title: Suspected new-onset autoimmune temporal lobe epilepsy with amygdala enlargement.
abstract: Recent reports define temporal lobe epilepsy with amygdala enlargement (TLE-AE) as a distinct electroclinical syndrome comparable to TLE with hippocampal sclerosis. In this retrospective observational study, we present the largest consecutive series of patients with new-onset TLE-AE to date and describe clinical characteristics and seizure outcome, and we aim to explore underlying autoimmune mechanisms within this syndrome.
  
  
  
  We reviewed all consecutive patients between 2004 and 2014 at our tertiary epilepsy center at the University of Bonn, Germany, with new-onset (<5 years) TLE-AE, negative serum antibody (ab) test results, and with available follow-up data for at least 12 months.
  
  
  
  We identified 40 patients (23 male) with TLE-AE with a median age at epilepsy onset of 51 years (range 10-73) and a median disease duration of 11 months (range 0.5-55) at first presentation. At follow-up, 50% of the entire cohort achieved seizure freedom. Of interest, patients with remittent features of AE at follow-up (N = 24) had a superior outcome compared to those with stable magnetic resonance imaging (MRI) features of AE (N = 16): 17 (71%) of 24 were seizure-free for at least 6 months compared to 3 (19%) of 16, respectively (p = 0.003). MRI volumetry confirmed significantly enlarged amygdalae in TLE-AE in relation to healthy controls, and additionally showed significantly greater volume reductions in patients with remittent AE compared to those with stable AE.
  
  
  
  TLE-AE is a clinical syndrome beginning mostly in middle age, and in addition to its known association with ab-positive limbic encephalitis, it occurs in an ab-negative condition. Remission of AE in the course of the disease could be identified as a predictor for a favorable clinical outcome and is suspicious of an autoimmune etiology, although we could not confirm this hypothesis unequivocally with currently available noninvasive diagnostic tools.
authors.affiliation: Department of Epileptology, University of Bonn, Bonn, Germany.|Department of Neurology, University of Cologne, Cologne, Germany.
authors.forename: Michael P
authors.initials: MP
authors.lastname: Malter
authors.affiliation: Department of Epileptology, University of Bonn, Bonn, Germany.
authors.forename: Guido
authors.initials: G
authors.lastname: Widman
authors.affiliation: Department of Neurology, University of Cologne, Cologne, Germany.|Institute of Neuroscience and Medicine, Research Center Juelich, Juelich, Germany.
authors.forename: Norbert
authors.initials: N
authors.lastname: Galldiks
authors.affiliation: Institute of Experimental Immunology, Affiliated to Euroimmun AG, Luebeck, Germany.
authors.forename: Winfried
authors.initials: W
authors.lastname: Stoecker
authors.affiliation: Department of Epileptology, University of Bonn, Bonn, Germany.
authors.forename: Christoph
authors.initials: C
authors.lastname: Helmstaedter
authors.affiliation: Department of Epileptology, University of Bonn, Bonn, Germany.|Department of NeuroCognition/Imaging, Life & Brain Centre, Bonn, Germany.
authors.forename: Christian E
authors.initials: CE
authors.lastname: Elger
authors.affiliation: Department of Epileptology, University of Bonn, Bonn, Germany.|Department of NeuroCognition/Imaging, Life & Brain Centre, Bonn, Germany.|Department of Neurology, Epilepsy Center Hessen-Marburg, University of Marburg Medical Centre, Marburg, Germany.
authors.forename: Jan
authors.identifier: 0000-0002-0459-8885
authors.initials: J
authors.lastname: Wagner
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000679:Amygdala / Q000000981:diagnostic imaging / Q000473:pathology*; D000927:Anticonvulsants / Q000627:therapeutic use; D001323:Autoantibodies / Q000378:metabolism*; D001327:Autoimmune Diseases / Q000150:complications*; D002648:Child; D003072:Cognition Disorders / Q000175:diagnosis / Q000209:etiology; D015331:Cohort Studies; D004569:Electroencephalography; D004833:Epilepsy, Temporal Lobe / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology*; D005260:Female; D006801:Humans; D007091:Image Processing, Computer-Assisted; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009483:Neuropsychological Tests; D018709:Statistics, Nonparametric; D055815:Young Adult
chemical_list: D000927:Anticonvulsants; D001323:Autoantibodies
keywords: Antineuronal antibodies; Limbic encephalitis; MRI volumetry