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Biomedicines
Special Issues
Molecular Epidemiology and Pathophysiology of Autoimmune Encephalitis
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Molecular Epidemiology and Pathophysiology of Autoimmune Encephalitis

A special issue of Biomedicines (ISSN 2227-9059). This special issue belongs to the section "Neurobiology and Clinical Neuroscience".

Deadline for manuscript submissions: closed (15 July 2022) | Viewed by 26472

Special Issue Editors

Dr. Agusti Alentorn

E-Mail Website
Guest Editor
Department of Neurology 2, Groupe Hospitalier Pitié Salpêtrière, AP-HP, Paris, France
Interests: neuro-oncology; brain tumors; cancer genomics; primary central nervous system lymphoma; bioinformatics
Dr. Dimitri Psimaras

E-Mail Website
Guest Editor
Department of Neurology 2, Groupe Hospitalier Pitié Salpêtrière, AP-HP, Paris, France
Interests: autoimmune encephalitis; neurotoxicity; neurologic paraneoplastic syndrome; peripheral neuropathy; peripheral neurotoxicity
Dr. Bastien Joubert

E-Mail Website
Guest Editor
1.French Reference Centre for Paraneoplastic Neurological Syndromes, Lyon Neurological Hospital, Lyon, France
2.Institut NeuroMyoGene, Université Claude Bernard Lyon 1, Lyon, France
Interests: acute cerebellitis; anti-GAD antibodies; autoimmune cerebellar ataxia; opsoclonus-myoclonus syndrome; neurologic paraneoplastic syndrome

Special Issue Information

Dear Colleagues,

Autoimmune encephalitis encompasses a broad range of diseases with different pathophysiology. Largely due to their rarity and heterogenous nature, these encephalitises are challenging to treat, and frequently recur. Over the last decade, significant progress has been made, providing a better understanding of their physiopathology.

The aim of this Special Issue is to provide a broad overview of the molecular epidemiology and pathophysiological mechanisms that drive autoimmune encephalitis. Overall, the goal is to highlight progress made in preclinical and translational research to improve our understanding of autoimmune encephalitis. We invite both original research and review articles highlighting recent advances in the field of autoimmune encephalitis.

Dr. Agusti Alentorn
Dr. Dimitri Psimaras
Dr. Bastien Joubert
Guest Editors

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Biomedicines is an international peer-reviewed open access monthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2600 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • autoimmune encephalitis
  • neuronal surface antibody
  • antineuronal nuclear antibody
  • paraneoplastic syndrome
  • limbic encephalitis
  • encephalomyelitis

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Published Papers (5 papers)

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Research

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16 pages, 1089 KiB  
Article
Brain Metabolic Alterations in Seropositive Autoimmune Encephalitis: An 18F-FDG PET Study
by Sébastien Bergeret, Cristina Birzu, Pierre Meneret, Alain Giron, Sophie Demeret, Clemence Marois, Louis Cousyn, Laura Rozenblum, Alice Laurenge, Agusti Alentorn, Vincent Navarro, Dimitri Psimaras and Aurélie Kas
Biomedicines 2023, 11(2), 506; https://doi.org/10.3390/biomedicines11020506 - 9 Feb 2023
Cited by 3 | Viewed by 2495
Abstract
Introduction: Autoimmune encephalitis (AE) diagnosis and follow-up remain challenging. Brain 18F-fluoro-deoxy-glucose positron emission tomography (FDG PET) has shown promising results in AE. Our aim was to investigate FDG PET alterations in AE, according to antibody subtype. Methods: We retrospectively included patients with [...] Read more.
Introduction: Autoimmune encephalitis (AE) diagnosis and follow-up remain challenging. Brain 18F-fluoro-deoxy-glucose positron emission tomography (FDG PET) has shown promising results in AE. Our aim was to investigate FDG PET alterations in AE, according to antibody subtype. Methods: We retrospectively included patients with available FDG PET and seropositive AE diagnosed in our center between 2015 and 2020. Brain PET Z-score maps (relative to age matched controls) were analyzed, considering metabolic changes significant if |Z-score| ≥ 2. Results: Forty-six patients were included (49.4 yrs [18; 81]): 13 with GAD autoantibodies, 11 with anti-LGI1, 9 with NMDAR, 5 with CASPR2, and 8 with other antibodies. Brain PET was abnormal in 98% of patients versus 53% for MRI. The most frequent abnormalities were medial temporal lobe (MTL) and/or striatum hypermetabolism (52% and 43% respectively), cortical hypometabolism (78%), and cerebellum abnormalities (70%). LGI1 AE tended to have more frequent MTL hypermetabolism. NMDAR AE was prone to widespread cortical hypometabolism. Fewer abnormalities were observed in GAD AE. Striatum hypermetabolism was more frequent in patients treated for less than 1 month (p = 0.014), suggesting a relation to disease activity. Conclusion: FDG PET could serve as an imaging biomarker for early diagnosis and follow-up in AE. Full article
(This article belongs to the Special Issue Molecular Epidemiology and Pathophysiology of Autoimmune Encephalitis)
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14 pages, 1597 KiB  
Article
Neuronal and Neuroaxonal Damage Cerebrospinal Fluid Biomarkers in Autoimmune Encephalitis Associated or Not with the Presence of Tumor
by Aigli G. Vakrakou, John S. Tzartos, Eleni Strataki, Fotini Boufidou, Eleni Dimou, Efstratios-Stylianos Pyrgelis, Vasilios C. Constantinides, George P. Paraskevas and Elisabeth Kapaki
Biomedicines 2022, 10(6), 1262; https://doi.org/10.3390/biomedicines10061262 - 28 May 2022
Cited by 4 | Viewed by 2683
Abstract
The aim of this study was to evaluate the association of neuronal damage biomarkers (neurofilament light chain (NFL) and total tau protein (T-tau)) in the CSF of patients with autoimmune encephalitis (AE) with the presence of an underlying malignancy and to determine correlations [...] Read more.
The aim of this study was to evaluate the association of neuronal damage biomarkers (neurofilament light chain (NFL) and total tau protein (T-tau)) in the CSF of patients with autoimmune encephalitis (AE) with the presence of an underlying malignancy and to determine correlations with patient characteristics. The study comprised 21 patients with encephalitis associated with antibodies against intracellular (n = 11) and surface/synaptic antigens (extracellular, n = 10) and non-inflammatory disease controls (n = 10). Patients with AE associated with intracellular antigens had increased CSF-NFL (p = 0.003) but not T-tau levels compared to controls. When adjusted for age, CSF-NFL but not CSF-T-tau was higher in patients with encephalitis associated with intracellular antigens as compared to those with encephalitis associated with extracellular antigens (p = 0.032). Total tau and NFL levels were not significantly altered in patients with encephalitis associated with extracellular antigens compared to controls. NFL in the total cohort correlated with neurological signs of cerebellar dysfunction, peripheral neuropathy, presence of CV2 positivity, presence of an underlying tumor and a more detrimental clinical outcome. AE patients with abnormal MRI findings displayed higher NFL levels compared to those without, albeit with no statistical significance (p = 0.07). Using receiver operating characteristic curve analysis, CSF-NFL levels with a cut-off value of 969 pg/mL had a sensitivity and specificity of 100% and 76.19%, respectively, regarding the detection of underlying malignancies. Our findings suggest that neuronal integrity is preserved in autoimmune encephalitis associated with extracellular antigens and without the presence of tumor. However, highly increased NFL is observed in AE associated with intracellular antigens and presence of an underlying tumor. CSF-NFL could potentially be used as a diagnostic biomarker of underlying malignancies in the clinical setting of AE. Full article
(This article belongs to the Special Issue Molecular Epidemiology and Pathophysiology of Autoimmune Encephalitis)
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Review

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19 pages, 1726 KiB  
Review
Seizures, Epilepsy, and NORSE Secondary to Autoimmune Encephalitis: A Practical Guide for Clinicians
by Alberto Vogrig, Gian Luigi Gigli, Annacarmen Nilo, Giada Pauletto and Mariarosaria Valente
Biomedicines 2023, 11(1), 44; https://doi.org/10.3390/biomedicines11010044 - 25 Dec 2022
Cited by 12 | Viewed by 8736
Abstract
The most recent International League Against Epilepsy (ILAE) classification has included “immune etiology” along with other well-known causes of epilepsy. This was possible thanks to the progress in detection of pathogenic neural antibodies (Abs) in a subset of patients, and resulted in an [...] Read more.
The most recent International League Against Epilepsy (ILAE) classification has included “immune etiology” along with other well-known causes of epilepsy. This was possible thanks to the progress in detection of pathogenic neural antibodies (Abs) in a subset of patients, and resulted in an increased interest in identifying potentially treatable causes of otherwise refractory seizures. Most autoimmune encephalitides (AE) present with seizures, but only a minority of cases evolve to long-term epilepsy. The risk of epilepsy is higher for patients harboring Abs targeting intracellular antigens (T cell-mediated and mostly paraneoplastic, such as Hu, CV2/CRMP5, Ma2, GAD65 Abs), compared with patients with neuronal surface Abs (antibody-mediated and less frequently paraneoplastic, such as NMDAR, GABAbR, LGI1, CASPR2 Abs). To consider these aspects, conceptual definitions for two entities were provided: acute symptomatic seizures secondary to AE, and autoimmune-associated epilepsy, which reflect the different pathophysiology and prognoses. Through this manuscript, we provide an up-to-date review on the current state of knowledge concerning diagnosis and management of patients with Ab-mediated encephalitis and associated epilepsy. Special emphasis is placed on clinical aspects, such as brain magnetic resonance imaging (MRI) and cerebrospinal fluid (CSF) specificities, electroencephalographic (EEG) findings, cancer screening and suggestions for a rational therapeutic approach. Full article
(This article belongs to the Special Issue Molecular Epidemiology and Pathophysiology of Autoimmune Encephalitis)
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Other

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15 pages, 2374 KiB  
Systematic Review
Spatial and Ecological Factors Modulate the Incidence of Anti-NMDAR Encephalitis—A Systematic Review
by Agustí Alentorn, Giulia Berzero, Harry Alexopoulos, John Tzartos, Germán Reyes Botero, Andrea Morales Martínez, Sergio Muñiz-Castrillo, Alberto Vogrig, Bastien Joubert, Francisco A. García Jiménez, Dagoberto Cabrera, José Vladimir Tobon, Carolina Delgado, Patricio Sandoval, Mónica Troncoso, Lorna Galleguillos, Marine Giry, Marion Benazra, Isaias Hernández Verdin, Maëlle Dade, Géraldine Picard, Véronique Rogemond, Nicolas Weiss, Marinos C. Dalakas, Pierre-Yves Boëlle, Jean-Yves Delattre, Jérôme Honnorat and Dimitri Psimarasadd Show full author list remove Hide full author list
Biomedicines 2023, 11(6), 1525; https://doi.org/10.3390/biomedicines11061525 - 25 May 2023
Cited by 4 | Viewed by 3472
Abstract
Anti-NMDAR encephalitis has been associated with multiple antigenic triggers (i.e., ovarian teratomas, prodromal viral infections) but whether geographic, climatic, and environmental factors might influence disease risk has not been explored yet. We performed a systematic review and a meta-analysis of all published papers [...] Read more.
Anti-NMDAR encephalitis has been associated with multiple antigenic triggers (i.e., ovarian teratomas, prodromal viral infections) but whether geographic, climatic, and environmental factors might influence disease risk has not been explored yet. We performed a systematic review and a meta-analysis of all published papers reporting the incidence of anti-NMDAR encephalitis in a definite country or region. We performed several multivariate spatial autocorrelation analyses to analyze the spatial variations in the incidence of anti-NMDA encephalitis depending on its geographical localization and temperature. Finally, we performed seasonal analyses in two original datasets from France and Greece and assessed the impact of temperature using an exposure-lag-response model in the French dataset. The reported incidence of anti-NMDAR encephalitis varied considerably among studies and countries, being higher in Oceania and South America (0.2 and 0.16 per 100,000 persons-year, respectively) compared to Europe and North America (0.06 per 100,000 persons-year) (p < 0.01). Different regression models confirmed a strong negative correlation with latitude (Pearson’s R = −0.88, p < 0.00001), with higher incidence in southern hemisphere countries far from the equator. Seasonal analyses showed a peak of cases during warm months. Exposure-lag-response models confirmed a positive correlation between extreme hot temperatures and the incidence of anti-NMDAR encephalitis in France (p = 0.03). Temperature analyses showed a significant association with higher mean temperatures and positive correlation with higher ultraviolet exposure worldwide. This study provides the first evidence that geographic and climatic factors including latitude, mean annual temperature, and ultraviolet exposure, might modify disease risk. Full article
(This article belongs to the Special Issue Molecular Epidemiology and Pathophysiology of Autoimmune Encephalitis)
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32 pages, 11929 KiB  
Systematic Review
Autoimmune Encephalitis in COVID-19 Infection: Our Experience and Systematic Review of the Literature
by Adina Stoian, Mircea Stoian, Zoltan Bajko, Smaranda Maier, Sebastian Andone, Roxana Adriana Cioflinc, Anca Motataianu, Laura Barcutean and Rodica Balasa
Biomedicines 2022, 10(4), 774; https://doi.org/10.3390/biomedicines10040774 - 25 Mar 2022
Cited by 30 | Viewed by 8073
Abstract
The neurologic complications of COVID-19 infection are frequent in hospitalized patients; a high percentage of them present neurologic manifestations at some point during the course of their disease. Headache, muscle pain, encephalopathy and dizziness are among the most common complications. Encephalitis is an [...] Read more.
The neurologic complications of COVID-19 infection are frequent in hospitalized patients; a high percentage of them present neurologic manifestations at some point during the course of their disease. Headache, muscle pain, encephalopathy and dizziness are among the most common complications. Encephalitis is an inflammatory condition with many etiologies. There are several forms of encephalitis associated with antibodies against intracellular neuronal proteins, cell surfaces or synaptic proteins, referred to as autoimmune encephalitis. Several case reports published in the literature document autoimmune encephalitis cases triggered by COVID-19 infection. Our paper first presents our experience in this issue and then systematically reviews the literature on autoimmune encephalitis that developed in the background of SARS-CoV-2 infections and also discusses the possible pathophysiological mechanisms of auto-immune-mediated damage to the nervous system. This review contributes to improve the management and prognosis of COVID-19-related autoimmune encephalitis. Full article
(This article belongs to the Special Issue Molecular Epidemiology and Pathophysiology of Autoimmune Encephalitis)
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