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 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 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 Autoimmune encephalitis in a South Asian population

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Nilanka Wickramasinghe ◽  
Dhanushka Dasanayake ◽  
Neelika Malavige ◽  
Rajiva de Silva ◽  
Thashi Chang
Keyword(s):  
Sri Lanka ◽  
Psychiatric Symptoms ◽  
South Asians ◽  
Laboratory Data ◽  
Clinical Manifestations ◽  
Autoimmune Encephalitis ◽  
Asian Population ◽  
South Asian Population ◽  
Clinical Presentations

Abstract Background Autoimmune encephalitis (AE) is now considered a main, potentially curable cause of encephalitis, but remains conspicuously underreported from South Asia. We studied the clinical characteristics in relation to their antibody status and outcomes of patients presenting with AE in Sri Lanka. Methods Patients admitting to government hospitals who were clinically suspected of AE by an on-site neurologist were prospectively recruited over a period of 12 months. Sera and cerebrospinal fluid were tested for NMDAR, AMPAR1, AMPAR2, LGI1, CASPR2, GABARB1/B2 antibodies (Ab) using commercial cell-based assays. Demographic, clinical and laboratory data were compiled into an investigator-administered proforma. Patients were reviewed at 1 year follow up either in person or via telephone. Results One-hundred and forty-two patients from 21 of 25 districts in Sri Lanka (median age = 20.5 years; range 1–86 years; females = 61.3%) were recruited. Of them, 65 (45.8%; median age = 19 years; range 1–86 years; females = 64.6%) fulfilled diagnostic criteria for probable NMDAR-antibody encephalitis (NMDARE) and 6 (4.2%; median age = 44 years; range 28–71 years; females = 83.3%) limbic encephalitis (LE). Abnormal behaviour (95.3%), seizures (81.5%) and movement disorders (69.2%) were the most frequent clinical manifestations of probable NMDARE. NMDAR-antibodies were detectable in 29 (44.6%) and not detectable in 36 in CSF of probable-NMDARE patients. Abnormal EEG was more frequent (p = 0.003) while a worse outcome (OR = 2.78; 95% CI = 0.88–9.09) and deaths (OR = 2.38; 95% CI = 0.67–8.33) were more likely in antibody-negative than antibody-positive probable-NMDARE. Most patients with LE had amnesia (50%) and/or confusion (100%) with agitation (83.3%) and seizures (100%) but none had detectable antibodies to any of the antigens tested. Conclusions NMDARE is the commonest type of AE among South Asians as is the case worldwide. Clinical presentations of NMDARAb-positive and NMDARAb-negative AE patients do not significantly differ but EEG may be a useful marker of an autoimmune basis for psychiatric symptoms.

scholarly journals ROHHAD(NET) Syndrome: Systematic Review of the Clinical Timeline and Recommendations for Diagnosis and Prognosis

2020 ◽  
Vol 105 (7) ◽  
pp. 2119-2131 ◽  
Author(s):  
Julie Harvengt ◽  
Caroline Gernay ◽  
Meriem Mastouri ◽  
Nesrine Farhat ◽  
Marie-Christine Lebrethon ◽  
...  
Keyword(s):  
Systematic Review ◽  
Early Diagnosis ◽  
Individual Patient Data ◽  
Psychiatric Symptoms ◽  
Clinical Manifestations ◽  
Rapid Onset ◽  
Patient Data ◽  
Autonomic Dysregulation ◽  
Hypothalamic Dysfunction

Abstract Context Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, autonomic dysregulation and neural crest tumor (ROHHHAD[NET]) is a rare and potentially fatal disease. No specific diagnostic biomarker is currently available, making prompt diagnosis challenging. Since its first definition in 2007, a complete clinical analysis leading to specific diagnosis and follow-up recommendations is still missing. Objective The purpose of this work is to describe the clinical timeline of symptoms of ROHHAD(NET) and propose recommendations for diagnosis and follow-up. Design We conducted a systematic review of all ROHHAD(NET) case studies and report a new ROHHAD patient with early diagnosis and multidisciplinary care. Methods All the articles that meet the definition of ROHHAD(NET) and provide chronological clinical data were reviewed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analysis individual patient data guidelines. The data were grouped into 7 categories: hypothalamic dysfunction, autonomic dysregulation, hypoventilation, NET, psychiatric symptoms, other clinical manifestations, and outcome. Results Forty-three individual patient data descriptions were analyzed. The timeline of the disease shows rapid-onset obesity followed shortly by hypothalamic dysfunction. Dysautonomia was reported at a median age of 4.95 years and hypoventilation at 5.33 years, or 2.2 years after the initial obesity. A NET was reported in 56% of the patients, and 70% of these tumors were diagnosed within 2 years after initial weight gain. Conclusion Because early diagnosis improves the clinical management and the prognosis in ROHHAD(NET), this diagnosis should be considered for any child with rapid and early obesity. We propose guidance for systematic follow-up and advise multidisciplinary management with the aim of improving prognosis and life expectancy.

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 Real-time fMRI neurofeedback as a new treatment for psychiatric disorders: a systematic review

2021 ◽  
Author(s):  
Pamela Pindi ◽  
Josselin Houenou ◽  
Camille Piguet ◽  
Pauline Favre
Keyword(s):  
Psychiatric Disorders ◽  
Real Time ◽  
Functional Mri ◽  
Psychiatric Symptoms ◽  
Psychotic Symptoms ◽  
Control Group ◽  
Controlled Trials ◽  
Brain Response ◽  
Double Blind

Background: Neurofeedback using real-time functional MRI (RT-fMRI-NF) is an innovative technique that allows to voluntarily modulating a targeted brain response and its associated behavior. Despite promising results in the current literature, its effectiveness on symptoms’ management in psychiatric disorders is not yet clearly demonstrated. This review aims to evaluate the effectiveness of RT-fMRI-NF in the treatment of psychiatric disorders and to provide methodological suggestions for future studies. Methods: Web of Science and PubMed databases were searched using the keywords: neurofeedback AND (fMRI OR “functional magnetic resonance imag*” OR “functional MRI”) AND (“real-time” OR “real time”). Twenty-six clinical trials focusing on psychiatric disorders were included and categorized according to the diagnostic categories. The RT-fMRI-NF efficacy was assessed by reporting changes in clinical endpoints before vs. after NF training and before or after NF training vs. follow-up. Results: Among the 26 studies, 18 were controlled trials, of which five showed significant clinical improvement in the experimental vs. control group after the training. Eight studies found an effect at follow-up on ADHD symptoms, emotion dysregulation, depressive symptoms, hallucinations, psychotic symptoms and specific fear. Limitations: Here, we only focused our review on fMRI-based NF training. Conclusion: The use of RT-fMRI-NF as a treatment for psychiatric symptoms is promising. However, further double blind, randomized-controlled trials are warranted.

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

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 M99. INVESTIGATING THE BEST PREDICTIVE CLINICAL FEATURES OF ANTI-N-METHYL-D-ASPARTATE RECEPTOR (NMDAR) ENCEPHALITIS IN THE 2010 AUSTRALIAN NATIONAL SURVEY OF HIGH IMPACT PSYCHOSIS (SHIP) COHORT

2020 ◽  
Vol 46 (Supplement_1) ◽  
pp. S172-S173
Author(s):  
Susan Rossell ◽  
Denny Meyer ◽  
Cyndi Shannon Weickert ◽  
Andrea Phillipou ◽  
Cherrie Galletly ◽  
...  
Keyword(s):  
Machine Learning ◽  
Medical History ◽  
Clinical Features ◽  
Clinical Data ◽  
Psychiatric Symptoms ◽  
Learning Algorithm ◽  
Psychotic Symptoms ◽  
Gradient Boosting ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis

Abstract Background Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis, a recently reported autoimmune disorder, can be mistakenly diagnosed as a psychotic disorder, especially schizophrenia, as patients can present with prominent psychotic symptoms, in particular persecutory ideation, hallucinations and disturbed speech. In this study we used machine learning of the clinical data in a large cohort of persons with a positive psychosis history to ascertain whether we could predict NMDAR-positive cases, and which variables most accurately distinguished between NMDAR-positive and -negative cases. Methods SHIP collected nationally representative data from 1825 individuals with a psychotic illness. Plasma samples were available for n=472. To investigate the prevalence of NMDAR autoantibodies a recombinant indirect immunofluorescence test was performed (EuroImmun AG, Lübeck, Germany), with NMDAR transfected human embryonic kidney (HEK) 293 cells quantified using NIS Elements software. NMDAR-positive cases were estimated. Gradient boosting machine learning (the data were randomly split: 60% for initial ascertainment and 40% for validation) was subsequently performed using the clinical data available: 120 variables in total across various domains of sociodemographic, medical history, psychiatric diagnosis and current psychiatric symptoms. Only the variables found to have significant (or near significant) association with being NMDAR-positive were used to develop rules for identifying cases. Results There were 38 NMDAR-positive cases. They were more likely to be associated with a schizophrenia /schizoaffective and a depressive psychosis diagnosis, and less likely to be associated with a bipolar diagnosis, than antibody-negative cases. They were also more likely to be associated with a single episode with good recovery, and with anxiety symptoms and dizziness in the prior 12 months (which included light headedness, feeling faint and unsteady). For the present state symptoms, restricted affect was more likely to be present whereas poverty of speech was rare. Initial insomnia and a medical history that included epilepsy were not present for any of the NMDAR-positive cases. The machine learning algorithm was able to successfully classify 94% of cases to the correct antibody group. Discussion In this significant Australian epidemiological cohort, we have identified key clinical features associated with anti-NMDAR encephalitis, including diagnosis, and symptoms and clinical course. The novel and insightful analyses afforded by using machine learning should be replicated in other samples to confirm the important clinical findings reported in the current work.

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