Anti- NMDAR Encephalitis: a House Afire

2012 ◽  
Vol 2 (4) ◽  
pp. 39-40
Author(s):  
Aldrin Anthony Dung Dung ◽  
◽  
Akhila Kumar Panda ◽  
Vachan Jayant Mehta
Keyword(s):  
2014 ◽  
Vol 45 (S 01) ◽  
Author(s):  
A. Berger ◽  
L. Schmid ◽  
C. Betzler ◽  
C. Bien ◽  
K. Rostasy ◽  
...  

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.


2021 ◽  
pp. 17-23
Author(s):  
Koji Obara ◽  
Tomoko Ono ◽  
Itaru Toyoshima

In anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis, progressive cerebellar atrophy potentially leads to severe sequelae. We encountered a patient with anti-NMDAR antibody encephalitis who showed a decrease of blood flow in the cerebellum. A 15-year-old girl presented with consciousness disturbance. Influenza encephalopathy was suspected, and she was treated with glucocorticoid pulse therapy, high-dose intravenous immunoglobulins, and plasma exchange sequentially. She subsequently underwent left oophorectomy due to the presence of anti-NMDAR antibodies and a left ovarian teratoma. In spite of the surgery, her neuropsychiatric symptoms persisted, and she recovered slowly after the introduction of oral methotrexate (MTX). Sequential cerebral blood flow monitoring with single-photon emission computed tomography showed marked cerebellar hypoperfusion. Although mild impairments including working memory and verbal fluency persisted, she eventually returned to high school 3 years after onset. Profound cerebellar hypoperfusion including lobules VI and VII may be the reason for her working memory impairment and speaking problems. Oral MTX may be a promising alternative treatment for some refractory cases of anti-NMDAR encephalitis.


CNS Spectrums ◽  
2019 ◽  
Vol 25 (4) ◽  
pp. 475-492 ◽  
Author(s):  
Inês Marques Macedo ◽  
João Gama Marques

AbstractAnti-N-methyl-D-aspartate receptor (NMDAr) encephalitis is a relatively recent autoimmune entity, as it was first described in 2007. Given that it is a condition with neuropsychiatric symptoms, its initial symptom is frequently psychiatric in nature. Hence, psychiatrists are often the first physicians to assess these patients and, as so, must recognize this type of encephalitis as a possible cause. Catatonia may be inaugural or develop throughout the course of the disease. Management of patients with anti-NMDAr encephalitis is based on etiologic treatment with immunotherapy and removal of the associated tumor, if any. However, these catatonic patients may have variable responses to etiologic treatment, sometimes with refractory catatonic symptoms, which attests to the necessary urgency to know how to manage these patients. In the clinical setting, physicians appear to be using guidelines originally created to the management of catatonia due to primary psychiatric conditions. In this literature review, catatonia was historically contextualized and anti-NMDAr encephalitis overall described. Finally, catatonia secondary to this type of encephalitis was discussed.


Biomolecules ◽  
2019 ◽  
Vol 9 (10) ◽  
pp. 572 ◽  
Author(s):  
Wang

MicroRNA (miRNA) is a small non-coding RNA that functions in the epigenetics control of gene expression, which can be used as a useful biomarker for diseases. Anti-NMDA receptor (anti-NMDAR) encephalitis is an acute autoimmune disorder. Some patients have been found to have tumors, specifically teratomas. This disease occurs more often in females than in males. Most of them have a significant recovery after tumor resection, which shows that the tumor may induce anti-NMDAR encephalitis. In this study, I review microRNA (miRNA) biomarkers that are associated with anti-NMDAR encephalitis and related tumors, respectively. To the best of my knowledge, there has not been any research in the literature investigating the relationship between anti-NMDAR encephalitis and tumors through their miRNA biomarkers. I adopt a phylogenetic analysis to plot the phylogenetic trees of their miRNA biomarkers. From the analyzed results, it may be concluded that (i) there is a relationship between these tumors and anti-NMDAR encephalitis, and (ii) this disease occurs more often in females than in males. This sheds light on this issue through miRNA intervention.


Author(s):  
Miguel Restrepo-Martínez ◽  
Jesús Ramírez-Bermúdez ◽  
Leo Bayliss ◽  
Mariana Espinola-Nadurille
Keyword(s):  

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