{
  "abstract": "Sjögren’s disease (SjD) is a chronic autoimmune connective tissue disorder primarily affecting the salivary and lacrimal glands. Extra-glandular involvement may occur, sometimes preceding the diagnosis of SjD or in the absence of sicca symptoms, contributing to diagnostic delay. Central nervous system (CNS) manifestations have been reported in SjD, though establishing a causal relationship is challenging given possible overlapping neuroinflammatory and autoimmune aetiologies.We report two patients who presented with acute encephalitis and new-onset refractory status epilepticus (NORSE), in whom features supportive of primary SjD were subsequently identified. Both patients underwent extensive evaluation that excluded infectious causes, other systemic autoimmune connective tissue diseases and known autoantibody-mediated forms of autoimmune encephalitis.These cases highlight the importance of considering systemic autoimmune diseases in the evaluation of unexplained encephalitis and NORSE, and support the early use of immunotherapy when an immune-mediated pathology is suspected.",
  "authors": [
    {
      "affiliations": [
        "Department of Neurology, National Neuroscience Institute, Tan Tock Seng Hospital Campus, Singapore"
      ],
      "name": "Gareth Zigui Lim"
    },
    {
      "affiliations": [
        "Department of Internal Medicine, Tan Tock Seng Hospital, Singapore"
      ],
      "name": "Justin Kian Guan Kwok"
    },
    {
      "affiliations": [
        "Department of Rheumatology, Allergy and Immunology, Tan Tock Seng Hospital, Singapore"
      ],
      "name": "Zhaoxiang Fan"
    },
    {
      "affiliations": [
        "Department of Pathology, Tan Tock Seng Hospital, Singapore"
      ],
      "name": "Nicholas Wee Chong Koh"
    },
    {
      "affiliations": [
        "Department of Dermatology, National Skin Centre, Singapore"
      ],
      "name": "Joyce Siong-See Lee"
    },
    {
      "affiliations": [
        "Department of Neurology, National Neuroscience Institute, Tan Tock Seng Hospital Campus, Singapore",
        "Duke-NUS Medical School, Singapore",
        "Lee Kong Chian School of Medicine, Nanyang Technological University, Singapore"
      ],
      "name": "Tianrong Yeo"
    }
  ],
  "title": "New-onset refractory status epilepticus associated with Sjögren’s disease",
  "uid": "d64b904b-b03a-5bde-ba1a-77c0218eaa0a"
}