108 Comparing autoimmune encephalitis variants in a pediatric cohort from Hamilton, Ontario

2021 ◽  
Vol 26 (Supplement_1) ◽  
pp. e76-e78
Author(s):  
Mark Grinberg ◽  
Michelle Schneeweiss ◽  
Christopher Povolo ◽  
Jeyanth Inkaran ◽  
Kevin Jones
Keyword(s):  
Nmda Receptor ◽  
Clinical Decision Making ◽  
Group Analysis ◽  
Autoimmune Encephalitis ◽  
Clinical Decision ◽  
Subject Area ◽  
Residual Symptoms ◽  
Receptor Antibody ◽  
Nmda Receptor Encephalitis ◽  
Csf Analysis

Abstract Primary Subject area Neurology Background Autoimmune Encephalitis (AE) is an emerging cause of epilepsy with numerous variants, including anti NMDA-receptor encephalitis, for which there is a detectable antibody. However, it is believed that there are many variants of AE for which an antibody has not yet been discovered. Objectives This study aimed to determine the differences in disease course of AE patients with and without detectable anti-NMDA receptor antibody. Design/Methods This retrospective analysis is part of a Canada-wide project aimed at evaluating the epidemiology and characteristics of AE. Cases with suspected AE were retrieved and screened by two independent reviewers against AE criteria. Those that met criteria were analyzed for trends and stratified into NMDA receptor antibody positive (NMDAr) and negative categories for inter-group analysis. Of 23 cases reviewed, 11 met criteria (aged 1-17 years, 27% males), of which 7 were NMDAr positive. Results The NMDAr subgroup was characterized by behavioural changes, focal seizures, and prodromal fever on presentation, whereas the receptor negative subset had a much higher variability of symptoms, without any distinctive patterns. On average, the NMDAr positive group showed an increase in white blood cell count on CSF analysis, and a slight increase in the proportion of patients presenting with supratentorial lesions on MRI. Both groups had abnormal findings on EEG. However, despite the lack of gross differences in findings, all of the NMDAr positive cases received IVIG (most with corticosteroids as well) while only 2 NMDAr negative patients received immunomodulatory therapy. At discharge 6/7 of the NMDAr patients had some form of residual movement disorder while the NMDAr negative group had more variable residual symptoms at discharge. Conclusion Our findings show that a high index of suspicion in the diagnosis of AE is required due to the indistinct distribution and variety in its presentation. Negative antibody findings should not rule out AE due to the possibility of unidentified antibodies. Future studies should explore why differences in treatment between the two groups exist, and if slight differences in presentation influence clinical decision-making.

scholarly journals ENSEFALITIS ANTIRESEPTOR NMDA: ENSEFALITIS DENGAN GEJALA AWAL PSIKOSIS

2020 ◽  
Vol 35 (1) ◽  
Author(s):  
Riwanti Estiasari ◽  
Darma Imran ◽  
Kartika Maharani ◽  
David Pangeran ◽  
Fitri Octaviana ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Nmda Receptor ◽  
Psychiatric Symptom ◽  
Autoimmune Encephalitis ◽  
Antibody Test ◽  
The Other ◽  
Receptor Antibody ◽  
The Third ◽  
Nmda Receptor Encephalitis ◽  
Cognitive Disturbance

     ANTI-NMDA RECEPTOR ENCEPHALITIS: ENCEPHALITIS PRESENTING WITH EARLY PSYCHOSIS SYMPTOMSABSTRACTAnti N-methyl-D-aspartate (NMDA) receptor encephalitis is seldom found in Indonesia. The atypical initial presentation of psychosis and cognitive disturbance results in underdiagnosed, or even undiagnosed case. This report described a 12-year-old girl presenting with initial psychiatric symptom followed by seizure and oropharyngeal dystonia. Anti-NMDA receptor antibody test carried out on both serum and  cerebrospinal  fluid (CSF) were positive and  no other pathogen was detected in the CSF. Clinical improvement was seen following the third day of methylprednisolone administration. This case demonstrates the importance of considering anti-NMDA receptor encephalitis or the other autoimmune encephalitis as the differential of infective encephalitis. Anti-NMDA receptor antibody test, which can already be performed in Indonesia, will greatly guide to the definitive diagnosis.Keywords: Encephalitis, NMDA, oropharyngeal dystonia, psychiatricABSTRAKEnsefalitis antireseptor N-metil-D-aspartat (NMDA) masih jarang ditemukan di Indonesia. Gejala awal yang tidak khas berupa psikosis dan gangguan kognitif menyebabkan keterlambatan diagnosis, atau bahkan tidak terdiagnosis, ensefalitis jenis ini. Berikut dilaporkan kasus seorang perempuan 12 tahun dengan gejala awal berupa gejala psikiatrik yang diikuti dengan kejang dan munculnya distonia orofaring. Pemeriksaan antibodi antireseptor NMDA pada serum dan cairan serebrospinal (CSS) positif dan tidak ditemukan patogen lain pada pemerikaan CSS. Perbaikan klinis terlihat setelah pemberian metilprednisolon selama 3 hari. Kasus ini memperlihatkan pentingnya mempertimbangkan diagnosis ensefalitis antireseptor NMDA atau ensefalitis autoimun lainnya sebagai diagnosis banding pada kasus ensefalitis infeksi. Oleh karena itu, pemeriksaan antibodi antireseptor NMDA yang telah dapat dilakukan di Indonesia akan sangat membantu proses penegakan diagnosis.Kata kunci: Distonia orofaring, ensefalitis, NMDA, psikiatrik

scholarly journals P.072 The spectrotemporal characteristics of NMDA receptor encephalitis

2016 ◽  
Vol 43 (S2) ◽  
pp. S38-S38
Author(s):  
A Richard ◽  
T Zanos ◽  
F Dubeau ◽  
E de Villers-Sidani
Keyword(s):  
Nmda Receptor ◽  
Clinical Decision Making ◽  
Clinical Status ◽  
Power Spectra ◽  
Disease Status ◽  
Autoimmune Disorder ◽  
Clinical Decision ◽  
Disease Progress ◽  
Nmda Receptor Encephalitis ◽  
Before And After

Background: NMDA receptor encephalitis (NMDA-RE) is an autoimmune disorder caused by antibodies to the NR1-NR2B heterodimer of the NMDA receptor. Currently, disease status is tracked primarily by the presence of auto-antibodies in the cerebrospinal fluid (CSF) and serum. Using serological and CSF markers along with clinical parameters to track disease progress can be challenging since patient symptoms and disease progress can vary widely. Methods: EEGs were reviewed in a 31 year old male patient with proven NMDA-RE. EEG data were sampled from various times before and after diagnosis, as well as during various stages of treatment. All analyses were performed using Matlab (Mathworks). Results: We showed that using a simple 1/f model of spectral behaviour (Buzsaki and Draguhn, 2004), we could fit the power spectra of the raw data at various instances during routine EEGs. We have demonstrated that the values of specific fitting parameters vary in relationship to the patient’s clinical status across various stages of illness. Conclusions: The aim of this project was to explore the potential utility of EEG as a complement to the usual clinical metrics used in monitoring NMDA-RE. The analysis techniques presented here highlight the use of EEG as a practical, minimaly-invasive tool to monitor progress and potentially aid in clinical decision making.

Persistent seropositivity in oophorectomy-resistant anti-NMDA receptor encephalitis

2021 ◽  
Vol 14 (9) ◽  
pp. e241878
Author(s):  
Susmit Tripathi ◽  
Nara M Michaelson ◽  
Alan Segal
Keyword(s):  
Clinical Decision Making ◽  
Serum Antibody ◽  
Disease Patient ◽  
Clinical Decision ◽  
High Serum ◽  
Nmda Receptor Encephalitis ◽  
Resistant Disease ◽  
Nmdar Encephalitis ◽  
Antibody Titres ◽  
Case Basis

To discuss (1) the significance of seropositivity in anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis and (2) clinical decision making in oophorectomy resistant disease. Patient A (a 35-year-old woman) had high CSF and serum anti-NMDA antibody titres, a complicated hospital course, little improvement with first and second-line therapies, and remained with high CSF and serum antibody titres despite unilateral oophorectomy, requiring a nearly 13-month long hospitalisation. Conversely, patient B (a 29-year-old woman) had low CSF titres, seronegative disease and quickly recovered to her baseline with first line therapies and oophorectomy. Anti-NMDAR antibodies are themselves pathological, causing signalling dysfunction and internalisation of the NMDAR. Seropositivity with anti-NMDAR antibodies likely reflects leakage from the blood–brain barrier, with high serum titres being a downstream effect of high CSF titres. Empiric bilateral oophorectomies is controversial but appropriate on a case-by-case basis in extremely treatment-resistant NMDAR encephalitis given the possibility of antigenic microteratomas, which may not be detected on imaging or even bilateral ovarian biopsies.

Frequency and temporal sequence of clinical features in adults with anti-NMDA receptor encephalitis presenting with psychiatric symptoms

2018 ◽  
Vol 49 (16) ◽  
pp. 2709-2716 ◽  
Author(s):  
Ronald J. Gurrera
Keyword(s):  
Nmda Receptor ◽  
Clinical Features ◽  
Psychiatric Symptoms ◽  
Autoimmune Encephalitis ◽  
Signs And Symptoms ◽  
Nmda Receptor Encephalitis ◽  
Nmdar Encephalitis ◽  
Unexplained Fever ◽  
Study Inclusion

AbstractBackgroundAnti-NMDA receptor (NMDAr) encephalitis is the most common autoimmune encephalitis in adults. It mimics psychiatric disorders so often that most patients are initially referred to a psychiatrist, and many are misdiagnosed. Without prompt and effective treatment, patients are likely to suffer a protracted course with significant residual disability, or death. This study focuses on the frequency and chronology of salient clinical features in adults with anti-NMDAr encephalitis who are likely to be first evaluated by a psychiatrist because their presentation suggests a primary psychiatric disorder.MethodsA systematic search of PubMed and EMBASE databases identified published reports of anti-NMDAr encephalitis associated with prominent behavioral or psychiatric symptoms. After eliminating redundancies, the frequencies and relative timing of clinical features were tabulated. Signs and symptoms were assigned temporal ranks based on the timing of their first appearance relative to the first appearance of other signs and symptoms in each patient; median ranks were used to compare temporal sequencing of both individual features and major symptom domains.ResultsTwo hundred thirty unique cases (185 female) met study inclusion criteria. The most common features were seizures (60.4%), disorientation/confusion (42.6%), orofacial dyskinesias (39.1%), and mutism/staring (37.4%). Seizures, fever, and cognitive dysfunction were often the earliest features to emerge, but psychiatric features predominated and sequencing varied greatly between individuals.ConclusionsClinicians should consider anti-NMDAr encephalitis when new psychiatric symptoms are accompanied by a recent viral prodrome, seizures or unexplained fever, or when the quality of the psychiatric symptoms is unusual (e.g. non-verbal auditory hallucinations).

scholarly journals RARE-36. BORTEZOMIB WOKE UP A PATIENT WITH ANTI-NMDA RECEPTOR ENCEPHALITIS REFRACTORY TO STANDARD THERAPY AND LONG TERM FOLLOW-UP

2019 ◽  
Vol 21 (Supplement_6) ◽  
pp. vi229-vi229
Author(s):  
Kong Xiao-Tang ◽  
Leonid Groysman ◽  
Cyrus Dastur ◽  
Beverly Fu ◽  
Daniela Bota
Keyword(s):  
Nmda Receptor ◽  
Standard Therapy ◽  
Autoimmune Encephalitis ◽  
Ivig Therapy ◽  
Term Care ◽  
Good Communication ◽  
Nmda Receptor Encephalitis ◽  
Long Term Follow Up ◽  
One Year

Abstract OBJECTIVE To report a case with refractory NMDA encephalitis in comatose for 18 months, who was treated successfully with bortezomib. BACKGROUND Anti-NMDA encephalitis is a rare autoimmune encephalitis. Standard therapy include corticosteroid, IVIG or plasma exchange, cyclophosphamide, rituximab, and tumor removal. Refractory cases are very severe and often stay in ICU on ventilation for several months to years. Bortezomib for the treatment of refractory anti-NMDA receptor encephalitis was reported. We have applied the treatment to our refractory case and successfully woke up the patient. And we have followed up the patient for 3 years. METHODS Case report. RESULTS A 40 yo male was diagnosed as anti-NMDA encephalitis. Standard therapy was applied. After stabilization, the patient was eventually discharged to ICU at a long term care subacute hospital. The patient was brought back for more Rituxan or steroid or IVIG therapy. The condition had not improved at all. Eighteen months in comatose, the patient had worsening NMDA titer in CSF to 1:640. Decision was made to start bortezumib as reported with modification: 1.3 mg/m2 bortezomib were administered on days 1, 8, 11 and 14 and allowed two weeks off therapy. After first cycle, the patent started to talk first word “hurt.” After 6 cycles, the patient sat up and started riding bicycles for physical therapy. The NMDA titer in CSF was reduced to 1:40 at the end of 6 cycles. One year later, the patent stood up and ambulated with a walker. One and half year later, the patient walks without assistance and his speech and cognition have significantly improved with good communication with family members and staff. CONCLUSIONS Proteasome inhibitor bortezomib might be considered to be the third line therapy as early as possible if the first line and second line are ineffective to treat anti-NMDA receptor encephalitis.

scholarly journals Decreased occipital lobe metabolism by FDG-PET/CT

2017 ◽  
Vol 5 (1) ◽  
pp. e413 ◽  
Author(s):  
John C. Probasco ◽  
Lilja Solnes ◽  
Abhinav Nalluri ◽  
Jesse Cohen ◽  
Krystyna M. Jones ◽  
...  
Keyword(s):  
Nmda Receptor ◽  
Fdg Pet ◽  
Medical Center ◽  
Academic Medical Center ◽  
Autoimmune Encephalitis ◽  
Occipital Lobe ◽  
Nmda Receptor Encephalitis ◽  
Pet Ct ◽  
Fdg Pet Ct ◽  
Occipital Lobes

Objective:To compare brain metabolism patterns on fluorodeoxyglucose (FDG)-PET/CT in anti–NMDA receptor and other definite autoimmune encephalitis (AE) and to assess how these patterns differ between anti–NMDA receptor neurologic disability groups.Methods:Retrospective review of clinical data and initial dedicated brain FDG-PET/CT studies for neurology inpatients with definite AE, per published consensus criteria, treated at a single academic medical center over a 10-year period. Z-score maps of FDG-PET/CT were made using 3-dimensional stereotactic surface projections in comparison to age group–matched controls. Brain region mean Z scores with magnitudes ≥2.00 were interpreted as significant. Comparisons were made between anti–NMDA receptor and other definite AE patients as well as among patients with anti–NMDA receptor based on modified Rankin Scale (mRS) scores at the time of FDG-PET/CT.Results:The medial occipital lobes were markedly hypometabolic in 6 of 8 patients with anti–NMDA receptor encephalitis and as a group (Z = −4.02, interquartile range [IQR] 2.14) relative to those with definite AE (Z = −2.32, 1.46; p = 0.004). Among patients with anti–NMDA receptor encephalitis, the lateral and medial occipital lobes were markedly hypometabolic for patients with mRS 4–5 (lateral occipital lobe Z = −3.69, IQR 1; medial occipital lobe Z = −4.08, 1) compared with those with mRS 0–3 (lateral occipital lobe Z = −0.83, 2; p < 0.0005; medial occipital lobe Z = −1.07, 2; p = 0.001).Conclusions:Marked medial occipital lobe hypometabolism by dedicated brain FDG-PET/CT may serve as an early biomarker for discriminating anti–NMDA receptor encephalitis from other AE. Resolution of lateral and medial occipital hypometabolism may correlate with improved neurologic status in anti–NMDA receptor encephalitis.

scholarly journals Long-term Functional Outcomes and Relapse of Anti-NMDA Receptor Encephalitis

2021 ◽  
Vol 8 (2) ◽  
pp. e958
Author(s):  
Xue Gong ◽  
Chu Chen ◽  
Xu Liu ◽  
Jingfang Lin ◽  
Aiqing Li ◽  
...  
Keyword(s):  
Nmda Receptor ◽  
Functional Outcomes ◽  
Western China ◽  
Residual Symptoms ◽  
Delayed Treatment ◽  
Nmda Receptor Encephalitis ◽  
Disturbance Of Consciousness ◽  
Related Risk ◽  
Multiple Relapses

ObjectiveTo study the factors associated with relapse and functional outcomes in patients with anti-NMDA receptor encephalitis in Western China.MethodsThe Outcome of the anti-NMDA receptor Encephalitis Study in Western China was initiated in October 2011 to collect prospective observational data from consecutively enrolled patients with anti-NMDA receptor encephalitis.ResultsWe consecutively enrolled 244 patients (median age: 26 years, range: 9–78 years; females: 128 [52.45%]) between October 2011 and September 2019. Fatality occurred in 17 (6.96%) patients, and tumors were found in 38 (15.57%) patients. The median follow-up duration was 40 (6–96) months. Of these patients, 84.8% showed clinical improvements within 4 weeks after immunotherapy, with a median modified Rankin Scale of 2 (interquartile range [IQR]: 2–3), and 80.7% (median: 1, IQR: 0–2) and 85.7% (median: 0, IQR: 0–1) had substantial recovery (i.e., mild or no residual symptoms) at 12 and 24 months, respectively. The overall prognosis was still improving at 42 months after onset. Disturbance of consciousness during the first month was the only independent predictor (OR: 2.91, 95% CI: 1.27–6.65; p = 0.01) of a poor functional neurologic outcome. Overall, 15.9% of the patients had one or multiple relapses, with 82.0% experiencing the first relapse within 24 months and 76.9% experiencing relapses that were less severe than the initial episodes. Relapse-related risk factors included the female sex and delayed treatment (p < 0.05).ConclusionsMost patients achieved favorable long-term functional outcomes. Some patients experienced one or multiple relapses, especially female patients. Timely immunotherapy at onset may reduce the risk of relapse.

scholarly journals Efficacy of tacrolimus as long-term immunotherapy for neuronal surface antibody-mediated autoimmune encephalitis

2022 ◽  
Vol 13 ◽  
pp. 204062232110630
Author(s):  
Chenchen Liu ◽  
Suqiong Ji ◽  
Huajie Gao ◽  
Zhuajin Bi ◽  
Qin Zhang ◽  
...  
Keyword(s):  
Nmda Receptor ◽  
Autoimmune Encephalitis ◽  
Efficacy And Safety ◽  
Nmda Receptor Encephalitis ◽  
Significant Difference ◽  
Observational Cohort ◽  
Surface Antibody ◽  
Retrospective Observational Cohort Study

Aims: We aimed to verify the efficacy and safety of tacrolimus as long-term immunotherapy for the treatment of neuronal surface antibody-mediated autoimmune encephalitis (AE) during the first attack. Methods: In this retrospective observational cohort study, patients with neuronal surface antibody-mediated AE who experienced the first attack were enrolled. We compared the outcomes of 17 patients who received tacrolimus with those of 47 patients treated without tacrolimus. Patients were assessed at onset and 3, 6, and 12 months, as well as at the last follow-up, by using the modified Rankin scale (mRS) and the Clinical Assessment Scale in Autoimmune Encephalitis (CASE). The efficacy of tacrolimus was also compared in a subgroup of patients with anti-NMDA receptor encephalitis. Results: Among all patients with neuronal surface antibody-mediated AE, those receiving tacrolimus had lower median mRS scores [1 (IQR = 0–1) versus 2 (IQR = 1–3) in controls, p = 0.001)], CASE scores [2 (IQR = 1–3) versus 3 (IQR = 2–7), p = 0.006], and more favorable mRS scores (94.1% versus 68.1%, p = 0.03) at the 3-month follow-up. No difference was found at the last follow-up. There was no significant difference in the occurrence of relapse and adverse events between the two groups (11.8% versus 14.9%, p = 0.75). In the subgroup of patients with anti-NMDA receptor encephalitis, patients treated with tacrolimus had a lower median mRS score at the 3-month follow-up [1 (IQR = 0–2) versus 2 (IQR = 1–3), p = 0.03]; however, no difference in the outcome was detected at the last follow-up. Conclusion: Tacrolimus can be used as long-term immunotherapy in patients with neuronal surface antibody-mediated AE during the first attack. Treatment with tacrolimus appears to accelerate the clinical improvement of neuronal surface antibody-mediated AE.

Sign in / Sign up

Export Citation Format

Share Document