Anti-NMDA receptor encephalitis

2021 ◽  
Vol 2 (3) ◽  
pp. 137-145
Author(s):  
Umida M. Azizova ◽  
Raisa Ts. Bembeeva ◽  
Anastasia A. Kozyreva ◽  
Nikolay N. Zavadenko
Keyword(s):  
Biological Fluids ◽  
Clinical Manifestations ◽  
Diagnostic Errors ◽  
Autoimmune Encephalitis ◽  
Diagnostic Algorithm ◽  
Epidemiological Data ◽  
Healthy Person ◽  
Favourable Outcome ◽  
Neurological Deficits ◽  
Nmdar Encephalitis

Autoimmune diseases of the central nervous system (CNS) are one of the most socially and economically significant problems of neurology. Despite the identification of new nosological forms of autoimmune encephalitis, the creation of diagnostic panels for the verification of autoantibodies in biological fluids, and the use of highly effective pathogenetic therapy, the number of diagnostic errors remains high, which poses a threat to the patient’s life and a high risk of developing severe complications. Anti-N-methyl-D-aspartate receptor encephalitis (anti-NMDAR encephalitis) is autoimmune encephalitis caused by the presence of antibodies (Ab) to the NR1 subunit of NMDA-receptors (NMDAR) characterized by the development of severe mental and neurological deficits in a previously healthy person. This article summarizes the recent literature on anti-NMDAR encephalitis. The literature search was carried out using the Scopus, Web of Science, Pubmed, CyberLeninka databases. The review presents the facts of the history of the study of the disease, epidemiological data, modern ideas about the pathogenetic mechanisms of the development of the disease, the spectrum of clinical manifestations and various forms of the course of the disease. The diagnostic criteria and research methods used to confirm the diagnosis are described, approaches to the treatment of anti-NMDAR encephalitis are outlined. Anti-NMDAR encephalitis is clinically manifested by a combination of mental disorders, epileptic seizures, speech and extrapyramidal disorders, and disturbances in the rhythm of sleep and wakefulness. The disease occurs at any age. The development of the disease can be associated with such immunological triggers as oncological process and herpetic encephalitis, or be idiopathic in nature. There are features of the course of the clinical picture depending on the age of the patient, paraneoplastic or postherpetic aetiology of the disease. The diagnostic algorithm, along with neuroimaging, determination of specific antibodies, electroencephalography, should also include the search for an oncological process. The recovery of patients can take from several months to years. In some cases, persistent neurological deficits develop. Predictors of a favourable outcome include early initiation and use of combination therapy, detection and removal of neoplasms, a low titer of anti-NMDAR antibodies, and age of patients over 12 years of age. In up to 25% of cases, a relapsing course of the disease is possible, and therefore requires long-term monitoring of these patients.

Anti-N-methyl-D-aspartate receptor encephalitis and ovarian teratomas: is there any association?

2020 ◽  
Vol 19 (5) ◽  
pp. 124-131
Author(s):  
N.M. Podzolkova ◽  
◽  
V.V. Korennaya ◽  
O.S. Levin ◽  
E.E. Vasenina ◽  
...  
Keyword(s):  
Pathological Condition ◽  
Rare Complication ◽  
Autoimmune Encephalitis ◽  
Neurological Deficits ◽  
Ovarian Teratoma ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis ◽  
Life Threatening ◽  
Causal Treatment ◽  
Ovarian Teratomas

This article aims to explore the problem of anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis and also describes our own experience in treating anti-NMDAR encephalitis in patients with ovarian teratomas. This pathological condition is a rare complication of cancer. Practicing gynecologists are often unfamiliar with it, although its consequences can be life-threatening. Early diagnosis and causal treatment, particularly teratoma removal, are critical for the outcome and can prevent death or the development of neurological deficits in patients. Key words: anti-NMDAR, autoimmune encephalitis, ovarian cysts, complications, ovarian teratoma, encephalitis, encephalitis in women

scholarly journals The diffuse involvement of anti-N-methyl-D-aspartate receptor encephalitis in brain: a case report

BMC Neurology ◽  
2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Yun Jiang ◽  
Jianpeng Ma ◽  
Tao Gong ◽  
Hongjun Hao ◽  
Haibo Chen
Keyword(s):  
Basal Ganglia ◽  
Brain Mri ◽  
Clinical Manifestations ◽  
Autoimmune Encephalitis ◽  
Ovarian Teratoma ◽  
Mass Effects ◽  
Glucose Levels ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis ◽  
Case Presentations

Abstract Background Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a severe and most common autoimmune encephalitis in patients under 40 years old. Anti-NMDAR encephalitis has various clinical and neuroimaging findings. Here we report a special case of an anti-NMDAR encephalitis who had diffuse lesions in bilateral hemispheres with mild mass effects in left basal ganglia area. Case presentations A 28-year-old female anti-NMDAR encephalitis patient mainly presented with headache and fever. Brain magnetic resonance image (MRI) showed slightly contrasted diffuse lesions, involving the left temporal and frontal lobes, left basal ganglia area and splenium of corpus callosum, as well as the right frontal lobe, with mild edema surrounded in the left basal ganglia area. Cerebrospinal fluid (CSF) revealed a moderate pleocytosis with normal protein and glucose levels. Anti-NMDAR antibodies were identified in CSF. Transvaginal ovarian ultrasound did not reveal an ovarian teratoma. The patient was treated with immunoglobulin and steroid, and had a good recovery. Conclusions Anti-NMDAR encephalitis has no special clinical manifestations and brain MRI is highly variable, which could be unremarkable or abnormal involving white and grey matters. The extensive lesions in frontal and temporal lobes, and basal ganglia area, with mild mass effects, have not been described previously. Recognition of various changes in brain MRI will enable the early detection of anti-NMDAR antibody and then effective treatments.

scholarly journals The diffuse involvement of Anti-N-methyl-D-aspartate receptor encephalitis in brain: a case report

2019 ◽  
Author(s):  
Yun Jiang ◽  
Jianpeng Ma ◽  
Tao Gong ◽  
Hongjun Hao ◽  
Haibo Chen
Keyword(s):  
Basal Ganglia ◽  
Brain Mri ◽  
Clinical Manifestations ◽  
Autoimmune Encephalitis ◽  
Ovarian Teratoma ◽  
Mass Effects ◽  
Glucose Levels ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis ◽  
Case Presentations

Abstract Background Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a severe and most common autoimmune encephalitis in patients under 40 years old. The anti-NMDAR encephalitis has various clinical and neuroimaging findings. Here we report a special anti-NMDAR encephalitis patient who had diffuse lesions in bilateral hemispheres with mild mass effects in left basal ganglia area. Case presentations A 28-year-old female anti-NMDAR encephalitis patient mainly presented with headache and fever. Brain magnetic resonance image (MRI) showed slightly contrasted diffuse lesions, involving the left temporal and frontal lobes, left basal ganglia area and splenium of corpus callosum, and the right frontal lobe, with mild edema surrounded in the left basal ganglia area. Cerebrospinal fluid (CSF) revealed a moderate pleocytosis with normal protein and glucose levels. Anti-NMDAR antibodies were identified in CSF. Transvaginal ovarian ultrasound did not reveal an ovarian teratoma. The patient was treated with immunoglobulin and steroid, and had a good recovery. Conclusions The anti-NMDAR encephalitis has no special clinical manifestations and brain MRI is highly variable, which could be unremarkable or abnormal involving white and grey matters. The extensive lesions in frontal and temporal lobes, and basal ganglia area, with mild mass effects, have not been described previously. The recognition of various changes in brain MRI will enable the early detection of anti-NMDAR antibody and then effective treatments.

scholarly journals Current Progress on Assessing the Prognosis for Anti-N-Methyl-D-Aspartate Receptor (NMDAR) Encephalitis

2020 ◽  
Vol 2020 ◽  
pp. 1-8
Author(s):  
Hao Wang ◽  
Zheng Xiao
Keyword(s):  
Positive Impact ◽  
Treatment Options ◽  
Clinical Manifestations ◽  
Autoimmune Encephalitis ◽  
Antibody Testing ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis ◽  
Behavioral Abnormalities ◽  
Positron Emission ◽  
The Impact

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is the most common antineuronal antibody encephalitis in autoimmune encephalitis found at present. It has complex clinical manifestations such as psychiatric and behavioral abnormalities, seizures, movement disorders, consciousness, and autonomic dysfunction. The relationship between those manifestations and prognosis is unclear. Electroencephalography (EEG) is gradually becoming useful in the evaluation of the prognosis of anti-NMDAR encephalitis patients, while imaging and antibody testing have a limited prognostic value. Starting the patients on adequate treatments (such as immunotherapy) in a timely fashion has a positive impact on their prognosis. Nevertheless, research on the prognosis of anti-NMDAR encephalitis remains scarce. Here, we review the current advances of prognosis-related research from the clinical manifestations of the disease and auxiliary examinations such as EEG, magnetic resonance imaging (MRI), 18F fluorodeoxyglucose positron emission tomography (FDG-PET), and antibody measurement. In addition, we also discuss the impact of different treatment options on prognosis. In-depth research on the prognosis of patients with anti-NMDAR encephalitis will contribute to a better understanding of this disease, leading to better treatments options and, ultimately, a better prognosis.

CLINICAL MANIFESTATIONS OF ANTI-NMDA RECEPTOR ENCEPHALITIS IN CHILDREN

2021 ◽  
Vol 100 (6) ◽  
pp. 113-120
Author(s):  
R.Ts. Bembeeva ◽  
◽  
U.M. Azizova ◽  
N.N. Zavadenko ◽  
E.S. Ilyina ◽  
...  
Keyword(s):  
Clinical Symptoms ◽  
Clinical Manifestations ◽  
Autoimmune Encephalitis ◽  
Neurological Dysfunction ◽  
Long Term Results ◽  
Impulsive Behavior ◽  
Speech Impairment ◽  
Prodromal Phase ◽  
Nmdar Encephalitis

The aim of the study was to determine the clinical features of anti-NMDAR encephalitis in children. Materials and methods of research: 11 patients were selected from the group of children with autoimmune encephalitis, who met the diagnostic criteria for reliable anti-NMDAR encephalitis. A retro-prospective analysis of clinical symptoms, laboratory, neurophysiological and neuroimaging data, treatment, duration of primary hospitalization and long-term results of treatment was carried out, neuropsychological testing of patients was performed in the follow-up. Results: the age of the patients was 8.5±4.4 years, the gender composition of boys/girls was 1/10 (9.1%/90.9%). The average follow-up period was 17±12 months. In 73% of cases, there was an acute onset of the disease without a prodromal phase and with rapidly growing signs of neurological dysfunction: behavior change (11/100%), epileptic seizures (11/100%), speech impairment (10/90.9%), movement disorders (10/90.9%), disturbed sleep/wakefulness rhythm (9/81.8%), hallucinations (5/45.5%), autonomic disorders (6/54.5%), sensory disturbance (1/9,1%). In 82% of cases, therapy was required in the intensive care unit. When analyzing EEG monitoring in children, the extreme delta brush pattern was revealed in only one patient. Neuroimaging revealed no specific changes in the substance of the brain. The paraneoplastic nature of the disease could not be established in any case. 4 patients (36.36%) had relapses of the disease after 1.5–27 months. from the onset of the disease. All children showed a favorable outcome without a gross neurological deficit, but complaints of increased fatigue, headaches, poor memory, a decrease in vocabulary, and impulsive behavior persisted.

scholarly journals Anti-NMDAR autoimmune encephalitis in children and herpes simplex virus-1

2019 ◽  
Vol 64 (3) ◽  
pp. 17-27
Author(s):  
E. Yu. Skripchenko ◽  
G. F. Zheleznikova ◽  
N. V. Skripchenko ◽  
L. A. Alekseeva ◽  
A. V. Surovtseva
Keyword(s):  
Herpes Simplex Virus ◽  
Herpes Simplex ◽  
Herpes Simplex Virus Infection ◽  
Clinical Manifestations ◽  
Autoimmune Encephalitis ◽  
Herpes Simplex Virus 1 ◽  
Nmdar Encephalitis ◽  
Adults And Children ◽  
The Central Nervous System ◽  
Simplex Virus

The review focuses on autoimmune encephalitis with antibodies to NMDA receptors, previously unknown autoimmune disease of the central nervous system, described in 2007. Anti-NMDAR encephalitis develops in adults and children, due to the tumor process and without it, it is manifested by characteristic syndrome of neurological and mental disorders. The authors separate anti-NMDAR encephalitis, which develops after the resolution of encephalitis caused by the herpes simplex virus-1. They describe the clinical manifestations of anti-NMDAR encephalitis in children and the effectiveness of early immunotherapy. Perspectively they discuss the pathogenesis of the disease, as well as the connection of herpes simplex virus infection and the development of anti-NMDAR encephalitis.

scholarly journals Algorithm of Radiological Diagnostics of Dystrophic Damage to Ligaments of the Lumbosacral Spine in the Lower Back Pain Syndrome: the Potential of Sonography

2018 ◽  
pp. 109-114
Author(s):  
A. N. Mihailov ◽  
A. M. Yurkovskiy ◽  
I. V. Nazarenko
Keyword(s):  
Back Pain ◽  
Lower Back Pain ◽  
Structural Changes ◽  
Clinical Manifestations ◽  
Diagnostic Errors ◽  
Diagnostic Algorithm ◽  
Pain Syndrome ◽  
Lumbosacral Spine ◽  
Lower Back ◽  
Magnetic Resonance Imaging Mri

Objective: to formulate the main points of the diagnostic algorithm in the lower back pain syndrome caused by pathology of the ilio-lumbar, posterior long sacroiliac, and sacrotuberal ligaments based on the limits and potential of visualization methods. Material. Data of X-ray, multispiral computer tomography (MSCT), magnetic resonance imaging (MRI) and sonography of 184 patients aged 19-79 with clinical manifestations of the lower back pain syndrome caused by pathology of the ilio-lumbar, posterior long sacroiliac, and sacrotuberal ligaments were analyzed. Results. The potential of the visualization methods in the assessment of structural changes of the ilio-lumbar, posterior long sacroiliac, and sacrotuberal ligaments has been identified. The main points of the algorithm which makes it possible to diagnose the lower back pain syndrome, caused by pathology of ilio-lumbar, posterior long sacroiliac and sacrotuberal ligaments have been formulated. Conclusion. The choice of the visualization method in the lower back pain syndrome, caused by ligamentosis of the ilio-lumbar, posterior long sacroiliac, and sacrotuberal ligaments must be made taking into account the limits and potential of MSCT, MRI and sonography, which will shorten the time of diagnostic search and reduce the risk of diagnostic errors.

scholarly journals Anti-N-Methyl-D-aspartate Receptor Encephalitis: A Severe, Potentially Reversible Autoimmune Encephalitis

2017 ◽  
Vol 2017 ◽  
pp. 1-14 ◽  
Author(s):  
Cai-yun Liu ◽  
Jie Zhu ◽  
Xiang-Yu Zheng ◽  
Chi Ma ◽  
Xu Wang
Keyword(s):  
Clinical Course ◽  
Clinical Manifestations ◽  
Autoimmune Encephalitis ◽  
Multidisciplinary Care ◽  
Autoimmune Disorder ◽  
Pathogenic Role ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis ◽  
Characteristic Features ◽  
Demyelinating Disorders

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is potentially lethal, but it is also a treatable autoimmune disorder characterized by prominent psychiatric and neurologic symptoms. It is often accompanied with teratoma or other neoplasm, especially in female patients. Anti-NMDAR antibodies in cerebrospinal fluid (CSF) and serum are characteristic features of the disease, thereby suggesting a pathogenic role in the disease. Here, we summarize recent studies that have clearly documented that both clinical manifestations and the antibodies may contribute to early diagnosis and multidisciplinary care. The clinical course of the disorder is reversible and the relapse could occur in some patients. Anti-NMDAR encephalitis coexisting with demyelinating disorders makes the diagnosis more complex; thus, clinicians should be aware of the overlapping diseases.

scholarly journals Decreased inflammatory cytokine production of antigen-specific CD4+ T cells in NMDA receptor encephalitis

2021 ◽  
Author(s):  
Le-Minh Dao ◽  
Marie-Luise Machule ◽  
Petra Bacher ◽  
Julius Hoffmann ◽  
Lam-Thanh Ly ◽  
...  
Keyword(s):  
T Cells ◽  
T Cell ◽  
Ex Vivo ◽  
Autoimmune Encephalitis ◽  
Active Immunization ◽  
Inflammatory Cytokine Production ◽  
T Cell Infiltration ◽  
Nmda Receptor Encephalitis ◽  
Nmdar Encephalitis ◽  
Disease Specific

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.

scholarly journals Gasperini Syndrome: A Case Report and Systematic Review and Proposing a New Definition

2020 ◽  
Vol 12 (3) ◽  
pp. 433-439
Author(s):  
Riwaj Bhagat ◽  
Siddharth Narayanan ◽  
Marwa Elnazeir ◽  
Thong Diep Pham ◽  
Robert Paul Friedland ◽  
...  
Keyword(s):  
Systematic Review ◽  
Brain Mri ◽  
Cranial Nerves ◽  
Clinical Manifestations ◽  
The Other ◽  
Neurological Deficits ◽  
Brainstem Stroke ◽  
Neurological Features ◽  
The Right ◽  
Core Features

Gasperini syndrome (GS), a rare brainstem syndrome, is featured by ipsilateral cranial nerves (CN) V–VIII dysfunction with contralateral hemibody hypoesthesia. While there have been 18 reported cases, the GS definition remains ambiguous. We report a new case and reviewed the clinical features of this syndrome from all published reports to propose a new definition. A 57-year-old man with acute brainstem stroke had right CN V–VIII and XII palsies, left body hypoesthesia and ataxia. Brain MRI showed an acute stroke in the right caudal pons and bilateral cerebellum. After a systematic review, we classified the clinical manifestations into core and associate features based on the frequencies of occurring neurological deficits. We propose that a definitive GS requires the presence of ipsilateral CN VI and VII palsies, plus one or more of the other three core features (ipsilateral CN V, VIII palsies and contralateral hemibody hemihypalgesia). Additionally, GS, similar to Wallenberg’s syndrome, represents a spectrum that can have other associated neurological features. The revised definition presented in this study may enlighten physicians with the immediate recognition of the syndrome and help improve clinical localization of the lesions and its management.

Sign in / Sign up

Export Citation Format

Share Document