title: Childhood Sjögren syndrome presenting as acute brainstem encephalitis.
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.
authors.affiliation: Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan.
authors.forename: Yoriko
authors.initials: Y
authors.lastname: Matsui
authors.affiliation: Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan. Electronic address: toshiki.take@keio.jp.
authors.forename: Toshiki
authors.initials: T
authors.lastname: Takenouchi
authors.affiliation: Department of Pediatrics, Kawasaki Municipal Hospital, Kanagawa, Japan.
authors.forename: Atsushi
authors.initials: A
authors.lastname: Narabayashi
authors.affiliation: Department of Pathology, Keio University School of Medicine, Tokyo, Japan.
authors.forename: Kentaro
authors.initials: K
authors.lastname: Ohara
authors.affiliation: Department of Radiology, Keio University School of Medicine, Tokyo, Japan.
authors.forename: Tadaki
authors.initials: T
authors.lastname: Nakahara
authors.affiliation: Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan.
authors.forename: Takao
authors.initials: T
authors.lastname: Takahashi
mesh_terms: D001933:Brain Stem / Q000473:pathology*; D002648:Child; D003937:Diagnosis, Differential; D004660:Encephalitis / Q000175:diagnosis* / Q000473:pathology*; D005260:Female; D006801:Humans; D011877:Radionuclide Imaging; D012469:Salivary Glands / Q000000981:diagnostic imaging / Q000473:pathology; D012859:Sjogren's Syndrome / Q000175:diagnosis* / Q000473:pathology*
keywords: Central nervous system; Children; Encephalitis; Sicca symptoms; Sjögren syndrome