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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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.
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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