title: Incidence and prevalence of major central nervous system involvement in systemic lupus erythematosus: a 3-year prospective study of 370 patients.
abstract: BACKGROUND
  The incidence and prevalence of CNS involvement in SLE remains unclear owing to conflicting results in the published studies. The aim of the study was to evaluate the incidence and prevalence of major definite CNS events in SLE patients.
  
  
  METHODS
  370 SLE patients with no previous history of CNS involvement were prospectively evaluated in a tertiary hospital referral center for 3 years. Major CNS manifestations were codified according to ACR definitions, including chorea, aseptic meningitis, psychosis, seizures, myelopathy, demyelinating syndrome, acute confusional state and strokes. Minor CNS events were excluded. ECLAM and SLEDAI-SELENA Modification scores were used to evaluate disease activity and SLICC/ACR Damage Index was used to assess accumulated damage.
  
  
  RESULTS
  16/370 (4.3%) patients presented with a total of 23 major CNS events. These included seizures (35%), strokes (26%), myelopathy (22%), optic neuritis (8.7%), aseptic meningitis (4.3%) and acute psychosis (4.3%). Incidence was 7.8/100 person years. Among hospitalizations for SLE, 13% were due to CNS manifestations. Epileptic seizures were associated with high disease activity, while myelopathy correlated with lower disease activity and NMO-IgG antibodies (P≤0.05). Stroke incidence correlated with APS coexistence (P = 0.06). Overall, CNS involvement correlated with high ECLAM and SLEDAI scores (P<0.001).
  
  
  CONCLUSIONS
  Clinically severe CNS involvement is rare in SLE patients, accounting for 7.8/100 person years. CNS involvement correlates with high disease activity and coexistence of specific features that define the respective CNS syndromes.
authors: Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece.
authors: Eleni I
authors: EI
authors: Kampylafka
authors: Haralampos
authors: H
authors: Alexopoulos
authors: Michalis L
authors: ML
authors: Kosmidis
authors: Demosthenes B
authors: DB
authors: Panagiotakos
authors: Panayiotis G
authors: PG
authors: Vlachoyiannopoulos
authors: Marinos C
authors: MC
authors: Dalakas
authors: Haralampos M
authors: HM
authors: Moutsopoulos
authors: Athanasios G
authors: AG
authors: Tzioufas
mesh_terms: D000328:Adult; D002490:Central Nervous System / Q000473:pathology* / Q000503:physiopathology*; D005260:Female; D005500:Follow-Up Studies; D006760:Hospitalization / Q000706:statistics & numerical data; D006801:Humans; D015994:Incidence; D008180:Lupus Erythematosus, Systemic / Q000453:epidemiology* / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D008297:Male; D015995:Prevalence; D011446:Prospective Studies