Bondish Kambadja1, Houot Marion2,3,4, Louis Cousyn5,6, Nicolas Mezouar7, Vincent Navarro5,6,7,8, Bastien Herlin1,7, Dupont Sophie9,10,11,12. 1. Rehabilitation Unit, Pitié-Salpêtrière Hospital, AP-HP, Paris, France. 2. Clinical Investigation Centre, Institut du Cerveau, Pitié-Salpêtrière Hospital, Paris, France. 3. Department of Neurology, Institute of Memory and Alzheimer's Disease (IM2A), Pitié-Salpêtrière Hospital, AP-HP, Paris, France. 4. Centre of Excellence of Neurodegenerative Disease (CoEN), Pitié-Salpêtrière Hospital, Paris, France. 5. Centre de Recherche de l'Institut du Cerveau, UMPC-UMR 7225 CNRS-UMRS 975 Inserm, Paris, France. 6. Université Paris Sorbonne, Paris, France. 7. Epilepsy Unit, Pitié-Salpêtrière Hospital, AP-HP, Paris, France. 8. Neurophysiology Unit Pitié-Salpêtrière Hospital, AP-HP, Paris, France. 9. Rehabilitation Unit, Pitié-Salpêtrière Hospital, AP-HP, Paris, France. sophie.dupont@aphp.fr. 10. Centre de Recherche de l'Institut du Cerveau, UMPC-UMR 7225 CNRS-UMRS 975 Inserm, Paris, France. sophie.dupont@aphp.fr. 11. Université Paris Sorbonne, Paris, France. sophie.dupont@aphp.fr. 12. Epilepsy Unit, Pitié-Salpêtrière Hospital, AP-HP, Paris, France. sophie.dupont@aphp.fr.
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.
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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