Table 1

Clinical and laboratory features of patients with epilepsy and positive neuronal surface autoantibodies

AgeSexAutoantibodyLive hippocampal neuron bindingAPE2 scoreCriteria AEClinical AEPiloEpil RFLowered MoodACE AttMRI ΔlimbicNumber of ASMsmRS
1 year
mRS 3 yearsImmunotherapy
79MCASPR2+3DefiniteYesNoNoneNo14Yes232IVMP, Pred, IVIg, PLEX
55FCASPR26DefiniteYesNoNoneYes18Yes322IVMP, Pred, IVIg, PLEX
55FGABABR+9DefiniteYesNoNoneNoNAYes66NAIVMP, Pred, PLEX
18FNMDAR+8DefiniteYesNoNoneYes15Yes322IVMP, Pred, PLEX, Aza, MMF
60FNMDAR4DefiniteYesNoNoneYes13No300Pred
77FGABAAR, GABABR+8PossibleYesNoNoneYes18Yes410Pred
54MLGI1+6PossibleYesYesNoneYes18Yes200None
59FLGI1+4NoYesNoNoneYes18No110None
69MUnknown+4NoYesNoNoneNo13No112Pred, Aza
63MAMPAR+2NoNoNoNoneNoNANo100None
28MCASPR22NoNoNoFHxYes17No100None
41FCASPR22NoNoNoNoneYes18No000None
75FContactin-21NoNoNoNoneNoNANo100None
37FGABAAR2NoNoNoNoneYes18No200None
36FGABABR2NoNoNoNoneNo18No110None
69FGlyR4NoNoNoNoneYes15No411None
61MLGI1+2NoNoYesNoneYes18No100None
36FNMDAR2NoNoNoNoneYes18No122None
68FNMDAR, GABAAR1NoNoNoNoneNo18No100None
77FUnknown+2NoNoNoNoneYesNANo00NANone
55FUnknown+4NoNoNoNoneYes18No522None
91MUnknown+1NoNoNoNoneNo9No16NANone
37MUnknown+2NoNoNoNoneYes18No200None
  • APE2 score of 4 or more is considered positive. Diagnosis of encephalitis based on clinical impression (‘Clinical AE’; corresponding rows shaded in grey) or published criteria (‘Criteria AE’), as per Graus et al.11

  • ‘Unknown’ autoantibody specificities reflect IgG binding to the surface of live hippocampal neurons, without a known antigenic target.

  • ACE Att, Addenbrooke’s Cognitive Examination attention domain score; AE, autoimmune encephalitis; APE2, Antibody Prevalence in Epilepsy and Encephalopathy Score; ASM, anti-seizure medication; Aza, azathioprine; Epil RF, epilepsy risk factors (neonatal, perinatal, focal neurological insults, family history of epilepsy or Alzheimer’s disease); FHx, family history; IVIg, intravenous immunoglobulin; IVMP, intravenous methylprednisolone; mRS, modified Rankin Score; Myco, mycophenolate; NA, not available; PLEX, plasma exchange; Pred, prednisolone.