scholarly journals Autoimmune encephalitis: clinical spectrum and management

2021 ◽  
pp. practneurol-2020-002567
Author(s):  
Christopher E Uy ◽  
Sophie Binks ◽  
Sarosh R Irani

Autoimmune encephalitis defines brain inflammation caused by a misdirected immune response against self-antigens expressed in the central nervous system. It comprises a heterogeneous group of disorders that are at least as common as infectious causes of encephalitis. The rapid and ongoing expansion of this field has been driven by the identification of several pathogenic autoantibodies that cause polysymptomatic neurological and neuropsychiatric diseases. These conditions often show highly distinctive cognitive, seizure and movement disorder phenotypes, making them clinically recognisable. Their early identification and treatment improve patient outcomes, and may aid rapid diagnosis of an underlying associated tumour. Here we summarise the well-known autoantibody-mediated encephalitis syndromes with neuronal cell-surface antigens. We focus on practical aspects of their diagnosis and treatment, offer our clinical experiences of managing such cases and highlight more basic neuroimmunological advances that will inform their future diagnosis and treatments.

Author(s):  
Marleen H. van Coevorden-Hameete ◽  
Maarten J. Titulaer ◽  
Marco W. J. Schreurs ◽  
Esther de Graaff ◽  
Peter A. E. Sillevis Smitt ◽  
...  

Marine Drugs ◽  
2019 ◽  
Vol 17 (2) ◽  
pp. 79 ◽  
Author(s):  
Beika Zhu ◽  
Yang Zhang ◽  
Karl Herrup

Cell cycle reentry is a unified mechanism shared by several neurodegenerative diseases, including Alzheimer’s disease (AD) and Ataxia Telangiectasia (A-T). This phenotype is often related to neuroinflammation in the central nervous system. To mimic brain inflammation in vitro, we adopted the previously established method of using conditioned medium collected from activated THP-1 cells and applied it to both differentiated HT22 cells and primary neurons. Unscheduled cell cycle events were observed in both systems, indicating the potential of this approach as an in vitro model of neurodegenerative disease. We used this assay to measure the neuroprotective effects of New Zealand green-lipped mussel extract, PCSO-524®, to protect post-mitotic cells from cell cycle reentry. We found that, both in vitro and in an animal model, PCSO-524® displayed promising neuroprotective effects, and thus has potential to postpone or prevent the onset of neurodegenerative disease.


2021 ◽  
Author(s):  
Saba Islam ◽  
Mirren Charnley ◽  
Guneet Bindra ◽  
Julian Ratcliffe ◽  
Jiangtao Zhou ◽  
...  

COVID-19 is primarily known as a respiratory disease caused by the virus SARS-CoV-2. However, neurological symptoms such as memory loss, sensory confusion, cognitive and psychiatric issues, severe headaches, and even stroke are reported in as many as 30% of cases and can persist even after the infection is over (so-called 'long COVID'). These neurological symptoms are thought to be caused by brain inflammation, triggered by the virus infecting the central nervous system of COVID-19 patients, however we still don't fully understand the mechanisms for these symptoms. The neurological effects of COVID-19 share many similarities to neurodegenerative diseases such as Alzheimer's and Parkinson's in which the presence of cytotoxic protein-based amyloid aggregates is a common etiological feature. Following the hypothesis that some neurological symptoms of COVID-19 may also follow an amyloid etiology we performed a bioinformatic scan of the SARS-CoV-2 proteome, detecting peptide fragments that were predicted to be highly amyloidogenic. We selected two of these peptides and discovered that they do rapidly self-assemble into amyloid. Furthermore, these amyloid assemblies were shown to be highly toxic to a neuronal cell line. We introduce and support the idea that cytotoxic amyloid aggregates of SARS-CoV-2 proteins are causing some of the neurological symptoms commonly found in COVID-19 and contributing to long COVID, especially those symptoms which are novel to long COVID in contrast to other post-viral syndromes.


2020 ◽  
pp. 1-12 ◽  
Author(s):  
Dominique Endres ◽  
Viktoria Maier ◽  
Frank Leypoldt ◽  
Klaus-Peter Wandinger ◽  
Belinda Lennox ◽  
...  

Abstract Background Autoimmune encephalitis (AE) is an important consideration during the diagnostic work-up of secondary mental disorders. Indeed, isolated psychiatric syndromes have been described in case reports of patients with underlying AE. Therefore, the authors performed a systematic literature review of published cases with AE that have predominant psychiatric/neurocognitive manifestations. The aim of this paper is to present the clinical characteristics of these patients. Methods The authors conducted a systematic Medline search via Ovid, looking for case reports/series of AEs with antineuronal autoantibodies (Abs) against cell surface/intracellular antigens combined with predominant psychiatric/neurocognitive syndromes. The same was done for patients with Hashimoto encephalopathy/SREAT. Only patients with signs of immunological brain involvement or tumors in their diagnostic investigations or improvement under immunomodulatory drugs were included. Results We identified 145 patients with AE mimicking predominant psychiatric/neurocognitive syndromes. Of these cases, 64% were female, and the mean age among all patients was 43.9 (±22.1) years. Most of the patients had Abs against neuronal cell surface antigens (55%), most frequently against the NMDA-receptor (N = 46). Amnestic/dementia-like (39%) and schizophreniform (34%) syndromes were the most frequently reported. Cerebrospinal fluid changes were found in 78%, electroencephalography abnormalities in 61%, and magnetic resonance imaging pathologies in 51% of the patients. Immunomodulatory treatment was performed in 87% of the cases, and 94% of the patients responded to treatment. Conclusions Our findings indicate that AEs can mimic predominant psychiatric and neurocognitive disorders, such as schizophreniform psychoses or neurodegenerative dementia, and that affected patients can be treated successfully with immunomodulatory drugs.


2021 ◽  
Author(s):  
Bruna de Freitas Dias

Background: Autoimmune encephalitis (AIE) is the main cause of non-infectious encephalitis and results from the peripheral immune response against cell surface antigens in the central nervous system. The clinical presentations are varied and known triggers are tumors and herpetic infections. Arboviruses Zika (ZIKV), Dengue (DENGV) and Chikungunya (CHIKV) are neurotropic infections that present neurological manifestations whose mechanism is unknown. Objective: Verify the frequency of arboviruses antibodies in patients with autoimmune encephalitis in a Brazilian cohort Design and setting: It is a transversal study performed by Hospital Israelita Albert Einstein in Brazil Methods: Patients who met the criteria for probable AIE (Graus 2016) evaluated at the 18 centers of the BrAIN network were included. Clinical, epidemiological and laboratory data were compiled. Antineuronal antibodies were detected using TBA, CBA and immunoblot in serum and CSF; antibodies against ZIKV, DENGV and CHIKV were detected by ELISA. The cohort was divided into two groups: seropositive encephalitic (SPE) and non-encephalitic (NE) and the frequencies of viral serologies were compared. Results: Among 619 patients included in the BrAIN cohort, serology for arboviruses was performed in 482 patients, being 79 SPE and 99 NE. The SPEgroup showed the following frequency of antibodies: 58.2% anti-NMDA, 7.6% antiLGI1, 6.3% anti-Caspr2, 2.5% anti-GABA B, 1.3% anti-GABA A, 3.8% anti-AMPAr, 1.3% anti-AQ4, 8.9% anti-MOG, 1.3% anti-IgLON5, 7.6% anti-GlyR and 5.1% others. The frequency of serology was IgG DENG (SPE 42.3% X NP 43.4%, p = 0.82); IgG CHIK (SPE 16.5% X 3.1% NP, p = 0.001); IgG ZIKV (SPE31.6% X NP 28.3%, p = 0.62). The frequency of triple positive serology (IgG DENG, ZIKV, CHIK) was 11.39% (SPE X 2.02% NP, p = 0.009). Conclusions: Patients with SPE have IgG CHIKV antibodies most commonly. In addition, they present a higher frequency of positivity for IgG CHIKV, ZIKV, DENGV simultaneously. Future studies should assess the association between arboviruses as a trigger for AIE or as a marker of susceptibility to immunological alteration.


2018 ◽  
Vol 390 ◽  
pp. 26-32 ◽  
Author(s):  
Juntaro Kaneko ◽  
Naomi Kanazawa ◽  
Naomi Tominaga ◽  
Atsushi Kaneko ◽  
Hiroki Suga ◽  
...  

Author(s):  
Anthony A. Paparo ◽  
Judith A. Murphy

The purpose of this study was to localize the red neuronal pigment in Mytilus edulis and examine its role in the control of lateral ciliary activity in the gill. The visceral ganglia (Vg) in the central nervous system show an over al red pigmentation. Most red pigments examined in squash preps and cryostat sec tions were localized in the neuronal cell bodies and proximal axon regions. Unstained cryostat sections showed highly localized patches of this pigment scattered throughout the cells in the form of dense granular masses about 5-7 um in diameter, with the individual granules ranging from 0.6-1.3 um in diame ter. Tissue stained with Gomori's method for Fe showed bright blue granular masses of about the same size and structure as previously seen in unstained cryostat sections.Thick section microanalysis (Fig.l) confirmed both the localization and presence of Fe in the nerve cell. These nerve cells of the Vg share with other pigmented photosensitive cells the common cytostructural feature of localization of absorbing molecules in intracellular organelles where they are tightly ordered in fine substructures.


Author(s):  
K. Chien ◽  
I.P. Shintaku ◽  
A.F. Sassoon ◽  
R.L. Van de Velde ◽  
R. Heusser

Identification of cellular phenotype by cell surface antigens in conjunction with ultrastructural analysis of cellular morphology can be a useful tool in the study of biologic processes as well as in diagnostic histopathology. In this abstract, we describe a simple pre-embedding, protein A-gold staining method which is designed for cell suspensions combining the handling convenience of slide-mounted cell monolayers and the ability to evaluate specimen staining specificity prior to EM embedding.


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