title: Neurosjögren: early therapy is associated with successful outcomes.
abstract: BACKGROUND
  Primary Sjögren syndrome (PSS) is a systemic autoimmune condition with an estimated prevalence of 0.6%. The frequency of neurologic manifestations in PSS varies widely from 0% to 60%.
  
  
  METHODS
  We report the characteristics of PSS patients with neurologic involvement seen at a single tertiary hospital in Singapore. Eight consecutive women (median age, 51 years [range, 38-67 years]) with neurologic manifestations of PSS seen between March 2009 to June 2011 were followed up for a mean duration of 19 months from the onset of neurologic manifestations.
  
  
  RESULTS
  Six of 8 patients with neurosjögren had their neurologic manifestation at time of PSS diagnosis. The lag times of neurologic manifestations from PSS diagnosis for the remaining 2 patients were 9 and 30 years, respectively. Sicca symptoms were not readily volunteered as a presenting complaint in the majority of patients. All our patients received early aggressive therapy with pulse corticosteroids and intravenously administered cyclophosphamide. The mean duration from initial presentation to initiation of treatment was 11 days (1-26 days). All achieved good recovery regardless of the type or site of neurologic involvement, initial erythrocyte sedimentation rate, immunoglobulin and complement levels.
  
  
  CONCLUSIONS
  Neurologic disease, when present, is a strong contributor to disease activity and damage. Confirmatory tests should be conducted early regardless of the presence of sicca symptoms. Vigilance for the development of new neurologic symptoms is imperative even in chronic, apparently stable patients. It is likely that early initiation of treatment contributed to good recovery in our patients.
authors.affiliation: University Medicine Cluster, Department of Medicine, Division of Rheumatology, National University Health System, Singapore. amelia.santosa@gmail.com
authors.forename: Amelia
authors.initials: A
authors.lastname: Santosa
authors.forename: Anita Y N
authors.initials: AY
authors.lastname: Lim
authors.forename: Sheila
authors.initials: S
authors.lastname: Vasoo
authors.forename: Tang Ching
authors.initials: TC
authors.lastname: Lau
authors.forename: Gim Gee
authors.initials: GG
authors.lastname: Teng
mesh_terms: D000328:Adult; D000368:Aged; D001799:Blood Sedimentation; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008775:Methylprednisolone / Q000008:administration & dosage; D008875:Middle Aged; D009422:Nervous System Diseases / Q000188:drug therapy* / Q000209:etiology*; D019965:Neurocognitive Disorders / Q000209:etiology; D020551:Pulse Therapy, Drug; D012189:Retrospective Studies; D012859:Sjogren's Syndrome / Q000150:complications*; D061665:Time-to-Treatment
chemical_list: D005938:Glucocorticoids; D007074:Immunoglobulin G; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D008775:Methylprednisolone