectrum disorders (NMOSD) are a heterogeneous group of potentially disabling central nervous system inflammatory disorders. Association with other organ-specific or multisystem autoimmune disorders is commonly encountered. We report the case of a Caucasian woman with AQP4-IgG positive NMOSD and primary Sjögren syndrome - previously reported as a poster at the 14th Congress of the Romanian Society of Neurology (abstract published in ROJN, Vol. XV, Suppl). The clinical onset followed the successful treatment of chronic hepatitis C with interferon alpha and ribavirin. To the best of our knowledge, similar clinical presentations have not been described. The etiopathogenic treatment included high dose methylprednisolone, immune suppression with cyclophosphamide, and periodic plasma exchanges. In spite of an active disease with very high baseline AQP4-IgG titres, during the four years of treatment the patient has had only one relapse and has no residual disability.
Primary Sjögren Syndrome in a Child with a Neuromyelitis Optica Spectrum Disorder
