Browse
Submit Data
Databases
API
Help

Dataset Information

6 Views

0 Connections

0 Citations

0 Reanalyses

0 Downloads

Omics score: 0

Neuropsychiatric Lupus with Antibody-Mediated Striatal Encephalitis.

ABSTRACT: Systemic lupus erythematosus is a chronic autoimmune disease characterized by the production of autoantibodies resulting in tissue injury across multiple organs; up to 50% of patients develop neurologic involvement, collectively referred to as neuropsychiatric systemic lupus erythematosus. The cases in this clinical report will highlight a subtype of neuropsychiatric systemic lupus erythematosus demonstrating imaging findings of striatal inflammation responsive to plasmapheresis similar to those in the subset of N-methyl-D-aspartate receptor autoimmune encephalitis that involves the striatum. Although the cause for this striking imaging appearance is not definitely known, literature will be presented supporting the hypothesis that it is due to peripheral anti-double-stranded DNA antibodies entering the central nervous system to cross-react with N-methyl-D-aspartate receptor antigens.

SUBMITTER: Kelley BP

PROVIDER: S-EPMC7655406 | biostudies-literature | 2018 Dec

REPOSITORIES: biostudies-literature

ACCESS DATA

Json Xml

Publications

Neuropsychiatric Lupus with Antibody-Mediated Striatal Encephalitis.

Kelley B P BP Corrigan J J JJ Patel S C SC Griffith B D BD

AJNR. American journal of neuroradiology 20181122 12

Systemic lupus erythematosus is a chronic autoimmune disease characterized by the production of autoantibodies resulting in tissue injury across multiple organs; up to 50% of patients develop neurologic involvement, collectively referred to as neuropsychiatric systemic lupus erythematosus. The cases in this clinical report will highlight a subtype of neuropsychiatric systemic lupus erythematosus demonstrating imaging findings of striatal inflammation responsive to plasmapheresis similar to those ...[more]

PMID: 30467216

Similar Datasets

The three pillars in treating antibody-mediated encephalitis.

Project description:The rapid initiation of immunotherapy has a decisive impact on the course of the disease in patients with antibody-mediated encephalitis (AE). The importance of treating AE with antiseizure medication and antipsychotics is discussed controversially; however, standardized procedures should be ensured, especially for the initiation of treatment in severe disease. Recommendations and guidelines for further interventions in refractory courses are needed. In this review, we contrast the three mainstays of treatment options in patients with AE and attempt to highlight the importance of 1) antiseizure therapy, 2) antipsychotic therapy, and 3) immunotherapy/tumor resection from today's perspective.

| S-EPMC10776469 | biostudies-literature

The gene expression analysis of human astrocyte exposure to anti-suprabasin antibody: identification of a novel antibody for neuropsychiatric systemic lupus erythematosus

Project description:[Objectives]: Neuropsychiatric systemic lupus erythematosus (NPSLE) is often difficult to diagnose and distinguish from those of other diseases, because no specific antibodies have yet been detected. [Methods]: We developed a novel proteomic strategy for identifying and profiling antigens in immune complexes (ICs) in the cerebrospinal fluid (CSF) of 26 NPSLE patients. We performed in vitro experiments using astrocytes and analyzed functional change by microarray. [Results]: We identified ICs of suprabasin (SBSN), a molecule which is thought to play a role in epidermal differentiation. Microarray data showed that the senescence and autophagy pathways were significantly changed in astrocytes with anti-SBSN antibody exposure compared to normal immunoglobulin G (IgG) exposure. [Conclusions]: These findings indicate that SBSN could be a novel autoantibody for the evaluation of suspected NPSLE, and may help elucidate the pathogenesis underlying this disease. The cultured human astrocyte exposed to non IgG, control rabbit IgG and anti suprabasin antibodies with or without lipopolysaccharide (1Î¼g/ml) for 24 h were collected for RNA isolation. We divided into the following 6 samples, Non IgG LPS(-), Non IgG LPS(+), Norm IgG LPS(-), Norm IgG LPS(+), SBSN LPS(-) and SBSN IgG LPS(+).

2016-09-01 | E-GEOD-79189 | biostudies-arrayexpress

Novel CSF biomarkers for diagnosis and integrated analysis of neuropsychiatric systemic lupus erythematosus: based on antibody profiling.

Project description:BackgroundNeuropsychiatric systemic lupus erythematosus (NPSLE), with various morbidities and multiple manifestations in the central nervous system, remains a limited standard for diagnosis. Our study was to discover novel biomarkers for improving the diagnostic efficiency for NPSLE.MethodsWe performed a quantitative planar protein antibody microarray to screen 1000 proteins in cerebrospinal fluid from controls, systemic lupus erythematosus (SLE, non-NPSLE) patients, and NPSLE patients. Differentially expressed proteins (DEPs) as candidate biomarkers were developed into a custom multiplexed protein antibody array for further validation in an independent larger cohort. Subsequently, we used least absolute shrinkage and selection operator regression (LASSO) analysis and multivariable logistic regression analysis for optimizing feature selection and constructing a diagnostic model. A receiver operating characteristic curve (ROC) was generated to assess the effectiveness of the models.ResultsThe expression of 29 proteins in CSF was significantly altered in the comparison of the three groups. We selected 17 proteins as candidate biomarkers in accordance with protein interaction analysis. In the larger cohort, we identified 5 DEPs as biomarkers for NPSLE, including TCN2, CST6, KLK5, L-selectin, and Trappin-2. The diagnostic model included 3 hub proteins (CST6, TCN2, KLK5) and was best at discriminating NPSLE from SLE patients. These CSF biomarkers were also highly associated with disease activity. In addition, there were 6 molecules with remarkable changes in NPSLE CSF and hippocampus, which indicated the consistency of the environment in the brain and the promising molecular targets in the pathogenesis of NPSLE.ConclusionsThe dual-chips screening strategy demonstrated KLK5, L-selectin, Trappin-2, TCN2, and CST6 as CSF biomarkers for diagnosing NPSLE.

| S-EPMC10486090 | biostudies-literature

Neuropsychiatric Disorders Due to Limbic Encephalitis: Immunologic Aspect.

Project description:Limbic encephalitis (LE) is a rare cause of encephalitis presenting as an acute and subacute onset of neuropsychiatric manifestations, particularly with memory deficits and confusion as core features, along with seizure occurrence, movement disorders, or autonomic dysfunctions. LE is caused by neuronal antibodies targeting the cellular surface, synaptic, and intracellular antigens, which alter the synaptic transmission, especially in the limbic area. Immunologic mechanisms involve antibodies, complements, or T-cell-mediated immune responses in different degree according to different autoantibodies. Sensitive cerebrospinal fluid markers of LE are unavailable, and radiographic findings may not reveal a typical mesiotemporal involvement at neurologic presentations; therefore, a high clinical index of suspicions is pivotal, and a neuronal antibody testing is necessary to make early diagnosis. Some patients have concomitant tumors, causing paraneoplastic LE; therefore, tumor survey and treatment are required in addition to immunotherapy. In this study, a review on the molecular and immunologic aspects of LE was conducted to gain awareness of its peculiarity, which we found quite different from our knowledge on traditional psychiatric illness.

| S-EPMC7795533 | biostudies-literature

C1q Deficiency and Neuropsychiatric Systemic Lupus Erythematosus.

Project description:C1q deficiency is a rare immunodeficiency, which is strongly associated with the development of systemic lupus erythematosus (SLE). A mutation in one of the C1q genes can either lead to complete deficiency or to low C1q levels with C1q polypeptide in the form of low-molecular weight (LMW) C1q. Patients with C1q deficiency mainly present with cutaneous and renal involvement. Although less frequent, neuropsychiatric (NP) involvement has also been reported in 20% of the C1q-deficient patients. This involvement appears to be absent in other deficiencies of early components of the complement classical pathway (CP) (C1r/C1s, C2, or C4 deficiencies). We describe a new case with C1q deficiency with a homozygous G34R mutation in C1qC-producing LMW-C1q presenting with a severe SLE flare with NP involvement. The serum of this patient contained very low levels of a LMW variant of C1q polypeptides. Cell lysates contained the three chains of C1q, but no intact C1q was detected, consistent with the hypothesis of the existence of a LMW-C1q. Furthermore, we provide a literature overview of NP-SLE in C1q deficiency and hypothesize about the potential role of C1q in the pathogenesis of NP involvement in these patients. The onset of NP-SLE in C1q-deficient individuals is more severe when compared with complement competent NP-SLE patients. An important number of cases present with seizures and the most frequent findings in neuroimaging are changes in basal ganglia and cerebral vasculitis. A defective CP, because of non-functional C1q, does not protect against NP involvement in SLE. The absence of C1q and, subsequently, some of its biological functions may be associated with more severe NP-SLE.

| S-EPMC5186770 | biostudies-literature

The gene expression analysis of human astrocyte exposure to anti-suprabasin antibody: identification of a novel antibody for neuropsychiatric systemic lupus erythematosus

2016-09-01 | GSE79189 | GEO

Neuropsychiatric presentation of Covid-19-related encephalitis: Case report.

Project description:Covid-19-related encephalitis is a heterogeneous syndrome characterized by a combination of clinical, laboratory, and imaging features related to inflammation of the brain, where the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is presumably the causative agent. We reported a case of Covid-19-related encephalitis presenting with neuropsychiatric symptoms, including intense agitation. Reverse-transcriptase polymerase-chain-reaction in cerebrospinal fluid was positive for SARS-CoV-2. Our case expands the literature about neurologic manifestations of Covid-19 and emphasizes the possibility of prominent behavioral symptoms as the initial manifestation.

| S-EPMC8552702 | biostudies-literature

Interferon-? exacerbates neuropsychiatric phenotypes in lupus-prone mice.

Project description:BACKGROUND:Neuropsychiatric systemic lupus erythaematosus (NP-SLE) is one of the major manifestations of lupus. However, the mechanisms involved in NP-SLE are still largely unknown. The abnormal activation of the type I IFN signalling pathway is involved in SLE pathogenesis and is linked to NP-SLE, but the effect of IFN-? on NP-SLE encephalopathy has not been systematically studied. METHODS:An intravenous injection of Adv-IFN-? (10 mice, 10?×?109 vp) was administered to the IFN-?-treated group, and Adv-ctrl (10 mice, 10?×?109 vp) (ViGene Biosciences, China) was administered to the control group. Gene expression was determined by real-time quantitative polymerase chain reaction (RT-qPCR). Enzyme-linked immunosorbent assay (ELISA) was used to detect antibodies in the serum, and urinary protein levels were measured with a BCA Protein Assay kit. Haematoxylin-eosin (H&E) and periodic acid-Schiff (PAS)-light green staining were used for kidney histology. The elevated plus-maze test, novelty-suppressed feeding assay, open-field test, tail suspension test, social dominance tube test, three-chamber social interaction test, step-down passive avoidance test and novelty Y-maze task were used to assess behaviour. RESULTS:In this study, we performed a series of behavioural tests to assess the neuropsychiatric phenotypes of IFN-?-treated NZB/NZW F1 mice and found that these mice developed a series of mental disorders such as anxiety-like phenotypes, depression-like phenotypes, deficits in sociability and cognitive impairments, which mimic the neuropsychiatric manifestations of NP-SLE, with a consistent onset and progression. CONCLUSIONS:Our research verified that IFN-? plays a critical role in NP-SLE and provides a comprehensive NP-SLE mouse model for dissecting the mechanisms of NP-SLE and developing novel therapies for intervention.

| S-EPMC6724270 | biostudies-literature

Diffusion tensor imaging in neuropsychiatric systemic lupus erythematosus.

Project description:BackgroundNeuropsychiatric systemic lupus erythematosus (NPSLE) is associated with increased morbidity and mortality.MethodsWe used Diffusion Tensor Imaging (DTI) to assess white matter abnormalities in seventeen NPSLE patients, sixteen SLE patients without NPSLE, and twenty age- and gender-matched controls.ResultsNPSLE patients differed significantly from SLE and control patients in white matter integrity of the body of the corpus callosum, the left arm of the forceps major and the left anterior corona radiata.ConclusionsSeveral possible mechanisms of white matter injury are explored, including vascular injury, medication effects, and platelet or fibrin macro- or microembolism from Libman-Sacks endocarditis.

| S-EPMC2919505 | biostudies-literature

Attribution of Neuropsychiatric Manifestations to Systemic Lupus Erythematosus.

Project description:Neuropsychiatric (NP) involvement in systemic lupus erythematosus (SLE) is one of the most severe manifestations of the disease that has a heavy impact on patient's functioning, quality of life, and disease outcome. The prevalence is highly variable and the clinical phenotypes vary from common syndromes to rare NP entities. Its occurrence may be the result of a primary manifestation of SLE, secondary to other conditions (such as infections or metabolic disturbances) or the effect of concomitant comorbidities that often complicate the disease course. Correct attribution of NP events may pose diagnostic challenges and it is a critical factor in selecting the correct management. Although there is still no diagnostic gold standard to rightly diagnose NPSLE syndromes, great advances have been made in improving the clinician judgment in the evaluation process. In this narrative review, we present and discuss available evidence concerning NPSLE with a special focus on the attribution models developed using composite decision rules to ascribe NP events to SLE.

| S-EPMC5861139 | biostudies-literature

OmicsDI is part of the ELIXIR infrastructure

OmicsDI is an Elixir interoperability service. Learn more ›

Tweets

OmicsDI Databases

PRIDE
PeptideAtlas
MassIVE
JPOST Repository
Physiome Model Repository

EGA
EVA
ENA
LINCS
PAXDB
Cell Collective

MetaboLights
Metabolomics Workbench
MetabolomeExpress
GNPS
BioModels
FAIRDOMHub

ArrayExpress
dbGaP
ExpressionAtlas
GEO
NODE

Information

Databases
Help
API
Contact us
Code on GitHub
Terms of use
Submit Data