Childhood Sjögren syndrome presenting as acute brainstem encephalitis

Yoriko Matsui, Toshiki Takenouchi, Atsushi Narabayashi, Kentaro Ohara, Tadaki Nakahara, Takao Takahashi

Research output: Contribution to journalArticlepeer-review

15 Citations (Scopus)


Sjögren syndrome is an autoimmune disease characterized by dry mouth and eyes, known as sicca symptoms. The exact spectrum of neurological involvement, especially of the central nervous system, in childhood Sjögren syndrome has not been well defined. We report a girl who presented with acute febrile brainstem encephalitis. In retrospect, she had exhibited a preceding history of recurrent conjunctivitis and strong halitosis that could be considered as sicca symptoms. The histopathology results of a minor salivary biopsy, the presence of anti-SSA/Ro antibody, and keratoconjunctivitis confirmed the diagnosis of Sjögren syndrome. Commonly observed features in previously reported patients with childhood Sjögren syndrome and central nervous system complications have included fever at the time of neurologic presentation, cerebrospinal fluid pleocytosis, abnormal neuroimaging, and positivity for several specific antibodies. In children presenting with unknown acute febrile encephalopathy, Sjögren syndrome should be included in the differential diagnosis, especially when sicca symptoms are present.

Original languageEnglish
Pages (from-to)158-162
Number of pages5
JournalBrain and Development
Issue number1
Publication statusPublished - 2016 Jan 1


  • Central nervous system
  • Children
  • Encephalitis
  • Sicca symptoms
  • Sjögren syndrome

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Developmental Neuroscience
  • Clinical Neurology


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