TY - JOUR T1 - IgG4 Syndrome Presenting as Leptomeningitis in a Young Woman JF - The Journal of Rheumatology JO - J Rheumatol SP - 828 LP - 830 DO - 10.3899/jrheum.151236 VL - 43 IS - 4 AU - SOWMINI MEDAVARAM AU - FEIFEI XUE AU - ROBERT LAHITA Y1 - 2016/04/01 UR - http://www.jrheum.org/content/43/4/828.abstract N2 - To the Editor:Immunoglobulin G4-related disease (IgG4-RD) is a recognized systemic condition that can affect almost every organ system1. The most commonly involved organs are the pancreas, salivary glands, and biliary tree. The common presentation with central nervous system (CNS) involvement is hypophysitis and pachymeningitis, but rarely leptomeningitis2. Only 3 reported cases of leptomeningitis have been reported, all of them presenting as cognitive decline, and 2 associated with rheumatoid arthritis (RA)3. We describe a patient with unilateral hearing loss and a right frontal mass on magnetic resonance imaging (MRI) who was subsequently diagnosed with IgG4-related leptomeningitis after biopsy. This is a unique case of IgG4-related leptomeningitis because of its presentation and lack of association with RA or positive serological markers.A 43-year-old woman with thyroid follicular cancer, post-thyroidectomy, had progressive right-side hearing loss for 2 months. She was also noted to have gait difficulty, pins-and-needles sensations in the feet, and facial droop on the left side. Physical examination showed that she was alert and oriented to time, place, and person. Her cranial nerves were intact except … Address correspondence to Dr. S. Medavaram, 3111 Bristol Station Court, Carteret, New Jersey 07008, USA. E-mail: sowmini.medavaram{at}gmail.com ER -