Case Presentation: A 21-year-old female with prior history of aseptic meningitis presented to the emergency department with progressively worsening headaches and drowsiness for 4 days. On physical examination, she was oriented only to herself but otherwise had no focal neurological deficits or nuchal rigidity. Kernig and Brudzinski sign were negative. Labs were unremarkable, except for mildly elevated inflammatory markers with a CRP of 59 and an ESR of 32. Lumbar puncture was performed and revealed CSF pleocytosis, with an elevated white blood cell count of 213. CT head revealed no acute pathology. MRI brain did not reveal any definite evidence of meningitis but did show mild enhancement of dura presumed secondary to CSF leak. She was placed on empiric antimicrobial therapy. An extensive infectious work-up that excluded bacterial, viral, fungal, and parasitic causes was conducted and was negative. Rheumatological workup revealed a very positive ANA (1:640) with an elevated Anti-SS-A/Anti-Ro, Anti-SS-B/Anti-La, and Ro-52kD Antibody. Rheumatology was consulted and the patient was accordingly started on steroids and told to follow up with their team as an outpatient.
Discussion: Sjögren’s Syndrome (SS) is a chronic autoimmune disorder that usually causes dry eyes and dry mouth due to lymphocytic infiltration in the exocrine glands. Like other autoimmune disorders, SS can cause nonspecific symptoms, such as joint pains and chronic fatigue. It can also affect the lungs, heart, kidneys, and central nervous system (CNS) (1). Although CNS involvement is rare, neurological complaints precede the sicca symptoms in 25-60% of the cases. On average, neurological manifestations precede the diagnosis of SS by 2 years (2). Documented CNS involvement includes transverse myelitis, encephalopathy, and aseptic meningitis (3). In 2020, Akiyma et al. presented two young patients with aseptic meningitis who were found to have underlying SS. One patient had three prior episodes of self-limiting aseptic meningitis before having a SS diagnosis. The study emphasized the importance of screening for Sjögren’s antibodies for patients with aseptic meningitis of unknown etiology (4). Similar to our patient, aseptic meningitis associated with Sjögren’s Syndrome often presents with flu-like symptoms, confusion, and fever (2). CSF findings in documented cases have been normal or have shown polymorphonuclear or lymphocytic pleocytosis (2). Several studies have reported specific MRI findings related to Sjogren’s syndrome. These include leptomeningitis, which affects the pia and arachnoid mater and is characterized by leptomeningeal enhancement, or pachymeningitis, notable for thickening and enhancement of the dura mater (5). A treatment plan for meningitis secondary to SS is not well studied. Similar to most cases of non-infectious meningitis, patients are typically managed with supportive care with fluids and pain medication. Treatment with glucocorticoids with or without Cyclophosphamide has been reported in case studies (6). Plasmapheresis was used in one case study, too (7).
Conclusions: While Sjögren’s Syndrome primarily affects the exocrine glands, it can also present as aseptic meningitis. The absence of dryness does not exclude the possibility of SS in a patient.