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.

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 Anti-N-methyl-D-Aspartate Receptor Encephalitis Mimicking Sporadic Creutzfeldt–Jakob Disease

2020 ◽  
Vol 11 ◽  
Author(s):  
Jiao Liu ◽  
Liyan Chen ◽  
Jing Yang ◽  
Lan Wang ◽  
Huifang Shang ◽  
...  
Keyword(s):  
Psychiatric Symptoms ◽  
Clinical Manifestations ◽  
Progressive Dementia ◽  
Memory Problems ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis ◽  
Jakob Disease ◽  
Laboratory Investigations ◽  
Positive Results

Objectives: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis and sporadic Creutzfeldt–Jakob disease (sCJD) share similar clinical features. Here, we present two unusual cases of anti-NMDAR encephalitis who were misdiagnosed as sCJD at first.Methods: We described two patients' clinical manifestations, as well as the string of symptomatological evolution, treatments, and follow-up results.Results: Our patients presented with rapidly progressive dementia, memory problems, psychiatric symptoms, and movement disorders, and we considered all these symptoms as a presenting feature of sCJD at first, but the cerebrospinal fluid examination showed positive results for both the 14-3-3 protein and antibodies against NMDAR. Immunomodulatory treatment led to a resolution of these deficits, and both of them remained in remission after treatment.Conclusion: Anti-NMDAR encephalitis can present with rapidly progressive cognitive decline, and sometimes laboratory investigations can be misleading. The examination for the presence of NMDAR antibodies is necessary, even with the presence of 14-3-3 protein. Early immunomodulatory therapy should be considered, especially for patients with high titers of NMDAR antibodies.

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

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 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 Relevance of Cerebrospinal Fluid Antibody Titers in Anti-N-Methyl-d-Aspartate Receptor Encephalitis

2021 ◽  
Vol 12 (1) ◽  
pp. 4
Author(s):  
Meng-Ting Cai ◽  
Yang Zheng ◽  
Sa Wang ◽  
Qi-Lun Lai ◽  
Gao-Li Fang ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Psychiatric Symptoms ◽  
Autoimmune Encephalitis ◽  
Memory Deficits ◽  
Clinical Severity ◽  
Weak Correlation ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis ◽  
Significant Difference ◽  
The Relationship

Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is the most common autoimmune encephalitis. To date, there has been no study on the relationship between antibody (Ab) titers and clinical phenotype. This study aims to clarify the relationship between cerebrospinal fluid Ab titers and clinical manifestations of anti-NMDAR encephalitis at onset. Seventy-six consecutive patients with a definite diagnosis were enrolled. The relationship between Ab titers and different onset symptoms including psychiatric symptoms, seizures, and memory deficits were analyzed. We further investigated the correlation between Ab titers and clinical severity as assessed by the modified Rankin scale (mRS) and the clinical assessment scale for autoimmune encephalitis (CASE), respectively. The Ab titers had a median value of 1:10 (range 1:1–1:100). There was no significant difference in titers among various clinical factors including gender and combination of tumor and other diseases (each p > 0.05). Patients presenting with psychiatric symptoms at onset had higher titers than those with seizures (p = 0.008) and memory deficits (p = 0.003). The mRS scores revealed a significant but weak correlation with Ab titers (r = 0.243, p = 0.034), while CASE scores did not correlate with the titers (p = 0.125). Our findings indicated that the Ab titers were associated with the type of onset symptoms, with a higher level of patients with psychiatric symptoms. Regarding the clinical severity, the titers showed a weak correlation with the mRS, but no correlation with the CASE.

scholarly journals Ovarian teratoma-associated anti-NMDAR encephalitis: a single-institute series of six patients from China

2020 ◽  
Author(s):  
Minhua Yu ◽  
Shanji Li ◽  
Jie Cheng ◽  
Liche Zhou ◽  
Zhou Jiang ◽  
...  
Keyword(s):  
Psychiatric Symptoms ◽  
Clinical Manifestations ◽  
Tumor Location ◽  
Ovarian Teratoma ◽  
Psychotic Symptoms ◽  
Consciousness Disorders ◽  
Aspartate Receptor ◽  
Radiological Data ◽  
Mental Symptoms

Abstract Purpose Ovarian teratoma-associated anti-N-methyl-d-aspartate receptor encephalitis is a rare disease with uncertain etiology and pathogenesis. The disorder is severe and rare with a great impact on young adults. This study aimed to improve the awareness of the disease from experience in our single center. Methods Between July 2012 and December 2019, six patients with ovarian teratoma-associated anti-N-methyl-d-aspartate receptor encephalitis were enrolled in Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University. All patients’ data like manifestations, laboratory and radiological data, treatment, and follow-up were reviewed. Results Typical psychotic symptoms, memory, and consciousness disorders accompanied by seizures were observed in all patients from this study. All six patients showed positive signals in serum and cerebrospinal fluid samples for N-methyl-d-aspartate receptor and received immunotherapy. Three patients underwent unilateral oophorocystectomy and the other three underwent unilateral oophorectomy through minimally invasive surgeries, including laparoscopic and single-port laparoscopic surgeries. The median follow-up time 24.5 months (range from 6 to 93 months). No death occurred. Two patients had recurrent psychotic symptoms while the left four patients had no mental symptoms or tumor recurrence during postoperative follow-up. Conclusions For patients with clinical manifestations of unexplained acute psychiatric symptoms accompanied by seizures, memory, and consciousness disorders, the possibility of anti-N-methyl-d-aspartate receptor encephalitis should be considered. To confirm the diagnosis, examinations of anti-N-methyl-d-aspartate receptor antibodies need to be completed as early as possible. Immunotherapy and tumor location should be given in time once the diagnosis is defined. We recommended removing the tumor as soon as possible without concerning whether the patient is in the acute phase or not. The surgical procedure should be decided based on pathology, age, fertility desire, and patients’ requirements and it should be ensured that tumors are completely removed during operation. Postoperative follow-up is particularly important.

scholarly journals Gray Matter Atrophy And Corresponding Impairments In Connectivity In Patients With Anti-N-Methyl-D-Aspartate Receptor Encephalitis

2022 ◽  
Author(s):  
Yuanyuan Guo ◽  
Xinyi Lv ◽  
Juanjuan Zhang ◽  
Chenglong Li ◽  
Ling Wei ◽  
...  
Keyword(s):  
Gray Matter ◽  
Clinical Symptoms ◽  
Psychiatric Symptoms ◽  
Inferior Frontal Gyrus ◽  
Gray Matter Volume ◽  
Cognitive Assessments ◽  
Aspartate Receptor ◽  
Gray Matter Atrophy ◽  
Nmdar Encephalitis ◽  
Correlation Analyses

Abstract Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a severe autoimmune disease that is commonly accompanied by cognitive impairment and various neurological and psychiatric symptoms, advanced image analyses help explore the pathogenesis of this disease. Therefore, this study aimed to explore specific structural and functional alterations and their relationship with the clinical symptoms of anti-NMDAR encephalitis. In this study, twenty-two patients with anti-NMDAR encephalitis after the acute stage and 29 controls received cognitive assessments and magnetic resonance imaging. Grey matter atrophy was measured using voxel-based morphometry, and functional alterations in abnormal regions were subsequently investigated using resting state functional connectivity (RSFC). Finally, correlation analyses were performed to explore the associations between imaging alterations and cognitive assessments. The patients demonstrated significant gray matter atrophy in the bilateral triangle part of the inferior frontal gyrus (triIFG.L and triIFG.R) and right precuneus, decreased RSFC between triIFG.L and bilateral Heschl gyrus (HES), decreased RSFC between triIFG.R and HES.R, decreased RSFC between right precuneus and left cerebellum, and increased RSFC between triIFG.R and left superior frontal gyrus. Further correlation analyses showed that the gray matter volume in triIFG.R and decreased RSFC between triIFG.L and HES.R were associated with decreased memory scores, whereas decreased RSFC between triIFG.R and HES.R was marginally correlated with the disease course in patients. In conclusion, this study suggests that cognitive impairments in patients with anti-NMDAR encephalitis may be mainly associated with gray matter atrophy and abnormal RSFC in the triIFG. These findings provide new insights into anti-NMDAR encephalitis pathogenesis and help explore potential treatments.

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.

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