Childhood Sjögren syndrome presenting as acute brainstem encephalitis

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

Research output: Contribution to journalArticle

7 Citations (Scopus)

Abstract

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
JournalBrain and Development
DOIs
Publication statusAccepted/In press - 2015

Fingerprint

Encephalitis
Brain Stem
Fever
Central Nervous System
Halitosis
Keratoconjunctivitis
Conjunctivitis
Leukocytosis
Neuroimaging
Nervous System
Autoimmune Diseases
Cerebrospinal Fluid
Mouth
Differential Diagnosis
Biopsy
Antibodies

Keywords

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

ASJC Scopus subject areas

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

Cite this

Childhood Sjögren syndrome presenting as acute brainstem encephalitis. / Matsui, Yoriko; Takenouchi, Toshiki; Narabayashi, Atsushi; Ohara, Kentaro; Nakahara, Tadaki; Takahashi, Takao.

In: Brain and Development, 2015.

Research output: Contribution to journalArticle

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abstract = "Sj{\"o}gren 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 Sj{\"o}gren 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 Sj{\"o}gren syndrome. Commonly observed features in previously reported patients with childhood Sj{\"o}gren 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, Sj{\"o}gren syndrome should be included in the differential diagnosis, especially when sicca symptoms are present.",
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AB - 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.

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