Malter, Michael P., Widman, Guido, Galldiks, Norbert ORCID: 0000-0002-2485-1796, Stoecker, Winfried, Helmstaedter, Christoph, Elger, Christian E. and Wagner, Jan ORCID: 0000-0002-0459-8885 (2016). Suspected new-onset autoimmune temporal lobe epilepsy with amygdala enlargement. Epilepsia, 57 (9). S. 1485 - 1495. HOBOKEN: WILEY. ISSN 1528-1167

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Abstract

Objective: 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. Methods: 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. Results: 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. Significance: 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.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Malter, Michael P.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Widman, GuidoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Galldiks, NorbertUNSPECIFIEDorcid.org/0000-0002-2485-1796UNSPECIFIED
Stoecker, WinfriedUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Helmstaedter, ChristophUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Elger, Christian E.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Wagner, JanUNSPECIFIEDorcid.org/0000-0002-0459-8885UNSPECIFIED
URN: urn:nbn:de:hbz:38-263609
DOI: 10.1111/epi.13471
Journal or Publication Title: Epilepsia
Volume: 57
Number: 9
Page Range: S. 1485 - 1495
Date: 2016
Publisher: WILEY
Place of Publication: HOBOKEN
ISSN: 1528-1167
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
NMDA RECEPTOR ANTIBODIES; GATED POTASSIUM CHANNEL; LIMBIC ENCEPHALITIS; CLINICAL-CHARACTERISTICS; HIPPOCAMPAL SCLEROSIS; CSF FINDINGS; NEUROMYOTONIA; VOLUMETRY; PROPOSAL; SURGERYMultiple languages
Clinical NeurologyMultiple languages
Refereed: Yes
URI: http://kups.ub.uni-koeln.de/id/eprint/26360

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