Review
Pediatrics
Claudio Ancona, Valentina Masenello, Matteo Tinnirello, Luca Mattia Toscano, Andrea Leo, Chiara La Piana, Irene Toldo, Margherita Nosadini, Stefano Sartori
Summary: This study conducted a systematic literature review on rare pediatric NSAS, and found that the clinical characteristics of pediatric NSAS differ from those in adults, including lower tumor association and decreased female preponderance. Anti-D2R, anti-GABAAR, and anti-GlyR autoimmunity were the most common types of NSAS in children.
FRONTIERS IN PEDIATRICS
(2022)
Article
Clinical Neurology
Simon Rinaldi, Alexander Davies, Janev Fehmi, Heidi N. Beadnall, Justine Wang, Todd A. Hardy, Michael H. Barnett, Simon A. Broadley, Patrick Waters, Stephen W. Reddel, Sarosh R. Irani, Fabienne Brilot, Russell C. Dale, Sudarshini Ramanathan
Summary: Antibodies to myelin oligodendrocyte glycoprotein (MOG) are associated with CNS demyelination including optic neuritis and transverse myelitis. The study of an Australasian MOGAD cohort revealed a subgroup possibly mediated by coexistent autoantibodies, presenting with myeloradiculitis, combined central and peripheral demyelination syndromes, and inflammatory neuropathies, being responsive to immunotherapy.
NEUROLOGY-NEUROIMMUNOLOGY & NEUROINFLAMMATION
(2021)
Review
Virology
Ivanildo P. Sousa Jr, Flavia B. dos Santos, Vanessa S. de Paula, Tuane C. R. G. Vieira, Helver G. Dias, Caroline A. Barros, Edson E. da Silva
Summary: Virus-induced infections of the central nervous system pose serious public health problems, especially in low- and middle-income countries. Common viruses include herpes simplex virus and varicella-zoster virus. More research is needed, particularly in countries like Brazil, to better understand the most common viruses involved in CNS infections.
Article
Chemistry, Multidisciplinary
Corleone S. Delaveris, Catherine L. Wang, Nicholas M. Riley, Sherry Li, Rishikesh U. Kulkarni, Carolyn R. Bertozzi
Summary: Neurons communicate through synapses, modulated by microglia using various means including the Neu3 enzyme. Neu3 disrupts neuronal network connectivity under inflammatory stimuli, impacting network-level activity of neurons and potentially contributing to neuroinflammatory diseases such as Parkinson's and Alzheimer's.
ACS CENTRAL SCIENCE
(2023)
Review
Clinical Neurology
Gelsomina Mansueto, Giuseppe Lanza, Francesco Fisicaro, Danielle Alaouieh, Emily Hong, Sara Girolami, Marco Montella, Alessandro Feola, Mario Di Napoli
Summary: This review provides a comprehensive update on the clinical assessment, diagnosis, complications, and treatment of PCNSV. Recent developments and advances in technology have improved diagnostic accuracy and provided new insights for preventing misdiagnosis. However, treatment for PCNSV remains largely empirical.
CURRENT NEUROLOGY AND NEUROSCIENCE REPORTS
(2022)
Review
Biochemistry & Molecular Biology
Polina Kameneva, Maria Eleni Kastriti, Igor Adameyko
Summary: Schwann cell precursors (SCPs) are a neural crest-derived multipotent progenitor type that can build enteric and parasympathetic neurons as well as neuroendocrine chromaffin cells. The similarities in markers and potential to crest cells raise many questions regarding medical side, fundamental developmental biology, and evolution.
CELLULAR AND MOLECULAR LIFE SCIENCES
(2021)
Article
Clinical Neurology
Bruna Klein da Costa, Paula de Oliveira Pinto, Lia Staub, Gisele Hansel, Guilherme Vanik Pinto, Lucas Porcello Schilling, Giordani Rodrigues dos Passos, William Alves Martins, Jefferson Becker, Raphael Machado Castilhos, Andre Palmini, Douglas Kazutoshi Sato
Summary: This study described the phenotype and environmental associations of patients with neurological syndromes associated with antibodies against neuronal surface antigens. The results showed that most patients had disease onset in spring and summer, and observed a different pattern of brain lesions in NMDAr-IgG encephalitis following herpes encephalitis, as well as an association with Rosai-Dorfman-Destombe disease. Importantly, most patients improved with immunotherapy, even when initiated later in the disease course.
MULTIPLE SCLEROSIS AND RELATED DISORDERS
(2023)
Review
Neurosciences
Jessica L. Mueller, Rhian Stavely, Ryo Hotta, Allan M. Goldstein
Summary: With the increasing prevalence of neurological disorders in an aging population, there is a critical need to better understand exogenous strategies for nervous system repair. Stem cell therapy offers a promising approach, but is limited by various factors. Researchers propose the use of autologous cells derived from the peripheral nervous system as an alternative source for CNS repair, which has shown neurogenic potential and eliminates concerns around tumorigenic risk, ethical considerations, and immune-mediated rejection.
FRONTIERS IN NEUROSCIENCE
(2022)
Review
Immunology
Lu Zhang, Cui Jiao, Lingjuan Liu, Aiping Wang, Li Tang, Yi Ren, Peng Huang, Jie Xu, Dingan Mao, Liqun Liu
Summary: NLRC5, the largest member of the NLR family, plays a significant role in inflammatory processes, angiogenesis, immunity, and apoptosis by regulating various signaling pathways. Recent studies have also linked NLRC5 with neuronal development and CNS diseases.
FRONTIERS IN IMMUNOLOGY
(2021)
Review
Biochemistry & Molecular Biology
Luca Marsili, Samuel Marcucci, Joseph LaPorta, Martina Chirra, Alberto J. Espay, Carlo Colosimo
Summary: Paraneoplastic neurological syndromes (PNS) can manifest as various clinical syndromes of the central nervous system. Early diagnosis and treatment are crucial for improving the long-term outcome of these conditions.
Review
Cell Biology
Devi Satarkar, Chinmoy Patra
Summary: This review provides a comprehensive description of the role of CXCR3 and its ligands in the immune system and diseases, highlighting the potential of CXCR3 as a therapeutic target and an early diagnostic biomarker.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2022)
Review
Biochemistry & Molecular Biology
Elise Lebigot, Manuel Schiff, Marie-Pierre Golinelli-Cohen
Summary: Mitochondrial proteins containing Fe-S clusters are crucial for cellular pathways, and mutations in certain genes can lead to multiple mitochondrial dysfunction syndromes. Patients with MMDS typically present with severe leukoencephalopathy, and diagnosis often involves testing for elevated lactate, pyruvate, and glycine levels, as well as genetic analysis.
Article
Clinical Neurology
Meng-Ting Cai, Qi-Lun Lai, Jin-Long Tang, Bing-Qing Du, Chun-Hong Shen, Yin-Xi Zhang, Yi Guo
Summary: This case report describes a patient initially diagnosed with MOG antibody-associated disease (MOGAD), which improved with corticosteroid therapy but relapsed four months later and was subsequently diagnosed with primary central nervous system lymphoma (PCNSL). This case broadens the phenotypic spectrum of PCNSL lesions and highlights the importance of considering PCNSL in patients with benign CNS inflammatory disorders who worsen clinically and radiographically despite steroid treatment. Timely biopsy is crucial for accurate diagnosis and appropriate therapy.
NEUROLOGICAL SCIENCES
(2023)
Review
Clinical Neurology
Lucy L. Gibson, Anna McKeever, Alexis E. Cullen, Timothy R. Nicholson, Dag Aarsland, Michael S. Zandi, Thomas A. Pollak
Summary: Neuronal antibodies can cause encephalopathy syndromes with cognitive impairment, but the association between NMDAR antibodies and atypical dementia was stronger than with all-cause dementia. Further studies are needed to clarify the role of neuronal antibodies in dementia and explore potential treatment options.
JOURNAL OF NEUROLOGY
(2021)
Review
Virology
Arash Hellysaz, Marie Hagbom
Summary: This qualitative review examines the relationship between rotavirus infection and CNS complications, indicating the involvement of gut-brain mechanisms in symptoms driven by the CNS. Rotavirus can affect the CNS through various pathways, including neural gut-brain communication and systemic spread, requiring further research to elucidate the underlying mechanisms.
Article
Clinical Neurology
Fatme Seval Ismail, Marianna Spatola, Friedrich G. Woermann, Stoyan Popkirov, Johannes Jungilligens, Christian G. Bien, Jorg Wellmer, Uwe Schlegel
Summary: In patients with temporal lobe seizures and additional signs of limbic involvement, 17% had neuronal antibodies confirming the diagnosis of ALE. Mediotemporal MRI changes were present in all antibody-positive cases and had a positive likelihood ratio of 2.11.
EUROPEAN JOURNAL OF NEUROLOGY
(2022)
Article
Clinical Neurology
Alessandra Morano, Emanuele Cerulli Irelli, Martina Fanella, Biagio Orlando, Enrico Michele Salamone, Emanuele Tinelli, Gabriele Ruffolo, Luigi Zuliani, Jinane Fattouch, Mario Manfredi, Anna Teresa Giallonardo, Carlo Di Bonaventura
Summary: This study investigated olfaction in a population of patients with autoimmune encephalitis (AE). It found that a significant proportion of AE cases had impaired olfaction, and this appeared to be more common in patients with severe AE, suggesting that olfactory deficits may be an additional feature of immune-mediated encephalitis.
JOURNAL OF NEUROLOGY
(2022)
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
Alessandro Dinoto, Matteo Gastaldi, Raffaele Iorio, Sofia Marini, Valentina Damato, Antonio Farina, Marco Zoccarato, Elia Sechi, Francesca Pinna, Giorgia Teresa Maniscalco, Ruggero Barnabei, Luigi Zuliani, Sergio Ferrari, Sara Mariotto
Summary: This retrospective multicenter study evaluated the safety of SARS-CoV-2 vaccination in patients with autoantibodies targeting neuronal surface and/or synaptic antigens. The results showed that a small percentage of patients experienced relapses after vaccination, but the majority did not experience any side effects.
MULTIPLE SCLEROSIS AND RELATED DISORDERS
(2022)
Article
Clinical Neurology
Alessandra Morano, Emanuele Cerulli Irelli, Enrico Michele Salamone, Biagio Orlando, Martina Fanella, Emanuele Tinelli, Gabriele Ruffolo, Luigi Zuliani, Jinane Fattouch, Mario Manfredi, Anna Teresa Giallonardo, Carlo Di Bonaventura
Summary: This study compares patients with autoimmune encephalitis (AE) and late-onset epilepsy of unknown origin (LOEUO) and finds that high-frequency focal seizures with autonomic manifestations may be indicative of AE in the elderly. It highlights the importance of using 24-h ambulatory electroencephalography (AEEG) to recognize AE and reduce diagnostic delay.
FRONTIERS IN NEUROLOGY
(2022)
Article
Clinical Neurology
Javier A. Lopez-Rivera, Costin Leu, Marie Macnee, Jean Khoury, Lucas Hoffmann, Roland Coras, Katja Kobow, Nisha Bhattarai, Eduardo Perez-Palma, Hajo Hamer, Sebastian Brandner, Karl Roessler, Christian G. Bien, Thilo Kalbhenn, Tom Pieper, Till Hartlieb, Elizabeth Butler, Giulio Genovese, Kerstin Becker, Janine Altmueller, Lisa-Marie Niestroj, Lisa Ferguson, Robyn M. Busch, Peter Nuernberg, Imad Najm, Ingmar Bluemcke, Dennis Lal
Summary: Lopez-Rivera et al. conducted a study on the genetic architecture of different types of epileptic brain lesions. They discovered novel somatic chromosomal alterations, identified new genes and genotype-phenotype associations, and supported the role of genetics in the histopathological diagnosis of epileptic lesions. The findings provide valuable insights for improving treatment of drug-resistant focal epilepsy.
Article
Clinical Neurology
Carmen Barba, Ingmar Blumcke, Melodie R. Winawer, Till Hartlieb, Hoon-Chul Kang, Laura Grisotto, Mathilde Chipaux, Christian G. Bien, Barbora Hermanovska, Brenda E. Porter, Hart G. W. Lidov, Valentina Cetica, Friedrich G. Woermann, Javier A. Lopez-Rivera, Peter D. Canoll, Irina Mader, Ludovico D'Incerti, Sara Baldassari, Edward Yang, Ahmed Gaballa, Hannes Vogel, Barbora Straka, Letizia Macconi, Tilman Polster, Gerald A. Grant, Lenka Krskova, Hui Jin Shin, Ara Ko, Peter B. Crino, Pavel Krsek, Jeong Ho Lee, Dennis Lal, Stephanie Baulac, Annapurna Poduri, Renzo Guerrini
Summary: This study describes the clinical, genetic, neuroimaging, EEG, and histopathologic findings in patients with refractory epilepsy and brain somatic SLC35A2 gene variants. It also assesses possible predictors of postoperative seizure and cognitive outcomes.
Article
Clinical Neurology
Sally Elakkary, Anne Hagemann, Dennis Klimpel, Christian G. Bien, Christian Brandt
Summary: This retrospective study evaluated the serum concentrations of clobazam and N-desmethylclobazam (NCLB) in five patients before and after co-medication with cenobamate. The addition of cenobamate resulted in an increase in NCLB serum concentration and concentration-to-dose ratio (CDR) in all patients. However, the degree of increase varied among patients. Therefore, concomitant administration of cenobamate and clobazam can substantially increase serum concentrations of NCLB, leading to both positive therapeutic effects and unwanted fatigue.
Article
Clinical Neurology
Louisa Hohmann, Christian G. G. Bien, Martin Holtkamp, Philip Grewe
Summary: This study investigated the relevance of patients' psycho-social profile for surgical decision-making in epilepsy. The results showed that employment was the only relevant psycho-social aspect for neurologists' surgery recommendations, while subjective antiseizure medication adverse events, subjective seizure severity, and subjective cognitive impairment were important predictors for patients' acceptance of surgery.
Article
Neurosciences
Anissa Benzait, Valentina Krenz, Martin Wegrzyn, Anna Doll, Friedrich Woermann, Kirsten Labudda, Christian G. Bien, Johanna Kissler
Summary: The ability to regulate emotions is crucial for maintaining psychological health. Patients with frontal lobe epilepsy exhibit emotional dysregulation and maladaptive emotion regulation strategies. Our study found that frontal lobe epilepsy patients showed diminished neural activity in specific areas during emotion regulation and displayed different activity patterns compared to healthy controls.
HUMAN BRAIN MAPPING
(2023)
Article
Clinical Neurology
Margherita Nosadini, Michael Eyre, Thea Giacomini, Massimiliano Valeriani, Marida Della Corte, Andrea D. Pratico, Pietro Annovazzi, Ramona Cordani, Duccio Maria Cordelli, Giovanni Crichiutti, Gabriella Di Rosa, Valentina Dolcemascolo, Anna Fetta, Elena Freri, Paolo Gallo, Matteo Gastaldi, Tiziana Granata, Luisa Grazian, Raffaele Iorio, Martina Lombardini, Monica Margoni, Sara Mariotto, Sara Matricardi, Federico Melani, Nardo Nardocci, Laura Papetti, Alice Passarini, Francesco Pisani, Chiara Po, Marco Puthenparampil, Francesca Ragona, Salvatore Savasta, Sabrina Siliquini, Irene Toldo, Alessandra Tozzo, Emanuela Claudia Turco, Antonio Varone, Alberto Vogrig, Luigi Zuliani, Samuela Bugin, Sara Rossato, Alessandro Orsini, Gaetano Cantalupo, Maria Margherita Mancardi, Michela Ada Noris Ferilli, Thomas Foiadelli, Stefano Sartori
Summary: This study aimed to identify early factors associated with relapse and outcome in pediatric-onset MOGAD. The study found that early immunotherapy, longer duration of corticosteroid treatment, and abnormal optic nerves on MRI were associated with a lower risk of relapse. Meanwhile, higher disease severity at onset was associated with a greater risk of final disability.
NEUROLOGY-NEUROIMMUNOLOGY & NEUROINFLAMMATION
(2023)
Article
Clinical Neurology
Thilo Kalbhenn, Thomas Cloppenborg, Friedrich G. G. Woermann, Anne Hagemann, Tilman Polster, Roland Coras, Ingmar Bluemcke, Christian G. G. Bien, Matthias Simon
Summary: Objective completeness is an important predictor of seizure freedom in epilepsy surgery. This study focuses on the requirements for complete hemispherotomy and suggests that disconnection of the insula can lead to favorable postoperative seizure outcomes. The results demonstrate that complete surgical disconnection is the most crucial factor in predicting seizure freedom following hemispherotomy.
Editorial Material
Clinical Neurology
Anna Rada, Christian G. Bien
Summary: Seizures resulting from cerebral autoimmunity can be either symptomatic in the context of autoimmune encephalitis (AIE) with neural surface antibodies or indicative of an enduring predisposition to seizures, known as autoimmune encephalitis-associated epilepsy (AEAE). AEAE can be diagnosed in cases where seizures persist for at least 2 years after immunotherapy initiation, no signs of encephalitis are observed on imaging, cerebrospinal fluid cell count is normal, and antibody titers decrease substantially. The distinction between AIE and AEAE has practical consequences for treatment and social limitations.
Article
Clinical Neurology
Izumi Kuramochi, Takayuki Iwayama, Christian Brandt, Haruo Yoshimasu, Christian G. Bien, Anne Hagemann
Summary: The German version of the Epilepsy Self-Stigma Scale (ESSS-G) was developed and validated in this study, allowing for reliable evaluation of self-stigma in German epilepsy patients. The results showed that higher ESSS-G scores were associated with higher anxiety and depression, lower self-esteem, overall lower quality of life and health. ESSS-G can be used to assess the efficacy of treatment strategies in reducing self-stigma and conducting intercultural comparisons.
Article
Clinical Neurology
Carmen Barba, Ingmar Blumcke, Melodie R. Winawer, Till Hartlieb, Hoon-Chul Kang, Laura Grisotto, Mathilde Chipaux, Christian G. Bien, Barbora Hermanovska, Brenda E. Porter, Hart G. W. Lidov, Valentina Cetica, Friedrich G. Woermann, Javier A. Lopez-Rivera, Peter D. Canoll, Irina Mader, Ludovico D'Incerti, Sara Baldassari, Edward Yang, Ahmed Gaballa, Hannes Vogel, Barbora Straka, Letizia Macconi, Tilman Polster, Gerald A. Grant, Lenka Krskova, Hui Jin Shin, Ara Ko, Peter B. Crino, Pavel Krsek, Jeong Ho Lee, Dennis Lal, Stephanie Baulac, Annapurna Poduri, Renzo Guerrini
Summary: Brain somatic SLC35A2 gene variants are associated with two main clinical phenotypes, early epileptic encephalopathy (EE) and drug-resistant focal epilepsy (DR-FE). Most patients showed unchanged cognitive performances after surgery, with better postoperative seizure control outcomes seen in cases where postoperative EEG was normal or improved.