scholarly journals Early multidisciplinary intensive-care therapy can improve outcome of severe anti-NMDA-receptor encephalitis presenting with extreme delta brush

2019 ◽  
Vol 10 (1) ◽  
pp. 241-243 ◽  
Author(s):  
R. Schneider ◽  
M. Brüne ◽  
TG. Breuer ◽  
C. Börnke ◽  
R. Gold ◽  
...  

Abstract Anti-N-methyl-D-aspartate receptor encephalitis (Anti-NMDARE) is a synaptic autoimmune encephalitis syndrome mainly affecting young females. An underlying tumor, most commonly ovarian teratomas in young females, may indicate a paraneoplastic syndrome. Prognostic factors of the clinical course of disease and outcome play a central role in view of early administration of second-line immunotherapy and intensive-care therapy. We report a case of severe Anti-NMDARE associated with unfavorable predictors including an extreme delta brush (EDB) electroencephalographic-pattern and high anti-NMDAR-antibody titers in the cerebral spinal fluid (CSF), which necessitated the admission to an intensive care unit. In spite of the poor prognosis, the patient completely recovered; we attribute this to an early escalation to second-line immunotherapy with rituximab and multidisciplinary intensive-care therapy. The present case underlines the relevance of multidisciplinary management for individuals with Anti-NMDARE.

2020 ◽  
Vol 33 (3) ◽  
pp. 208
Author(s):  
André Ponte ◽  
Ana Brito ◽  
Camila Nóbrega ◽  
Sofia Pinheiro ◽  
João Gama Marques

Anti-N-Mmethyl-D-aspartate receptor encephalitis is an autoimmune disease of the central nervous system with prominent neurologic and psychiatric features. Symptoms appear progressively and sometimes with an exclusively psychiatric initial presentation. The patient’s evaluation should be meticulous, and we should use all the diagnostic tests required for the exclusion of entities that can mimic this disease. We report the diagnostic investigation of a case of anti-N-methyl-D-aspartate receptor encephalitis in a patient with a previous diagnosis of schizophrenia with poor response to antipsychotics. The aim of this case report is to highlight the importance of close surveillance for neuropsychiatric symptoms, especially catatonia, and to recognize autoimmune encephalitis in the differential diagnosis of psychotic disorders with neurological symptoms and resistance or intolerance to antipsychotics. A prompt diagnosis will contribute to a faster onset of therapy and an overall improvement in prognosis.


Author(s):  
Joshua A. Vova

Anti-N-Methyl-D-Aspartate Receptor Encephalitis (ANMDARE) is one of the most common autoimmune encephalitis in the pediatric population. Patients with ANMDARE initially present with a prodrome of neuropsychiatric symptoms followed by progressively worsening seizures, agitation, and movement disorders. Complications can include problems such as aggression, insomnia, catatonia, and autonomic instability. Due to the complexity of this disease process, symptom management can be complex and may lead to significant polypharmacy. The goal of this review is to educate clinicians about the challenges of managing this disorder and providing guidance in symptom management.


2016 ◽  
Vol 32 (2) ◽  
pp. 243-245 ◽  
Author(s):  
Ilana Kahn ◽  
Guy Helman ◽  
Adeline Vanderver ◽  
Elizabeth Wells

Anti– N-methyl-d-aspartate receptor (NMDAR) encephalitis has been shown to be a treatable form of autoimmune encephalitis, but there remains no standardized approach to immunotherapy. We designed an anonymous survey sent to members of the Child Neurology Society to identify the current practices among child neurologists. A total of 151 pediatric neurologists responded to the survey. With these responses we were able to highlight areas of practice uniformity, including first-line treatment with intravenous immunoglobulin and intravenous methylprednisone and initiation of disease-modifying therapy with rituximab alone. The survey also identifies existing gaps in knowledge, specifically, when to add disease-modifying therapy and how long to continue therapy. We propose that the areas of agreement can be used as a step toward establishing standard treatment guidelines and research protocols directed at evidence-based clinical trials.


Author(s):  
Le-Minh Dao ◽  
Marie-Luise Machule ◽  
Petra Bacher ◽  
Julius Hoffmann ◽  
Lam-Thanh Ly ◽  
...  

AbstractAnti-N-methyl-D-aspartate-receptor (NMDAR) encephalitis is the most common autoimmune encephalitis with psychosis, amnesia, seizures and dyskinesias. The disease is mediated by pathogenic autoantibodies against the NR1 subunit that disrupt NMDAR function. Antibody infusion into mouse brains can recapitulate encephalitis symptoms, while active immunization resulted also in strong T cell infiltration into the hippocampus. However, whether T cells react against NMDAR and their specific contribution to disease development are poorly understood. Here we characterized the ex vivo frequency and phenotype of circulating CD4+ T helper (TH) cells reactive to NR1 protein using antigen-reactive T cell enrichment (ARTE) in 24 patients with NMDAR encephalitis, 13 patients with LGI1 encephalitis and 51 matched controls. Unexpectedly, patients with NMDAR encephalitis had lower frequencies of CD154-expressing NR1-reactive TH cells than healthy controls and produced significantly less inflammatory cytokines. No difference was seen in T cells reactive to the synaptic target LGI1 (Leucine-rich glioma-inactivated 1), ubiquitous Candida antigens or neoantigens, suggesting that the findings are disease-specific and not related to therapeutic immunosuppression. Also, patients with LGI1 encephalitis showed unaltered numbers of LGI1 antigen-reactive T cells. The data reveal disease-specific functional alterations of circulating NMDAR-reactive TH cells in patients with NMDAR encephalitis and challenge the idea that increased pro-inflammatory NMDAR-reactive T cells contribute to disease pathogenesis.


2018 ◽  
Vol 49 (16) ◽  
pp. 2709-2716 ◽  
Author(s):  
Ronald J. Gurrera

AbstractBackgroundAnti-NMDA receptor (NMDAr) encephalitis is the most common autoimmune encephalitis in adults. It mimics psychiatric disorders so often that most patients are initially referred to a psychiatrist, and many are misdiagnosed. Without prompt and effective treatment, patients are likely to suffer a protracted course with significant residual disability, or death. This study focuses on the frequency and chronology of salient clinical features in adults with anti-NMDAr encephalitis who are likely to be first evaluated by a psychiatrist because their presentation suggests a primary psychiatric disorder.MethodsA systematic search of PubMed and EMBASE databases identified published reports of anti-NMDAr encephalitis associated with prominent behavioral or psychiatric symptoms. After eliminating redundancies, the frequencies and relative timing of clinical features were tabulated. Signs and symptoms were assigned temporal ranks based on the timing of their first appearance relative to the first appearance of other signs and symptoms in each patient; median ranks were used to compare temporal sequencing of both individual features and major symptom domains.ResultsTwo hundred thirty unique cases (185 female) met study inclusion criteria. The most common features were seizures (60.4%), disorientation/confusion (42.6%), orofacial dyskinesias (39.1%), and mutism/staring (37.4%). Seizures, fever, and cognitive dysfunction were often the earliest features to emerge, but psychiatric features predominated and sequencing varied greatly between individuals.ConclusionsClinicians should consider anti-NMDAr encephalitis when new psychiatric symptoms are accompanied by a recent viral prodrome, seizures or unexplained fever, or when the quality of the psychiatric symptoms is unusual (e.g. non-verbal auditory hallucinations).


2021 ◽  
Vol 11 ◽  
Author(s):  
Gabriel de Albuquerque Vasconcelos ◽  
Rodrigo Montenegro Barreira ◽  
Karmelita Emanuelle Nogueira Torres Antoniollo ◽  
Alina Maria Nuñez Pinheiro ◽  
Cíntia Fernandes Rodrigues Maia ◽  
...  

Autoimmune encephalitis is an increasingly recognized cause of encephalitis. The majority of case series report patients residing in developed countries in the northern hemisphere. The epidemiologic features of autoimmune encephalitis in Latin America are still unclear. The aim of the study was to perform a review of the clinical presentation of autoimmune encephalitis in Latin America and compare to world literature. References were identified by an in-depth literature search and selected on the basis of relevance to the topic and authors' judgment. We selected clinical studies and case reports published from 2007 to July, 2020 including patients from Latin American countries. Of the 379 patients included, the majority were cases of anti-NMDA receptor encephalitis (93.14%), followed by anti-VGKC-complex encephalitis (N = 17; 4.48%), anti-GAD encephalitis (N = 9; 2.37%), anti-AMPA receptor encephalitis (N = 1; 0.26%), anti-GABA receptor encephalitis (N = 1; 0. 26%), anti-mGluR5 encephalitis (N = 1; 0. 26%), and anti-mGluR1 encephalitis (N = 1; 0. 26%). Reported cases of Anti-NMDA encephalitis in Latin-America had a very slight female predominance, lower prevalence of associated tumors and a lower incidence of extreme delta brush on electroencephalogram. Autoimmune encephalitis is possibly underdiagnosed in underdeveloped countries. Its outcome after treatment, however, appears to be similarly favorable in Latin American patients as has been reported in developed countries based on available case reports and case series. Regional specificities in the manifestation of autoimmune encephalitis could be related to epidemiologic factors, such as the presence of different triggers and different genetic and immunologic background, that need to be studied by future research.


Author(s):  
A.K. Gergen ◽  
P. Hosokawa ◽  
C. Irwin ◽  
M.J. Cohen ◽  
F.L. Wright ◽  
...  

Objectives: Elderly patients requiring emergency general surgery (EGS) are at high risk for complications due to preexisting malnutrition. Thus, correcting nutritional deficits perioperatively is essential to improve outcomes. However, even in patients unable to tolerate enteral nutrition, initiation of parenteral nutrition (PN) is often delayed due to concerns of associated complications. In this study, we hypothesized that in elderly EGS patients with relative short-term contraindications to enteral nutrition, early administration of PN is as safe as delayed administration. Furthermore, early PN may improve outcomes by enhancing caloric intake and combatting malnutrition in the immediate perioperative period. Design and Setting: A single-institution, retrospective review was performed at a quaternary academic medical center. Participants: Participants consisted of 58 elderly patients >65 years of age admitted to the EGS service who required PN between July 2017 and July 2020. Measurements: Postoperative outcomes of patients started on PN on hospital day 0-3 (early initiation) were compared to patients started on PN on hospital day 4 or later (late initiation). Bivariate analysis was conducted using the Chi-square or Fisher’s exact test for categorical variables and the Wilcoxon-Mann-Whitney test and F-test for continuous variables. Results: Fifty-eight patients met inclusion criteria, with 27 (46.6%) patients receiving early PN and 31 (53.4%) receiving late PN. Both groups shared similar baseline characteristics, including degree of frailty, body mass index, and nutritional status at time of admission. Complications associated with PN administration were negligible, with no instances of central venous catheter insertion-related complications, catheter-associated bloodstream infection, or factors leading to early termination of PN therapy. A significantly higher proportion of patients in the early administration group met 60% of their caloric goal within 72 hours of admission (62.9% versus 19.5%, p=0.0007). Patients receiving late PN demonstrated a significantly higher rate of unplanned admission to the intensive care unit (38.7% versus 14.8%, p=0.04). Moreover, there was a 21.5% reduction in mortality among patients in the early initiation group compared to patients in the late initiation group (33.3% versus 54.8%, p=0.10). Conclusions: Early initiation of PN in hospitalized elderly EGS patients was not associated with increased adverse events compared to patients undergoing delayed PN administration. Furthermore, patients receiving early PN demonstrated a 2.6-fold decrease in the rate of unplanned admission to the intensive care unit and trended toward improved mortality. Based on these results, further prospective studies are warranted to further explore the safety and potential benefits of early PN administration in elderly surgical patients unable to receive enteral nutrition.


2002 ◽  
Vol 9 (5) ◽  
pp. 987-993
Author(s):  
M. Kameswaran ◽  
K. Shetty ◽  
M. K. Ray ◽  
M. A. Jaleel ◽  
G. V. Kadival

ABSTRACT A radioassay for the detection of antitubercular antibody has been developed. The technique involves the addition of 125I-labeled Mycobacterium tuberculosis antigen as a tracer, diluted clinical sample (serum or cerebrospinal fluid [CSF]), and heat-inactivated Staphylococcus aureus to capture the antibody, incubation for 4 h, and quantitation of the amount of antibody present in the sample. A total of 330 serum samples from patients with pulmonary tuberculosis and 138 control serum samples from individuals who were vaccinated with M. bovis BCG and from patients with pulmonary disorders of nontubercular origin were analyzed. Also, 26 CSF samples from patients with tuberculous meningitis and 24 CSF samples as controls from patients with central nervous system disorders of nontuberculous origin were analyzed. Sensitivities of 80 and 73% were observed for patients with pulmonary tuberculosis and tuberculous meningitis, respectively, and specificities of 90 and 88% were seen for the two groups of patients, respectively. The sensitivity was lower, however, for human immunodeficiency virus-infected patients coinfected with M. tuberculosis. The control population could be differentiated from the patient population. This assay is rapid and user friendly and, with its good sensitivity and specificity, should benefit the population by providing diagnoses early in the course of disease and, hence, permit the early administration of appropriate chemotherapy.


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