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Timing of MOG-IgG Testing Is Key to 2023 MOGAD Diagnostic Criteria Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-11-17 Forcadela, M., Rocchi, C., San Martin, D., Gibbons, E. L., Wells, D., Woodhall, M. R., Waters, P. J., Huda, S., Hamid, S.
Background and Objectives Myelin oligodendrocyte glycoprotein (MOG) antibody–associated disease (MOGAD) is a recently identified autoimmune demyelinating disorder of the CNS affecting both adults and children. Diagnostic criteria for MOGAD have recently been published. We aimed to validate the 2023 MOGAD diagnostic criteria in a real-world cohort of patients with atypical CNS inflammation. Methods
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Antibody Investigations in 2,750 Children With Suspected Autoimmune Encephalitis Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-11-15 Chen, L.-W., Guasp, M., Olive-Cirera, G., Martinez-Hernandez, E., Ruiz Garcia, R., Naranjo, L., Saiz, A., Armangue, T., Dalmau, J.
Objectives To assess the frequency and types of neuronal and glial (neural) antibodies in children with suspected autoimmune encephalitis (AE). Methods Patients younger than 18 years with suspected AE other than acute disseminated encephalomyelitis, whose serum or CSF samples were examined in our center between January 1, 2011, and April 30, 2022, were included in this study. Samples were systematically
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Glial Fibrillary Acidic Protein Autoimmunity After Aseptic Meningitis: A Report of 2 Cases Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-11-10 Bien, C. G., Büttner, T., Reichen, I. C., Thomas, A., Vlad, B., Woermann, F., Bien, C. I., Jelcic, I.
Objectives We describe 2 patients with glial fibrillary acidic protein (GFAP) autoimmunity secondary to aseptic viral meningitis or meningoencephalomyelitis. Methods This study involved a retrospective chart review. Results Two female patients, 45 and 55 years of age, developed aseptic meningoencephalomyelitis or meningitis; in one patient, it was likely caused by herpes simplex virus 2. The patients
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Neuropsychological Testing in Autoimmune Encephalitis: A Scoping Review Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-11-10 Galioto, R., Grezmak, T., Swetlik, C., Abbatemarco, J. R., Titulaer, M. J., Finke, C., Kunchok, A.
Background and Objectives Identifying optimal methods for evaluation and monitoring of cognitive outcomes in AE is important for clinical care and research. This scoping review aimed to evaluate neuropsychological tests (NPT) that are most frequently impaired in AE cohorts to provide recommendations for a standardized NPT battery for AE outcome. Methods PubMed search for studies examining NPT in patients
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Clinical Determinants of Longitudinal Disability in LGI-1-IgG Autoimmune Encephalitis Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2024-01-01 Albert Aboseif, Yadi Li, Moein Amin, Brittany Lapin, Alex Milinovich, Justin R. Abbatemarco, Jeffrey A. Cohen, Vineet Punia, Alex D. Rae-Grant, Rachel Galioto, Amy Kunchok
Background and Objectives Longitudinal outcome studies in leucine-rich glioma inactivated-1 (LGI-1) immunoglobulin G (IgG) autoimmune encephalitis (AE) are needed to inform clinical management and prognostication. This study aims to evaluate longitudinal predictors of disability and disease severity in LGI-1-IgG AE. Methods This retrospective observational study of patients with LGI-1-IgG AE was conducted
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Mitoxantrone in NMO Spectrum Disorder in a Large Multicenter Cohort in French Caribbean Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2024-01-01 Hugo Chaumont, Nicolas Bérard, Jean-Pierre Karam, Quentin Lobjois, Benoit Tressieres, Aissatou Signate, Annie Lannuzel, Philippe Cabre
Background and Objectives Preventing relapses in neuromyelitis Optica spectrum disorder (NMOSD) is a primary goal. New effective molecules are often expensive and not readily available in regions with fragile health systems. Assessing the efficacy and safety of less costly therapeutic alternatives is necessary. We aim to evaluate the efficacy and safety of mitoxantrone (MiTX) in NMOSD. Methods This
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Resident Memory-like CD8+ T Cells Are Involved in Chronic Inflammatory and Neurodegenerative Diseases in the CNS Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2024-01-01 Kimitoshi Kimura, Ryusei Nishigori, Mio Hamatani, Masanori Sawamura, Shinji Ashida, Chihiro Fujii, Masaki Takata, Youwei Lin, Wakiro Sato, Tomoko Okamoto, Akira Kuzuya, Ryosuke Takahashi, Takashi Yamamura, Takayuki Kondo
Background and Objectives Resident memory T (Trm) cells are a unique population that can survive and function in a compartmentalized tissue with inflammatory potential. We aim to investigate the alteration of Trm population in acute/chronic inflammatory and neurodegenerative diseases in the CNS. Methods The frequencies of CD4+ and CD8+ T cells expressing both CD69 and CD103, the markers for Trm cells
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Increased Intracranial Pressure in Pediatric Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2024-01-01 Linda Nguyen, Darryl K. Miles, Lana Harder, Sumit Singh, Brett A. Whittemore, Benjamin M. Greenberg, Cynthia X. Wang
Background and Objectives Elevated intracranial pressure (ICP) in myelin oligodendrocyte glycoprotein (MOG) antibody–associated disease (MOGAD) has been largely unexplored. The objectives of this study were to determine the frequency of increased ICP in MOGAD and its association with disease course and outcomes and to highlight cases requiring medical and/or surgical management of increased ICP. Methods
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Early Neuroaxonal Damage in Neurologic Disorders Associated With GAD65 Antibodies Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2024-01-01 Katharina Eisenhut, Jennifer Faber, Daniel Engels, Ramona Gerhards, Jan Lewerenz, Kathrin Doppler, Claudia Sommer, Robert Markewitz, Kim K. Falk, Rosa Rössling, Harald Pruess, Carsten Finke, Jonathan Wickel, Christian Geis, Dominica Ratuszny, Lena K. Pfeffer, Stefan Bittner, Johannes Piepgras, Andrea Kraft, Jaqueline Klausewitz, Brigitte Nuscher, Tania Kümpfel, Franziska S. Thaler, for the German Network
Objectives Neurodegeneration is considered a relevant pathophysiologic feature in neurologic disorders associated with antibodies against glutamic acid decarboxylase 65 (GAD65). In this study, we investigate surrogates of neuroaxonal damage in relation to disease duration and clinical presentation. Methods In a multicentric cohort of 50 patients, we measured serum neurofilament light chain (sNfL) in
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Immune Checkpoint Inhibition-Related Myasthenia-Myositis-Myocarditis Responsive to Complement Blockade Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2024-01-01 Christopher Nelke, Marc Pawlitzki, Ruth Kerkhoff, Christina B. Schroeter, Orhan Aktas, Eva Neuen-Jacob, Amin Polzin, Sven G. Meuth, Tobias Ruck
Objective Immune checkpoint inhibitors (ICIs) have revolutionized cancer therapy but come with immune-related adverse events (irAEs) that provide a novel challenge for treating physicians. Neuromuscular irAEs, including myositis, myasthenia gravis (MG), and demyelinating polyradiculoneuropathy, lead to significant morbidity and mortality. Methods We present a case of severe myasthenia-myositis-myocarditis
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Antibody-Negative Autoimmune Encephalitis: A Single-Center Retrospective Analysis Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-10-25 Mojzisova, H., Krysl, D., Hanzalova, J., Dargvainiene, J., Wandinger, K.-P., Leypoldt, F., Elisak, M., Marusic, P.
Background and Objectives Autoimmune encephalitis (AE) refers to a heterogenous group of inflammatory CNS diseases. Subgroups with specified neural autoantibodies are more homogeneous in presentation, trigger factors, outcome, and response to therapy. However, a considerable fraction of patients has AE features but does not harbor detectable autoantibodies and is referred to as antibody-negative AE
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Long-term Effects of IL-6 Receptor Blockade Therapy on Regulatory Lymphocytes and Neutrophils in Neuromyelitis Optica Spectrum Disorder Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2024-01-01 Takako Matsuoka, Manabu Araki, Youwei Lin, Tomoko Okamoto, Ralf Gold, Norio Chihara, Wakiro Sato, Atsuko Kimura, Hisateru Tachimori, Katsuichi Miyamoto, Susumu Kusunoki, Takashi Yamamura
Background and Objectives Neuromyelitis optica spectrum disorder (NMOSD) is a disabling autoimmune neurologic disease. Anti–IL-6 receptor (IL-6R) therapy prevents relapses in patients with anti–aquaporin 4 (AQP4)-IgG-positive NMOSD; however, it remains unclear how cellular immune components are altered by anti–IL-6R therapy. In this study, we examined the long-term effects of the anti–IL-6R monoclonal
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Electrolyte Imbalance in Anti LGI1 Encephalitis: It Is Not All in Your Head Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-11-01 Avi Gadoth
In the Research Article "Electrolyte Imbalance in Anti LGI1 Encephalitis: It Is Not All in Your Head" by Gadoth et al.,1 the second author's name should be listed as "Michal Nisnboym." The article has been replaced by a corrected version. The authors regret the error.
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Chronic Enterovirus Meningoencephalitis in Prolonged B-Cell Depletion After Rituximab Therapy: Case Report Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-10-09 Jacksch, C., Dargvainiene, J., Böttcher, S., Diedrich, S., Leypoldt, F., Stürner, K., Berg, D., Schäffer, E.
Objectives Persistent impaired immunity is possible even years after B-cell depleting therapies. This may favor the occurrence of infections, including infectious meningitis and encephalitis. In this study, we report a case of chronic enterovirus meningoencephalitis in prolonged B-cell depletion years after rituximab therapy. Methods This is a case report from a German academic hospital. In addition
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The OSCAR-MP Consensus Criteria for Quality Assessment of Retinal Optical Coherence Tomography Angiography Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-11-01 Rebecca Wicklein, Charmaine Yam, Christina Noll, Lilian Aly, Nicolas Banze, Eva Feodora Romahn, Elisabeth Wolf, Bernhard Hemmer, Frederike C. Oertel, Hanna Zimmermann, Philipp Albrecht, Marius Ringelstein, Carmen Baumann, Nikolaus Feucht, Josef Penkava, Joachim Havla, Jonathan A. Gernert, Christian Mardin, Eleni S. Vasileiou, Anneke Van Der Walt, Omar Al-Louzi, Sergio Cabello, Angela Vidal-Jordana
Background and Objectives Optical coherence tomography angiography (OCTA) is a noninvasive high-resolution imaging technique for assessing the retinal vasculature and is increasingly used in various ophthalmologic, neuro-ophthalmologic, and neurologic diseases. To date, there are no validated consensus criteria for quality control (QC) of OCTA. Our study aimed to develop criteria for OCTA quality assessment
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Increasing Neurofilament and Glial Fibrillary Acidic Protein After Treatment Discontinuation Predicts Multiple Sclerosis Disease Activity Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-11-01 Gauruv Bose, Brian C. Healy, Shrishti Saxena, Fermisk Saleh, Bonnie I. Glanz, Rohit Bakshi, Howard L. Weiner, Tanuja Chitnis
Background and Objectives Stable patients with multiple sclerosis (MS) may discontinue treatment, but the risk of disease activity is unknown. Serum neurofilament light chain (sNfL) and serum glial fibrillary acidic protein (sGFAP) are biomarkers of subclinical disease activity and may help risk stratification. In this study, sNfL and sGFAP levels in stable patients were evaluated before and after
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Multiple Sclerosis, Disease-Modifying Therapies, and Infections Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-11-01 Annette M. Langer-Gould, Jessica B. Smith, Edlin G. Gonzales, Fredrik Piehl, Bonnie H. Li
Background and Objectives The use of highly effective multiple sclerosis (MS) disease-modifying therapies (DMTs) is rapidly increasing. Yet, little is known about their real-world risks of infections. The goals of this study were to assess the comparative risk of outpatient and serious infections across DMTs in a large, diverse, U.S. cohort and determine whether such risks are attributable to DMTs
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Natalizumab Treatment Induces Proinflammatory CD4 T Cells Preferentially in the Integrin {beta}7+ Compartment Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-11-01 Mélanie Nguyen Ky, Adrien Duran, Iris Hasantari, Agnès Bru, Mathilde Deloire, Bruno Brochet, Aurélie Ruet, Nathalie Schmitt
Background and Objectives Natalizumab, a monoclonal humanized antibody targeting integrin α4, inhibits the transmigration of lymphocytes into the CNS by preventing the interaction of integrin α4β1 with V-CAM expressed on brain vascular endothelial cells. Although natalizumab treatment reduces the clinical relapse rate in patients with relapsing-remitting MS, its discontinuation after reactivation of
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Prevalence, Clinical Profiles, and Prognosis of Stiff-Person Syndrome in a Japanese Nationwide Survey Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-11-01 Naoko Matsui, Keiko Tanaka, Mitsuyo Ishida, Yohei Yamamoto, Yuri Matsubara, Reiko Saika, Takahiro Iizuka, Koshi Nakamura, Nagato Kuriyama, Makoto Matsui, Kokichi Arisawa, Yosikazu Nakamura, Ryuji Kaji, Satoshi Kuwabara, Yuishin Izumi, for the Japanese SPS Study
Background and Objectives To elucidate current epidemiologic, clinical, and immunologic profiles and treatments of stiff-person syndrome (SPS) in Japan. Methods A nationwide mail survey was conducted using an established method. Data processing sheets were sent to randomly selected departments of internal medicine, neurology, pediatrics, psychiatry, and neurosurgery in hospitals and clinics throughout
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mRNA COVID-19 Vaccination Does Not Exacerbate Symptoms or Trigger Neural Antibody Responses in Multiple Sclerosis Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-11-01 Yolanda Blanco, Domingo Escudero, Cinta Lleixà, Sara Llufriu, Natalia Egri, Raquel Ruiz García, Mercedes Alba, Esther Aguilar, Montse Artola, Marta Aldea Novo, Silvia Alvarez, Eva Caballero, Jose Maria Cabrera-Maqueda, Elianet Fonseca, Mar Guasp, Ana Hernando, Eugenia Martinez-Hernandez, Gemma Olivé-Cirera, Joaquin Lopez-Contreras, Lorena Martín-Aguilar, Laura Martinez-Martinez, Alexander Rombauts
Background and Objective In people with multiple sclerosis (pwMS), concern for potential disease exacerbation or triggering of other autoimmune disorders contributes to vaccine hesitancy. We assessed the humoral and T-cell responses to SARS-CoV-2 after mRNA vaccination, changes in disease activity, and development of antibodies against central or peripheral nervous system antigens. Methods This was
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A 28-Year-Old Woman With Left-Sided Weakness and Atypical MRI Lesions: From the National Multiple Sclerosis Society Case Conference Proceedings Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-11-01 Amy L. Safadi, Benjamin Osborne, Tanuja Chitnis, Jennifer S. Graves, Scott D. Newsome, Scott S. Zamvil, Isaac H. Solomon, Robert K. Shin
A 28-year-old woman presented with subacute relapsing left-sided weakness. MRI demonstrated both enhancing C3-C6 and nonenhancing T2-T4 lesions. Initial provisional diagnosis was inflammatory/autoimmune. Her left-sided weakness progressed despite immunosuppressive therapies. We reassessed our original suspected diagnosis because of an atypical clinicoradiologic course, leading to biopsy and a definitive
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Peptides From the Variable Domain of Immunoglobulin G as Biomarkers in Chronic Inflammatory Demyelinating Polyradiculoneuropathy Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-11-01 Joris Godelaine, Yamini Chitale, Bart De Moor, Chantal Mathieu, Lina Ancheva, Philip Van Damme, Kristl G. Claeys, Xavier Bossuyt, Sebastien Carpentier, Koen Poesen
Background and Objectives Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a clinically heterogeneous immune-mediated disease. Diagnostic biomarkers for CIDP are currently lacking. Peptides derived from the variable domain of circulating immunoglobulin G (IgG) have earlier been shown to be shared among patients with the same immunologic disease. Because humoral immune factors are
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Immunotherapy-Responsive Neuropathic Pain and Allodynia in a Patient With Glycine Receptor Autoantibodies: A Case Report Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-11-01 Babak Soleimani, Callum Board, Tony Yu, Irene Tracey, Sarosh R. Irani, Peter Foley
Objectives Neuropathic pain is common and distressing. Improved mechanistic understanding and pharmacotherapies are urgently needed. Molecularly specific pain syndromes may provide insights with translational relevance. Glycine receptors are known to play a key role in inhibitory neurotransmission in the spinal dorsal horn and have therefore been considered as targets for analgesic development. While
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Kappa Free Light Chain Index Predicts Disease Course in Clinically and Radiologically Isolated Syndromes Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-11-01 Michael Levraut, Antoine Gavoille, Cassandre Landes-Chateau, Mikael Cohen, Saskia Bresch, Barbara Seitz-Polski, Lydiane Mondot, Christine Lebrun-Frenay
Background and Objectives To evaluate whether the kappa free light chain index (K-index) can predict the occurrence of new T2-weighted MRI lesions (T2L) and clinical events in clinically isolated syndrome (CIS) and radiologically isolated syndrome (RIS). Methods All consecutive patients presenting for the diagnostic workup, including CSF analysis, of clinical and/or MRI suspicion of multiple sclerosis
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SARS-CoV-2-Specific Immune Responses in Patients With Postviral Syndrome After Suspected COVID-19 Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-11-01 Zachary S. Orban, Lavanya Visvabharathy, Gina S. Perez Giraldo, Millenia Jimenez, Igor J. Koralnik
Background and Objectives Millions of Americans were exposed to SARS-CoV-2 early in the pandemic but could not get diagnosed with COVID-19 due to testing limitations. Many have developed a postviral syndrome (PVS) including neurologic manifestations similar to those with postacute sequelae of SARS-CoV-2 infection (Neuro-PASC). Owing to those circumstances, proof of SARS-CoV-2 infection was not required
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Efficacy of Rituximab Outlasts B-Cell Repopulation in Multiple Sclerosis: Time to Rethink Dosing? Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-09-01 Roxane Claverie, Marine Perriguey, Audrey Rico, Clemence Boutiere, Sarah Demortiere, Pierre Durozard, Frederic Hilezian, Clea Dubrou, Frederic Vely, Jean Pelletier, Bertrand Audoin, Adil Maarouf
Background and Objectives Patients with multiple sclerosis (PwMS) receiving extended dosing of rituximab (RTX) have exhibited no return of disease activity, which suggests that maintenance of deep depletion of circulating B cells is not necessary to maintain the efficacy of RTX in MS. Methods This was a prospective monocentric observational study including all consecutive PwMS who started or continued
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Trigeminal Nerve Involvement in Bulbar-Onset Anti-IgLON5 Disease Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-11-01 Florent Cluse, Marc Hermier, Genevieve Demarquay, Veronique Rogemond, Martial Mallaret, Juliette Svahn, Antoine Pegat, Jerome Honnorat, Emilien Bernard
Objectives Anti-IgLON5 disease (IgLON5-D) may present with a bulbar-onset motor neuron disease-like phenotype, mimicking bulbar-onset amyotrophic lateral sclerosis. Recognition of their distinctive clinical and paraclinical features may help for differential diagnosis. We report 2 cases of atypical trigeminal neuropathy in bulbar-onset IgLON5-D. Methods Trigeminal nerve involvement was assessed using
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Cenobamate and Clobazam Combination as Personalized Medicine in Autoimmune-Associated Epilepsy With Anti-Gad65 Antibodies Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-11-01 Pedro J. Serrano-Castro, Juan J. Rodríguez-Uranga, Pablo Cabezudo-García, Guillermina García-Martín, Jorge Romero-Godoy, Guillermo Estivill-Torrús, Nicolás Lundahl Ciano-Petersen, Begoña Oliver, Jesús Ortega-Pinazo, Yolanda López-Moreno, Maria J. Aguilar-Castillo, Antonio L. Gutierrez-Cardo, Teresa Ramírez-García, Lorenzo Sanchez-Godoy, Mar Carreño
Background and Objectives Autoimmune-associated epilepsy (AAE) with antiglutamic acid decarboxylase 65 (GAD65) antibodies is considered a T-cell–mediated encephalitis that evolves to drug-resistant epilepsy. We do not have an effective therapeutic strategy for these patients. Because the GAD enzyme is primarily responsible for the conversion of glutamate to GABA, the mechanism of epileptogenesis in
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Electrolyte Imbalance in Anti-LGI1 Encephalitis: It Is Not All in Your Head Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-11-01 Avi Gadoth, Michal Nisnboym Ziv, Yifat Alcalay, Asia Zubkov, Idit Schwartz, Doron Schwartz, Marana Abboud, Tamar Rubinek, Ofer Yossepowitch, Talia Weinstein
Background and Objectives Antileucine-rich glioma-inactivated 1 (anti-LGI1) autoimmune encephalitis was first described in 2010 and is today the most common type of limbic encephalitis. During the course of the disease, 60%–88% of the patients develop hyponatremia. The etiology of the sodium disorder is unclear, often presumed to be the result of the syndrome of inappropriate antidiuretic hormone secretion
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Visual System Involvement in Glial Fibrillary Acidic Protein Astrocytopathy: Two Case Reports and a Systematic Literature Review Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-09-01 Giacomo Greco, Stefano Masciocchi, Luca Diamanti, Paola Bini, Elisa Vegezzi, Enrico Marchioni, Elena Colombo, Eleonora Rigoni, Pietro Businaro, Ottavia E. Ferraro, Stefania Bianchi Marzoli, Roberto Bergamaschi, Diego Franciotta, Matteo Gastaldi
Background and Objectives Glial fibrillary acidic protein (GFAP) antibodies can associate with an astrocytopathy often presenting as a meningoencephalitis. Visual involvement has been reported but scarcely defined. We describe 2 cases of GFAP astrocytopathy with predominant visual symptoms and present a systematic review of the literature. Methods We describe 2 patients with GFAP astrocytopathy from
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LGI1 Encephalitis and IgG4-Related Disease: Rare Conditions Collide Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-11-01 Aaron Jesuthasan, Tara Barwick, Luke Dixon, Sophie Molloy, Michael R. Johnson, James A.P. Tomlinson
Objectives Leucine-rich glioma-inactivated 1 (LGI1) encephalitis and IgG4-related disease (IgG4RD) have traditionally been regarded as 2 distinct disease entities. Methods We detail the presentation, investigations, and management of a patient who showed typical signs and symptoms of LGI1 encephalitis and also found to possess pancreatic changes and a serum profile in keeping with IgG4RD. Results Serum
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Dramatic Response to Anti-IL-6 Receptor Therapy in Children With Life-Threatening Myelin Oligodendrocyte Glycoprotein-Associated Disease Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-11-01 Loren A. McLendon, Claudia Gambrah-lyles, Angela Viaene, Nina A. Fainberg, Elizabeth I. Landzberg, Alexander M. Tucker, Peter J. Madsen, Jimmy Huh, Maya R. Silver, John D. Arena, Martha F. Kienzle, Brenda Banwell
Objectives Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an immune-mediated neuroinflammatory disorder leading to demyelination of the CNS. Interleukin (IL)-6 receptor blockade is under study in relapsing MOGAD as a preventative strategy, but little is known about the role of such treatment for acute MOGAD attacks. Methods We discuss the cases of a 7-year-old boy and a
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Mimics of Autoimmune Encephalitis: Validation of the 2016 Clinical Autoimmune Encephalitis Criteria Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-11-01 Robin W. Van Steenhoven, Juna M. de Vries, Arlette L. Bruijstens, Manuela Paunovic, Mariska M. Nagtzaam, Suzanne C. Franken, Anna E. Bastiaansen, Marienke A. De Bruijn, Agnes Van Sonderen, Marco W.J. Schreurs, Mayke Gardeniers, Robert M. Verdijk, Rutger K. Balvers, Peter A. Sillevis Smitt, Rinze F. Neuteboom, Maarten J. Titulaer
Background and Objectives The clinical criteria for autoimmune encephalitis (AE) were proposed by Graus et al. in 2016. In this study, the AE criteria were validated in the real world, and common AE mimics were described. In addition, criteria for probable anti-LGI1 encephalitis were proposed and validated. Methods In this retrospective cohort study, patients referred to our national referral center
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IgG1-3 MuSK Antibodies Inhibit AChR Cluster Formation, Restored by SHP2 Inhibitor, Despite Normal MuSK, DOK7, or AChR Subunit Phosphorylation Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-11-01 Michelangelo Cao, Wei-Wei Liu, Susan Maxwell, Saif Huda, Richard Webster, Amelia Evoli, David Beeson, Judith A. Cossins, Angela Vincent
Background and Objectives Up to 50% of patients with myasthenia gravis (MG) without acetylcholine receptor antibodies (AChR-Abs) have antibodies to muscle-specific kinase (MuSK). Most MuSK antibodies (MuSK-Abs) are IgG4 and inhibit agrin-induced MuSK phosphorylation, leading to impaired clustering of AChRs at the developing or mature neuromuscular junction. However, IgG1-3 MuSK-Abs also exist in MuSK-MG
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Severe Neuroinvasive West Nile Virus in Association With Anti-CD20 Monotherapy for Multiple Sclerosis Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-09-01 Simon Thebault, Stephanie Gandelman, Camryn Lane, Erin J. Kim, Caitlin Pileggi, Leah Zuroff, Luana D. Yamashita, Matthew K. Schindler, Charles Chiu, Michael R. Wilson, Joseph R. Berger, Clyde Markowitz, Amit Bar-Or, Ryan Fuller, Rachel Brandstadter, Amy A. Pruitt, Dina A. Jacobs
Objectives The objective of this study was to report on the development of neuroinvasive West Nile virus (WNV) infection in the context of anti-CD20 monotherapy for multiple sclerosis (MS). Methods This is a case series study. Results In 2021–2022, we observed 4 cases of neuroinvasive WNV infection in our patient population of 2009 patients with MS on ocrelizumab, compared with a total of 46 cases
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EBNA1 Inhibitors Block Proliferation of Spontaneous Lymphoblastoid Cell Lines From Patients With Multiple Sclerosis and Healthy Controls Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-09-01 Maria Chiara G. Monaco, Samantha S. Soldan, Chenhe Su, Annaliese Clauze, John F. Cooper, Rishi J. Patel, Fang Lu, Randall J. Hughes, Troy E. Messick, Frances C. Andrada, Joan Ohayon, Paul M. Lieberman, Steven Jacobson
Background and Objectives Epstein-Barr virus (EBV) is a ubiquitous herpesvirus that establishes lifelong latency in memory B cells and has been identified as a major risk factor of multiple sclerosis (MS). B cell depletion therapies have disease-modifying benefit in MS. However, it is unclear whether this benefit is partly attributable to the elimination of EBV+ B cells. Currently, there are no EBV-specific
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Disease Evolution in Women With Highly Active MS Who Suspended Natalizumab During Pregnancy vs Rituximab/Ocrelizumab Before Conception Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-09-01 Sarah Demortiere, Adil Maarouf, Audrey Rico, Clemence Boutiere, Frederic Hilezian, Pierre Durozard, Jean Pelletier, Bertrand Audoin
Background and Objectives In women with highly active multiple sclerosis (MS), suspending rituximab (RTX) for planning pregnancy is associated with low disease reactivation. Whether this strategy reduces the risk of disease reactivity as compared with suspending natalizumab (NTZ) 3 months after conception is unclear. Methods We retrospectively included women with MS followed in our department during
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Utility of Protein Microarrays for Detection of Classified and Novel Antibodies in Autoimmune Neurologic Disease Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-09-01 Andrew McKeon, Connie Lesnick, Nisa Vorasoot, Monica W. Buckley, Surendra Dasari, Eoin P. Flanagan, Michael Gilligan, Reghann Lafrance-Corey, Ramona Miske, Sean J. Pittock, Madeleine Scharf, Binxia Yang, Anastasia Zekeridou, Divyanshu Dubey, John Mills
Background and Objectives Neural antibodies are detected by tissue-based indirect immunofluorescence assay (IFA) in Mayo Clinic's Neuroimmunology Laboratory practice, but the process of characterizing and validating novel antibodies is lengthy. We report our assessment of human protein arrays. Methods Assessment of arrays (81% human proteome coverage) was undertaken using diverse known positive samples
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Unilateral Relapsing Primary CNS Vasculitis: Description of 3 Cases From a Single-Institutional Cohort of 216 Cases Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-09-01 Carlo Salvarani, Gene G. Hunder, Caterina Giannini, John Huston, Robert D. Brown
Objectives To define the frequency and characteristics of patients with unilateral relapsing involvement in a cohort of patients with adult primary CNS vasculitis (PCNSV). Methods We retrospectively studied a cohort of 216 patients with PCNSV seen at the Mayo Clinic, Rochester, MN from 1983 to 2022. Twenty-five patients (19.8%) had at least 2 flares. Three of them (1.4%) had unilateral relapsing vasculitis
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Structural Changes of Cutaneous Immune Cells in Patients With Type 1 Diabetes and Their Relationship With Diabetic Polyneuropathy Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-09-01 Xiaoli Hu, Christian S. Buhl, Marie B. Sjogaard, Karoline Schousboe, Hatice I. Mizrak, Huda Kufaishi, Christian S. Hansen, Knud B. Yderstræde, Troels S. Jensen, Jens R. Nyengaard, Pall Karlsson
Background and Objectives Diabetic polyneuropathy (DPN) is a complication of diabetes characterized by pain or lack of peripheral sensation, but the underlying mechanisms are not yet fully understood. Recent evidence showed increased cutaneous macrophage infiltration in patients with type 2 diabetes and painful DPN, and this study aimed to understand whether the same applies to type 1 diabetes. Methods
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Upregulation of the CD155-CD226 Axis Is Associated With Muscle Inflammation and Disease Severity in Idiopathic Inflammatory Myopathies Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-09-01 Wenli Li, Chuiwen Deng, Hanbo Yang, Xiaolan Tian, Lida Chen, Qingyan Liu, Chang Gao, Xin Lu, Guochun Wang, Qinglin Peng
Background and Objectives The CD155-CD226/T-cell Ig and immunoreceptor tyrosine-based inhibitory motif (ITIM) domain (TIGIT) pathway plays a critical role in regulating T-cell responses and is being targeted clinically. However, research on the role of this pathway in autoimmune diseases is limited. This study aimed to investigate the expression and tissue-specific roles of CD155-CD226/TIGIT pathway
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Modeling MOG Antibody-Associated Disorder and Neuromyelitis Optica Spectrum Disorder in Animal Models: Visual System Manifestations Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-09-01 Jana Remlinger, Maud Bagnoud, Ivo Meli, Marine Massy, Robert Hoepner, Christopher Linington, Andrew Chan, Jeffrey L. Bennett, Volker Enzmann, Anke Salmen
Background and Objectives Mechanisms of visual impairment in aquaporin 4 antibody (AQP4-IgG) seropositive neuromyelitis optica spectrum disorder (NMOSD) and myelin oligodendrocyte glycoprotein antibody (MOG-IgG)–associated disorder (MOGAD) are incompletely understood. The respective impact of optic nerve demyelination and primary and secondary retinal neurodegeneration are yet to be investigated in
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Immune Repertoire Profiling Reveals Its Clinical Application Potential and Triggers for Neuromyelitis Optica Spectrum Disorders Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-09-01 Yu Miao, Ziyan Shi, Wei Zhang, Lin Zhu, Shanshan Tang, Hongxi Chen, Xiaofei Wang, Qin Du, Shuaicheng Li, Ying Zhang, Wenqin Luo, Xin Jin, Mingyan Fang, Hongyu Zhou
Background and Objectives Neuromyelitis optica spectrum disorders (NMOSD) is widely recognized as a CNS demyelinating disease associated with AQP4-IgG (T cell–dependent antibody), and its trigger is still unclear. In addition, although the treatment of NMOSD currently can rely on traditional immunosuppressive and modulating agents, effective methods to predict the efficacy of these therapeutics are
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Synthetic Cell-Based Immunotherapies for Neurologic Diseases Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-09-01 Louisa von Baumgarten, Hans J. Stauss, Jan D. Lünemann
The therapeutic success and widespread approval of genetically engineered T cells for a variety of hematologic malignancies spurred the development of synthetic cell-based immunotherapies for CNS lymphoma, primary brain tumors, and a growing spectrum of nononcologic disease conditions of the nervous system. Chimeric antigen receptor effector T cells bear the potential to deplete target cells with higher
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Adoptive Allogeneic T-Cell Therapy Improves the Clinical Outcome of JC Virus Granule Cell Neuronopathy: A Case Report Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-09-01 Lea Grote-Levi, Nora Möhn, Agnes Bonifacius, Sabine Tischer-Zimmermann, Finja Schweitzer, Nima Mahmoudi, Steffi Silling, Clemens Warnke, Britta Maecker-Kolhoff, Mike P. Wattjes, Britta Eiz-Vesper, Günter U. Höglinger, Thomas Skripuletz
Objectives JC virus granule cell neuronopathy is a potentially fatal otherwise highly disabling disease without an approved therapeutic option. This case report presents the positive record to T-cell therapy in JC virus granule cell neuronopathy. Methods The patient represented with subacute cerebellar symptoms. Diagnosis of JC virus granule cell neuronopathy was made because of infratentorially accentuated
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Intrathecal CD8+CD20+ T Cells in Primary Progressive Multiple Sclerosis Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-09-01 Marina Rode von Essen, Jacob Talbot, Rikke Holm Holm Hansen, Helene Højsgaard Chow, Henrik Lundell, Hartwig Roman Siebner, Finn Sellebjerg
Background and Objective Despite accumulating evidence of intrathecal inflammation in patients with primary progressive multiple sclerosis (PPMS), immunomodulatory and suppressive treatment strategies have proven unsuccessful. With this study, we investigated the involvement of CD20+ T cells and the effect of dimethyl fumarate on CD20+ T cells in PPMS. Methods The main outcomes in this observational
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TSPO-Detectable Chronic Active Lesions Predict Disease Progression in Multiple Sclerosis Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-09-01 Eero Polvinen, Markus Matilainen, Marjo Nylund, Marcus Sucksdorff, Laura M. Airas
Background and Objectives In the multiple sclerosis (MS) brain, chronic active lesions can be detected using MRI- and PET-based methods. In this study, we investigated whether the frequency of TSPO-PET–detectable chronic active lesions associates with disease progression measured using the Expanded Disability Status Scale (EDSS) at 5-year follow-up. Methods Chronic lesion–associated innate immune cell
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Complement Factor I Gene Variant as a Treatable Cause of Recurrent Aseptic Neutrophilic Meningitis: A Case Report Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-09-01 Mary Rolfes, Adil Harroud, Kelsey C. Zorn, Asritha Tubati, Charles Omura, Kenneth Kurtz, Mehrdad Matloubian, Amy Berger, Charles Y. Chiu, Michael R. Wilson, Prashanth S. Ramachandran
Mutations in the complement factor I (CFI) gene have previously been identified as causes of recurrent CNS inflammation. We present a case of a 26-year-old man with 18 episodes of recurrent meningitis, who had a variant in CFI(c.859G>A,p.Gly287Arg) not previously associated with neurologic manifestations. He achieved remission with canakinumab, a human monoclonal antibody targeted at interleukin-1
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Frequency and Referral Patterns of Neural Antibody Studies During the COVID-19 Pandemic: Experience From an Autoimmune Neurology Center Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-07-01 Helena Ariño, Raquel Ruiz García, Beatriz Rioseras, Laura Naranjo, Eugenia Martinez-Hernandez, Albert Saiz, Francesc Graus, Josep Dalmau
Objective To determine whether the frequency of paraneoplastic or autoimmune encephalitis antibodies examined in a referral center changed during the COVID-19 pandemic. Methods The number of patients who tested positive for neuronal or glial (neural) antibodies during pre–COVID-19 (2017–2019) and COVID-19 (2020–2021) periods was compared. The techniques used for antibody testing did not change during
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Subacute Horizontal Diplopia, Jaw Dystonia, and Laryngospasm Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-07-01 Nanthaya Tisavipat, Bryce K. Chang, Farwa Ali, Sean J. Pittock, Ryan Kammeyer, Anthony Declusin, Samuel J. Cohn, Eoin P. Flanagan
Jaw dystonia and laryngospasm in the context of subacute brainstem dysfunction have been described in a small number of diseases, including antineuronal nuclear antibody type 2 (ANNA-2, also known as anti-Ri) paraneoplastic neurologic syndrome. Severe episodes of laryngospasms causing cyanosis are potentially fatal. Jaw dystonia can also cause eating difficulty, resulting in severe weight loss and
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Antibodies Associated With Autoimmune Encephalitis in Patients With Presumed Neurodegenerative Dementia Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-09-01 Anna E.M. Bastiaansen, Robin W. van Steenhoven, Esmee S. te Vaarwerk, Wiesje M. van der Flier, Charlotte Teunissen, Esther de Graaff, Mariska M.P. Nagtzaam, Manuela Paunovic, Suzanne C. Franken, Marco W.J. Schreurs, Frank Leypoldt, Peter A.E. Smitt, Juna M. de Vries, Harro Seelaar, John van Swieten, Frank Jan de Jong, Yolande A.L. Pijnenburg, Maarten J. Titulaer
Background & Objectives Autoimmune encephalitis (AIE) may present with prominent cognitive disturbances without overt inflammatory changes in MRI and CSF. Identification of these neurodegenerative dementia diagnosis mimics is important because patients generally respond to immunotherapy. The objective of this study was to determine the frequency of neuronal antibodies in patients with presumed neurodegenerative
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CSF Concentrations of CXCL13 and sCD27 Before and After Autologous Hematopoietic Stem Cell Transplantation for Multiple Sclerosis Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-09-01 Katarina Lundblad, Christina Zjukovskaja, Anders Larsson, Honar Cherif, Kim Kultima, Joachim Burman
Background and Objectives In the past decade, autologous hematopoietic stem cell transplantation (AHSCT) has emerged as a treatment for relapsing-remitting multiple sclerosis (RRMS). How this procedure affects biomarkers of B- and T-cell activation is currently unknown. The objective of this study was to investigate CXCL13 and sCD27 concentrations in CSF before and after AHSCT. Methods This prospective
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Inflammatory Biomarkers in Newly Diagnosed Patients With Parkinson Disease and Related Neurodegenerative Disorders Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-07-01 Camilla Christina Pedersen, Anastasia Ushakova, Ragnhild Eide Skogseth, Guido Alves, Ole-Bjørn Tysnes, Dag Aarsland, Johannes Lange, Jodi Maple-Grødem
Background and Objectives Neuroinflammation contributes to Parkinson disease (PD) pathology, and inflammatory biomarkers may aid in PD diagnosis. Proximity extension assay (PEA) technology is a promising method for multiplex analysis of inflammatory markers. Neuroinflammation also plays a role in related neurodegenerative diseases, such as dementia with Lewy bodies (DLB) and Alzheimer disease (AD)
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International Delphi Consensus on the Management of AQP4-IgG+ NMOSD: Recommendations for Eculizumab, Inebilizumab, and Satralizumab Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-07-01 Friedemann Paul, Romain Marignier, Jacqueline Palace, Georgina Arrambide, Nasrin Asgari, Jeffrey L. Bennett, Bruce Anthony Campbell Cree, Jérôme De Sèze, Kazuo Fujihara, Ho Jin Kim, Rebecca Hornby, Saif Huda, Najib Kissani, Ingo Kleiter, Satoshi Kuwabara, Marco Lana-Peixoto, Lisa Law, M. Isabel Leite, Lekha Pandit, Sean J. Pittock, Chao Quan, Sudarshini Ramanathan, Dalia Rotstein, Albert Saiz, Douglas
Background and Objectives Neuromyelitis optica spectrum disorder (NMOSD) is a rare debilitating autoimmune disease of the CNS. Three monoclonal antibodies were recently approved as maintenance therapies for aquaporin-4 immunoglobulin G (AQP4-IgG)–seropositive NMOSD (eculizumab, inebilizumab, and satralizumab), prompting the need to consider best practice therapeutic decision-making for this indication
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MRI Features and Their Association With Outcomes in Children With Anti-NMDA Receptor Encephalitis Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-07-01 Grace Gombolay, J. Nicholas Brenton, Jennifer H. Yang, Coral M. Stredny, Ryan Kammeyer, Catherine E. Otten, NgocHanh Vu, Jonathan D. Santoro, Karla Robles-Lopez, Andrew Christiana, Claude Steriade, Morgan Morris, Mark Gorman, Manikum Moodley, Duriel Hardy, Alexandra B. Kornbluh, Ilana Kahn, Leigh N. Sepeta, Anusha Yeshokumar, and the Conquering Neuroinflammation and Epilepsies Consortium (CONNECT)
Objectives How brain MRI lesions associate with outcomes in pediatric anti-NMDA receptor encephalitis (pNMDARE) is unknown. In this study, we correlate T2-hyperintense MRI brain lesions with clinical outcomes in pNMDARE. Methods This was a multicenter retrospective cohort study from 11 institutions. Children younger than 18 years with pNMDARE were included. One-year outcomes were assessed by the modified
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Immunohistologic Features of Cerebral Venous Thrombosis Due to Vaccine-Induced Immune Thrombotic Thrombocytopenia Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-07-01 Thomas Geeraerts, Céline Guilbeau-Frugier, Cédric Garcia, Vincent Memier, Nicolas Raposo, Fabrice Bonneville, Céline Gales, Jean Darcourt, Sophie Voisin, Agnès Ribes, Marie Piel-Julian, Fanny Bounes, Jean François Albucher, Franck-Emmanuel Roux, Jacques Izopet, Norbert Telmon, Jean Marc Olivot, Pierre Sié, Jan Bauer, Bernard Payrastre, Roland S. Liblau
Objectives Vaccine-induced immune thrombotic thrombocytopenia (VITT), a recently described entity characterized by thrombosis at unusual locations such as cerebral venous sinus and splanchnic vein, has been rarely described after adenoviral-encoded COVID-19 vaccines. In this study, we report the immunohistological correlates in 3 fatal cases of cerebral venous thrombosis related to VITT analyzed at
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Late-Onset Anti-GABAB Receptor Encephalitis: Clinical Characteristics and Outcomes Differing From Early-Onset Patients Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-07-01 Tangna Sun, Daidi Zhao, Gejuan Zhang, Yue Huang, Jia Guo, Wen Jiang, Rui Jia, Maynur Maimaiti, Jianguo Liu, Ning Bu, Zunbo Li, Yaping Yan, Xiaoyan Zhang, Chenjing Sun, Cong Zhao, Xiaotao Jia, Baoyi Mao, Hui Tian, Yan Liu, Zheng Chen, Zilian Fan, Xiaoyan Guo, Jiarui Lu, Kaixi Ren, Hongzeng Li, Jun Guo
Background and Objectives Existing evidence indicates anti-GABAB receptor encephalitis (GABABR-E) seems to occur more commonly later in life, yet the age-associated differences in clinical features and outcomes are not well determined. This study aims to explore the demographic, clinical characteristics, and prognostic differences between late-onset and early-onset GABABR-E and identify predictors
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Mesiotemporal Volumetry, Cortical Thickness, and Neuropsychological Deficits in the Long-term Course of Limbic Encephalitis Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-07-01 Antonia Harms, Tobias Bauer, Juri-Alexander Witt, Tobias Baumgartner, Randi von Wrede, Attila Racz, Leon Ernst, Albert J. Becker, Christoph Helmstaedter, Rainer Surges, Theodor Rüber
Background and Objectives Limbic encephalitis (LE) is an autoimmune disease often associated with temporal lobe epilepsy and subacute memory deficits. It is categorized into serologic subgroups, which differ in clinical progress, therapy response, and prognosis. Using longitudinal MRI analysis, we hypothesized that mesiotemporal and cortical atrophy rates would reveal serotype-specific patterns and
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Acknowledgment to Reviewers Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-07-01 Josep O. Dalmau
The success of Neurology® Neuroimmunology & Neuroinflammation derives from the publication of excellent manuscripts which requires the time and effort of our reviewers. Their thoughtful comments on clinical trials, experimental research, and novel methodologies or techniques are critical to that success. We appreciate their dedication to the journal and their commitment to excellence.
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Anti-LGI1 Encephalitis With Co-occurring IgLON5 Antibodies: Clinical Features and Human Leukocyte Antigen Haplotypes Neurol. Neuroimmunol. Neuroinflamm. (IF 8.8) Pub Date : 2023-07-01 Pierre Schiff, Sergio Muñiz-Castrillo, Le Duy Do, Maria Livia Fantini, Eve Chanson, Veronique Rogemond, Jerome Honnorat, Louis Poncet-Megemont
Objectives Autoimmune encephalitis (AE) with antibodies against LGI1 and IgLON5 are clinically distinctive but share some particularities such as a strong association with specific human leukocyte antigen (HLA) class II alleles. Methods We clinically describe a patient with double positivity for LGI1 and IgLON5 antibodies. In addition, we conducted specific immunodepletion with the patient's serum