Esme Ekizoglu1, Betul Baykan2, Mine Sezgin2, Ece Erdag3, Gokçen Gundogdu-Unverengil4, Ebru Nur Vanlı-Yavuz5, Pinar Tekturk2, Ebru Yılmaz6, Nerses Bebek2, Erdem Tuzun3. 1. Department of Neurology, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Turkey. Electronic address: esmeekizoglu@yahoo.com. 2. Department of Neurology, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Turkey. 3. Department of Neuroscience, Istanbul University, Institute of Experimental Medicine, Istanbul, Turkey. 4. Department of Pathology, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Turkey. 5. Department of Neurology, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Turkey; Department of Neurology, Koc University School of Medicine, Turkey. 6. Department of Nuclear Medicine, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Turkey.
Abstract
OBJECTIVE: The long-term follow-up of patients with epilepsy harboring autoantibodies against the glycine receptor (also glycine receptor antibodies or GlyR-Ab) is not well-known. Our aim was to investigate the 5-year prognosis and treatment response of patients with epilepsy who were seropositive for GlyR-Ab. METHODS: Clinical features; electroencephalogram (EEG), neuroradiological, and neuropathological findings; and treatment responses of patients with epilepsy with GlyR-Ab seropositivity were investigated. RESULTS: Thirteen (5.46%) of 238 patients with epilepsy were GlyR-Ab positive: focal epilepsy of unknown cause (FEoUC) was diagnosed in four (7.27%) out of 55 patients, mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS) in five (4.5%) out of 111 patients, epileptic encephalopathy (EE) in two (4%) out of 50 patients, and status epilepticus (SE) in two (9.09%) out of 22 patients. None of the patients developed any other neurological symptoms or cancer during the 5-year follow-up. Seven of them had seizures that were resistant to antiepileptic drug (AED). Immunotherapy was used in two patients (with FEoUC and EE) improving seizure control. Three patients with MTLE-HS benefited from epilepsy surgery, and another patient with EE showed spontaneous remission. CONCLUSION: Glycine receptor antibodies are detected in a wide spectrum of epileptic disorders with unclear pathogenic significance. Two GlyR-Ab seropositive patients with AED-resistant epilepsy treated with intravenous immunoglobulin (IVIg) showed clear benefit from immunotherapy. Future studies will be valuable in determining the role of screening patients with drug-resistant epilepsy for GlyR-Ab in order to identify patients who may benefit or respond to immunotherapy.
OBJECTIVE: The long-term follow-up of patients with epilepsy harboring autoantibodies against the glycine receptor (also glycine receptor antibodies or GlyR-Ab) is not well-known. Our aim was to investigate the 5-year prognosis and treatment response of patients with epilepsy who were seropositive for GlyR-Ab. METHODS: Clinical features; electroencephalogram (EEG), neuroradiological, and neuropathological findings; and treatment responses of patients with epilepsy with GlyR-Ab seropositivity were investigated. RESULTS: Thirteen (5.46%) of 238 patients with epilepsy were GlyR-Ab positive: focal epilepsy of unknown cause (FEoUC) was diagnosed in four (7.27%) out of 55 patients, mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS) in five (4.5%) out of 111 patients, epileptic encephalopathy (EE) in two (4%) out of 50 patients, and status epilepticus (SE) in two (9.09%) out of 22 patients. None of the patients developed any other neurological symptoms or cancer during the 5-year follow-up. Seven of them had seizures that were resistant to antiepileptic drug (AED). Immunotherapy was used in two patients (with FEoUC and EE) improving seizure control. Three patients with MTLE-HS benefited from epilepsy surgery, and another patient with EE showed spontaneous remission. CONCLUSION:Glycine receptor antibodies are detected in a wide spectrum of epileptic disorders with unclear pathogenic significance. Two GlyR-Ab seropositive patients with AED-resistant epilepsy treated with intravenous immunoglobulin (IVIg) showed clear benefit from immunotherapy. Future studies will be valuable in determining the role of screening patients with drug-resistant epilepsy for GlyR-Ab in order to identify patients who may benefit or respond to immunotherapy.
Authors: Attila Rácz; Chiara A Hummel; Albert Becker; Christoph Helmstaedter; Fabiane Schuch; Tobias Baumgartner; Randi von Wrede; Valeri Borger; László Solymosi; Rainer Surges; Christian E Elger Journal: Front Neurol Date: 2022-04-15 Impact factor: 4.003
Authors: Gabriela Dumitrita Stanciu; Veronica Bild; Daniela Carmen Ababei; Razvan Nicolae Rusu; Sorin Ioan Beschea Chiriac; Elena Rezuş; Andrei Luca Journal: Int J Mol Sci Date: 2019-09-13 Impact factor: 5.923