TY - JOUR T1 - Primary Sjögren Syndrome in a Child with a Neuromyelitis Optica Spectrum Disorder JF - The Journal of Rheumatology JO - J Rheumatol SP - 1260 LP - 1261 DO - 10.3899/jrheum.151207 VL - 43 IS - 6 AU - JEFFREY M. KORNITZER AU - YUKIKO KIMURA AU - GINGER L. JANOW Y1 - 2016/06/01 UR - http://www.jrheum.org/content/43/6/1260.abstract N2 - To the Editor:Neuromyelitis optica spectrum disorders (NMOSD) are a group of rare autoimmune diseases typically affecting women in their 40s and 50s, as does Sjögren syndrome (SS), another autoimmune disease. We describe a 6-year-old girl who presented with clinical and laboratory findings of both an NMOSD and SS.A 6-year-old previously healthy Korean girl presented with a 1-day history of fever, headache, progressive right-sided weakness, and altered mental status. Cerebrospinal fluid (CSF) analysis revealed an elevated protein without pleocytosis or oligoclonal bands. Magnetic resonance imaging (MRI) of the spinal cord showed contiguous T2 hyperintensity, and MRI of the brain showed extensive white matter lesions bilaterally (Figure 1), suspicious for demyelinating disease. She was treated with high-dose methylprednisolone, resulting in marked improvement, and continued treatment with tapering doses of prednisone, but flared when the prednisone was discontinued.Figure 1. A. MRI of the cervical and thoracic spine (T2 FLAIR with contrast shown) obtained at the onset of symptoms, prior to treatment. There is a contiguous hyperintense, centrally located spinal cord lesion. B. MRI of the head (T2 FLAIR with contrast shown) obtained at the … Address correspondence to Dr. G. Janow, Section of Pediatric Rheumatology, Joseph M. Sanzari Children’s Hospital, Hackensack University Medical Center, 30 Prospect Ave., WFAN Bldg. 3rd Floor, Hackensack, New Jersey 07601, USA. E-mail: GJanow{at}HackensackUMC.org ER -