Abstract
Background and purpose
Seizures represent a core symptom of autoimmune encephalitides with specific therapeutic issues. To date, patients with new-onset seizures or established epilepsy are not systematically tested for autoimmune antibodies. We aimed to identify clinical and paraclinical criterion that could help to select patients requiring additional autoimmune antibodies serum and cerebrospinal fluid (CSF) detection.
Methods
In this retrospective single center study from the French Salpêtrière Hospital, data from 286 adult patients with epilepsy who received an autoantibody assay for the first time were analyzed. All patients were evaluated at our institution between January 2007 and December 2018 for assessment of new-onset epilepsy (n = 90) or established epilepsy (n = 196). We only analyzed patients that were screened for autoimmune antibodies. Demographic, clinical and neuroimaging measures were compared between patients with and without autoimmune encephalitis using Fisher’s exact test for categorical variables and Welch’s t test for continuous variables. Our primary goal was to identify significant factors that differentiated patients with and without autoimmune encephalitis.
Results
We identified 27 patients with autoimmune epilepsy (9.4% of the patients who had been tested for autoantibodies). The significant factors differentiating patients with and without autoimmune encephalitis were: (i) the existence of a new-onset focal epilepsy + (e.g., newly diagnosed epilepsy < 6 months associated with additional symptoms, mainly cognitive or psychiatric symptoms), (ii) the presence of faciobrachial dystonic seizures very suggestive of anti- Leucine-rich glioma inactivated 1 (LGI1) encephalitis, and (iii) the presence of magnetic resonance imaging (MRI) abnormalities suggestive of encephalitis.
Conclusion
New-onset focal seizures combined with cognitive or psychiatric symptoms support the test for autoimmune antibodies. Further clinical already known red flags for an autoimmune origin are the presence of faciobrachial dystonic seizures and MRI signal changes consistent with encephalitis. On the other hand, isolated new-onset seizures and chronic epilepsy, even with associated symptoms, seem rarely linked to autoimmune encephalitis and should not lead to systematic testing.
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Data availability
Anonymized data will be shared by request from any qualified investigator.
Code availability
Text and cover letter: Microsoft word.
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All authors contributed to the study conception and design. Material preparation, data collection and analysis were performed by BK (material preparation, data collection), MH (statistical analysis), LC (data collection), NM (data collection), Vincent Navarro (analysis), BH (analysis), and SD (analysis). The first draft of the manuscript was written by SD and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.
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On behalf of all authors, Sophie Dupont states that there is no conflict of interest.
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The study was conducted in accordance with the ethical standards of the AP-HP according to the Declaration of Helsinki and authorized by the CNIL committee (No. 2020051517525).
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Patients provided written informed consent for participation in the study and further publications.
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Kambadja, B., Marion, H., Cousyn, L. et al. When should we test patients with epilepsy for autoimmune antibodies? Results from a French retrospective single center study. J Neurol 269, 3109–3118 (2022). https://doi.org/10.1007/s00415-021-10894-y
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DOI: https://doi.org/10.1007/s00415-021-10894-y