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. 2023 Dec;64(12):e229-e236.
doi: 10.1111/epi.17755. Epub 2023 Oct 5.

The role of neuronal antibodies in cryptogenic new onset refractory status epilepticus

Amanda Eisele  1 , Matthias Schwager  2 , Stefan Yu Bögli  1 , Ina Reichen  1 , Justina Dargvainiene  3 , Klaus-Peter Wandinger  3 , Lukas Imbach  4 , Marcellina Haeberlin  1 , Emanuela Keller  2   5 , Ilijas Jelcic  1 , Marian Galovic  1 , Giovanna Brandi  2
Affiliations

The role of neuronal antibodies in cryptogenic new onset refractory status epilepticus

Amanda Eisele et al. Epilepsia. 2023 Dec.

Abstract

Most cases with new onset refractory status epilepticus (NORSE) remain cryptogenic despite extensive diagnostic workup. The aim of this study was to analyze the etiology and clinical features of NORSE and investigate known or potentially novel autoantibodies in cryptogenic NORSE (cNORSE). We retrospectively assessed the medical records of adults with status epilepticus at a Swiss tertiary referral center between 2010 and 2021. Demographic, diagnostic, therapeutic, and outcome parameters were characterized. We performed post hoc screening for known or potentially novel autoantibodies including immunohistochemistry (IHC) on rat brain with cerebrospinal fluid (CSF) and serum samples of cNORSE. Twenty patients with NORSE were identified. Etiologies included infections (n = 4), Creutzfeldt-Jakob disease (n = 1), CASPR2 autoimmune encephalitis (n = 1), and carotid artery stenosis with recurrent perfusion deficit (n = 1). Thirteen cases (65%) were cryptogenic despite detailed evaluation. A posteriori IHC for neuronal autoantibodies yielded negative results in all available serum (n = 11) and CSF (n = 9) samples of cNORSE. Our results suggest that neuronal antibodies are unlikely to play a major role in the pathogenesis of cNORSE. Future studies should rather focus on other-especially T-cell- and cytokine-mediated-mechanisms of autoinflammation in this devastating disease, which is far too poorly understood so far.

Keywords: NORSE; autoimmune; autoinflammation; etiology; outcome.

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Conflict of interest statement

I.J. has received speaker honoraria or unrestricted grants from Biogen Idec and Novartis and has received compensation for advice or lecturing from Alexion, Biogen, Bristol Myers Squibb, Celgene, Janssen-Cilag, Neuway, Merck, Novartis, Roche, and Sanofi Genzyme; none of these is related to this study. M.G. reports fees from Advisis, Arvelle, Bial, Eisai, Nestlé Health Science, and UCB outside the submitted work. None of the other authors has any conflict of interest to disclose.

References

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