title: Sjogren's syndrome-associated meningoencephalomyelitis: cerebrospinal fluid cytokine levels and therapeutic utility of tacrolimus.
abstract: Serial changes in the circulating and cerebrospinal fluid (CSF) cytokine levels were assessed in a patient with Sjogren's syndrome (SS)-associated meningoencephalomyelitis. A 16-yr-old girl diagnosed as having primary SS at 8 yr of age presented headache and vomiting. CSF studies revealed lymphocyte-dominant pleocytosis and high IgM index, but no evidence of infection. Disturbed consciousness and diffuse slow waves on electroencephalogram led to the diagnosis of SS-meningoencephalitis. The clinical condition subsided after a cycle of dexamethasone therapy, however, 2 months later urinary retention and paresthesia of the lower body developed. Craniospinal magnetic resonance imaging (MRI) showed extensive intraparenchymal lesions with high T2-weighted signal intensity adjacent to the posterior left horn of lateral ventricle of the brain and the longitudinal lesion from C5 to T10 of the spinal cord. High-dose methyl-prednisolone and subsequent tacrolimus therapy has effectively controlled the activity of SS-meningoencephalomyelitis. Monitoring of systemic and CSF cytokine levels during the course of illness revealed that CSF interleukin-6, but not interferon-gamma or tumor necrosis factor-alpha levels were the sensitive indicator of disease activity. The unique cytokine profile, differing from those of infectious meningitis may be useful for predicting the central nervous system involvement in autoimmune disease.
authors.affiliation: Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Fukuoka 812-8582, Japan.
authors.forename: Takayuki
authors.initials: T
authors.lastname: Hoshina
authors.forename: Yui
authors.initials: Y
authors.lastname: Yamaguchi
authors.forename: Shouichi
authors.initials: S
authors.lastname: Ohga
authors.forename: Ryutaro
authors.initials: R
authors.lastname: Kira
authors.forename: Masataka
authors.initials: M
authors.lastname: Ishimura
authors.forename: Hidetoshi
authors.initials: H
authors.lastname: Takada
authors.forename: Tamami
authors.initials: T
authors.lastname: Tanaka
authors.forename: Toshiro
authors.initials: T
authors.lastname: Hara
mesh_terms: D000293:Adolescent; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage; D015415:Biomarkers / Q000032:analysis / Q000097:blood / Q000134:cerebrospinal fluid; D001921:Brain / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D016207:Cytokines / Q000134:cerebrospinal fluid*; D004305:Dose-Response Relationship, Drug; D005260:Female; D006261:Headache / Q000276:immunology / Q000503:physiopathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D015850:Interleukin-6 / Q000032:analysis / Q000097:blood / Q000134:cerebrospinal fluid; D008279:Magnetic Resonance Imaging; D008590:Meningoencephalitis / Q000134:cerebrospinal fluid / Q000188:drug therapy / Q000276:immunology; D008775:Methylprednisolone / Q000008:administration & dosage; D008991:Monitoring, Physiologic / Q000592:standards; D009187:Myelitis / Q000150:complications / Q000188:drug therapy / Q000276:immunology*; D011237:Predictive Value of Tests; D012859:Sjogren's Syndrome / Q000150:complications*; D013116:Spinal Cord / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D016559:Tacrolimus / Q000627:therapeutic use*; D016896:Treatment Outcome; D014839:Vomiting / Q000209:etiology / Q000503:physiopathology
chemical_list: D000893:Anti-Inflammatory Agents; D015415:Biomarkers; D016207:Cytokines; D007166:Immunosuppressive Agents; D015850:Interleukin-6; D016559:Tacrolimus; D008775:Methylprednisolone