We performed a retrospective review of the electronic medical records at the University of Utah Health and queried charts of patients with a diagnosis of SS with a positive lip biopsy and seronegative antibody status who were also evaluated on at least one occasion by a Neurologist between January 2010 to October 2018. 

Of the seronegative SS patients identified, we describe the type of neurologic involvement (central or peripheral nervous system; with or without autonomic symptoms), comorbid conditions, associated autoimmunity, and response to immunomodulatory and anti-inflammatory treatments, as well as demographic and clinical data. 

25-60% of SS patients present for evaluation of neurologic symptoms prior to receiving a formal diagnosis of Sjögren’s Syndrome.  Therefore, Neurologists should be familiar with the range of manifestations of SS to avoid misdiagnosis, especially as those patients who are seronegative for SS may suffer even longer delays in diagnosis.