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Autoimmune encephalitis (AE) comprises a group of diseases mediated by antibodies against neuronal cell surface or synaptic antigens, such as ion channels or neurotransmitter receptors. New clinical syndromes and their associated antibodies were and are still being characterized over the last two decades. The fact that their main clinical features are interdisciplinary, - encompassing neuropsychiatric symptoms, cognitive dysfunction, epileptic seizures, movement and sleep disorders - has led to a surge of interest in this field. Some of these diseases present with a well-defined syndrome, being recognizable on clinical grounds. Correct diagnosis is important since AE are potentially treatable diseases, despite their severity. On the other hand, an increasing number of neuronal antibodies being described casts doubt upon the way we should utilize antibody testing and interpret results. In this article we review, summarize and update the current knowledge on antibody mediated encephalitis.
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http://dx.doi.org/10.1590/0004-282X-ANP-2022-S122 | DOI Listing |
J Neurol
September 2025
Department of General Practice, The First People's Hospital of Lin'an District, Hangzhou, Lin'an People's Hospital Affiliated to Hangzhou Medical College, Hangzhou, 310000, Zhejiang Province, China.
Anti-mGluR1 encephalitis is a rare autoimmune disorder manifesting with cerebellar syndrome with varying levels of severity. However, limited data exist regarding the clinical features and treatment strategies for patients suffering from encephalitis associated with anti-mGluR1 antibodies. Herein, we comprehensively review and discuss clinical features of anti-mGluR1 encephalitis to enhance our understanding of this rare disorder.
View Article and Find Full Text PDFMethods Cell Biol
September 2025
Instituto de Biomedicina y Genética Molecular de Valladolid (IBGM-CSIC/UVA), Valladolid, Spain. Electronic address:
Multiple sclerosis (MS) is a chronic inflammatory disease of the central nervous system (CNS) that is characterized by a severe and progressive demyelinating process. It is considered a neurodegenerative autoimmune disorder driven by immune cell infiltration, overproduction of cytokines and reactive oxygen species (ROS) accumulation that leads to axonal and neuronal injury. Experimental autoimmune encephalomyelitis (EAE) is the most commonly used pre-clinical model of multiple sclerosis (MS), since it resembles many aspects of the human disease.
View Article and Find Full Text PDFBrain Behav Immun
September 2025
Department of Neurology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China. Electronic address:
Background: The proteome is a valuable resource for pinpointing therapeutic targets. Therefore, we conducted a proteome-wide Mendelian randomization (MR) study aimed at identifying potential protein markers and therapeutic targets for Anti-N-Methyl-D-Aspartate Receptor Encephalitis (NMDAR-E).
Methods: Protein quantitative trait loci (pQTLs) were obtained from seven published genome-wide association studies (GWASs) focusing on the plasma proteome, resulting in summary-level data for 734 circulating protein markers.
Redox Biol
September 2025
Department of Neurology with Institute of Translational Neurology, University Hospital Münster, Münster, Germany. Electronic address:
Anti-IgLON5 disease is an autoimmune encephalitis with more chronic presentation including memory decline, sleep disorder, bulbar symptoms and movement disorder. Post-mortem brains of patients with anti-IgLON5 disease show neurodegeneration with tau deposition sparking interest in this 'acquired tauopathy' as a disease model for neurodegeneration, yet mechanisms of neurodegeneration remain unknown. Using a reductionist human iPSC-derived neuron-antibody model, we applied proteomics approach, electrophysiology and live cell imaging.
View Article and Find Full Text PDFNeurol Neuroimmunol Neuroinflamm
November 2025
Department of Neurology, UC Davis Medical Center, Sacramento, CA.
Objectives: Complement factor I (CFI) deficiency is a rare condition that can present with fulminant relapsing CNS autoinflammation. In this report, we highlight the utility of genetic testing in unexplained CNS autoinflammation.
Methods: This case report describes a young adult with partial CFI deficiency, presenting with acute hemorrhagic leukoencephalitis and longitudinally extensive transverse myelitis.