Article
Immunology
Mia Levite, Hadassa Goldberg
Summary: Autoimmune Epilepsy is a complex disorder involving autoimmune antibodies targeting glutamate receptors and inhibitory neurotransmitter receptors, leading to neuronal damage and behavioral issues. The findings suggest a potential link between autoimmune mechanisms and epilepsy pathogenesis, highlighting the importance of understanding the role of autoimmunity in neurological disorders.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Clinical Neurology
Kelsey M. M. Smith, Jeffrey W. W. Britton, Smathorn Thakolwiboon, Nicholas H. H. Chia, Pranjal Gupta, Eoin P. P. Flanagan, Anastasia Zekeridou, Alfonso S. Lopez S. Chiriboga (Sebastian), Cristina Valencia Sanchez, Andrew McKeon, Sean J. J. Pittock, Divyanshu Dubey
Summary: This study aimed to describe the seizure characteristics and outcomes in patients with high-risk paraneoplastic autoantibodies and identify factors associated with ongoing seizures. The results showed that ANNA1-IgG and Ma2-IgG antibodies, high seizure frequency, and EEG and imaging abnormalities were associated with ongoing seizures. Although some patients may respond to immunotherapy and achieve seizure freedom, poor outcomes are frequently encountered, especially among patients with limbic encephalitis.
Review
Neurosciences
Pablo Cabezudo-Garcia, Natalia Mena-Vazquez, Nicolas L. Ciano-Petersen, Guillermina Garcia-Martin, Guillermo Estivill-Torrus, Pedro J. Serrano-Castro
Summary: This systematic review and meta-analysis found a small but not irrelevant prevalence of neural autoantibodies in patients with epilepsy of unknown etiology, with different prevalence rates for each antibody. None of the controls had a positive result, but there was high heterogeneity among studies, suggesting the need for a uniform protocol for testing neural autoantibodies in the future.
Article
Clinical Neurology
Anna Rada, Robert Birnbacher, Claudio Gobbi, Martin Kurthen, Albert Ludolph, Markus Naumann, Ulrike Neirich, Tim J. von Oertzen, Gerhard Ransmayr, Matthias Riepe, Mareike Schimmel, Oliver Schwartz, Rainer Surges, Christian G. Bien
Summary: Patients with surface antibodies are more likely to achieve seizure freedom and require less long-term use of anti-seizure medication. Changes in antibody titers are closely related to clinical courses.
JOURNAL OF NEUROLOGY
(2021)
Article
Medicine, General & Internal
Pablo Cabezudo-Garcia, Nicolas L. Ciano-Petersen, Natalia Mena-Vazquez, Jesus Ortega-Pinazo, Maria J. Postigo-Pozo, Guillermina Garcia-Martin, Helena Antoli-Martinez, Violeta Sanchez-Sanchez, Pablo Quiroga-Subirana, Pedro J. Serrano-Castro, Guillermo Estivill-Torrus
Summary: The study revealed a high prevalence of neural autoantibodies in adult patients with drug-resistant temporal lobe epilepsy of unknown etiology, indicating a possible autoimmune mechanism. The presence of bitemporal, independent, interictal epileptiform discharges and a history of status epilepticus may serve as markers for autoimmune-associated epilepsy in drug-resistant temporal lobe epilepsy of unknown etiology. Further validation of the proposed ARTE score in larger independent cohorts is warranted.
JOURNAL OF CLINICAL MEDICINE
(2021)
Article
Clinical Neurology
Sara Matricardi, Sara Casciato, Silvia Bozzetti, Sara Mariotto, Andrea Stabile, Elena Freri, Francesco Deleo, Stefano Sartori, Margherita Nosadini, Irene Pappalardo, Stefano Meletti, Giada Giovannini, Elisabetta Zucchi, Carlo Di Bonaventura, Giancarlo Di Gennaro, Sergio Ferrari, Luigi Zuliani, Marco Zoccarato, Alberto Vogrig, Simona Lattanzi, Roberto Michelucci, Antonio Gambardella, Edoardo Ferlazzo, Lucia Fusco, Tiziana Granata, Flavio Villani
Summary: This study describes the clinical and paraclinical findings, treatment options, and long-term outcomes in autoimmune encephalitis (AE), with a focus on the relationship with epilepsy. The study found that early immunotherapy and detection of antineuronal surface antibodies were independent predictors of favorable outcomes in AE. Autoimmune-associated epilepsy was a common long-term sequelae, often accompanied by cognitive and psychiatric disturbances. The severity of seizures at onset was the major risk factor for the development of chronic epilepsy.
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY
(2022)
Article
Clinical Neurology
Stephanie H. Chen, Pamela K. O'Dea, Bahareh Sianati, David R. Benavides
Summary: Autoimmune encephalitis (AE) often presents with seizures that are refractory to anti-seizure medications (ASM). A subset of patients with AE, called autoimmune-associated epilepsy (AAE), continue to have refractory epilepsy for years. Optimal treatment for AAE is still unknown, and the efficacy of neuromodulation and immunotherapy in managing AE has not been well established.
FRONTIERS IN NEUROLOGY
(2022)
Article
Immunology
Atsushi Hara, Norio Chihara, Ritsu Akatani, Ryusei Nishigori, Asato Tsuji, Hajime Yoshimura, Michi Kawamoto, Yoshihisa Otsuka, Yasufumi Kageyama, Takayuki Kondo, Frank Leypoldt, Klaus-Peter Wandinger, Riki Matsumoto
Summary: This study compared the characteristics of patients with autoimmune epilepsy who tested positive and negative for anti-neuronal antibodies. Patients in the AE/Ab(+) group showed increased differentiation of B cells and cTfh subsets associated with antibody production.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Immunology
Tingting Qiao, Lanlan Chen, Li Jiang, Hua Wei, Xin Chen, Xiaobo Li, Yingzhu Chen, Yao Xu
Summary: Anti-LGI1 encephalitis is a type of autoimmune encephalitis characterized by cognitive impairment, psychiatric disorders, and faciobrachial dystonic seizures. This report describes a rare neurological presentation of unilateral hyperhidrosis in a patient with anti-LGI1 encephalitis. The symptoms did not respond to antiepileptic drug treatment but showed favorable response to high-dose steroid therapy and intravenous immunoglobulin.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Clinical Neurology
Yongheng Zhao, Jun Li, Liang Gao, Xiaofan Yang, Haiqing Zhao, Yumei Li, Li Su, Xiaoyu Zhao, Hao Ding, Baomin Li
Summary: This study retrospectively analyzed the clinical characteristics and treatment plan of 9 children with short-term, frequent epileptic seizures who were autoimmune-antibody negative and responded well to immunotherapy. The results showed that symptomatic relief was achieved with immunotherapy in all patients, with most of them experiencing no recurrence.
FRONTIERS IN NEUROLOGY
(2022)
Article
Clinical Neurology
Ronan N. McGinty, Adam Handel, Teresa Moloney, Archana Ramesh, Andrew Fower, Emma Torzillo, Holger Kramer, Stephen Howell, Patrick Waters, Jane Adcock, Arjune Sen, Bethan Lang, Sarosh R. Irani
Summary: This study aimed to identify surface-directed autoantibodies in adults with new-onset focal epilepsy and evaluate the value of immunotherapy in this setting. The results showed that patients with detected autoantibodies but without encephalitis had excellent outcomes without immunotherapy, suggesting that immunotherapy administration should be guided by clinical features of encephalitis rather than autoantibody positivity. The findings indicate that seizure syndromes with autoantibodies largely fall under the category of autoimmune encephalitis in this cohort.
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY
(2021)
Article
Clinical Neurology
Ariane Soldatos, Thomas B. Nutman, Tory Johnson, Scott F. Dowell, James J. Sejvar, Michael R. Wilson, Joseph L. DeRisi, Sara K. Inati, Catherine Groden, Colleen Evans, Elise M. O'Connell, Bernard Opar Toliva, Jane R. Aceng, Josephine Aryek-Kwe, Camilo Toro, Constantine A. Stratakis, A. Gretchen Buckler, Cathy Cantilena, Tara N. Palmore, Audrey Thurm, Eva H. Baker, Richard Chang, Harper Fauni, David Adams, Ellen F. Macnamara, C. Christopher Lau, May Christine Malicdan, Barbara Pusey-Swerdzewski, Robert Downing, Sudhir Bunga, Jerry D. Thomas, William A. Gahl, Avindra Nath
Summary: This study characterized the phenotype of patients with Nodding Syndrome and investigated potential contributors to disease aetiology. They also evaluated the response to immunotherapy.
Article
Immunology
Maria Pia Giannoccaro, Matteo Gastaldi, Giovanni Rizzo, Leslie Jacobson, Veria Vacchiano, Giulia Perini, Sabina Capellari, Diego Franciotta, Alfredo Costa, Rocco Liguori, Angela Vincent
Summary: The study found that antibodies against neuronal surface antigens are relatively common in patients with neurodegenerative disorders, especially in those with unclear dementia presentation. An unclassified diagnosis and an irregular disease progression can predict positive antibody status.
BRAIN BEHAVIOR AND IMMUNITY
(2021)
Article
Clinical Neurology
Ebru Nur Vanli Yavuz, Ebru Altindag, Erdem Tuzun, Betul Baykan
Summary: This survey study on the attitudes of neurologists towards autoimmune epilepsy (AE) suggests that there is a relatively high level of awareness and understanding of AE, but there are still some deficiencies. Participants reported a lack of satisfactory guidelines for diagnosis and treatment, as well as widespread laboratory support. In addition, neurologists with less experience and those working outside of training hospitals tend to seek more consultation from experienced clinicians and conduct more detailed investigations.
NEUROLOGICAL SCIENCES
(2022)
Article
Clinical Neurology
Marie Suesse, Maria Zank, Viola von Podewils, Felix von Podewils
Summary: Late-onset seizures due to autoimmune encephalitis (AE) are associated with mesial temporal neuropsychological deficits, malignancy, pleocytosis, and oligoclonal bands in cerebrospinal fluid. Patients with AE showed favorable outcome with antiseizure drug and immunosuppressive therapy, highlighting the importance of neural antibody testing, neuroimaging, cerebrospinal fluid analysis, and neuropsychological testing in the diagnostic protocol for AE following late-onset seizures.
FRONTIERS IN NEUROLOGY
(2021)