Autoimmune encephalitis epidemiology and a comparison to infectious encephalitis

Dubey, Divyanshu; Pittock, Sean J.; Kelly, Cecilia; McKeon, Andrew; López-Chiriboga, A. Sebastian; Lennon, Vanda A.; Gadoth, Avi; Smith, Carin Y.; Bryant, Sandra C.; Klein, Christopher J.; Aksamit, Allen J.; Toledano, Michel; Boeve, Bradley F.; Tillema, Jan Mendelt; Flanagan, Eoin P.

doi:10.1002/ana.25131

Cited by 568 publications

(451 citation statements)

References 33 publications

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“…15,16 Unlike patients with MOG-IgG or NMDAR-IgG, who typically have inflammatory CSF, 16 only 42% of pediatric patients with LGI1-IgG or CASPR2-IgG seropositivity have CSF abnormalities and only 33% have brain MRI abnormalities. 15,16 Unlike patients with MOG-IgG or NMDAR-IgG, who typically have inflammatory CSF, 16 only 42% of pediatric patients with LGI1-IgG or CASPR2-IgG seropositivity have CSF abnormalities and only 33% have brain MRI abnormalities.…”

Section: Discussionmentioning

confidence: 99%

“…N-methyl-D-aspartate receptor (NMDAR)-IgG and myelin oligodendrocyte glycoprotein (MOG)-IgG are the most commonly identified neural autoantibodies in the setting of autoimmune encephalitis . 15,16 Unlike patients with MOG-IgG or NMDAR-IgG, who typically have inflammatory CSF, 16 only 42% of pediatric patients with LGI1-IgG or CASPR2-IgG seropositivity have CSF abnormalities and only 33% have brain MRI abnormalities. This is an important observation, because one-fifth of the children in this report were initially misdiagnosed as having a functional neurological disorder.…”

Section: Discussionmentioning

confidence: 99%

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LGI1 and CASPR2 neurological autoimmunity in children

López-Chiriboga¹,

Klein²,

Ζεκερίδου³

et al. 2018

Annals of Neurology

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The clinical phenotype of leucine-rich glioma-inactivated protein 1 (LGI1) and contactin-associated proteinlike 2 (CASPR2) autoimmunity is well defined in adults. Data for children are limited (<10 cases). Among 13,319 pediatric patients serologically tested for autoimmune neurological disorders (2010-2017), 264 were seropositive for voltage-gated potassium channel-complex-IgG (radioimmunoprecipitation). Only 13 (4.9%) were positive by transfected cell-binding assay for LGI1-IgG (n = 7), CASPR2-IgG (n = 3), or both (n = 3). This is significantly less than in adults. Encephalopathy, seizures, and peripheral nerve hyperexcitability were common, as was coexisting autoimmunity. No faciobrachial dystonic seizures or cancers were identified. Functional neurologic disorders were frequently the initial diagnosis, and immunotherapy appeared beneficial. Ann Neurol 2018;84:473-480.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

LGI1 and CASPR2 neurological autoimmunity in children

López-Chiriboga¹,

Klein²,

Ζεκερίδου³

et al. 2018

Annals of Neurology

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“…With the increasing awareness of autoantibody-positive cases, autoimmune encephalitis has received increasing attention (Dubey et al 2018). This autoimmune disease is related to genetic susceptibility, infection, and stressful life events, and is caused by excessive self-response and antigen-driven immune responses (David et al 2018;Karagianni et al 2019;Skopouli and Katsiougiannis 2018).…”

Section: Possible Mechanisms Of Neurological Symptomsmentioning

confidence: 99%

Involvement of the Nervous System in SARS-CoV-2 Infection

2020

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As a severe and highly contagious infectious disease, coronavirus disease 2019 (COVID-19) has caused a global pandemic. Several case reports have demonstrated that the respiratory system is the main target in patients with COVID-19, but the disease is not limited to the respiratory system. Case analysis indicated that the nervous system can be invaded by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and that 36.4% of COVID-19 patients had neurological symptoms. Importantly, the involvement of the CNS may be associated with poor prognosis and disease worsening. Here, we discussed the symptoms and evidence of nervous system involvement (directly and indirectly) caused by SARS-CoV-2 infection and possible mechanisms. CNS symptoms could be a potential indicator of poor prognosis; therefore, the prevention and treatment of CNS symptoms are also crucial for the recovery of COVID-19 patients.

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“…Indeed, the third most common category of autoantibody-mediated causes of encephalitis belonged to patients with unidentified autoantigens that have defied identification by more conventional methods. 48 Furthermore, high-resolution datasets characterizing the co-occurrence of specific antigens and antigenic determinants to specific diseases, symptoms, and clinical outcomes may ultimately serve to identify early biomarkers and may even stratify patient outcomes and inform treatment decisions. Finally, higher resolution epitope mapping and analysis of antigenic signatures across multiple patients should better inform the currently incomplete models describing oncoimmunologic processes and the loss of self-tolerance underpinning the rise of PND and autoimmune disease in general.…”

Section: Discussionmentioning

confidence: 99%

Exploration of Anti-Yo and Anti-Hu paraneoplastic neurological disorders by PhIP-Seq reveals a highly restricted pattern of antibody epitopes

O’Donovan

Mandel‐Brehm

Vazquez

et al. 2018

Preprint

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Paraneoplastic neurological disorders (PNDs) are immune-mediated diseases of the nervous system understood to manifest as part of a misdirected anti-tumor immune response.Identifying PND-associated autoantibodies and their cognate antigens can assist with proper diagnosis and treatment while also enhancing our understanding of tumor-associated immune processes, triggers for autoimmune disease, and the functional significance of onconeuronal proteins. Here, we employed an enhanced version of phage display immunoprecipitation and sequencing (PhIP-Seq) leveraging a library of over 731,000 unique phage clones tiling across the entire human proteome to detect autoantibodies and create high-resolution epitope profiles in serum and CSF samples from patients suffering from two common PNDs, the anti-Yo (n = 36 patients) and anti-Hu syndromes (n = 44 patients). All patient samples positive for anti-Yo antibody by a validated clinical assay yielded polyspecific enrichment of phage presenting peptides from the canonical anti-Yo (CDR2 and CDR2L) antigens, while 38% of anti-Hu patients (17/44) had a serum and/or CSF sample that significantly enriched peptides deriving from the ELAVL family of proteins, the anti-Hu autoantigenic target. The anti-Hu antibodies showed a remarkably convergent antigenic signature across 15/17 patients corresponding to residues surrounding and including the degenerate motif, RLDxLL, shared by ELAVL2, 3 and 4. Lastly, PhIP-Seq identified several known and novel autoantigens in these same patient samples, representing potential biomarkers that could aid in the diagnosis and prognosis of PND and cancer.

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Autoimmune encephalitis epidemiology and a comparison to infectious encephalitis

Abstract: This study shows that the prevalence and incidence of autoimmune encephalitis are comparable to infectious encephalitis, and its detection is increasing over time. Ann Neurol 2018;83:166-177.

Cited by 568 publications

References 33 publications

LGI1 and CASPR2 neurological autoimmunity in children

LGI1 and CASPR2 neurological autoimmunity in children

Involvement of the Nervous System in SARS-CoV-2 Infection

Exploration of Anti-Yo and Anti-Hu paraneoplastic neurological disorders by PhIP-Seq reveals a highly restricted pattern of antibody epitopes

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