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01-08-2021 | Status Epilepticus | Original Communication

Recurrent seizures of autoimmune origin: emerging phenotypes

Authors: Mathilde Goudot, Solène Frismand, Lucie Hopes, Antoine Verger, Bastien Joubert, Jérôme Honnorat, Louise Tyvaert

Published in: Journal of Neurology | Issue 8/2021

Abstract

Objective

Recurrent seizures of autoimmune origin (AEp) are one of the most frequent causes of recurrent seizures or suspected epilepsy of unknown cause. The aim of this study was to identify specific phenotypes corresponding to AEp.

Methods

We retrospectively reviewed features of patients with recurrent seizures of unknown cause and investigated for suspected AEp (January 2015–May 2018). Patients were separated in: (1) AEpAb+: AEp with positive autoantibodies; (2) AEpAb−: suspected AEp (inflammatory central nervous system (CNS) profile) without autoantibodies; (3) NAEp: epilepsy without CNS inflammation.

Results

Eighty-nine epileptic patients underwent a CSF antibody detection. From the remaining 57 epileptic patients (32 excluded for a differential diagnosis), 61.4% were considered as AEp. 21% were AEpAb+ (4 NMDAR, 2 GABAbR, 3 GAD-Ab, 2 LGi1, 1 CASPR2), 40.4% AEpAb−, and 38.6% NAE. AE (AEpAb+ and AEpAb−) was significantly associated with antibody prevalence in epilepsy (APE) score ≥ 4 (80%), encephalitic phase (71.4%), psychiatric involvement (64.7%), cognitive impairment (50%), and status epilepticus (41.2%). Within the group of 29 patients without encephalitic phase and with chronic epilepsy (NEPp), 34.5% were defined as AEp. 10.4% were AEpAb+ (2 GAD, 1 CASPR2) and 24.1% were AEpAb−. NEP AEp was associated with non-cerebral autoimmune disorders, short epileptic disease duration, and cognitive impairment.

Conclusions

Autoimmune cause (AEp) should be assessed in patient suffering from recurrent seizures of unknown cause. Acute encephalitis is clearly the main AEp phenotype. AEp was also defined in more than one-third of chronic epilepsy patients (NEP) of unknown cause. Then, AEp may be combined with other autoimmune comorbidities, a shorter evolution of recurrent seizures, and cognitive impairment.

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Title: Recurrent seizures of autoimmune origin: emerging phenotypes
Authors: Mathilde Goudot
Solène Frismand
Lucie Hopes
Antoine Verger
Bastien Joubert
Jérôme Honnorat
Louise Tyvaert
Publication date: 01-08-2021
Publisher: Springer Berlin Heidelberg
Keywords: Status Epilepticus
Epilepsy
Encephalitis
Published in: Journal of Neurology / Issue 8/2021
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-021-10457-1

2024 ASCO Annual Meeting

Springer Medicine

Recurrent seizures of autoimmune origin: emerging phenotypes

Abstract

Objective

Methods

Results

Conclusions

2024 ASCO Annual Meeting

Springer Medicine

Abstract

Objective

Methods

Results

Conclusions

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Correction to: Routine diagnostics for neural antibodies, clinical correlates, treatment and functional outcome