Recent research on cytokines associated with anti-N-methyl-D-aspartate receptor encephalitis / 中国当代儿科杂志

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune inflammatory disease of the central nervous system, and little is known about its immune mechanism at present. There is a lack of disease-related biomarkers in cerebrospinal fluid except anti-NMDAR antibody, which leads to delayed diagnosis and treatment in some patients. Therefore, there has been an increasing number of studies on related cytokines in recent years to assess whether they can be used as new biomarkers for evaluating disease conditions and assisting diagnosis and treatment. Current studies have shown that some cytokines may be associated with the progression of anti-NMDAR encephalitis, and this article reviews the research advances in such cytokines associated with anti-NMDAR encephalitis.

Clinical features of autoimmune encephalitis secondary to epidemic encephalitis B in 5 children / 中国当代儿科杂志

OBJECTIVES@#To study the clinical features of children with autoimmune encephalitis (AE) secondary to epidemic encephalitis B (EEB).@*METHODS@#A retrospective analysis was performed on the medical data of five children with EEB with "bipolar course" who were treated in Children's Hospital Affiliated to Zhengzhou University from January 2020 to June 2022.@*RESULTS@#Among the five children, there were three boys and two girls, with a median age of onset of 7 years (range 3 years 9 months to 12 years) and a median time of 32 (range 25-37) days from the onset of EEB to the appearance of AE symptoms. The main symptoms in the AE stage included dyskinesia (5/5), low-grade fever (4/5), mental and behavioral disorders (4/5), convulsion (2/5), severe disturbance of consciousness (2/5), and limb weakness (1/5). Compared with the results of cranial MRI in the acute phase of EEB, the lesions were enlarged in 3 children and unchanged in 2 children showed on cranial MRI in the AE stage. In the AE stage, four children were positive for anti-N-methyl-D-aspartate receptor antibody (one was also positive for anti-γ-aminobutyric acid type B receptor antibody), and one was negative for all AE antibodies. All five children in the AE stage responded to immunotherapy and were followed up for 3 months, among whom one almost recovered and four still had neurological dysfunction.@*CONCLUSIONS@#EEB can induce AE, with anti-N-methyl-D-aspartate receptor encephalitis as the most common disease. The symptoms in the AE stage are similar to those of classical anti-N-methyl-D-aspartate receptor encephalitis. Immunotherapy is effective for children with AE secondary to EEB, and the prognosis might be related to neurological dysfunction in the acute phase of EEB.

Analysis of the risk factors for poor prognosis and recurrence in patients with anti-NMDAR encephalitis / 中华预防医学杂志

To investigate the risk factors of poor prognosis and recurrence in patients with anti-NMDAR encephalitis. A single center, observational cohort study was used to retrospectively analyze 44 patients with anti NMDAR encephalitis hospitalized in the Department of Neurology of Beijing Tong Ren Hospital from January 2014 to October 2020. The results showed that the interval from onset to immunotherapy in the poor prognosis group was significantly longer than that in the good prognosis group (t=2.045,P=0.047), and the course of disease in the poor prognosis group was significantly longer than that in the good prognosis group (t=4.127,P=0.000 2). The number of patients with clinical manifestations of dyskinesia was significantly increased (Fisher exact test: P=0.014). The patients with abnormal brain MRI in the poor prognosis group were significantly more than those in the good prognosis group (Fisher exact test: P=0.017), and the patients with slow wave>50% in the poor prognosis group were significantly more than those with slow wave <50% (Fisher exact test: P<0.001). Patients with the first onset of immunotherapy time <3 months, long course of disease, high intracranial pressure, and high cerebrospinal fluid protein are prone to relapse. Bivariate logistic regression analysis showed that patients with dyskinesia, abnormal brain MRI, and slow wave EEG more than 50% were risk factors for poor prognosis (OR values were 4.687, 4.978, and 24.500, respectively; P values were 0.018, 0.016, and 0.000, respectively). The time of first-line immunotherapy for the first onset<3 months was the risk factor for recurrence (OR 17.231, P=0.010). In conclusion, dyskinesia, abnormal brain MRI and slow wave of EEG more than 50% may be the risk factors for poor prognosis of patients. The duration of immunotherapy less than 3 months after the first onset might be the risk factor for recurrence.

Anti-NMDA-receptor encephalitis in Filipino adults: Case series and outcomes in a tertiary government hospital in the Philippines

INTRODUCTION@#We performed a case series of all five (5) confirmed adult Filipino cases of Anti-N-Methyl-D-Aspartate receptor (anti-NMDA-R) encephalitis in a tertiary government hospital in the Philippines admitted in the past three years. Two cases were identified with unique features: (1) a 23-year old female who presented with combined refractory seizures and persistent chorea and orofacial dyskinesias; and (2) a 22-year old male who presented with refractory epilepsia partialis continuua. The rest of the patients were hereby presented.@*BACKGROUND@#In the past years, anti-NMDA-R encephalitis has been considered a diagnosis of exclusion in lieu of other infectious causes of encephalitis. It is rare and an emerging disease with an incidence estimated at approximately 2-3 cases per million. Recent literature recorded severe cases of anti-NMDA-R encephalitis that presented as intractable first onset seizures, combined with hyperkinetic movement disorders, acute psychosis without a premorbid condition, and dysautonomia. @*OBJECTIVES@#To present the clinicodemographic profile and to discuss the management and outcomes of patients with anti-NMDAR encephalitis in a tertiary hospital in the Philippines.@*RESULTS@#Here, we report five confirmed cases of anti-NMDA-R encephalitis admitted in 2019-2021. The mean age is 23 years old, with 4:1 female to male ratio with a median length of hospitalization of 58 days. All patients presented with acute psychiatric symptoms without premorbid condition, focal and generalized seizures, decreased consciousness, dyskinesias, and autonomic instability. Four patients needed airway support for central hypoventilation, one had first onset seizure that developed into refractory epilepsia partialis continuua, one had persistent chorea and orofacial dyskinesia. Imaging studies of the brain included contrast-enhanced CT Scan and MRI with unremarkable findings. No female patients had an ovarian teratoma as revealed in the whole abdominal ultrasound. All CSF analysis for anti-NMDA-receptor was done in the same laboratory outside the hospital which revealed positive for NMDA-receptor antibodies, while CSF lymphocytic pleocytosis was only seen in 1/5 and protein elevation in 4/5. All of the patients underwent electroencephalogram (EEG) studies which revealed diffuse delta-theta slowing without epileptiform discharges. The patient who had persistent chorea and orofacial dyskinesias showed extreme delta brush, while one had normal EEG findings. They all received high-dose steroid and intravenous Immunoglobulin (IVIg); three patients were able to undergo Rituximab infusion. Only one female patient had mild deficits, one female was discharged fully functional and ambulatory from being weaned off from the mechanical ventilator, one female had aborted cardiac arrest and was discharged bedridden at GCS 10, and two died due to the other concomitant medical conditions. The Modified Rankin Scale (MRS) and Mini-mental Status Examination (MMSE) were used to assess the neurological and functional outcomes of our patients. @*CONCLUSION@#Anti-NMDA-R encephalitis is an emerging neurological disorder that warrants early identification as it impacts timeliness of management and long-term outcomes.

Reporte de un caso fatal de encefalitis autoinmunitaria asociada a teratoma ovárico / Report of a fatal case of autoimmune encephalitis associated with an ovarian teratoma

INTRODUCCIÓN: El teratoma del ovario es el tumor de células germinales más frecuente. Entre sus complicaciones se describen algunos síndromes paraneoplásicos, como la encefalitis por anticuerpos contra el receptor N-metil-D-aspartato (NMDA). CASO CLÍNICO: Mujer de 22 años sin antecedentes de importancia que consulta por cuadro clínico de 4 días de evolución caracterizado por síntomas psiquiátricos y un episodio convulsivo. Se considera un cuadro de meningoencefalitis y se indica manejo antibiótico de amplio espectro y antiviral. Ante el deterioro se sospecha una encefalitis autoinmunitaria, se identifica un tumor anexial en los estudios imagenológicos compatible con teratoma y se confirma el diagnóstico con el hallazgo de anticuerpos NMDA en el líquido cefalorraquídeo. A pesar de su resección y manejo sistémico, fallece a los 5 meses. DISCUSIÓN: La encefalitis autoinmunitaria asociada a un teratoma es inusual, pero es una complicación que debe sospecharse como diagnóstico de exclusión. La mayoría tienen un pronóstico favorable, aunque hasta una cuarta parte de los casos puede asociarse a daño irreversible en la corteza del hipocampo e incluso la muerte, principalmente cuando el diagnóstico y el tratamiento son tardíos. CONCLUSIONES: Este caso es un reto clínico que representa un vacío en la evidencia actual, puesto que no existe un estándar de manejo de los teratomas. Se propone que, una vez diagnosticado un teratoma, se realice una cistectomía ovárica. Son necesarios más estudios para validar esta recomendación.

INTRODUCTION: The ovarian teratoma is the most common cell germ tumor. Some paraneoplastic syndromes have been described, including the anti-N-methyl-D-aspartate (NMDA) receptor encephalitis as part of its complications. CASE REPORT: A 22 years old female patient with no important medical history, consults due to an acute psychotic disorder and a convulsion. A meningoencephalitis was considered and broad-spectrum antibiotics and antivirals were started. Faced with deterioration, an autoimmune encephalitis is considered as well. Imaging studies revealed an ovarian teratoma and diagnosis was confirmed with antibodies against NMDA receptor in cerebrospinal fluid. Despite its resection and systemic management, the patient dies after 5 months. DISCUSSION: The autoimmune encephalitis associated with an ovarian teratoma is rare, its a complication that must be suspected as an exclusion diagnosis and most have a favorable prognosis, however up to a quarter of cases can be associated with irreversible damage to the hippocampal cortex and even death, mainly when late diagnosis and treatment are made. CONCLUSIONS: This case is a clinical challenge, no evidence is available since there is no standard for teratoma management. It is proposed that once a teratoma is diagnosed, an ovarian cystectomy is performed. Further studies are necessary to validate this recommendation.

Encefalitis autoinmune en pediatría. Receptor antirreceptor N-metil D-aspartato (Anti-R-NMDA) / Autoimmune encephalitis in pediatrics. N-methyl D-aspartate Receptor Receiver (Anti-R-NMDA)

La encefalitis es un desorden inflamatorio del encéfa- lo que deriva en un estado mental alterado, crisis con- vulsivas, déficits neurológicos focales, acompañado usualmente de signos de inflamación en el líquido ce- falorraquídeo y hallazgos en la resonancia magnética que pueden ir desde normalidad hasta anormalidades extensas. El anticuerpo anti RNMDA es el que origi- na la mayoría de los casos de encefalitis autoinmune en niños y adultos jóvenes, seguido por el anticuerpo anti LGI1 de presentación en el adulto. Se caracteri- zan por estar frecuentemente asociadas a algún tumor, responder a la inmunoterapia y provocar daños cere- brales variables que se manifiestan como cuadros clí- nicos polimorfos (desde la disfunción límbica hasta una encefalopatía multifocal o difusa). La prevalencia exacta de las encefalitis autoinmunes es incierta, exis- tiendo un aumento importante del reporte de casos en los últimos 10 años, asociado al avance en la pesqui- sa de los anticuerpos contra un antígeno celular espe- cífico. Todas las encefalitis autoinmunes responden a terapia inmunomoduladora, generalmente la evolución a la mejoría es lenta y puede completarse en meses o incluso en un año o más. Revisaremos generalidades de la encefalitis autoinmune, su forma más común y discutiremos su enfoque diagnóstico y tratamiento...(AU)

Clinical characteristics of antiNmethylaspartate receptor encephalitis in children / 中南大学学报(医学版)

OBJECTIVES@#To analyze the clinical characteristics and prognosis of children with anti-N-methyl--aspartate receptor (NMDAR) encephalitis and to provide a basis for early clinical identification of this disease.@*METHODS@#The clinical data of 42 cases of anti-NMDAR encephalitis at Department of Pediatrics, Second Xiangya Hospital, Central South University from January 2015 to March 2018 were collected. The clinical features and followed-up outcomes were analyzed retrospectively.@*RESULTS@#There were 15 cases (35.7%) of males and 27 cases (64.3%) of females in 42 children, with a ratio of 1꞉1.8. They were aged from 4 months to 17 years, with an average of (9.20±4.66) years. The most common initial symptoms were seizures (47.6%, 20/42) and mental behavior disorder (35.7%, 15/42). During the course of the disease, 85.7% patients(36/42) had mental and behavior disorder, 85.7% patients (36/42) had epilepsy, 76.2% (32/42) had speech disorder, 66.7% patients (28/42) had dyskinesia, 66.7% patients (28/42) had the decreased level of consciousness, 61.9% patients (26/42) had autonomic instability, and 57.1% (24/42) patients had sleep disorder. All the children had positive antibody against NMDA receptor resistance encephalitis in cerebrospinal fluid. Head MRI showed the abnormal incidence was 50.0% (21/42), and the lesions involved in parietal lobe, frontal lobe, temporal lobe, occipital lobe, midbrain, thalamus, basal ganglia and optic nerve. There was a patient with optic nerve damage combined with myelin oligodendrocyte glycoprotein (MOG) antibody positive. Forty cases were examined by electroencephalogram (EEG), 92.5% cases (37/40) were abnormal, mainly showing diffuse slow waves, and δ brushes could be seen in severe cases. And there was 1 patient (2.4%) complicated with mesenteric teratoma. The mRS score (2.14±1.46) at discharge was significantly lower than the highest mRS score (3.88±1.38) during hospitalization (<0.05). After 3-39 months of follow-up, mRS score at 3 months after discharge was only 0.81±1.29, which was still improved compared with that at discharge, 76.2% cases (32/42) experienced complete or near-complete recovery (mRS score≤2), and 4.8% (2/42) cases relapsed. There was no mortality; the initial time of immunotherapy and the highest mRS score in the course of the disease were the factors affecting the prognosis. The earlier the starting time for immunotherapy and the lower mRS score in the course of the disease were, the better the prognosis was.@*CONCLUSIONS@#Seizures, mental and behavior disorder, dyskinesias, speech disorder and autonomic instability are common clinical manifestations of anti-NMDAR encephalitis in children. The effect of immunotherapy is significant, and the time to start immunotherapy and the severity of the disease are important factors affecting the prognosis. Anti-NMDAR encephalitis can be combined with other autoantibodies, but its clinical significance and mechanism need further study.

A Case of Anti-NMDA Receptor Encephalitis with Normal Findings on Initial Diagnostic Tests

No abstract available.

Cryptococcal Encephalitis Complicating Anti-N-methyl-D-aspartate Receptor Encephalitis in an Immunosuppressed Patient / 中国医学科学院学报

Cryptococcal encephalitis is a fatal central nervous system infectious disease,whereas anti-N-methyl-D-aspartate(NMDA)receptor encephalitis(NMDARE)is an autoimmune syndrome associated with psychological symptoms,behavioural abnormalities,seizures,and dyskinesias.Despite their distinct pathologies and pathogenic mechanisms,both of them can lead to cognitive dysfunction and abnormal behaviors,although anti-NMDARE can also have mood and mental disorders as its core manifestations.A patient with nephrotic syndrome accompanied by both cryptococcal encephalitis and anti-NMDARE was treated in our center,which for the first confirmed that these two conditions could coexist in one patient.The underlying mechanism may be similar to that of anti-NMDARE after other infections.

Anti N-Methyl-D-Aspartate Receptor (NMDR) encephalitis in a 28-year old female with ovarian follicular cyst presenting with status epilepticus

@#Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a potentially fatal autoimmune condition where antibodies are produced and destroy NMDA receptors in the brain, thereby causing profound dysfunctional neurotransmission. This rare disease is often associated with a tumor, usually a teratoma. A 28-year-old female previously diagnosed with seizure disorder presented with chronic history of neuropsychiatric symptoms leading to status epilepticus on the day of confinement. Investigation showed the presence of anti-NMDAR antibodies both in serum and cerebrospinal fluid. A right ovarian cyst was noted on ultrasound. Methylprednisolone and immunoglobulin were given followed by surgical removal of the ovarian cyst. Histopathology result showed a follicular cyst. The patient was discharged improved, and remained asymptomatic and seizure free. Repeat serum NMDAR antibody test was negative. Early diagnosis and prompt treatment of patients with NMDA receptor encephalitis is lifesaving. Although mostly associated with an ovarian teratoma, the case reported a possible link between NMDAR encephalitis and benign ovarian cysts.

Research progress on electroencephalogram characteristics of anti-N-methyl-D-aspartate receptor encephalitis / 浙江大学学报·医学版

Anti-N-methyl-d-aspartate (NMDA) receptor encephalitis is a kind of autoimmune disease aiming at NR1 subunit of NMDA receptor. In the early stage, functional damage is the main cause. Electroencephalogram (EEG) can reflect the abnormal brain function by recording the changes of EEG signals. The common EEG patterns of anti NMDA receptor encephalitis are slow wave abnormality, epileptic discharge, a large number of β activity, extreme delta brush, etc. Here we review the waveform characteristics, origin, pathogenesis and clinical value of EEG in patients with NMDA receptor encephalitis.

Encefalitis por anticuerpos contra el receptor de NMDA. Reporte de un caso y revisión de la literatura / Anti-NMDA receptor encephalitis. Case Report and Literature Review / Encefalite causada por anticorpos contra o receptor NMDA. Relatório de caso e revisão da literatura

Resumen: La encefalitis por anticuerpos contra el receptor de NMDA es un desorden grave, tratable y potencialmente reversible, caracterizado por la presencia de alteraciones en el comportamiento, convulsiones y trastornos del movimiento. La presencia de anticuerpos contra el receptor del glutamato (anti-NMDA) en plasma o líquido cefalorraquídeo es específico para el diagnóstico de la enferme -dad. El reconocimiento temprano de la enfermedad es vital para el pronóstico del paciente, dado que el manejo precoz facilita la recuperación y reducción de la morbimortalidad. El tratamiento consiste en la utilización de corticoides, inmunoglobulina intravenosa o plasmaféresis como primera línea de terapia; además de otros inmunomoduladores, como ciclofosfamida o rituximab, como segunda línea. La recuperación es lenta, pero presenta mejoría en la medida en que los títulos de anticuerpos disminuyen y llega a una recuperación completa hasta en 75 % de los pacientes.

Abstract: Anti-NMDA receptor encephalitis is a severe, treatable, and potentially reversible disorder characterized by the presence of behavioral disturbances, seizures, and movement disorders. The presence of antibodies against the glutamate receptor (anti-NMDA) in plasma or cerebrospinal fluid is specific to the diagnosis of the disease. Early recognition of the disease is vital for the patient's prognosis since early management facilitates recovery and reduction of morbidity and mortality. Treatment consists of corticosteroids, intravenous immunoglobulin, or plasmapheresis as the first line of therapy, in addition to other immunomodulators, such as cyclophosphamide or rituximab, as the second line. Recovery is slow but improves as antibody titers decrease. Complete recovery occurs in up to 75 % of patients.

Resumo: A encefalite causada por anticorpos contra o receptor NMDA é um distúrbio grave, tratável e potencialmente reversível, caracterizado pela presença de alterações comportamentais, convulsões e transtornos de movimento. A presença de anticorpos contra o receptor de glutamato (anti-NMDA) no plasma ou no líquido cefalorraquidiano é específica para o diagnóstico da doença. O reconhecimento precoce da doença é vital para o prognóstico do paciente, visto que o tratamento precoce facilita a recuperação e a redução da morbimortalidade. O tratamento consiste no uso de corticoides, imunoglobulina intravenosa ou plasmaférese como primeira linha de terapia. Além de outros imunomoduladores, como ciclofosfamida ou rituximabe, como segunda linha. A recuperação é lenta, mas apresenta melhora à medida que os títulos de anticorpos diminuem e a recuperação completa ocorre em até 75% dos pacientes.

Encefalitis autoinmunes: Receptor anti-NMDA y nuevos inmunofenotipos / Autoimmune encephalitis. Anti-NMDA receptor and new immunophenotypes

Las encefalitis autoinmunes (EA) se definen como síndromes neurológicos de instalación subaguda de compromiso de conciencia, alteración de la memoria de trabajo y trastornos psiquiátricos frecuentemente asociados a movimientos anormales y crisis epilépticas y que se producen por la acción de anticuerpos anti neuronales específicos que se fijan a receptores de neurotransmisores o proteínas de membrana. El anticuerpo anti NMDAR es el que origina la mayoría de los casos de EA en niños y adultos jóvenes, seguido por el anticuerpo anti LGI1 de presentación en el adulto. Las EA han aumentado en la última década, en la que se ha descrito un gran número de nuevos anticuerpos que producen en su mayoría síndromes neurológicos que involucran al sistema nervioso central, con predominio de signología psiquiátrica, excepto en niños en los que predominan movimientos anormales, crisis epilépticas y compromiso de conciencia. Se asocian frecuentemente a tumores en el adulto pero en los niños esta asociación es más rara. Todas las EA responden a terapia inmunomoduladora aunque en diferente medida según el tipo de anticuerpo involucrado. Generalmente la evolución a la mejoría es lenta y puede completarse en meses o incluso en un año o más. En esta revisión se destaca los principales cuadros de EA relacionados con anticuerpos específicos mencionando también los inmunofenotipos descubiertos recientemente.

Autoimmune encephalitis (AE) is defined as neurological syndromes of subacute installation of compromise of consciousness, alteration of working memory and psychiatric disorders associated with abnormal movements and epileptic seizures and that are produced by the action of anti-neuronal antibodies. They bind to neurotransmitter receptors or membrane proteins. Antibody to NMDAR is the origin of the majority of cases of AD in children and young adults, followed by anti-LGI1 antibody for presentation in adults. The AE has increased in the last decade, with a large number of new agents described that produce mostly neurological syndromes that involve the central nervous system, with predominance of psychiatric signaling, except in children and the predominant abnormal movements, epileptic seizures and compromise of conscience. They are frequently associated with tumors in adults but in children this association is more infrecuent. All AEs respond to immunomodulatory therapy although in different measures depending on the type of antibody involved. In general, the evolution to improvement is slow and can be completed in months or even in one year or more. In this review, the main EA clinical pictures related to specific antibodies are highlighted, also mentioning recently discovered immunophenotypes.

Banderas rojas para sospechar encefalitis anti-NMDAr en un primer episodio psicótico: reporte de dos casos / Red flags for suspecting anti-NMDAr encephalitis in a first psychotic episode: report of two cases

RESUMEN La encefalitis asociada a anticuerpos contra el receptor de N-metil-D-Aspartato (NMDAr) es una entidad clínica recientemente descrita con un número creciente de casos reporta dos. Los síntomas psiquiátricos en etapas tempranas de la enfermedad conforman un reto diagnóstico para el médico tratante. Presentamos dos casos clínicos: el caso clínico 1, un hombre de 26 arios y el caso clínico 2, un joven de 18 arios, ambos abordados como primer episodio de psicosis y hospitalizados en instituciones psiquiátricas. Posteriormente, ambos casos fueron diagnosticados como encefalitis anti-NMDAr. La alta prevalencia de síntomas psiquiátricos en la encefalis anti-NMDAr demanda a los médicos psiquiatras y neurólogos a tener un alto índice de sospecha en presencia de síntomas atípicos en pacientes evaluados por primer episodio de psicosis.

ABSTRACT Anti-N-methyl-D-Aspartate receptor (NMDAr) encephalitis is a recently described clinical entity with an increasing number of reported cases. Psychiatric symptoms in the early stages of the disease constitute a diagnostic challenge for the treating physician. We present two clinical cases: clinical case 1, a 26-year-old man, and clinical case 2, an 18-year-old man; both presented with a first episode of psychosis and were hospitalized as psychiatric disorders. Subsequently, both cases were diagnosed as anti-NMDAr encephalitis. The high prevalence of psychiatric symptoms in anti-NMDAr encephalitis forces psychiatrists and neurologists to have a high degree of suspicion in the presence of atypical symptoms in patients evaluated for the first episode of psychosis.

Clinical features of anti-N-methyl-D-aspartate receptor encephalitis and the concomitant seizure / 中南大学学报(医学版)

To investigate the clinical features, auxiliary examination and characteristics for anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis and its concomitant seizure.
 Methods: A total of 20 patients diagnosed as anti-NMDAR encephalitis were enrolled from January 2016 to September 2018 in Xiangya Hospital. The data including the clinical features, auxiliary examination, characteristics of seizure, treatment and prognosis were collected. The discharged patients were followed up for half a year.
 Results: The initial symptom in patients with anti-NMDAR encephalitis were mainly psychiatric symptom and seizure. Most of the EEG result were diffused slow waves. The mainly type of seizure in patients with anti-NMDAR encephalitis showed generalized tonic-clonic seizure. Patients occurred consciousness during the onset of the disease. MRI showed that patients with temporal lobe were more inclined to occur seizure than patients with anti-NMDAR encephalitis (P<0.05). After standardized treatment, 20 patients showed a significant improvement in modified Rankin Scale (mRS) scores and the seizure was under control within half a year. 
 Conclusion: Patients with temporal lobe affected in MRI should pay attention to the possibility of seizure occurrence. Anti-epileptic drugs and immunotherapy should be used promptly in patient with seizure. After standardized treatment, the prognosis of patients will be mostly good.

Childhood Onset of Anti-N-Methyl-D-Aspartate Receptor Encephalitis Without Teratoma Masquerading as a Psychotic Disorder

Many neurologic disorders manifest as psychiatric symptoms. Anti-N-Methyl-D-Aspartate (NMDA) receptor encephalitis is an autoimmune disease of the brain characterized by numerous neurological and psychiatric features. Despite being rare, its prevalence is rapidly increasing and early management is critical in ensuring successful and sustainable recovery. Therefore, the illness should be considered as a differential diagnosis when clinically assessing patients. This report presents a case of a female child who was hospitalized for acute psychiatric manifestations, which was later confirmed as anti-NMDA receptor encephalitis. She recovered relatively successfully after combined neurological and psychiatric treatment. This report provides information on the clinical course of early onset anti-NMDA receptor encephalitis, including treatment strategy and prognosis.

Factors Affecting the Response to First-Line Treatments in Patients with Anti-N-Methyl-D-Aspartate Receptor Encephalitis

BACKGROUND AND PURPOSE: Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is the most common type of autoimmune encephalitis. This study aimed to explore the possible factors affecting the response to first-line treatments in patients with anti-NMDAR encephalitis. METHODS: We enrolled 29 patients who were diagnosed as anti-NMDAR encephalitis between January 1, 2015, and June 30, 2018. They were divided into the remission and nonremission groups according to their response to first-line treatments. The demographics, clinical manifestations, main ancillary examinations, follow-up treatments, and prognosis of patients were recorded. The symptoms reported on in this study occurred before treatments or during the course of first-line treatments. RESULTS: There were 18 patients (62.07%) in the remission group and 11 patients (37.93%) in the nonremission group. Compared to the remission group, a higher proportion of the patients in the nonremission group exhibited involuntary movements, decreased consciousness, central hypoventilation, lung infection, and hypoalbuminemia. The nonremission group had a high incidence of increased intracranial pressure and significant elevations of the neutrophil-to-lymphocyte ratio in peripheral blood (NLR), aspartate aminotransferase, and fibrinogen. Six patients (54.55%) in the nonremission group received second-line immunotherapy. Only one patient (3.45%) died, which was due to multiple-organ failure. CONCLUSIONS: Anti-NMDAR-encephalitis patients with more symptoms—especially involuntary movements, disturbance of consciousness, central hypoventilation, and accompanying hypoalbuminemia and pulmonary infection—may respond poorly to first-line treatments. Positive second-line immunotherapy therefore needs to be considered. Admission to an intensive-care unit, increased cerebrospinal fluid pressure, and increased NLR might be the significant factors affecting the response to first-line treatments.

Anti-N-methyl-D-aspartate Receptor Encephalitis in a Patient with Thyroid Eye Disease

No abstract available.

A Case of Anti-N-Methyl-D-Aspartate Receptor Encephalitis Associated with Glioma of the Pons

No abstract available.

Ovarian teratoma associated Anti-N-methyl-D-aspartate receptor encephalitis: a difficult diagnosis with a favorable prognosis

Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a recently described auto-immune and paraneoplastic encephalitis with prominent neuropsychiatric manifestations affecting young adults with ovarian teratoma. The availability of a novel assay to measure these antibodies might suggest an etiology for this potentially life-threatening disease, which if early recognized can be treated promptly with surgery with chances of a good clinical outcome. Reported prognostic indicators for a good outcome depend on the presence of a tumor, prompt treatment and no admission to an intensive care unit. However, due to the rarity and unawareness of this disease, the diagnosis may be delayed as primary psychiatric disorders, and infective encephalitis is taken more into consideration and ruled out first. Here we report a case of anti-NMDAR encephalitis in a 22-year-old female prompted by an ovarian teratoma with a gradual and complete resolution of symptoms after surgical excision of the teratoma and immunomodulating therapies.