Maria Ilyas-Feldmann1, Harald Prüß2, Martin Holtkamp3. 1. Department of Neurology and Experimental Neurology, Epilepsy-Center Berlin-Brandenburg, Charité - Universitätsmedizin Berlin, Berlin, Germany. Electronic address: maria.feldmann@charite.de. 2. Department of Neurology and Experimental Neurology, Charité - Universitätsmedizin Berlin, Berlin, Germany; German Center for Neurodegenerative Diseases (DZNE) Berlin, Berlin, Germany. 3. Department of Neurology and Experimental Neurology, Epilepsy-Center Berlin-Brandenburg, Charité - Universitätsmedizin Berlin, Berlin, Germany.
Abstract
PURPOSE: To determine long-term seizure outcome, use of antiseizure medication (ASM) and seizure recurrence risk after its withdrawal in patients with autoimmune encephalitis (AE) due to neuronal surface and GAD antibodies. METHODS: In patients from a specialized AE outpatient clinic, we assessed seizure manifestation, ASM and immunotherapy at onset of AE as well as seizure occurrence, development of autoimmune-associated epilepsy (AAE) and use of ASM in the long-term. Data were collected from patients via telephone interviews and medical records. RESULTS: Out of 94 AE patients, 75 were analyzed; 47 patients had NMDAR, 17 LGI1, 7 GAD, 3 CASPR2 and 1 mGluR5 antibodies. Fifty-three of the 75 patients (71 %) experienced seizures, all of which for the first time occurred at AE onset. After a median follow-up of 6 years (range, 1-15), 47 of the 53 AE patients had 1-year terminal seizure remission, median duration of terminal seizure freedom was 5 years. Rate of 1-year terminal seizure remission was significantly higher in patients with neuronal surface antibodies (NMDAR 97 %, LGI1 93 %, CASPR2 100 %) compared to patients with GAD antibodies (20 %, p < 0.001). In seizure-free patients, ASM was withdrawn after 13 months (median) without any relapse seizures. CONCLUSIONS: Seizures are common in most forms of AE manifesting at disease onset in all cases. However, the development of AAE is rare and typically occurs in patients with GAD antibodies. Thus, in most AE cases with neuronal surface antibodies, ASM can be withdrawn after the acute phase of AE with low risk of seizure relapse.
PURPOSE: To determine long-term seizure outcome, use of antiseizure medication (ASM) and seizure recurrence risk after its withdrawal in patients with autoimmune encephalitis (AE) due to neuronal surface and GAD antibodies. METHODS: In patients from a specialized AE outpatient clinic, we assessed seizure manifestation, ASM and immunotherapy at onset of AE as well as seizure occurrence, development of autoimmune-associated epilepsy (AAE) and use of ASM in the long-term. Data were collected from patients via telephone interviews and medical records. RESULTS: Out of 94 AE patients, 75 were analyzed; 47 patients had NMDAR, 17 LGI1, 7 GAD, 3 CASPR2 and 1 mGluR5 antibodies. Fifty-three of the 75 patients (71 %) experienced seizures, all of which for the first time occurred at AE onset. After a median follow-up of 6 years (range, 1-15), 47 of the 53 AE patients had 1-year terminal seizure remission, median duration of terminal seizure freedom was 5 years. Rate of 1-year terminal seizure remission was significantly higher in patients with neuronal surface antibodies (NMDAR 97 %, LGI1 93 %, CASPR2 100 %) compared to patients with GAD antibodies (20 %, p < 0.001). In seizure-free patients, ASM was withdrawn after 13 months (median) without any relapse seizures. CONCLUSIONS:Seizures are common in most forms of AE manifesting at disease onset in all cases. However, the development of AAE is rare and typically occurs in patients with GAD antibodies. Thus, in most AE cases with neuronal surface antibodies, ASM can be withdrawn after the acute phase of AE with low risk of seizure relapse.
Authors: Anusha Yeshokumar; Eliza Gordon-Lipkin; Ana Arenivas; Mark Rosenfeld; Kristina Patterson; Raia Blum; Brenda Banwell; Arun Venkatesan; Eric Lancaster; Jessica Panzer; John Probasco Journal: Neurol Neuroimmunol Neuroinflamm Date: 2022-07-06