Frontiers in Neurology (May 2021)

Case Report: Anti-N-Methyl-D-Aspartate Receptor Encephalitis in an Elderly Patient With Primary Sjögren's Syndrome

  • Xia Li,
  • Rui Kong,
  • Qiuju Liao,
  • Jing Ye,
  • Yi Zhao

DOI
https://doi.org/10.3389/fneur.2021.656024
Journal volume & issue
Vol. 12

Abstract

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Neurological manifestations of primary Sjögren's syndrome (SS) are diverse involving the peripheral and central nervous system. Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis, as the most prevalent autoimmune encephalitis, was rarely reported to be complicated with primary SS. Herein, we present an elderly patient with a 15-year history of primary SS presenting with progressive cognitive dysfunction due to anti-NMDAR encephalitis that was once misdiagnosed as primary degenerative dementia. Early recognition of anti-NMDAR encephalitis and initiation of treatment with steroids and immunosuppressant gained a favorable outcome. Our findings enhance the awareness that autoimmune encephalitis should be taken into account in the patients with primary SS presenting with progressive cognitive impairment.

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