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 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 ◽  
Magnetic Resonance ◽  
Magnetic Resonance Image ◽  
Brain Mri ◽  
Clinical Manifestations ◽  
Ovarian Teratoma ◽  
Mass Effects ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis ◽  
Brain Magnetic Resonance Image

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. Key words: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis, clinic, brain, magnetic resonance image

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 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.

N-Methyl-D-Aspartate Receptor Encephalitis with Psychiatric Symptoms and an Ovarian Teratoma Detected by MRI in a 17-Year-Old Girl

2019 ◽  
Vol 50 (04) ◽  
pp. 253-256
Author(s):  
A. Barth ◽  
I. Nassenstein ◽  
R.B. Tröbs ◽  
A. Tannapfel ◽  
H. Dercks ◽  
...  
Keyword(s):  
Magnetic Resonance ◽  
Mr Imaging ◽  
Ovarian Tumor ◽  
Psychiatric Symptoms ◽  
Autoimmune Encephalitis ◽  
Adolescent Females ◽  
Ovarian Teratoma ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis

AbstractN-methyl-D-aspartate receptor (NMDAR) encephalitis is a rare antibody-mediated autoimmune encephalitis often associated with an ovarian teratoma in adolescent females. Here we present a 17-year-old girl with only and unusual psychiatric symptoms as part of her NMDAR encephalitis in combination with a very small ovarian teratoma suspected by magnetic resonance (MR) imaging and finally histologically confirmed. We further review the literature of NMDAR encephalitis in combination with an ovarian teratoma and discuss the recommended radiological workup in children with a suspected ovarian tumor.

scholarly journals Distinct cerebral 18F-FDG PET metabolic patterns in anti-N-methyl-D-aspartate receptor encephalitis patients with different trigger factors

2021 ◽  
Vol 14 ◽  
pp. 175628642199563
Author(s):  
Jingjie Ge ◽  
Bo Deng ◽  
Yihui Guan ◽  
Weiqi Bao ◽  
Ping Wu ◽  
...  
Keyword(s):  
Positron Emission Tomography ◽  
Basal Ganglia ◽  
Viral Encephalitis ◽  
Emission Tomography ◽  
Ovarian Teratoma ◽  
Trigger Factors ◽  
Temporal Lobes ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis ◽  
Positron Emission

Aim: Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a subgroup of treatable autoimmune encephalitis, characterized by rapid development of psychosis, cognitive impairments and seizures. Etiologically, anti-NMDAR encephalitis could be divided into three subgroups, which are paraneoplastic (especially associated with ovarian teratoma), viral encephalitis-related and cryptogenic. Each type is different in clinical course, treatment strategies and prognosis. In this study, we aim to investigate whether anti-NMDAR encephalitis patients with different trigger factors exhibit distinct cerebral metabolic patterns detected by 18F-fluorodeoxyglucose positron emission tomography imaging. Methods: 24 patients with anti-NMDAR encephalitis in acute phase from Huashan Hospital, Fudan University (Shanghai, China) were recruited in this study. Each patient was classified into one of etiological subgroups. Positron emission tomography images of individual patients were analyzed with both routine visual reading and computer-supported reading by comparison with those of the same 10 healthy controls using a voxel-wise statistical parametric mapping analysis. Results: Patients in both the cryptogenic (13 patients) and paraneoplastic (five patients) subgroups showed hypermetabolism in the frontal-temporal lobes and basal ganglia, covarying with hypometabolism in the occipital regions. Notably, the abnormal metabolism was usually asymmetric in the cryptogenic subgroup, but relatively symmetric in the paraneoplastic subgroup. Moreover, the other six patients secondary to viral encephalitis presented with significant hypometabolism in the bilateral occipital regions, as well as in the unilateral temporal lobes and part of basal ganglia (also is virus infection side), but hypermetabolism in the contralateral temporal areas. Conclusion: This study revealed that patients with anti-NMDAR encephalitis triggered by different factors presented distinct cerebral metabolic patterns. Awareness of these patterns may help to better understand the varying occurrence and development of anti-NMDAR encephalitis in each subgroup, and could offer valuable information to the early diagnosis, treatment and prognosis of this disorder. Trial registration number ChiCTR2000029115 (Chinese clinical trial registry site, http://www.chictr.org )

scholarly journals Anti-N-methyl-D-aspartate receptor encephalitis presenting as atypical psychosis in multiple sclerosis: a case report

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Khushminder Chahal ◽  
Tara Endeman ◽  
Sarah Scapinello ◽  
Michal Sapieha
Keyword(s):  
Multiple Sclerosis ◽  
Psychiatric Symptoms ◽  
Neuropsychiatric Symptoms ◽  
Psychiatric Inpatient ◽  
Treatment Protocol ◽  
Autoimmune Encephalitis ◽  
Ovarian Teratoma ◽  
Atypical Symptoms ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis

Abstract Background Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is an autoimmune disorder which often presents with neuropsychiatric symptoms. A large proportion of cases are associated with an identifiable tumor, most commonly ovarian teratoma. However, recent literature has also described an overlap of anti-NMDAR encephalitis and demyelinating syndromes. Cases have been reported of anti-NMDAR encephalitis in patients with ADEM, optic neuritis, myelitis and multiple sclerosis. This link is considered rare, however has important clinical implications as treatments and prognosis may differ. Case presentation A 33-year-old female with a history of multiple sclerosis presented with new-onset neuropsychiatric symptoms. After substance-induced psychosis was ruled out, she was admitted to the medical ward for work up of psychosis secondary to multiple sclerosis. However, the consultation-liaison psychiatry service noted atypical symptoms which were concerning for autoimmune encephalitis. Admission to a psychiatric inpatient ward was deferred. Anti-NMDAR encephalitis was diagnosed with CSF analysis demonstrating lymphocytic pleocytosis and anti-NMDAR antibodies. In addition to first-line treatment of encephalitis with steroids, second-line immunotherapies were also implemented given the patient’s underlining demyelinating syndrome. The patient’s neurologic and psychiatric symptoms began to improve. Conclusions There is literature to demonstrate a possible connection between anti-NMDAR encephalitis and demyelinating syndromes. As such, autoimmune encephalitis should be considered in patients with multiple sclerosis presenting with atypical symptoms. Determining the correct diagnosis is crucial to inform the appropriate treatment protocol, and to improve prognosis.

scholarly journals Clinical Characteristics and Outcome of Neuronal Surface Antibody-Mediated Autoimmune Encephalitis Patients in a National Cohort

2021 ◽  
Vol 12 ◽  
Author(s):  
Zsófia Hayden ◽  
Beáta Bóné ◽  
Gergely Orsi ◽  
Monika Szots ◽  
Ferenc Nagy ◽  
...  
Keyword(s):  
Clinical Characteristics ◽  
Clinical Symptoms ◽  
Psychiatric Symptoms ◽  
Brain Mri ◽  
Disease Onset ◽  
Neuronal Cell ◽  
Autoimmune Encephalitis ◽  
Ovarian Teratoma ◽  
Test Results ◽  
Nmdar Encephalitis

Background: In our previous single-center study of autoimmune encephalitis (AE) related autoantibody test results we found positivity in 60 patients out of 1,034 with suspected AE from 2012 through 2018 as part of a Hungarian nationwide program. In our current multicenter retrospective study, we analyzed the clinical characteristics and outcome of AE patients with positive neuronal cell surface autoantibody test results.Methods: A standard online questionnaire was used to collect demographic and clinical characteristics, laboratory and imaging data, therapy and prognosis of 30 definitive AE patients in four major clinical centers of the region.Results: In our study, 19 patients were positive for anti-NMDAR (63%), 6 patients (20%) for anti-LGI1, 3 patients for anti-GABABR (10%) and 3 patients for anti-Caspr2 (10%) autoantibodies. Most common prodromal symptoms were fever or flu-like symptoms (10/30, 33%). Main clinical features included psychiatric symptoms (83%), epileptic seizures (73%) and memory loss (50%). 19 patients (63%) presented with signs of central nervous system (CNS) inflammation, which occurred more frequently in elder individuals (p = 0.024), although no significant differences were observed in sex, tumor association, time to diagnosis, prognosis and immunotherapy compared to AE patients without CNS inflammatory markers. Anti-NMDAR encephalitis patients were in more severe condition at the disease onset (p = 0.028), although no significant correlation between mRS score, age, sex and immunotherapy was found. 27% of patients (n = 8) with associated tumors had worse outcome (p = 0.045) than patients without tumor. In most cases, immunotherapy led to clinical improvement of AE patients (80%) who achieved a good outcome (mRS ≤ 2; median follow-up 33 months).Conclusion: Our study confirms previous publications describing characteristics of AE patients, however, differences were observed in anti-NMDAR encephalitis that showed no association with ovarian teratoma and occurred more frequently among young males. One-third of AE patients lacked signs of inflammation in both CSF and brain MRI, which emphasizes the importance of clinical symptoms and autoantibody testing in diagnostic workflow for early introduction of immunotherapy, which can lead to favorable outcome in AE patients.

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 Anti-N-Methyl-D-Aspartate Receptor Encephalitis with Ovarian Teratoma Improved by Prompt Surgery

2021 ◽  
Vol 39 (4) ◽  
pp. 340-342
Author(s):  
Seok-Yeol Yang ◽  
Wooryang Byun ◽  
Sung-Pa Park ◽  
Jong-Geun Seo
Keyword(s):  
Cerebrospinal Fluid ◽  
Clinical Outcome ◽  
Clinical Symptoms ◽  
Psychiatric Symptoms ◽  
Clinical Manifestations ◽  
Early Surgery ◽  
Ovarian Teratoma ◽  
Aspartate Receptor ◽  
Early Removal ◽  
Nmdar Encephalitis

Anti-N-methyl-D aspartate receptor (NMDAR) encephalitis is often accompanied with ovarian teratomas. It has a variety of clinical manifestations including psychiatric symptoms, seizure, and motor dysfunctions. The diagnosis can be definite when clinical symptoms are present and anti-NMDAR antibodies in cerebrospinal fluid are detected. However, in patients with suspected anti-NMDAR encephalitis with teratomas, early surgery may help the clinical outcome even if the antibodies are initially negative. The authors report a patient whose clinical symptoms improved significantly after early removal of teratoma.

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