title: Analysis of relapses in anti-NMDAR encephalitis.
abstract: OBJECTIVE
  The clinical characteristics of patients with relapsing anti-NMDA receptor (NMDAR) encephalitis are not well-defined. In this study, we report the clinical profile and outcome of relapses in a series of anti-NMDAR encephalitis.
  
  
  METHODS
  We did a retrospective review of relapses that occurred in 25 patients with anti-NMDAR encephalitis. Relapses were defined as any new psychiatric or neurologic syndrome, not explained by other causes, which improved after immunotherapy or, less frequently, spontaneously.
  
  
  RESULTS
  A total of 13 relapses were identified in 6 patients. Four of them had several, 2 to 4, relapses. There was a median delay of 2 years (range 0.5 to 13 years) for the first relapse. Median relapse rate was 0.52 relapses/patient-year. Relapse risk was higher in patients who did not receive immunotherapy in the first episode (p = 0.009). Most cases (53%) presented partial syndromes of the typical anti-NMDAR encephalitis. Main symptoms of relapses were speech dysfunction (61%), psychiatric (54%), consciousness-attention disturbance (38%), and seizures (31%). Three relapses (23%) presented with isolated atypical symptoms suggestive of brainstem-cerebellar involvement. An ovarian teratoma was detected at relapse in only 1 patient (17%). Relapses did not add residual deficit to that caused by the first episode.
  
  
  CONCLUSIONS
  Relapses in anti-NMDAR encephalitis are common (24%). They may occur many years after the initial episode. Relapses may present with partial aspects or with isolated symptoms of the full-blown syndrome. Immunotherapy at first episode reduces the risk of relapses.
authors.affiliation: Center for Neuroimmunology, Service of Neurology, Hospital Clinic and Institut d’Investigació Biomèdica August Pi i Sunyer, Barcelona, Spain.
authors.forename: I
authors.initials: I
authors.lastname: Gabilondo
authors.forename: A
authors.initials: A
authors.lastname: Saiz
authors.forename: L
authors.initials: L
authors.lastname: Galán
authors.forename: V
authors.initials: V
authors.lastname: González
authors.forename: R
authors.initials: R
authors.lastname: Jadraque
authors.forename: L
authors.initials: L
authors.lastname: Sabater
authors.forename: A
authors.initials: A
authors.lastname: Sans
authors.forename: A
authors.initials: A
authors.lastname: Sempere
authors.forename: A
authors.initials: A
authors.lastname: Vela
authors.forename: F
authors.initials: F
authors.lastname: Villalobos
authors.forename: M
authors.initials: M
authors.lastname: Viñals
authors.forename: P
authors.initials: P
authors.lastname: Villoslada
authors.forename: F
authors.initials: F
authors.lastname: Graus
mesh_terms: D000293:Adolescent; D000328:Adult; D001323:Autoantibodies / Q000096:biosynthesis* / Q000097:blood; D002648:Child; D002675:Child, Preschool; D004660:Encephalitis / Q000175:diagnosis / Q000276:immunology*; D005260:Female; D005500:Follow-Up Studies; D057809:HEK293 Cells; D006801:Humans; D007167:Immunotherapy / Q000379:methods; D007223:Infant; D008297:Male; D008875:Middle Aged; D016194:Receptors, N-Methyl-D-Aspartate / Q000037:antagonists & inhibitors / Q000097:blood / Q000276:immunology*; D012008:Recurrence; D012189:Retrospective Studies; D055815:Young Adult
chemical_list: D001323:Autoantibodies; D016194:Receptors, N-Methyl-D-Aspartate