Case Of anti-NMDAR Encephalitis Research Articles

Giant immature teratoma in a 15 year old causing anti-n-methyl-d-aspartate (anti-nmda) receptor encephalitis-like and Guillain-Barré Syndrome as paraneoplastic manifestations: A case zreport

Germ cell tumor is the most prevalent ovarian tumor in young women between 10 and 30 years of age.[1] However, immature teratomas account for only 20% of the malignant ovarian tumors found in the adolescent age group.[2] More uncommon is the occurrence of immature teratoma causing anti-N-methyl-D-aspartate receptor (Anti-NMDAR) encephalitis and Guillain-Barré Syndrome (GBS). This is a case of a 15-year-old female, nulligravid, who initially presented sudden behavioral change and symmetrical weakness of both lower and upper extremities with concomitant seizure episodes and with palpable lower abdominal mass. The patient was diagnosed to have GBS and treated with intravenous immunoglobulin causing resolution of neurologic symptoms months after. During the management of GBS, the patient noted increasing abdominal girth. Tumor markers showed elevated alpha-fetoprotein, CA125, and lactate dehydrogenase. An imaging study revealed predominantly solid ovarian mass hence malignancy was considered. Once the medical condition stabilized, the patient underwent fertility-sparing surgery with the final histopathologic result of immature teratoma. The aim of this report is to present a case of anti-NMDAR encephalitis and GBS as paraneoplastic manifestation of immature teratoma.

Anti-NMDAR Encephalitis with Relapsing Optic Neuritis

Autoimmune encephalitis with antibodies to NMDA receptors (anti-NMDAR encephalitis) is a frequent form of autoimmune encephalitis. The disease is curable, though the lack of timely treatment can lead to disability in patients or death. Difficulties in the diagnosis of anti-NMDAR encephalitis arise from its heterogenous manifestations, its possible combination with other autoimmune diseases, and lack of doctors’ awareness of this type of encephalitis. This article reviews published data and describes a case of anti-NMDAR encephalitis with atypical features in the form of relapsing optic neuritis; it was difficult to link optic nerve lesions with comorbidity with multiple sclerosis or optic neuromyelitis spectrum disorders.

Anti-NMDAR encephalitis presenting with early and prominent orolingual dyskinesia and its association with ovarian serous cystadenoma- case report

Autoimmune encephalitis is relatively a new entity. Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is one of the most common and well studied autoimmune encephalitis. We report a case of anti-NMDAR encephalitis presenting initially with orolingual dyskinesia and its association with large ovarian serous cystadenoma. She was given complete course of intravenous immunoglobulin (2GM/KG) as well as removal of the tumor. Histopathology of the cystic lesion revealed serous cystadenoma of the ovary. The patient is now on regular follow up and is doing well. To best of our knowledge, this is the first case in literature showing association of anti-NMDAR encephalitis with serous cystadenoma of ovary and orolingual dyskinesia as initial clinical presentation. Keywords: AntiNMDAR encephalitis, Intravenous immunoglobulin (IVIG), Serous cystadenoma of ovary.

A case of anti-NMDAR encephalitis with peculiar gyratory events that reignites the epilepsy versus movement disorders debate

A case of anti-NMDAR encephalitis with peculiar gyratory events that reignites the epilepsy versus movement disorders debate

Cerebrospinal fluid CD20 positive B-cell expansion in a case of anti-NMDAR encephalitis

Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is a potentially fatal autoimmune encephalitis with a strong B-cell response. We measured the proportion of CD20 positive and CD19 positive B-cells in the CSF from a case of severe anti-NMDAR encephalitis. The proportion of CD20 positive B-cells in the CSF was 15.0%, higher than CD19 positive B cells (10.1%), and higher than that seen in non-inflammatory neurological disorders (<1%). After the treatments of steroids, intravenous immunoglobulin and rituximab to eliminate B-cells, she recovered. These findings further support the use of rituximab that targets CD20 positive B-cells in anti-NMDAR encephalitis.

A case of NMDAR encephalitis treated in the third trimester - novel arterial spin labeling findings and a review of literature

Diagnosis and treatment of anti-NMDAR encephalitis during pregnancy are challenging. We report a case of anti-NMDAR encephalitis in a 28-year-old, 24 weeks pregnant woman. Her brain magnetic resonance imaging was normal, but arterial spin labeling (ASL) showed increased cerebral blood flow in the right insula and temporal area. She was successfully treated with emergent cesarean section and immunotherapies including intravenous immunoglobulin, steroids, and rituximab. The newborn was healthy and she recovered fully. Early suspicion and proper management is important in treatment of anti-NMDAR encephalitis during pregnancy. ASL can help diagnosing anti-NMDAR encephalitis during pregnancy.

Clinical characteristics of anti-N-methyl-D-aspartate receptor encephalitis in children.

To analyze the clinical characteristics and prognosis of children with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis and to provide a basis for early clinical identification of this disease. 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. 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 (P<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. 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.

New Onset Focal Seizure Clusters in Children: Expanding the Spectrum of Anti NMDAR Encephalitis.

Anti-N-methyl-d-aspartate receptor (Anti-NMDAR) Encephalitis classically presents with polysymptomatic presentation of behavioral or cognitive changes, seizures, and focal deficits. Large series in adults and children have described the above features. Monosymptomatic presentation of Anti NMDAR encephalitis is rare and in literature single case reports in adults and children are available. Here we report a series of 6 children presenting with seizure alone and thus expanding the clinical spectrum of Anti NMDAR encephalitis. This is a a retrospective case series of 6 cases of anti NMDAR encephalitis treated in our institute, which is a tertiary referral center between 2010 and 2014. All the patients with NMDA encephalitis were initially included. The baseline demographics, clinical presentations, investigations (CSF, MRI and EEG), course in the hospital, details of treatment, short and long term outcomes were documented from the available medical records. Children presenting with monosymptomatic seizure clusters were only included in the final analysis. Twenty eight children were diagnosed with ant NMDA R encephalitis during the study period. 22 children had classical polysymptomatic presentations and were not included in this analysis. The remaining 6 children (5 girls and one boy), who presented with only acute seizure clusters were included in the study. All children presented with acute cluster of focal seizures. Four out of six had focal status epilepticus while 2 out of six had recurrent focal seizures. Commonest semiology was clonic seizure in 4/6 and one child had dystonic seizure and one had tonic seizure. All patients were started on steroids and antiepileptic drugs. No other immunomodulators or immunosuppressants were used. On discharge all patients where seizure free and with no focal deficits. This is the first series of Anti NMDAR encephalitis presenting as new onset seizure clusters in children. Unlike the existing literature, these children did not develop any other symptoms. We propose that focal encephalitis could be the reason for this monosymptomatic presentation.

Ovarian teratoma with anti-N-methyl-D-aspartate receptor encephalitis, a type of limbic encephalitis: a review of the literature and a case report in korea

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a type of limbic encephalitis that is resulted by an autoimmune processes; it is a rare autoimmune encephalitis caused by the NMDA receptor antibody secreted by all kinds of tumors. This paraneoplastic syndrome is frequently associated with ovarian teratomas; however, neural cells expressing anti-NMDAR may also be involved in the disease. We report a patient with a case of anti-NMDAR encephalitis associated with an ovarian teratoma, and present a literature review of 16 cases of anti-NMDAR encephalitis in Korean women.

1941 Ovarian Dermoid Cyst as the Cause of Anti-Nmda Receptor Encephalitis: A Case Series

Study Objective Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune condition often presenting in the form of a neuropsychiatric disorder. Ovarian dermoid cyst has been described as a possible instigator of this state with surgical therapy often leading to substantial clinical improvement. We aim to describe our hospital's experience and highlight the importance of ultra sound in the diagnosis and treatment of Anti-NMDAR encephalitis. Design A case series. Setting All women were treated at the gynecology department of a tertiary teaching hospital between January 2013 and December 2018. Patients or Participants We describe 3 cases of anti-NMDAR encephalitis. In the first case a 33 y/o woman presented with headaches, dizziness, and seizures for the first time in her life and later, developed aphasia. In the second case, a 37 y/o woman was referred to the emergency room with acute confusion, delusions and hallucinations and in the third case, a 32 y/o woman appeared with psychosis and seizures. All three were diagnosed with ANTI NMDA antibodies and an ultrasound examination revealed a dermoid cyst ranging from 1 to 4 cm, and in one case the cyst was not detected by CT-scan. Interventions All women underwent ovarian resection and continued immunosuppressive medical therapy with gradual improvement in their condition. Measurements and Main Results We show that even small dermoid cysts may evoke antibody titer rise emphasizing the importance of performing early gynecologic ultrasound for women with new-onset encephalitis. Conclusion ANTI-NMDAR encephalitis caused by dermoid cyst is an important entity which can easily go misdiagnosed. Early cystectomy or ovarian resection and immunosuppressive treatment are key elements in the management of such cases.

Clinical features of the seizures in the neuronal surface antibody-associated autoimmune encephalitis - Report of 18 Cases

To investigate clinical features of seizures in different types of neuronal surface antibody-related autoimmune encephalitis (AE). The clinical data of seizures in 18 cases of neuronal surface antibody-related AEs diagnosed in our hospital were retrospectively analyzed. Among 18 cases, 9 was LGI1 antibody-related encephalitis, 1 anti-GABABR encephalitis, 6 anti-NMDAR encephalitis, and 2 AE with undefinable antibodies. Epileptic seizures in 9 cases of LGI1 antibody-associated AE manifested in 3 forms: faciobranchial dystonia seizure (FBDS) in 4, mesial temporal lobe epilepsy (MTLE) in 6, and GTCS in 7 patients. The brain MRI of 4 patients showed high T2 flair signal on one hippocampus, 2 showed high T1/T2 signal on one side basal ganglia. For EEG, 6 revealed interictal epileptic discharges; ictal EEG were recorded in 5 patients, 2 FBDS, 3 MTLE. Epileptic seizures in patient with anti-GABABR encephalitis manifested in 2 forms: GTCS and MTLE. Epileptic seizures in 6 cases of anti-NMDAR encephalitis manifested in 2 forms: partial seizures in 3, GTCS in 5 cases. For EEG, 2 cases showed interictal epileptic discharges, 3 showed δ activity or rhythm in frontal area. Most neuronal surface antibody-associated AE have seizures. LGI1 antibody-associated AE usually affected limbic system or basal ganglia, seizure type accordingly were MTLE or FBDS. Anti-GABABR encephalitis showed severe and refractory seizures, GTCS or MTLE, which rapidly progress to status epilepticus and AEDs were ineffective. Anti-NMDAR encephalitis showed more generalized than partial seizures. The δ activity or rhythm in frontal area in EEG was helpful for diagnosis.

Severe relapse of anti-NMDA receptor encephalitis 5 years after initial symptom onset.

Anti-NMDAR encephalitis is characterized by abnormal behavior, cognitive dysfunction, seizures, disturbance of consciousness, central hypoventilation, and movement disorders, with a tendency to occur in younger women. Immunotherapy and tumor removal, where applicable, are effective for this disorder. However, previous papers have shown neurological relapse in 12–24% of cases. We present a case of anti-NMDAR encephalitis relapse 5 years after the initial episode. Although the relapse was much more severe than the initial episode, she recovered with aggressive therapy using first- and second-line immunotherapies. Anti- NMDAR encephalitis could relapse with a more severe clinical course after several years. Aggressive immunotherapy including cyclophosphamide must be necessary even for recurrent cases of anti-NMDAR encephalitis.

Anti-N-Methy-D-Aspartate receptor encephalitis in pediatric age

Introduction: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an immune-mediated syndrome characterized by psychiatric symptoms, movement disorders, insomnia, seizures, altered level of consciousness and autonomic dysfunction. The aim of this study was described the cases of anti-NMDAR encephalitis admitted to a Neuropediatrics Department, in order to alert for the importance of early recognition of this clinical entity due to its implications in prognosis. Material and Methods: A retrospective review of the clinical processes of patients with anti-NMDAR encephalitis admitted to the Neuropediatrics Department of a University Hospital in the period between May 2009 and May 2016 was conducted. Results: Diagnosis of anti-NMDAR encephalitis was established in nine patients. Mean age at diagnosis was 9.4 years and five patients were male. Most cases (88.9%) presented with neuropsychiatric symptomatology. Movement disorders were another frequent finding (88.9%) and include chorea, dystonia and dyskinesias. Altered level of consciousness occurred in 77.8% of cases, insomnia and speech disturbance in 66.7%, seizures in 55.6% and autonomic dysfunction in 22.2%. Anti-NMDAR antibodies were identified in the cerebrospinal fluid of all patients. One patient had an underlying neoplasm. All patients underwent immunosuppressive therapy and seven patients fully recovered. In the remaining patients, sequelae included refractory epilepsy and cognitive impairment. Discussion: The diagnosis of anti-NMDAR encephalitis should be considered in children and adolescents presenting with psychiatric symptoms associated and movement disorders. In this sample, although most patients responded very favorably to treatment, severe sequelae were also observed. This highlights the importance of an early diagnosis to initiate treatment as soon as possible.

Bimodal presentation of Anti-NMDAR encephalitis

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an immune-mediated disease characterized by a complex neuropsychiatric syndrome, which is related to tumors, such as teratomas of the ovaries. To our knowledge, this is the first reported case of anti-NMDAR encephalitis concomitantly occurring in a pediatric patient with newly identified urothelial bladder neoplasms.A 5-year-old boy presented with a flu-like illness, disturbance of consciousness, hemiplegia, generalized tonic-clonic seizures last for 40 min, and central hypoventilation. The Computed tomography (CT) of his chest, abdomen and pelvis showed that there was a polypoid lesion on the right anterior wall of the urinary bladder. Then, a transurethral resection of the bladder tumor (TURBT) was performed, and the histopathology revealed a papillary urothelial neoplasm. He recovered gradually and had complete resolution of symptoms after the surgery and immunotherapy.Anti-NMDAR encephalitis can be caused by urothelial bladder neoplasms. Early tumor resection is beneficial to the rapid and complete recovery of patients with anti-NMDAR encephalitis.

Anti-N-Methyl-D-Aspartate Receptor Encephalitis in Psychiatry.

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a recently-discovered autoimmune disorder in which antibodies target NMDAR in the brain. The number of reported cases of anti-NMDAR encephalitis has increased rapidly. Anti-NMDAR encephalitis can be mistakenly diagnosed as psychiatric disorders because many patients present with prominent psychiatric symptoms and visit psychiatric institutions first. Thus, psychiatrists should cultivate a better understanding of anti-NMDAR encephalitis. In this review, we present the mechanisms, epidemiology, symptoms and clinical course, diagnostic tests, treatment and outcomes of patients with anti-NMDAR encephalitis. Furthermore, we discuss the diversity of clinical spectra of anti-NMDAR encephalitis, and demonstrate a differential diagnosis of psychiatric disease from the perspective of psychiatry.

Effect of Clozapine on Anti-N-Methyl-D-Aspartate Receptor Encephalitis With Psychiatric Symptoms: A Series of Three Cases.

The main clinical manifestations of anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis are acute or subacute seizures, cognition impairment, and psychiatric symptoms. Nowadays, the scheme of antipsychotic therapy for this disease has not been established. This study reports three cases of anti-NMDAR encephalitis with psychiatric symptoms. The anti-NMDAR antibodies in cerebrospinal fluid (CSF) and serum were positive. The psychiatric symptoms still existed after intravenous immunoglobulin (IVIG) treatment; thus, clozapine was used for antipsychotic therapy. Case 1 was a 37-year-old man who suffered from bad mood and suicide behaviors for 1 month. Hallucination and delusion still existed after IVIG treatment and hormone therapy, and the symptoms were relieved when given clozapine for 12 months. Case 2 was a 28-year-old man who was admitted to our hospital due to injuring other people and destructive behaviors for 2 days. He showed irritability, bad temper, declined cognition, and severe delusion of persecution after IVIG treatment and hormone therapy, but the psychiatric symptoms disappeared when given clozapine for 3 months. Case 3 was a 23-year-old man who suffered from headache and babbing for 7 days. Symptoms such as irritability, bad temper, babbing, and injuring other people still existed after IVIG treatment and hormone therapy, but they disappeared when given clozapine for 2 months. Therefore, we suggest that during the treatment of anti-NMDAR encephalitis with psychiatric symptoms, if the anti-NMDAR antibodies in CSF and serum were positive, and psychiatric symptoms could not be controlled after IVIG and hormone therapy, clozapine may work.

A Case of Anti-NMDAR Encephalitis Successfully Treated with Cyclophosphomide

A Case of Anti-NMDAR Encephalitis Successfully Treated with Cyclophosphomide

A rare case of anti-NMDAR encephalitis complicating pregnancy (P5.391)

A rare case of anti-NMDAR encephalitis complicating pregnancy (P5.391)

Anti-N-methyl-D-aspartate receptor encephalitis relapse in the brainstem

Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is an autoimmune disorder involving IgG antibody reaction against NMDAR. It typically develops in women with ovarian teratoma, and its characteristic clinical symptoms include acute behavioural...

Two cases of anti-NMDAR encephalitis with atypical clinical features

Two cases of anti-NMDAR encephalitis with atypical clinical features