Treatment Of anti-NMDA Receptor Encephalitis Research Articles

Anti-NMDA receptor autoimmune encephalitis associated with ovarian teratoma: A case series and literature review.

Autoimmune encephalitis is a group of disorders characterized by symptoms of dysfunction of the limbic and extra-limbic systems that occur in association with antibodies against intracellular antigens, synapses, or proteins located on the surface of nerve cells. Anti-NMDA (N-methyl-D-aspartate) receptor encephalitis was first described in 2007 and has become one of the most commonly recognized autoimmune encephalitides. The disease is predominantly female (81%) and affects young patients (37% <18 years old, 95% <45 years old), with up to 58% of women over 18 years old having ovarian teratoma. Clinical symptoms of anti-NMDA receptor encephalitis include psychiatric disorders (anxiety, agitation, bizarre behavior, hallucinations, delusions, and memory impairment); seizures and epileptic states; and other neurologic symptoms (muscle tone abnormalities, eye movement disorders, and central ventilatory disorders). Diagnosis of this condition can be difficult due to the non-specific nature of the symptoms, often leading to delays in appropriate treatment. Early diagnosis and timely treatment of anti-NMDA receptor encephalitis, especially in young women with ovarian teratoma, has significantly improved the chances of full recovery and improved reproductive health outcomes for patients.

Does new knowledge allow to improve the diagnosis of mental disorders: the problem of anti-NMDA receptor encephalitis?

A narrative review of research studies discusses the clinical features, differential diagnosis, and treatment of anti-NMDA receptor encephalitis. Special attention is given to the controversial aspects of the etiology and pathogenesis of this disorder. The need for further research into on the role of antibodies to NMDA receptors in the development of psychiatric disorders is emphasized.

A Rare Case of Anti-N-Methyl-D-Aspartate Receptor Encephalitis in an Infant Presenting with Regression and Movement Disorder.

A Rare Case of Anti-N-Methyl-D-Aspartate Receptor Encephalitis in an Infant Presenting with Regression and Movement Disorder.

Staged Treatment for Anti-NMDA Receptor Encephalitis Based on Combined Western and Traditional Chinese Medicine

Staged Treatment for Anti-NMDA Receptor Encephalitis Based on Combined Western and Traditional Chinese Medicine

Driving Out of Coma: Treatment of Anti-NMDA Receptor Encephalitis with Bortezomib (5437)

Driving Out of Coma: Treatment of Anti-NMDA Receptor Encephalitis with Bortezomib (5437)

Immunotherapy for anti-NMDA receptor encephalitis: Experience from a single center in Taiwan

Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is an anti-neuronal antibody-mediated inflammatory brain disease that causes severe psychiatric and neurological deficits in previously healthy patients. The aims of this study were to demonstrate the clinical characteristics of patients diagnosed with anti-NMDA receptor encephalitis and to compare the different treatment strategies among these patients. Patients presenting with newly acquired psychiatric and/or neurological deficits were studied retrospectively from 2009 to 2017. Patients with evidence of anti-NMDA receptor antibodies in serum and/or cerebrospinal fluid were enrolled. The modified Rankin scale was used to assess the initial status and outcomes of the enrolled patients. Details of the clinical presentations and results of investigations were analyzed. All (n=24) of the patients received first-line immunotherapy (steroids, and/or intravenous immunoglobulin, and/or plasma exchange), and 14 patients received second-line immunotherapy (rituximab and/or cyclophosphamide). The mean time between the first- and second-line treatment was 13 days. During the first 6 months, 20 patients (20/24, 83%) achieved a good outcome (modified Rankin Scale score ≤2) and 15 patients (15/24, 62.5%) completely recovered. Four patients (17.7%) relapsed, and three patients (12.5%) had associated tumors. Immunotherapy is an effective treatment for anti-NMDA receptor encephalitis. Rituximab and/or cyclophosphamide are treatment options for those who cannot tolerate or do not respond to first-line immunotherapy. Prospective studies are necessary to investigate the role of rituximab and cyclophosphamide in anti-NMDA receptor encephalitis.

Lower dosages of rituximab used successfully in the treatment of anti-NMDA receptor encephalitis without tumour

ObjectiveThe aim of this study was to evaluate the use and efficacy of lower dosages of rituximab for treating anti N-methyl-d-aspartate receptor (NMDAR) encephalitis without tumour. MethodsWe performed a prospective study of 10 patients with anti-NMDAR encephalitis who did not respond to 10 to 14days first-line immunotherapy and received rituximab administered intravenously (IV) at a dosage of 100mg once per week for 4 consecutive weeks. Reinfusion of rituximab was given when CD19+ B-cell counts of total lymphocytes in peripheral blood >1%. The annualized relapse rate (ARR), modified Rankin scale (mRS) and CD19+ B-cell counts were measured every 4 to 10weeks after initial rituximab treatment in order to assess the clinical outcome and efficacy of rituximab. ResultsLower dosages of rituximab led to a significant reduction of mRS and CD19+ B-cells when compared with before the rituximab infusion (P<0.05) and allowed 9 (90%) patients to maintain a stabilised neurological status. One patient experienced a relapse at 19weeks after initial rituximab infusion. Although ARR reduction of all 10 patients did not achieve statistical significance (P>0.05), in the 4 patients who had relapses before rituximab treatment there was an apparent reduction in ARR over 56weeks. At the last follow up, 9 patients (90%) had a good outcome (mRS≤2) including 3 patients (30%) who recovered completely (mRS=0). Transient infusion adverse events occurred in 2 patients. We observed no serious delayed adverse events during the 56weeks follow-up. ConclusionsIn patients with anti-NMDAR encephalitis who did not respond to first-line immunotherapy, early application of lower dosages of rituximab could efficiently reduce CD19+ B-cell counts of peripheral blood and improve the prognosis of anti-NMDAR encephalitis.

Differences in treatment of anti-NMDA receptor encephalitis: results of a worldwide survey.

The objective of the study was to identify differences in treatment strategies for anti-NMDA receptor encephalitis based on specialty of treating physicians, geographic location, and years in practice. We conducted an anonymous worldwide electronic survey through the Practice Current section of Neurology® Clinical Practice to appraise differences in decisions about first- and second-line treatment and timing for initiation of second-line treatment for anti-NMDA receptor encephalitis. 399 participants answered all questions of the survey and were included in the analysis. 261 (65%) were adult neurologists, 86 (22%) were neurologists treating children, and 52 (13%) were pediatric rheumatologists. 179 (45%) responders practiced in the US. The majority agreed on the use of steroids and/or IVIg for first-line therapy and rituximab alone as second line. Differences in initial treatment regimen based on specialty included increased use of plasma exchange by adult neurologists (27%) and rituximab by pediatric rheumatologists (29%) (χ 2(4)=27.43, p<0.001). Trainees opted for plasma exchange (35%) and junior faculty picked rituximab (15%) more as part of first line (χ 2(4)=13.37, p=0.010). There was greater usage of anti-metabolites for second-line therapy outside of the US (15%) (χ 2(4)=11.67, p=0.020). US physicians also utilized second-line treatment earlier than their mostly European counterparts (14 vs. 23% used later than 2weeks; χ 2(1)=4.96, p=0.026). Although treatment patterns were similar, differences observed across specialties and geographic locations may guide the development of consensus-driven guidelines by multi-disciplinary task forces. These guidelines may promote treatment trials of immunomodulators in autoimmune encephalitides.

抗NMDA受容体脳炎の治療後に卵巣嚢腫の再発を認めた症例に関する検討

抗NMDA受容体脳炎の治療後に卵巣嚢腫の再発を認めた症例に関する検討

Successful treatment of anti-NMDA receptor encephalitis with a prompt ovarian tumour removal and prolonged course of plasmapheresis: A case report.

Anti-N-methyl-d-aspartate-receptor (NMDAR) encephalitis is an uncommon autoimmune disorder with a wide spectrum of neuropsychiatric symptoms. There is a great requirement to emphasize the importance of a multidisciplinary team approach in the process of diagnosis and treatment of the potentially fatal condition, including psychiatrists, neurologists, gynaecologists and intensivists. Physicians must be aware that psychiatric and neurological disorders, which are typical features for NMDAR encephalitis in young women with ovarian tumours, may progress into status epilepticus and respiratory insufficiency. This disease can only be successfully treated with prompt surgical intervention and an early implementation of a wide array of immunosuppressive therapies. Optimal timing of initiation of therapeutic plasma exchange, as well as duration of treatment necessary to achieve desirable outcomes in patients with NMDAR remains unknown. The present case report aims to raise awareness about the importance of early implementation of this potentially life-saving therapy and continuing the treatment courses until full subsidence of symptoms.

Anti N-Methyl-D-Aspartate (NMDA) Receptor Encephalitis with Frustrated Diagnosis Course: A Case Report

Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a rare disease with uncertain etiology and pathogenesis that affects young women. Its diagnosis can be delayed because of the nonspecific neuropsychiatric symptoms in the foreground. This article describes the details of a recent complicated case of a patient with this condition which is related to an ovarian teratoma. Correct diagnostic and prompt treatment of anti-NMDA receptor encephalitis remains a serious clinical challenge due to its unspecific manifestations and varying response to treatments. The information will be of interest to clinicians working with encephalitis patients.

Could coenzyme Q10 supplementation have a role in the treatment of anti-NMDA receptor encephalitis?

Could coenzyme Q10 supplementation have a role in the treatment of anti-NMDA receptor encephalitis?

Anti-N-methyl-d-aspartate receptor encephalitis associated with ovarian teratoma: clinical presentation, diagnosis, treatment, and surgical management

Anti-N-methyl-d-aspartate (NMDA) receptor encephalitis is a newly recognized autoimmune disorder with prominent neuropsychiatric symptoms, usually part of a paraneoplastic syndrome. We present two cases of patients with anti-NMDA receptor encephalitis related to ovarian teratoma. Our two patients developed neuropsychiatric symptoms and later evolved into a state of impaired consciousness. The physical examinations and investigations, including routine laboratory testing, a basic metabolic panel, MRI of the brain, and the cerebrospinal fluid (CSF) analysis were non-specific. CT scan and MRI of the pelvis revealed a calcified tumor that was suspected to be an ovarian teratoma in both patients. Each patient underwent salpingo-oophorectomy, and histopathological diagnosis of the patient of case 1 was mature cystic teratoma and that of case 2 was immature teratoma. Finally, the result of the anti-NMDA receptor antibody assay demonstrated high antibody titers in CSF, leading to definite diagnosis of anti-NMDA receptor encephalitis triggered by ovarian teratoma in both cases. Given that anti-NMDA receptor encephalitis often results in severe symptoms regardless of the tumor size of the ovarian teratoma, accurate diagnosis of the teratoma followed by prompt surgical resection of the tumor is crucial for the treatment of anti-NMDA receptor encephalitis. The clinical presentation, diagnosis, treatment, surgical management, and prognosis, as well as a review of this disorder, were discussed in this case conference.

Rituximab Used Successfully in the Treatment of Anti-NMDA Receptor Encephalitis

We report the case of a young woman with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis, without tumor, who was successfully treated with rituximab. Because conventional immunotherapy, including corticosteroids, immunoglobulin (IVIg), and plasma exchange showed little improvement in our patient, we introduced another treatment using rituximab. A week after the first administration of rituximab, her symptoms improved gradually and significantly. This case provides in vivo evidence that rituximab is an effective agent for treating anti-NMDAR encephalitis, even in those cases where conventional immunotherapies have been ineffective. Rituximab should be regarded as a beneficial therapeutic agent for this disease.

Therapeutic plasma exchange for the treatment of anti‐NMDA receptor encephalitis

Anti-N-methyl-D-aspartate receptor (NMDA-R) encephalitis is thought to be one of the common paraneoplastic-associated encephalitides. Between February 2001 and February 2011, nine patients were diagnosed with this disorder at Columbia University Medical Center: eight females (mean age 23 years) and one male (3 years of age). Four female patients had ovarian teratomas, which were removed as part of their treatment. Therapeutic plasma exchange (TPE) was used as one of the treatment modalities in addition to immunosuppressive therapy, including corticosteroids, intravenous immunoglobulin (IVIG), and/or rituximab. A total of 56 TPE procedures were performed in these patients on alternate days (range, 5-14 procedures/patient). Approximately 1 plasma volume (PV) was processed for all patients; 5% albumin and 0.9% normal saline were used as replacement fluid. Complications occurred in 20% of TPE procedures; 9% were possibly due to underlying disease. The remaining 11% of complications were hypotensive episodes that rapidly responded to either a fluid bolus or a vasopressor treatment. One patient demonstrated immediate clinical improvement after three TPE treatments, and four patients had significant improvement at time of discharge from the hospital. Long-term follow-up showed that early initiation of TPE appears to be beneficial, and patients who received IVIG after TPE did better than those who received IVIG before TPE. However, the number of patients in this series is too small to provide statistically significant conclusions. Overall, TPE is a relatively safe treatment option in patients with anti-NMDA-R encephalitis. Further studies are needed to elucidate the benefit of TPE in this disease.