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Recurrent seizures of autoimmune origin: emerging phenotypes

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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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Author information

Authors and Affiliations

  1. Department of Neurology, University Hospital of Nancy, Nancy, France

    Mathilde Goudot, Solène Frismand, Lucie Hopes & Louise Tyvaert

  2. Faculty of Medicine, CRAN CNRS UMR 7039, University of Lorraine, Nancy, France

    Louise Tyvaert

  3. Department of Nuclear Medicine, IADI, INSERM U1254, University Hospital of Nancy, Nancy, France

    Antoine Verger

  4. Department of Neurology, French Reference Center of Paraneoplastic Neurological Syndrome, Hospices Civils de Lyon, INSERM U1217/CNRS, UMR5310, Lyon, France

    Bastien Joubert & Jérôme Honnorat

  5. Epilepsy Unit, Department of Neurology, Nancy University Medical Centre, Central Hospital, 54000, Nancy, France

    Louise Tyvaert

Authors
  1. Mathilde Goudot
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  2. Solène Frismand
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  3. Lucie Hopes
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  4. Antoine Verger
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  5. Bastien Joubert
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  7. Louise Tyvaert
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Contributions

MG and LT: designed and conceptualized the study; acquisition of data; analyzed the data; draft the manuscript for intellectual content. SF and LH: designed and conceptualized the study; interpreted the data; revised the manuscript for intellectual content. AV, BJ, and JH: interpreted the data; revised the manuscript for intellectual content.

Corresponding author

Correspondence to Louise Tyvaert.

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On behalf of all authors, the corresponding author states that there is no conflict of interest.

Ethical standard statement

This study was conducted in accordance with the ethical standards recognized internationally.

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Goudot, M., Frismand, S., Hopes, L. et al. Recurrent seizures of autoimmune origin: emerging phenotypes. J Neurol 268, 3000–3010 (2021). https://doi.org/10.1007/s00415-021-10457-1

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  • DOI: https://doi.org/10.1007/s00415-021-10457-1

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