Bastien Joubert1,2, Aude Belbezier1, Julie Haesebaert3,4, Sylvain Rheims5,6, François Ducray1, Géraldine Picard1, Véronique Rogemond1,2, Dimitri Psimaras1,7, Giulia Berzero7, Virginie Desestret1,2, Jérôme Honnorat8,9,10. 1. French Reference Center on Paraneoplastic Neurological Syndromes, Hospices Civils de Lyon, Hôpital Neurologique, 69677, Bron, France. 2. Institut NeuroMyoGene INSERM U1217/CNRS UMR 5310, Université de Lyon, Université Claude Bernard Lyon 1, 69372, Lyon, France. 3. Public Health Department, Hospices Civils de Lyon, 69424, Lyon, France. 4. HESPER, Université de Lyon, Université Claude Bernard Lyon 1, EA 7425, 69008, Lyon, France. 5. Departement of Functional Neurology and Epileptology, Hospices Civils de Lyon, Hôpital Neurologique, 69677, Bron, France. 6. Lyon Neuroscience Research Center INSERM U1058/CNRS UMR 5292, Université de Lyon, Université Claude Bernard Lyon 1, 69372, Lyon, France. 7. Neurology Department, Groupe Hospitalier Pitié-Salpêtrière, 75013, Paris, France. 8. French Reference Center on Paraneoplastic Neurological Syndromes, Hospices Civils de Lyon, Hôpital Neurologique, 69677, Bron, France. jerome.honnorat@chu-lyon.fr. 9. Institut NeuroMyoGene INSERM U1217/CNRS UMR 5310, Université de Lyon, Université Claude Bernard Lyon 1, 69372, Lyon, France. jerome.honnorat@chu-lyon.fr. 10. Neuro-Oncologie, Hôpital Neurologique Pierre Wertheimer, 59 Boulevard Pinel, 69677, Bron Cedex, France. jerome.honnorat@chu-lyon.fr.
Abstract
OBJECTIVE: To assess the long-term outcomes of patients with temporal lobe epilepsy and CSF anti-glutamate decarboxylase antibodies (GAD65-Abs). METHODS: We retrospectively analyzed the clinical records of 35 patients with temporal lobe epilepsy and CSF GAD65-Abs, collected from January 1993 to December 2016 and assessed cognitive impairment and seizure activity at last visit. Cognitive impairment was considered significant if impacting on daily life activities. Immunohistochemistry on rat brain slices and ELISA were used for antibody detection and titration. RESULTS: Median age was 30 years (range 2-63), 32/35 (91%) patients were female, and median follow-up was 68 months (range 7-232). At presentation, 20 patients had isolated temporal lobe epilepsy and 15 patients had other limbic symptoms, including anterograde amnesia (n = 10) and behavioral disturbances (n = 5). Progressive clinical deterioration over follow-up was reported in 28/35 patients (80%), including gradual increase of memory impairment (n = 25), and apparition of behavioral disturbances (n = 4) or mood disorders (n = 18). At last follow-up, 24/35 (69%) patients had cognitive disturbances with an impact on patient's daily life activities, and 28/35 (80%) still had active seizures. CONCLUSION: Most patients with temporal lobe epilepsy and CSF GAD65-Abs develop a chronic disease with progressive cognitive impairment and refractory epilepsy regardless of the presence of additional limbic symptoms at onset.
OBJECTIVE: To assess the long-term outcomes of patients with temporal lobe epilepsy and CSF anti-glutamate decarboxylase antibodies (GAD65-Abs). METHODS: We retrospectively analyzed the clinical records of 35 patients with temporal lobe epilepsy and CSFGAD65-Abs, collected from January 1993 to December 2016 and assessed cognitive impairment and seizure activity at last visit. Cognitive impairment was considered significant if impacting on daily life activities. Immunohistochemistry on rat brain slices and ELISA were used for antibody detection and titration. RESULTS: Median age was 30 years (range 2-63), 32/35 (91%) patients were female, and median follow-up was 68 months (range 7-232). At presentation, 20 patients had isolated temporal lobe epilepsy and 15 patients had other limbic symptoms, including anterograde amnesia (n = 10) and behavioral disturbances (n = 5). Progressive clinical deterioration over follow-up was reported in 28/35 patients (80%), including gradual increase of memory impairment (n = 25), and apparition of behavioral disturbances (n = 4) or mood disorders (n = 18). At last follow-up, 24/35 (69%) patients had cognitive disturbances with an impact on patient's daily life activities, and 28/35 (80%) still had active seizures. CONCLUSION: Most patients with temporal lobe epilepsy and CSFGAD65-Abs develop a chronic disease with progressive cognitive impairment and refractory epilepsy regardless of the presence of additional limbic symptoms at onset.
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