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Autoimmune encephalitis

J M Lieb, N Naumann, F J Ahlhelm
CLINICAL ISSUE: Autoimmune disorders of the central nervous system (CNS) are common but are also a heterogeneous group of diseases. The most common form is multiple sclerosis (MS), others are clinically isolated syndrome (CIS), acute demyelinating encephalomyelitis (ADEM) and neuromyelitis optica spectrum disorders (NMOSD). Paraneoplastic syndromes are rare and tumor-associated, they are not induced by direct invasion of tumor tissue but by tumor-associated autoantibodies mostly against specific CNS proteins, e...
September 20, 2018: Der Radiologe
Julia C Nantes, Adam G Thomas, Natalie L Voets, Jonathan G Best, Clive R Rosenthal, Adam Al-Diwani, Sarosh R Irani, Charlotte J Stagg
This is the first study to investigate functional brain activity in patients affected by autoimmune encephalitis with faciobrancial dystonic seizures (FBDS). Multimodal 3T MRI scans, including structural neuroimaging (T1-weighted, diffusion weighted) and functional neuroimaging (scene-encoding task known to activate hippocampal regions), were performed. This case series analysis included eight patients treated for autoimmune encephalitis with FBDS, scanned during the convalescent phase of their condition (median 1...
2018: Frontiers in Neurology
Christina Hermetter, Franz Fazekas, Sonja Hochmeister
In recent years, new antibodies have been discovered which mediate autoimmune encephalitis. This immunological response can be triggered by an infection or a tumor. Classical onconeuronal antibodies are directed against intracellular neuronal agents but recently, a novel group of antibodies to neuronal cell-surface and synaptic antigens associated with different CNS-syndromes, has been discovered. Interestingly, the syndromes in this group can be successfully treated with immunotherapy and frequently do not have underlying tumors...
2018: Frontiers in Neurology
Satinder Aneja, Suvasini Sharma
Acute encephalitis is a common cause of death and neurodevelopmental problems in children. The causative agents are numerous including infectious agents such as viruses, bacteria, mycobacteria and protozoa; para-infectious and immune mediated disorders such as acute disseminated encephalomyelitis (ADEM) and autoimmune encephalitis, especially the recently described anti-NMDA receptor encephalitis. Also, many viral associated encephalopathies such as acute necrotizing encephalopathy can mimic the presentation of acute encephalitis...
September 19, 2018: Indian Journal of Pediatrics
Margherita Nosadini, Jonathan Gadian, Ming Lim, Stefano Sartori, Terrence Thomas, Russell C Dale
AIM: To gather data on mycophenolate mofetil (MMF) in paediatric autoimmune/immune-mediated central nervous system (CNS) conditions, focusing on safety and factors that may affect MMF efficacy. METHOD: Retrospective, multicentre study based on four paediatric neurology centres. RESULTS: Forty-four children were included (30 females, 14 males): 19 had proven/suspected autoimmune encephalitis, 14 had inflammatory demyelinating CNS diseases, and 11 had other autoimmune/immune-mediated CNS conditions...
September 17, 2018: Developmental Medicine and Child Neurology
Antoine Poulet, Hanna Bou Ali, Helene Savini, Elsa Kaphan, Philippe Parola
Post-malaria neurological syndrome (PMNS) is a complication that occurs after recovery from a severe Plasmodium falciparum attack. Over the past two decades, the description of several imported cases has confirmed that this syndrome is a clearly distinct entity, different from other post malarial neurological disorders. However, the underlying mechanisms are not yet elucidated. Herein, we present five imported PMNS cases managed in Marseille, France. The detection of neuronal surface antibodies to an encephalitic syndrome of unknown origin allowed us to reveal positivity of anti Voltage-Gated-Potassium Channel antibodies (anti VGKC) in one of them...
September 11, 2018: Travel Medicine and Infectious Disease
Jindřich Kopecký, Ondřej Kubeček, Tomáš Geryk, Birgita Slováčková, Petr Hoffmann, Miroslav Žiaran, Peter Priester
BACKGROUND: Great progress has recently been made in the treatment of metastatic renal cell carcinoma, including the introduction of nivolumab, an immune checkpoint inhibitor. Despite promising results, this treatment brings a completely new spectrum of adverse events, distinct from those experienced with small-molecule kinase inhibitors. Neurologic immune-related adverse events may be serious and potentially life-threatening complications requiring immediate immunosuppressive therapy...
September 15, 2018: Journal of Medical Case Reports
Zhan Zhang, Feng Fang
Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is an autoimmune encephalitis that was reported in recent years. At present, there remains no unified treatment regimen for the children and adolescents with this disease around the world. Immunotherapy is still the preferred way of treatment, mainly including first-line immunotherapy, second-line immunotherapy, and long-term immunotherapy. In recent years, some researchers have tried new immunosuppressants or modified immunotherapy regimens to improve the treatment outcome of the patients...
September 2018: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
Rubén L Caparó Oblitas
Autoimmune encephalitis is a new group of diseases of great clinical and therapeutic importance due to the good response in most cases to the immunomodulatory therapy indicated, with a large percentage of healing without significant neurological effects (cognitive, motor, seizures or involuntary movements). Since 2007, the presence of neuronal autoantibodies in the pathogenesis of this group of diseases has been demonstrated, with psychotic symptoms and involuntary movements as clinical markers of the disease...
2018: Medicina
Leila Shakerian, Samira Ghorbani, Farideh Talebi, Farshid Noorbakhsh
PU.1 is a transcription factor which is expressed in myeloid cells. Herein, we investigated the expression of PU.1 and its potentially targeting miRNAs in the central nervous system (CNS) of mice with experimental autoimmune encephalitis (EAE) and in cultured primary macrophages. PU.1 levels where highly induced in EAE spinal cords and in activated macrophages; this was associated with a significant reduction in miR-150-5p levels at chronic phase of disease and in activated cells. Luciferase assays confirmed the PU...
October 15, 2018: Journal of Neuroimmunology
Laura Midgley, Eric Kelleher, Michael S Zandi
No abstract text is available yet for this article.
September 2, 2018: British Journal of Hospital Medicine
Lam-Thanh Ly, Jakob Kreye, Betty Jurek, Jonas Leubner, Franziska Scheibe, Johannes Lemcke, Nina Kerstin Wenke, Sebastian Momsen Reincke, Harald Prüss
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a common autoimmune encephalitis presenting with psychosis, dyskinesias, autonomic dysfunction and seizures. The underlying autoantibodies against the NR1 subunit are directly pathogenic by disrupting synaptic NMDAR currents. However, antibody titers correlate only partially with the clinical outcome, suggesting the relevance of other factors such as antibody affinity. We thus determined the binding curves of human monoclonal autoantibodies and patients' cerebrospinal fluid (CSF) against NR1-expressing HEK293 cells using flow cytometry...
September 5, 2018: Journal of Neurology
Nan-Chang Chiu, Yi-Jie Lin, Ruu-Fen Tzang, Ying-Syuan Li, Hui-Ju Lin, Subir Das, Caleb G Chen, Chiao-Chicy Chen, Kate Hsu
Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is one of the most frequently encountered autoimmune encephalitis. The pathogenesis of both anti-NMDAR encephalitis and schizophrenia involve down-regulation of NMDA receptors. Whether autoantibody-mediated destruction of neuronal NMDA receptors is associated with schizophrenia or first-episode psychosis (FEP) remains unclear, as the current findings from different groups are inconsistent. The main culprits are likely due to heterogeneity of autoantibodies (autoAbs) in a patient's blood or cerebrospinal fluid (CSF), as well as due to limitation of the current detection methods for anti-NMDAR autoAbs...
2018: Frontiers in Neurology
Keiko Hatano, Hideyuki Matsumoto, Akihiko Mitsutake, Junko Yoshimura, Aya Nomura, Sumihisa Imakado, Yukitoshi Takahashi, Hideji Hashida
We report on a 44-year-old woman who was diagnosed with toxic epidermal necrolysis (TEN) during the recovery phase from autoimmune limbic encephalitis with anti-glutamate receptor antibodies. Both, autoimmune limbic encephalitis and TEN are very rare diseases. The co-existence of the two diseases has not yet been reported. We speculate that the total of 18 drugs needed for the treatment of encephalitis might have increased the risk of TEN. Similar reports would be required to elucidate the pathophysiology of the co-existence...
May 2018: Case Reports in Neurology
Luciana I Melamud, Victoria C Fernández, Analisa Manin, Andrés M Villa
Autoimmune encephalitis (AE) is a common cause of noninfectious acute encephalitis. We aimed to provide the first review of immune therapy regimens used for patients with AE in Latin America, as well as the safety and efficacy associated with them, by reviewing the medical records of Argentine patients with AE treated between 2013 and 2018. Data included clinical symptoms, laboratory tests, electroencephalography, magnetic resonance imaging, cerebral spinal fluid, and neoplasm screenings. We examined ten AE patients who received first-line immunotherapy at a median of 2...
September 4, 2018: Acta Neurologica Belgica
Rachel L Randell, Ashley V Adams, Heather Van Mater
BACKGROUND: Autoimmune encephalitis can result in significant neurological and psychiatric morbidity and mortality in patients of all ages and often does not respond to standard therapies. Recent reports suggest efficacy of tocilizumab, a monoclonal antibody against interleukin 6, in refractory autoimmune encephalitis. RESULTS: We describe three children with refractory autoimmune encephalitis who experienced a robust, immediate clinical response following treatment with tocilizumab...
August 3, 2018: Pediatric Neurology
Devon A Cohen, A Sebastian Lopez-Chiriboga, Sean J Pittock, Avi Gadoth, Anastasia Zekeridou, Barry A Boilson, William J Hogan, John J Poterucha, Katelynn M Wilton, Yi Lin, Eoin P Flanagan
No abstract text is available yet for this article.
November 2018: Neurology® Neuroimmunology & Neuroinflammation
Vijay K Ramanan, Brian A Crum, Andrew McKeon
No abstract text is available yet for this article.
September 2018: Neurology® Neuroimmunology & Neuroinflammation
Giovanni Di Liberto, Stanislav Pantelyushin, Mario Kreutzfeldt, Nicolas Page, Stefano Musardo, Roland Coras, Karin Steinbach, Ilena Vincenti, Bogna Klimek, Thomas Lingner, Gabriela Salinas, Nathalie Lin-Marq, Ori Staszewski, Marta Joana Costa Jordão, Ingrid Wagner, Kristof Egervari, Matthias Mack, Camilla Bellone, Ingmar Blümcke, Marco Prinz, Daniel D Pinschewer, Doron Merkler
Inflammatory disorders of the CNS are frequently accompanied by synaptic loss, which is thought to involve phagocytic microglia and complement components. However, the mechanisms accounting for aberrant synaptic connectivity in the context of CD8+ T cell-driven neuronal damage are poorly understood. Here, we profiled the neuronal translatome in a murine model of encephalitis caused by CD8+ T cells targeting antigenic neurons. Neuronal STAT1 signaling and downstream CCL2 expression were essential for apposition of phagocytes, ensuing synaptic loss and neurological disease...
August 28, 2018: Cell
Kenji Iwai, Kiyoko Amo, Ichiro Kuki, Masataka Fukuoka, Kiyohiro Kim, Chiharu Yamairi, Masao Togawa
Sjögren syndrome (SS) is a systemic inflammatory and autoimmune disease characterized by systemic disorders of the exocrine glands, predominantly the salivary and lacrimal glands. Here, we report a 4-year-old boy who presented with the repetition of generalized tonic-clonic seizures for 1-2 min. Initially, he was diagnosed with idiopathic autoimmune encephalitis and was treated with steroids. He was eventually diagnosed with SS based on the examination results, such as inflammatory cell infiltration into the minor salivary glands and positive serum anti-SSA/Ro antibody...
August 28, 2018: Brain & Development
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