Pediatric anti-NMDAR Encephalitis Research Articles

Pediatric anti-NMDAR encephalitis with demyelination on brain MRI: A single center study

ObjectiveTo explore the clinical characteristics, immunotherapy response, and prognosis of pediatric anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis associated with demyelination on brain magnetic resonance (MRI). MethodsWe retrospectively reviewed the medical records of children diagnosed with anti-NMDAR encephalitis in our hospital between January 2016 and December 2021. All children with evidence of demyelination on brain MRI were included. ResultsA total of 183 anti-NMDAR encephalitis children were included; 8.7 % (16/183) of them had demyelination on brain MRI. Nine were positive for myelin oligodendrocyte glycoprotein (MOG)-IgG, while two were positive for both MOG-IgG and glial fibrillary acidic protein (GFAP)-IgG. Four patients had a history of acquired demyelinating syndromes and encephalitis, respectively, while nine (56.3 %) had atypical symptoms of anti-NMDAR encephalitis. All children had supratentorial demyelination on brain MRI; four of them had additional infratentorial lesions. All children received first-line immunotherapy; four were administered repeated first-line immunotherapy and/or rituximab because of poor initial response. During the follow-up, 37.5 % (6/16) of the children relapsed, but all responded well to immunotherapy. There were no significant differences in mRS score before immunotherapy, response to first-line immunotherapy, and long-term prognosis between anti-NMDAR encephalitis children with and without demyelination. However, patients with demyelination were more likely to have a history of acquired demyelinating syndromes or unexplained cortical encephalitis and to relapse. ConclusionPediatric anti-NMDAR encephalitis can co-occur with demyelination and has a high rate of MOG-IgG positivity. A history of acquired demyelinating syndromes or unexplained cortical encephalitis and atypical symptoms may indicate demyelination in children with anti-NMDAR encephalitis. Pediatric anti-NMDAR encephalitis with demyelination is more likely to relapse and needs a closer follow-up. However, it remains unknown whether more intensive immunotherapy is required in these patients.

Analysis of the relation between cerebrospinal fluid antibody titers and clinical characteristics in pediatric patients with anti-N-methyl-D-aspartate receptor encephalitis

ObjectiveThis retrospective study aimed to investigate the relationship between cerebrospinal fluid (CSF) antibody titers of N-methyl-d-aspartate receptor (NMDAR), and clinical characteristics in pediatric patients with anti-NMDAR encephalitis. MethodsThe clinical and laboratory characteristics of hospitalized patients with anti-NMDAR encephalitis, stratified by antibody titers in CSF and disease severity, were retrospectively studied. The demographics, clinical characteristics, main accessory examinations, immunotherapy, and prognosis of patients were recorded, and each observed indicator was statistically analyzed. ResultsA total of 103 pediatric anti-NMDAR encephalitis patients were enrolled in the study, including 41 males (39.8%) and 62 females (60.2%) with a mean age of 8.0 ± 4.0 years. The proportion of patients with cognitive dysfunction and the positive pathogen was higher in the high-titer group than in the low-titer group (p = 0.023, p = 0.042). Consciousness disturbance that occurred as an initial symptom or during the course in the severe group was higher than in the non-severe group (p = 0.002, p < 0.001). More patients in the high-titer group received plasma exchange (PE) than in the low-titer group (p = 0.022). The cure rate of patients was higher with PE (65.2%) than with Corticosteroids and (or) intravenous immunoglobulin (58.1%). ConclusionsPatients with symptoms of disturbance of consciousness may be severer. The severity in pediatric anti-NMDAR encephalitis patients was not correlated with anti-NMDAR antibody titers. Patients with high CSF antibody titers had a better prognosis after early PE therapy.

The Texas Children's Hospital Experience With Pediatric Anti-NMDAR Encephalitis: 2010-2021

Objective Our objective was to characterize the patients with anti-NMDAR encephalitis and identify the most common presenting symptoms and etiologies. Background Anti N-methyl-d-aspartate receptor (NMDAR) encephalitis is a specific autoimmune CNS disorder that decouples electrochemical synapses from their neuronal network, causing seizures, neuropsychiatric symptoms, movement problems, and autonomic dysfunction. Although well studied in the adult population, the clinical characteristics and potential triggers in pediatric cases of anti-NMDAR encephalitis are not well understood. Design/Methods We retrospectively analyzed patients with anti-NMDAR at Texas Children's Hospital (TCH) between 2010 and 2021, characterizing the demographics, presenting symptoms, and underlying etiologies. Results Of the 65 pediatric cases at TCH, our cohort is 65% female and 62% Hispanic, which is 1.6 times higher than the demographics of our TCH patient population and that of the Houston Metropolitan area at large (Harris County, 39% Hispanic). The average age of onset in our pediatric cohort was 7.2 years (range 3 months to 17.9 years). Post-herpetic NMDAR encephalitis and ovarian teratoma associated encephalitis made up 12.8% and 4.2% respectively. Among the idiopathic NMDAR encephalitis group, the most common presenting symptom was focal weakness associated with altered gait and speech regression (39.6%), while 12% presented with isolated psychiatric symptoms. Within our cohort, 100% had behavioral/cognitive symptoms, 79% had seizures, 73% had speech problems, 67% had movement disorder, and 61% had memory deficits. Conclusions Our study describes the clinical characteristics which help define the presenting symptoms and potential etiologies in a heterogenous population from the largest single center pediatric cohort of anti-NMDAR encephalitis to date.

Outcome of Pediatric Anti-N-Methyl-D-Aspartate (NMDA) Receptor Encephalitis in Rural Area of Thailand

AbstractAnti-N-methyl-d-aspartate receptor (NMDAR) encephalitis has a very good outcome; however, there are few reports in developing countries regarding the outcome of pediatric anti-NMDAR encephalitis. We aimed to report the clinical outcome of pediatric anti-NMDAR encephalitis in the rural area of Thailand. This retrospective cohort study recruited children between the age of 1 month and 15 years with the diagnosis of anti-NMDAR encephalitis admitted at Maharat Nakhon Ratchasima Hospital from 1st May 2015 to 31th March 2020. Baseline characteristics and investigation were recorded. The first-line treatment was intravenous pulse methylprednisolone alone, pulse methylprednisolone plus intravenous immune globulin (IVIG), or IVIG alone. The second-line treatment was monthly intravenous cyclophosphamide or azathioprine. The modified Rankin Scale (MRS) was used for evaluation at 1, 3, 6, 12, 18, and 24 months after receiving first-line treatment to determine clinical outcomes. The factors that may affect the outcomes were evaluated. In total, 17 patients were recruited, with the mean age of 8 years, and 76.46% were female. 82.35% of patients developed seizures, and 52.94% turned to status epilepticus. 70.59% received first-line treatment as pulse methylprednisolone plus IVIG. 82.35% received complete follow-up at 12 months. According to MRS score, at first diagnosis 94.12% reported severe disability, at 1 month after receiving treatment, 47.06% improved to moderate–mild disability, and at 6 and 12 months, 40% and 78.57% had complete recovery, respectively. Different types of first-line and second-line treatments did not affect the clinical outcome. Comorbidity of status epilepticus affected MRS. Anti-NMDAR encephalitis has a very good prognosis; prompt diagnosis is necessary. Comorbidity as status epilepticus may affect the clinical outcome.

Childhood encephalitis in the Greater Mekong region: critical to public health policy

Childhood encephalitis in the Greater Mekong region: critical to public health policy

Thyroid Function and Anti-thyroid Antibodies in Pediatric Anti-NMDAR Encephalitis.

Objective: Recent studies found that changes of thyroid antibodies (ATAbs), thyroid hormone, and non-thyroidal illness syndrome (NTIS) characterized by thyroid hormone inactivation with low triiodothyronine and high reverse triiodothyronine followed by suppressed thyroid-stimulating hormone (TSH) in adult anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis were associated with disease severity. This study aimed to explore thyroid function and ATAbs in pediatric anti-NMDAR encephalitis and their clinical association.Methods: We retrospectively analyzed the clinical data of 51 pediatric cases with anti-NMDAR encephalitis hospitalized in Guangzhou Women and Children's Medical Center from August 2016 to 2019.Results: A percentage of 52.9% of patients belonged to the ATAb (+) group, with 26 cases both positive for anti-thyroid peroxidase antibodies (TPOAb) and anti-thyroglobulin antibodies (TGAb), and one patient only positive for TPOAb. A percentage of 62.7% of patients had at least one abnormality in terms of FT3, free thyroxin (FT4), or TSH levels. Meanwhile, 45.1% of patients were diagnosed with NTIS. Among 25 cases retested for thyroid function 2 months after the initial test, the respectively decreased FT3 and FT4 in 13 and 11 cases on admission returned to normal or closer normal than before; TPOAb in eight cases and TGAb in 12 cases were changed from positivity to negativity. Compared with onset, the level of TPOAb and TGAb at relapse remained stable or significantly decreased, respectively. Compared with the ATAb (–) group, the ATAb (+) group had an older onset age, a higher ratio of movement disorders, elevated rate of sleep disorders, increased anti-nuclear antibody positivity rate, and higher ratio of more than one course of intravenous immunoglobulin treatment. There were no significant differences between the NTIS and non-NTIS groups in clinical characteristics.Conclusion: Anti-thyroid antibody positivity, abnormality of FT3, FT4, or TSH levels and NTIS are frequent in pediatric anti-NMDAR encephalitis. Thyroid antibody and thyroid hormone abnormalities could be improved through the course of treatment of anti-NMDAR encephalitis. Cases with ATAbs (+) are at older onset ages and more likely to be treated by intravenous immunoglobulin therapy more than once. Unlike adult anti-NMDAR encephalitis, NTIS might not be associated with the clinical characteristics of anti-NMDAR encephalitis in pediatric patients.

Pediatric anti-N-methyl-d-aspartate receptor encephalitis in southern China: Analysis of 111 cases

ObjectiveTo study the clinical features of children diagnosed with anti-NMDAR encephalitis in southern China. MethodsClinical data of children diagnosed with anti-NMDAR encephalitis from October 2014 to June 2020 from one national regional medical center were analyzed. Neurological disability was assessed by modified Rankin Scale (mRS) throughout the course of disease. Results111 children (M/F = 49/62; mean onset age = 6.8 y) with anti-NMDAR encephalitis were involved. Prodromal events occurred in 34.2% of patients with infectious events being the most common. Seizure was the most common initial symptom, though movement disorder served as the most common event throughout the course of disease. 9.9% of patients had overlapped with other neuronal autoantibodies. Electroencephalogram showed abnormalities with slow wave (100.0%), epileptic discharge (31.5%) and delta brush (8.1%) respectively. 41.4% of patients had abnormal brain MRI, with focal lesions being the most common. None patients had tumor. 80.9% of patients had good response to first line therapy (steroid plus immunoglobulin), while 14 patients accepted second-line therapy (Rituximab) and all had a good response. Boys were significantly more likely to need more course of steroid. 13.8% of patients relapsed. 2 male patients died. mRS score was significantly improved after treatment. 51.4% of patients had a full recovery and 81.7% had mRS score ≤ 2. The median mRS score of boys after treatment was higher than that of girls. Non-infectious prodromal event, past medical history, perivascular lesions in brain MRI, hospital stay, initial mRS score higher than 3, and RTX treatment were independent risk factors associated with poor prognosis, defined as mRS score > 2. ConclusionOf pediatric anti-NMDAR encephalitis in southern China: median onset age around 7 years; girls more common; boys might have poor outcome than girls; seizure or movement disorder respectively being most common onset or course symptom; a few overlapped with other neuronal autoantibodies; rare combined with tumor; most had a good response to immunotherapy and a good prognosis; relapse rate relatively high; fatality rate relatively low; some risk factors associated with poor prognosis.

Diagnosis of pediatric anti-NMDAR encephalitis at the onset: A clinical challenge.

To investigate the clinical and instrumental features at the onset addressing to the diagnosis of anti-NMDAR encephalitis. Twenty children (age: 15 months-17 years; 7 males, 13 females) with initial suspected diagnosis of autoimmune encephalitis, observed between January 2008 and March 2018, were included. The final diagnosis was anti-NMDAR encephalitis in 7 children, other/probable autoimmune encephalitis in 7 children, and primary psychosis in the remaining 6 children. At the clinical onset, anxiety disorder was the main symptom that helped in distinguishing the group of psychotic children from children with non-infectious encephalitis (P=0.05 OR=0.001), while epileptic seizures strongly predicted anti-NMDAR encephalitis (P=0.04 OR=28.6). At the onset, anti-NMDAR encephalitis could be distinguished from other/probable autoimmune encephalitis for the presence of sleep/wake rhythm alteration (P=0.05 OR=15). Among the symptoms occurring during the hospitalization, movement disorders (P=0.031 OR=12) were predictive of non-infectious encephalitis rather than primary psychosis. More specifically, the occurrence of language impairment (P=0.03 OR=33), epileptic seizures (P=0.04 OR=28.6) and catatonia (P=0.03, OR=33), were predictive of anti-NMDAR encephalitis. Also at this stage, anxiety disorder (P=0.03 OR=0.033) was predictive of primary psychosis. Our findings suggest that at the clinical onset epileptic seizures and sleep/wake rhythm alteration represent the main features addressing to the diagnosis of anti-NMDAR encephalitis rather than primary psychosis and other/probable autoimmune encephalitis, while anxiety disorder could be a solid predictor of primary psychosis.

Effectiveness of Mycophenolate Mofetil in the Treatment of Pediatric Anti-NMDAR Encephalitis: A Retrospective Analysis of 6 Cases.

Objective: To explore the effectiveness and safety of mycophenolate mofetil (MMF) as a second-line medication in the treatment of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis, the most common and severe autoimmune encephalitis.Methods: The clinical data of six children with anti-NMDAR encephalitis admitted to the First Hospital of Jilin University were retrospectively analyzed, and the effectiveness and safety of MMF were evaluated.Results: Six children with anti-NMDAR encephalitis were treated with MMF in the 2nd or 3rd treatment disease event (3 cases vs. 3 cases). MMF initiation was mean 19.2 months (range 6–39 months) after disease onset at a mean dose of 25.6 mg/kg (range 19.6–28.4 mg/kg) for 14 months (range 6–26 months). Only two patients had transient mild diarrhea within 2 weeks of MMF application. During follow-up, one patient relapsed whilst on MMF, one patient discontinued MMF, and 4 cases were still on MMF.Conclusion: The use of MMF in anti-NMDAR encephalitis may be effective and safe. MMF can be used as one of the relapse prevention options in patients who already have relapsed or possibly even after the first event. Delayed use may be the main reason for MMF failure.

Clinical outcomes of pediatric Anti-NMDA receptor encephalitis.

To investigate the clinical features and long-term outcomes of pediatric Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. Thirty-two anti-NMDAR encephalitis patients with positive anti-NMDAR antibody test results were recruited. Clinical outcomes were evaluated using the Clinical Assessment Scale in Autoimmune Encephalitis (CASE) and the modified Rankin Scale (mRS). The median age of onset was 9.0 years (range, 0.7-17.2 years). Twenty-four patients (75.0%) were female. All patients received first-line immunotherapy including intravenous immunoglobulin and/or steroid therapy. The second-line immunotherapy was administered to 22 patients (68.8%). Clinical outcomes were evaluated in 27 patients who were followed for longer than 6 months after onset, among whom the median follow-up duration was 31.2 months (range, 6.3-82.9 months). The proportion of patients with ≤2 points on the mRS at their 12-month follow-up was 79.2% (19/24). The CASE scores of these 19 patients ranged from 0 to 5, with language and memory deficits accounting for most of these disabilities. When the outcome was assessed according to onset age (<12 years or 12-18 years), the younger group tended to show a slower recovery over their clinical course. Despite overall favorable clinical outcomes, mild cognitive problems, including language and memory, may persist in pediatric anti-NMDAR encephalitis patients. A specific outcome measure, such as CASE, should be adopted to delineate clinical outcomes and aid the development of individualized treatment plans.

Brain MRI Findings in Pediatric Anti-NMDAR Encephalitis

Anti–N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a treatable autoimmune disorder. MRI findings have been well described in adults but

Early diagnosis of autoimmune encephalitis

Autoimmune encephalitis is a group of treatable autoimmune diseases of central nervous system.Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is one of the most common autoimmune encephalitis.Some of autoimmune encephalitis can be confirmed by specific autoantibodies, but excessive dependence on autoantibody detection usually leads to delayed treatment.This article reviews the clinical presentations of pediatric anti-NMDAR encephalitis and the antibody-negative autoimmune encephalitis so as to improve the pediatricians understanding on the diseases. Key words: Autoimmune encephalitis; Anti-N-methyl-D-aspartate receptor encephalitis; Antibody-negative autoimmune encephalitis

Clinical manifestations, treatment outcomes, and prognostic factors of pediatric anti-NMDAR encephalitis in tertiary care hospitals: A multicenter retrospective/prospective cohort study

ObjectiveAnti-NMDAR encephalitis is an acute autoimmune neurological disorder that is increasingly recognized in pediatric populations. Several studies of the disorder have been conducted worldwide but there are few publications in Thailand. Here, we describe the clinical manifestations, treatment outcomes, and prognostic factors in children with anti-NMDAR encephalitis. MethodsBetween January 2007 and September 2017, we conducted a retrospective/prospective cohort study of children diagnosed with anti-NMDAR encephalitis from three tertiary care hospitals in Thailand: King Chulalongkorn Memorial Hospital, Chonburi Hospital, and Prapokklao Hospital. We assessed the Modified Rankin Score (mRS) score for each participant to measure severity of disease and treatment outcome at baseline, 12, and 24 months. ResultsWe recruited 14 participants (1–13 years with median age 8.4 years). Participants were followed up for a median of 20.5 months. Clinical manifestations included behavioral dysfunction (100%), movement disorder (93%), speech disorder (79%), sleep disorder (79%), and seizures (79%). All patients received first-line immunotherapy (corticosteroids: 100%, intravenous immunoglobulin: 79%, plasma exchange: 21%). Second-line immunotherapy (cyclophosphamide) was administered to 57% of patients. During the first 12 months, 8 patients (62%) achieved a good outcome (mRS ≤ 2). At 24 months, 9 patients (81%) had achieved a good outcome. Altered consciousness and central hypoventilation were predictors of poor outcome. (p < 0.05). ConclusionsWe observed similar clinical manifestation of anti-NMDAR encephalitis in Thai children to those reported in other countries. Furthermore, the percentage of patients with good outcomes in our study was comparable with previous studies. Further studies are required to investigate other populations in other regions of Thailand.

Neuropsychological And Psychopathological Profile Of Anti-Nmdar Encephalitis: A Possible Pathophysiological Model For Pediatric Neuropsychiatric Disorders.

Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is a severe, but treatable, autoimmune disorder, characterized by autoantibodies causing hypofunction of blocking NMDA receptors leading to a unique constellation of cognitive, motor, and psychiatric symptoms. Neuropsychological and psychopathological outcome has not been fully explored, particularly in children. Aim of this study was to investigate pediatric anti-NMDAR encephalitis as a model of impairment of the complex frontal-subcortical circuits who are implicated in several of the childhood neuropsychiatric disorders. Seven children diagnosed with anti-NMDAR encephalitis at our department underwent an evaluation of the global mental functioning before discharge, a neuropsychological and psychological/behavioral standardized examination within one month after discharge and subsequently were followed up longitudinally for mean 35 months (range 24-48 months). Collected neuropsychological data were evaluated retrospectively. Deficits in attention, executive functions and/or visual motor functions involving executive functions were seen in all children within one month after discharge. These deficits were long lasting in about a half of the patients. In addition, four patients developed persistent psychopathological dysfunctions: difficulties to regulate their own behavior, impulsivity, hyperactivity, irritability, apathy, and obsessive-compulsive symptoms. Our data are in line with research suggesting a crucial role of the executive functions impairments in cognitive outcome disturbance of anti-NMDAR encephalitis. We found also behavioral and psychological deficits pointing to a more comprehensive framework of frontal-subcortical dysfunction, in which the NMDA mediated transmission appear to have a role, as suggested by neurobiological, pharmacological, and neuroimaging studies.

Long-term neuropsychological outcome following pediatric anti-NMDAR encephalitis.

ObjectiveTo provide detailed long-term outcome data of children and adolescents following pediatric anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis, to identify neuropsychological impairments, and to evaluate the influence of these factors on quality of life (QoL).MethodsAll Dutch children diagnosed with anti-NMDAR encephalitis were identified. Patients currently aged 4 years or older were included in the follow-up study, consisting of a visit to our clinic for a detailed interview and a standardized neuropsychological assessment. The following domains were included: attention, memory, language, executive functioning, QoL, and fatigue. Primary outcome measures were z scores on sustained attention, long-term verbal memory, QoL, fatigue, and working memory.ResultsTwenty-eight patients were included. Median Pediatric Cerebral Performance Category at last visit was 1 (interquartile range 1–2, range 1–4), and 64% (18/28) of patients returned consistently to their previous school level. Twenty-two patients were included in the cross-sectional part of the long-term follow-up study. Median follow-up time was 31 months (interquartile range 15–49, range 5–91). There were problems with sustained attention (z = −2.10, 95% confidence interval = −2.71 to −1.46, p < 0.0001) and fatigue (z = −0.96, 95% confidence interval = −1.64 to −0.28, p = 0.008). Cognitive deficits were not correlated with QoL, while fatigue was strongly correlated with QoL (r = 0.82, p < 0.0001).ConclusionsAlthough follow-up is often reported as “good” following pediatric anti-NMDAR encephalitis, many patients have cognitive problems and fatigue, even up until adolescence, resulting in academic achievement problems and lower QoL. For physicians, it is essential to be aware of these problems, to provide valuable advice to patients and caregivers in the acute and follow-up phase, and to consider early neuropsychological counseling.

Pediatric Relapsing Anti-NMDAR Encephalitis Crossing with Tumefactive Demyelinating Lesion

AbstractAnti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is an autoimmune encephalopathy characterized by neuropsychiatric symptoms, autonomic instability, and abnormal movements. Most children who undergo tumor removal and receive appropriate immunosuppressants have substantial neurological improvement. We report a 14-year-old girl with relapsing anti-NMDAR encephalitis who presented with paroxysmal tingling and weakness of the right lower limb. Despite long-term prophylaxis with immunotherapy (azathioprine), tumefactive demyelinating lesion and multifocal subcortical white matter lesions were identified on magnetic resonance imaging, which improved after high doses of steroid therapy. There may be overlap between the clinical manifestations of anti-NMDAR encephalitis and acquired demyelination syndromes in children.

Clinical characteristics and pathogen screening of 38 children with anti-N-methyl-D-aspartate receptor encephalitis

Objective To analyze the clinical characteristics and screen pathogens and prognosis of anti-N-methyl-D-aspartate receptor(NMDAR) encephalitis in children and so as to improve the diagnosis and treatment of anti-NMDAR encephalitis in children. Methods A retrospective and follow-up analysis of 38 cases of pediatric anti-NMDAR encephalitis was performed, who were admitted to the West China Second University Hospital, Sichuan University from May 2012 to June 2016, and their clinical features, associated pathogen screening, treatment and prognosis were reviewed. Results Those 38 cases included 14 males and 24 females.The age ranged from 1 year and 10 months to 17 years and 6 months.The most common symptoms were psychiatric symptoms (33 cases), and dyskinesias and movement disorders (33 cases), speech disturbances (31 cases). Twenty-four cases (63.2%) received positive pathogenic screening and only one case had right ovarian mature cystic teratoma.Among them, 11 cases had positive mycoplasma pneumonia immunoglobulin M(IgM) antibodies, 10 cases had positive virus screening, and 1 case had toxoplasma gondii IgM antibodies positive, and 2 cases had mix infection.Symptoms of one case were relieved after anti-toxoplasma treatment without immunosuppression treatment, and another case had partial symptom relief after mycoplasma pneumonia treatment.Thirty-seven cases received immunosuppressant therapy(4 cases were treated with glucocorticoid, 12 cases were treated with glucocorticoid combined with immune globulin, 2 cases were treated with glucocorticoid combined with plasma exchange, still 19 cases were treated with glucocorticoid combined with immune globulin and plasma exchange). After follow-up for median 12 months, 3 cases relapsed, 29 cases were fully recovered, 5 cases had mild language impairment, 2 cases had mild memory impairment, and 2 cases had involuntary movement. Conclusions The most common symptoms of the patients were psychiatric symptoms, dyskinesias and movement disorders, and speech disturbances.Children with anti-NMDAR encephalitis had a low incidence of tumors, while various pathogen infections including mycoplasma pneumonia, virus and toxoplasma gondii, which may trigger abnormal immune response of anti-NMDAR encephalitis.Pathogen screening needs to be done for pediatric anti-NMDAR encephalitis and anti-pathogenic treatment may be helpful for disease remission. Key words: Anti-N-methyl-D-aspartate receptor encephalitis; Pathogen; Child

Intravenous immunoglobulin accompanied with high-dose methylprednisolone therapy for 17 children with anti-N-methyl-D-aspartate receptor encephalitis: Clinic and nursing

ObjectiveAn increasing number of pediatric patients are being diagnosed with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis, whose treatment requires immunotherapy through nursing interventions. This study aimed to analyze the clinical features and long-term prognosis of pediatric anti-NMDAR encephalitis and to gather nursing experiences of immunotherapy. MethodsSeventeen children diagnosed with anti-NMDAR encephalitis were admitted to the pediatric department. They were subjected to a therapy of intravenous immunoglobulin (IVIG) accompanied with high-dose methylprednisolone (HDMP). Multidisciplinary cooperation and intensive care were used to manage them. The effects of nursing intervention and therapy were repeatedly assessed and analyzed throughout the course of treatment and recovery. ResultsNone of the patients manifested adverse drug reaction (ADR) during IVIG administration. At the first administration of HDMP, ADRs were promptly and efficiently treated in four patients (24%; i.e., one case each of hyperglycosemia, hypertension, aggravated symptoms, and gastrointestinal bleed). Two patients underwent rehabilitation, and six patients received hyperbaric oxygenation during hospitalization. Nine patients with indwelling gastric tubes experienced four times of unplanned extubation. Hospital stay ranged from 11 days to 59 days, with the mean duration of 26 days. Discharge evaluation revealed that 16 patients who scored 0–2 on the modified Rankin scale presented obvious remission, and one patient who had a mRS score of 4 exhibited less improvement. The mRS scores of hospitalization, discharge, and six-month follow-up displayed statistically significant differences. ConclusionsNursing interventions of immunotherapy ensures the security of IVIG administration. Multidisciplinary cooperation promotes remission. Our findings can serve as reference for healthcare teams.

Pediatric Anti-N-methyl-D-Aspartate Receptor Encephalitis—Clinical Analysis and Novel Findings in a Series of 20 Patients

To report the clinical features of 20 pediatric patients with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. Review of clinical data, long-term follow-up, and immunologic studies performed in a single center in Spain in the last 4 years. The median age of the patients was 13 years (range, 8 months-18 years), 70% were female. In 12 patients (60%), the initial symptoms were neurologic, usually dyskinesias or seizures, and in the other 40% psychiatric. One month into the disease, all patients had involuntary movements and alterations of behavior and speech. All patients received steroids, intravenous immunoglobulin or plasma exchange, and 7 rituximab or cyclophosphamide. With a median follow up of 17.5 months, 85% had substantial recovery, 10% moderate or severe deficits, and 1 died. Three patients had previous episodes compatible with anti-NMDAR encephalitis, 2 of them with additional relapses after the diagnosis of the disorder. Ovarian teratoma was identified in 2 patients, 1 at onset of encephalitis and the other 1 year later. Two novel observations (1 patient each) include, the identification of an electroencephalographic pattern ("extreme delta brush") considered characteristic of this disorder, and the development of anti-NMDAR encephalitis as post herpes simplex encephalitis choreoathetosis. The initial symptoms of pediatric anti-NMDAR encephalitis vary from those of the adults (more neurologic and less psychiatric in children), the development of a mono-symptomatic illness is extremely rare (except in relapses), and most patients respond to treatment. Our study suggests a link between post herpes simplex encephalitis choreoathetosis and anti-NMDAR encephalitis.