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
Neurosciences
Timo Kirschstein, Ruediger Koehling
Summary: Autoimmune-mediated encephalitis syndromes are important clinical entities that can present with various neurological symptoms. Antibody identification and early diagnosis are crucial to prevent brain tissue damage. This review focuses on the current understanding of antibody interaction mechanisms in the central nervous system and discusses the challenges in explaining hyperexcitability, seizures, and cognitive dysfunction.
NEUROBIOLOGY OF DISEASE
(2023)
Article
Immunology
Dong Li, Teng Huang, Fangyuan Zhang, Xiaoyu Zhang, Jingjing Dou, Chunjuan Wang, Shougang Guo
Summary: Objective: To compare the efficacy and safety of different-course corticosteroids plus mycophenolate mofetil (MMF) as maintenance therapy in autoimmune encephalitis (AE) without tumor. Results: The beneficial effects of oral corticosteroid treatment may not persist beyond 12 months and may even contribute to an increased incidence of adverse effects. Conclusion: To optimize treatment effectiveness and safety, a corticosteroid course of 3-12 months is recommended. Patients with reduced levels of consciousness may be more inclined to choose longer courses of corticosteroids for long-term treatment.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Immunology
Shan Qiao, Shan-Chao Zhang, Zhi-Hao Wang, Lei Wang, Ran-Ran Zhang, Hai-Yun Li, Yang Jin, Ling-Ling Liu, Mei-Ling Wang, Ai-Hua Wang, Xue-Wu Liu
Summary: This study investigated the clinical characteristics and significance of overlapping multiple anti-neuronal antibodies in patients with autoimmune encephalitis (AE). Among the 276 AE antibody-positive patients, 7.97% had two or more antibodies. The main symptoms of patients with coexisting antibodies included fever, seizures, memory impairment, cognitive decline, and sleep disorders. The study also found that the presence of multiple antibodies may indicate an underlying malignancy.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Cell Biology
Susann Ludewig, Leonie Salzburger, Alexander Goihl, Jana Rohne, Frank Leypoldt, Daniel Bittner, Emrah Duezel, Burkhart Schraven, Dirk Reinhold, Martin Korte, Peter Koertvelyessy
Summary: Autoimmune encephalitis (AE) associated with autoantibodies against leucine-rich glioma-inactivated protein-1 (LGI1) can present with faciobrachial dystonic seizures (FBDS) and/or limbic encephalitis (LE). The reasons for this heterogeneity in phenotypes are unclear. The study characterized the autoantibodies from patients with LE and FBDS, identified distinct epitope patterns, and found that LE patients' antibodies had more significant effects on hippocampal neurons' function and spine density compared to FBDS patients' antibodies.
Article
Immunology
Luigi Zuliani, Sabrina Marangoni, Piera De Gaspari, Irene Rosellini, Margherita Nosadini, Joanne M. Fleming, Valentina De Riva, Elisabetta Galloni, Francesco Perini, Anna Chiara Frigo, Stefano Sartori, Marco Zoccarato, Bruno Giometto
Summary: This study reviewed 13 years of biobank data to assess the incidence of NSAE in two Italian provinces over a 5-year period, finding an estimated rate of 1.54 per 1,000,000 population. LGI1-E was the most common NSAE among adults.
JOURNAL OF NEUROIMMUNOLOGY
(2021)
Review
Biochemistry & Molecular Biology
Maria Pia Giannoccaro, Federico Verde, Luana Morelli, Giovanni Rizzo, Fortuna Ricciardiello, Rocco Liguori
Summary: Autoimmune encephalitis and neurodegenerative disorders share clinical features and antibodies against neuronal proteins may occur in both types of diseases. This article reviews the link between these antibodies and neurodegeneration, exploring the possibility of autoimmune encephalitis presenting as a neurodegenerative disease and the role of antibodies in the disease course. The long-term cognitive and neuroradiological changes associated with autoimmune encephalitis are also discussed, along with potential biomarkers for predicting cognitive outcome.
Article
Pharmacology & Pharmacy
Sara Merlo, Grazia Ilaria Caruso, Laura Bonfili, Simona Federica Spampinato, Giuseppe Costantino, Anna Maria Eleuteri, Maria Angela Sortino
Summary: This study explored the effects of melatonin on microglial cells in Alzheimer's disease and found that melatonin can inhibit inflammation, restore the expression of beneficial genes, and protect neuronal cells from damage caused by beta-amyloid.
BIOCHEMICAL PHARMACOLOGY
(2022)
Article
Psychiatry
Lidia Hau, Tamas Tenyi, Natalia Laszlo, Marton Aron Kovacs, Szabina Erdoe-Bonyar, Zsuzsanna Csizmadia, Timea Berki, Diana Simon, Gyoergyi Csabi
Summary: This study examined the presence of anti-neuronal autoantibodies in synthetic cannabinoid users presenting with psychosis and found a small number of positive or borderline cases. However, there was no significant correlation between these autoantibodies and the severity of psychiatric symptoms. The alteration of anti-CS autoantibody levels may indicate immunological dysfunctions in these cases.
FRONTIERS IN PSYCHIATRY
(2022)
Article
Biochemistry & Molecular Biology
Matthew J. Sticco, Perla A. Pena Palomino, David Lukacsovich, Brianna L. Thompson, Csaba Foeldy, Susanne Ressl, David C. Martinelli
Summary: Synapses are the key structures through which neurons communicate, regulated by a variety of proteins. C1QL proteins, part of the C1q superfamily, act as synaptic organizers involved in crucial neuronal processes. The study shows that C1QL3 mediates a novel cell-cell adhesion complex involving ADGRB3 and two neuronal pentraxins, suggesting the formation of a new trans-synaptic adhesion complex.
Article
Biochemistry & Molecular Biology
Pedro Valada, Sofia Alcada-Morais, Rodrigo A. Cunha, Joao Pedro Lopes
Summary: Theobromine, a metabolite of caffeine, affects synaptic transmission and plasticity by antagonizing adenosine receptors, resulting in improved cognitive function and neuroprotection against disease-related conditions.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Neurosciences
Nadine Huber, Dorit Hoffmann, Raisa Giniatullina, Hannah Rostalski, Stina Leskela, Mari Takalo, Teemu Natunen, Eino Solje, Anne M. Remes, Rashid Giniatullin, Mikko Hiltunen, Annakaisa Haapasalo
Summary: Frontotemporal lobar degeneration (FTLD) is a progressive neurodegenerative disease with no validated biomarkers or effective treatments. The pathological C9orf72 gene expansion in FTLD leads to synaptic dysfunction and morphological changes in dendritic spines, increasing the susceptibility of neurons to glutamate-induced excitotoxicity.
NEUROBIOLOGY OF DISEASE
(2022)
Article
Biochemistry & Molecular Biology
Vilmos Toth, Henrietta Vadaszi, Lilla Ravasz, Daniel Mittli, Dominik Matyas, Tamas Molnar, Andras Micsonai, Tamas Szaniszlo, Reka A. Kovacs, Tunde Juhasz, Tamas Beke-Somfai, Gabor Juhasz, Balazs Andras Gyorffy, Katalin A. Kekesi, Jozsef Kardos, Peter Lorincz
Summary: This study found that neuronal-specific septin-3 is associated with synaptic/neuronal autophagy, can bind autophagy protein Atg8, and localize to autophagic membranes. The elevated levels of septin-3 may indicate enhanced or impeded autophagy in neurons.
CELLULAR AND MOLECULAR LIFE SCIENCES
(2022)
Article
Biochemistry & Molecular Biology
Ana Catarina Ferreira, Brittany M. Hemmer, Sarah M. Philippi, Alejandro B. Grau-Perales, Jacob L. Rosenstadt, Hanxiao Liu, Jeffrey D. Zhu, Tatyana Kareva, Tim Ahfeldt, Merina Varghese, Patrick R. Hof, Joseph M. Castellano
Summary: The functional output of the hippocampus, which supports memory function, is regulated by cellular and molecular processes. This study reveals that TIMP2, a molecule highly expressed in the hippocampus, plays a role in cellular programs related to neurogenesis and dendritic spine turnover. Its loss leads to impairments in hippocampus-dependent memory. This research provides insights into the regulation of synaptic plasticity by TIMP2.
MOLECULAR PSYCHIATRY
(2023)
Article
Multidisciplinary Sciences
Elodie Fels, Marie-Eve Mayeur, Estelle Wayere, Clementine Vincent, Celine Malleval, Jerome Honnorat, Olivier Pascual
Summary: LGI1, a neuronal secreted protein highly expressed in the hippocampus, plays an essential role in regulating the balance between inhibition and excitation in the neuronal network. The altered function of LGI1 could lead to epileptic seizures. In this study, mice were infused with LGI1 IgG to investigate its effect on AMPA-receptor expression and network excitability. The findings suggest that LGI1 IgG reduces AMPA-R expression, increases network hyperexcitability, and more significantly impairs the inhibitory network activity, shedding light on the crucial role of the inhibitory network in triggering epileptic seizures in LGI1 LE patients.
Editorial Material
Clinical Neurology
Francesc Graus, Josep Dalmau
Editorial Material
Clinical Neurology
Josep Dalmau, Francesc Graus
Editorial Material
Clinical Neurology
Amy Kunchok, Josep Dalmau
Summary: Anti-NMDA receptor encephalitis is characterized by behavioral, cognitive, and psychiatric alterations, along with seizures, abnormal movements, dysautonomia, or decreased consciousness. These symptoms develop sequentially over several weeks in association with NMDAR-IgG in cerebrospinal fluid. After the initial presentation, persistent symptoms may last for weeks or months and often require ICU care, followed by a slow recovery. Changes in CSF antibody titers may correlate with relapse, but serum titers are unreliable biomarkers of disease activity.
Article
Clinical Neurology
Mar Guasp, Josep Dalmau
Summary: In recent years, there have been numerous reports on individual symptoms of autoimmune encephalitis (AE) in neurology and psychiatry journals. However, the presence of antibodies in psychiatric diseases has not defined any specific disorder or affected the diagnosis and treatment of patients. These studies often lack rigorous investigations and fail to address the clinical significance and limitations of antibody testing techniques. Clinical judgment is emphasized as important in interpreting antibody findings.
Article
Immunology
Jon Landa, Ana Beatriz Serafim, Carles Gaig, Albert Saiz, Inga Koneczny, Romana Hoftberger, Joan Santamaria, Josep Dalmau, Francesc Graus, Lidia Sabater
Summary: This study explores the interactome of IgLON5 and demonstrates that IgLON5 antibodies can interfere with its protein interactions. The findings reveal a potentially novel pathogenic mechanism of IgLON5 antibodies in blocking its interactions with binding partners, extending our knowledge about the physiological role of IgLON5 and contributing to the understanding of the pathological mechanisms of anti-IgLON5 disease.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Immunology
Cristina Arnaldos-Perez, Andreu Vilaseca, Laura Naranjo, Lidia Sabater, Josep Dalmau, Raquel Ruiz-Garcia, Francesc Graus
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Clinical Neurology
Susanna Asseyer, Nasrin Asgari, Jeffrey Bennett, Omer Bialer, Yolanda Blanco, Francesca Bosello., Anna Camos-Carreras., Edgar Carnero Contentti., Sara Carta, John Chen, Claudia Chien, Mashina Chomba, Russell C. Dale, Josep Dalmau, Kristina Feldmann, Eoin P. Flanagan, Caroline Froment Tilikete, Carolina Garcia-Alfonso, Joachim Havla, Mark Hellmann, Ho Jin Kim, Philipp Klyscz, Frank Konietschke, Chiara La Morgia, Marco Lana-Peixoto, Maria Isabel Leite, Netta Levin, Michael Levy, Sara Llufriu, Pablo Lopez, Itay Lotan, Alessandra Lugaresi, Romain Marignier, Sara Mariotto, Susan P. Mollan, Cassandra Ocampo, Frederike Cosima Oertel, Maja Olszewska, Jacqueline Palace, Lekha Pandit, Jose Luis Peralta Uribe, Sean Pittock, Sudarshini Ramanathan, Natthapon Rattanathamsakul, Albert Saiz, Sara Samadzadeh, Bernardo Sanchez-Dalmau, Deanna Saylor, Michael Scheel, Tanja Schmitz-Huebsch, Jemal Shifa, Sasitorn Siritho, Pia S. Sperber, Prem S. Subramanian, Alon Tiosano, Adi Vaknin-Dembinsky, Alvaro Jose Mejia Vergara, Adi Wilf-Yarkoni, Luis Alfonso Zarco, Hanna G. Zimmermann, Friedemann Paul, Hadas Stiebel-Kalish
Summary: This is a global study aimed at investigating the effect of early high-dose corticosteroid treatment on optic neuritis. The study will collect various data to evaluate treatment strategies and the accuracy of diagnostic criteria.
FRONTIERS IN NEUROLOGY
(2023)
Article
Clinical Neurology
Thais Armangue, Gemma Olive-Cirera, Eugenia Martinez-Hernandez, Maria Rodes, Vicente Peris-Sempere, Mar Guasp, Raquel Ruiz, Eduard Palou, Azucena Gonzalez, Ma angeles Marcos, Maria Elena Erro, Luis Bataller, Inigo Corral-Corral, Jesus Planaguma, Eva Caballero, Alexandru Vlagea, Jie Chen, Paul Bastard, Marie Materna, Astrid Marchal, Laurent Abel, Aurelie Cobat, Laia Alsina, Claudia Fortuny, Albert Saiz, Emmanuel Mignot, Adeline Vanderver, Jean-Laurent Casanova, Shen-Ying Zhang, Josep Dalmau, Spanish HSE Study Grp
Summary: Patients with herpes simplex virus encephalitis often develop neuronal autoantibody-associated encephalitis post-infection. This study found that risk factors for post-HSE AE may include genetic variants, HLA haplotypes, and the blood innate immune response against HSV. These findings have potential clinical applications for the diagnosis and monitoring of HSE complications.
Correction
Immunology
Jon Landa, Ana Beatriz Serafim, Carles Gaig, Albert Saiz, Inga Koneczny, Romana Hoftberger, Joan Santamaria, Josep Dalmau, Francesc Graus, Lidia Sabater
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Clinical Neurology
Josep Dalmau, Francesc Graus
Summary: Increased awareness of autoimmune encephalitis has led to misdiagnoses and inappropriate use of diagnostic criteria for antibody-negative disease. Misdiagnoses occur due to non-adherence to clinical requirements, inadequate assessment, and limited use of assays. Clinicians should adhere to published criteria and substantiate absence of neural antibodies. Accurate diagnosis will help assess treatment response and outcomes.
Article
Clinical Neurology
Mar Guasp, Mireia Rosa-Justicia, Amaia Munoz-Lopetegi, Eugenia Martinez-Hernandez, Thais Armangue, Gisela Sugranyes, Heike Stein, Roger Borras, Laia Prades, Helena Arino, Jesus Planaguma, Elena De-La-Serna, Domingo Escudero, Sara Llufriu, Raquel Sanchez-Valle, Joan Santamaria, Albert Compte, Josefina Castro-Fornielest, Josep Dalmau
Summary: The study described the clinical features of NMDA receptor encephalitis in the post-acute stage, found similarities with schizophrenia spectrum disorders, identified factors predicting cognitive-psychiatric outcomes, and explored their potential as prognostic biomarkers.
Meeting Abstract
Clinical Neurology
E. Fonseca Perez, G. Olive-Cirera, C. del-Prado-Sanchez, G. Romeu, S. Llufriu, J. M. Cabrera-Maqueda, E. Martinez-Hernandez, M. Sepulveda, M. San Miguel, Y. Blanco, A. Saiz, T. Armangue
MULTIPLE SCLEROSIS JOURNAL
(2022)
Meeting Abstract
Clinical Neurology
J. L. Chico Garcia, M. Morales De la Prida, J. M. Cabrera-Maqueda, F. Rodriguez-Jorge, R. Sainz-Amo, S. Sainz De la Maza, E. Monreal, G. Valero-Lopez, J. Millan-Pascual, M. Sepulveda, E. Solana, E. Martinez-Heras, E. Lopez-Soley, E. Fonseca, E. Martinez-Hernandez, L. Costa-Frossard, J. E. Meca-Lallana, Y. Blanco, A. Saiz, S. Llufriu
MULTIPLE SCLEROSIS JOURNAL
(2022)
Article
Clinical Neurology
Diana Esteller, Antonio Doncel-Moriano, Marfa Claro, Laia Tardon, Judith Navarro-Otano, Eugenia Martinez-Hernandez, Maria Suarez-Lledo, Aida Alejaldre
Summary: Neuroleukemia is a rare disorder of the peripheral nervous system caused by leukemic cell infiltration. This case report describes a patient with a history of acute myelomonoblastic leukemia in remission who presented with progressive paresis of multiple nerves. The diagnosis was confirmed by electromyogram and PET-CT, and the patient showed improvement with chemotherapy.
REVISTA DE NEUROLOGIA
(2022)
Meeting Abstract
Clinical Neurology
M. Guasp, L. Martin-Aguilar, L. Sabater, M. Bioque, T. Armangue, E. Martinez-Hernandez, J. Landa, E. Maudes, A. Saiz, J. Castro-Fornieles, F. Graus, E. Parellada, L. Querol, J. Dalmau
EUROPEAN JOURNAL OF NEUROLOGY
(2022)