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.
Humans ; Anti-N-Methyl-D-Aspartate Receptor Encephalitis/cerebrospinal fluid* ; Retrospective Studies ; Neoplasm Recurrence, Local ; Risk Factors ; Dyskinesias

Adolescent ; Anti-N-Methyl-D-Aspartate Receptor Encephalitis ; blood ; cerebrospinal fluid ; drug therapy ; Autoantibodies ; blood ; cerebrospinal fluid ; Female ; Follow-Up Studies ; Humans ; Immunoglobulins, Intravenous ; therapeutic use ; Immunotherapy ; Methylprednisolone ; therapeutic use

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 ; Aspartate Aminotransferases ; Cerebrospinal Fluid Pressure ; Consciousness ; Demography ; Dyskinesias ; Encephalitis ; Fibrinogen ; Follow-Up Studies ; Humans ; Hypoalbuminemia ; Hypoventilation ; Immunotherapy ; Incidence ; Intracranial Pressure ; Lung ; Prognosis

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a disease that is characterized by acute psychiatric symptoms, seizures, and central hypoventilation. Patients with anti-NMDAR encephalitis exhibit speech alterations, insomnia, seizures, and movement disorders. We describe a previously healthy 6-year-old girl who presented with seizures, disorientation, and fever. Over the five weeks of treatment, the patient exhibited progressive neurologic symptoms, including a change in mental status. Her serum and cerebrospinal fluid contained high titers of antibodies against the NMDAR, and she was diagnosed with anti-NMDAR encephalitis. She was treated with plasmapheresis, steroid pulse therapy, intravenous immunoglobulins, and repeated doses of rituximab. After the patient was diagnosed with a concomitant ovarian teratoma, a unilateral salpingo-oophorectomy was performed. A histopathologic examination revealed that neuronal elements accounted for 60% of the resected ovarian teratoma. The patient's clinical symptoms and antibody titers improved after the surgical treatment and rituximab therapy. These observations suggested that patients with high titers of anti-NMDAR antibodies should be examined for the presence and quantity of neuronal components in concurrent ovarian teratomas.
Anti-N-Methyl-D-Aspartate Receptor Encephalitis ; Antibodies ; Cerebrospinal Fluid ; Child ; Encephalitis* ; Epilepsy ; Female ; Fever ; Humans ; Hypoventilation ; Immunoglobulins, Intravenous ; Movement Disorders ; Neurologic Manifestations ; Neurons* ; Plasmapheresis ; Rituximab ; Seizures ; Sleep Initiation and Maintenance Disorders ; Teratoma*

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a disease that is characterized by acute psychiatric symptoms, seizures, and central hypoventilation. Patients with anti-NMDAR encephalitis exhibit speech alterations, insomnia, seizures, and movement disorders. We describe a previously healthy 6-year-old girl who presented with seizures, disorientation, and fever. Over the five weeks of treatment, the patient exhibited progressive neurologic symptoms, including a change in mental status. Her serum and cerebrospinal fluid contained high titers of antibodies against the NMDAR, and she was diagnosed with anti-NMDAR encephalitis. She was treated with plasmapheresis, steroid pulse therapy, intravenous immunoglobulins, and repeated doses of rituximab. After the patient was diagnosed with a concomitant ovarian teratoma, a unilateral salpingo-oophorectomy was performed. A histopathologic examination revealed that neuronal elements accounted for 60% of the resected ovarian teratoma. The patient's clinical symptoms and antibody titers improved after the surgical treatment and rituximab therapy. These observations suggested that patients with high titers of anti-NMDAR antibodies should be examined for the presence and quantity of neuronal components in concurrent ovarian teratomas.
Anti-N-Methyl-D-Aspartate Receptor Encephalitis ; Antibodies ; Cerebrospinal Fluid ; Child ; Encephalitis* ; Epilepsy ; Female ; Fever ; Humans ; Hypoventilation ; Immunoglobulins, Intravenous ; Movement Disorders ; Neurologic Manifestations ; Neurons* ; Plasmapheresis ; Rituximab ; Seizures ; Sleep Initiation and Maintenance Disorders ; Teratoma*

Anti-N-methyl D-aspartate receptor (anti-NMDAR) encephalitis, recently recognized as a form of paraneoplastic encephalitis, is characterized by a prodromal phase of unspecific illness with fever that resembles a viral disease. The prodromal phase is followed by seizures, disturbed consciousness, psychiatric features, prominent abnormal movements, and autonomic imbalance. Here, we report a case of anti-NMDAR encephalitis with initial symptoms of epilepsia partialis continua in the absence of tumor. Briefly, a 3-year-old girl was admitted to the hospital due to right-sided, complex partial seizures without preceding febrile illness. The seizures evolved into epilepsia partialis continua and were accompanied by epileptiform discharges from the left frontal area. Three weeks after admission, the patient's seizures were reduced with antiepileptic drugs; however, she developed sleep disturbances, cognitive decline, noticeable oro-lingual-facial dyskinesia, and choreoathetoid movements. Anti-NMDAR encephalitis was confirmed by positive detection of NMDAR antibodies in the patient's serum and cerebrospinal fluid, and her condition slowly improved with immunoglobulin, methylprednisolone, and rituximab. At present, the patient is no longer taking multiple antiepileptic or antihypertensive drugs. Moreover, the patient showed gradual improvement of motor and cognitive function. This case serves as an example that a diagnosis of anti-NMDAR encephalitis should be considered when children with uncontrolled seizures develop dyskinesias without evidence of malignant tumor. In these cases, aggressive immunotherapies are needed to improve the outcome of anti-NMDAR encephalitis.
Anti-N-Methyl-D-Aspartate Receptor Encephalitis* ; Antibodies ; Anticonvulsants ; Antihypertensive Agents ; Cerebrospinal Fluid ; Child* ; Child, Preschool ; Cognition ; Consciousness ; D-Aspartic Acid ; Diagnosis ; Dyskinesias ; Encephalitis ; Epilepsia Partialis Continua* ; Female ; Fever ; Humans ; Immunoglobulins ; Immunotherapy ; Korea* ; Methylprednisolone ; Rituximab ; Seizures ; Virus Diseases

Encephalitis associated with antibodies to the N-methyl-D-aspartate receptor (NMDAR) has variable clinical manifestations. Patients are often diagnosed with infectious processes because of prodromal symptoms and autonomic manifestations. Approximately 70% of patients have prodromal symptoms consisting of headache, fever, nausea, vomiting, and diarrhea, along with frequent autonomic manifestations, including tachycardia, and fluctuating blood pressure. A 36-year-old woman presented with uncontrolled fever and skin and soft tissue infections. She had shown psychiatric symptoms and abnormal behavior, and had been diagnosed with bipolar disorder. Antibodies to NMDAR were positive in cerebrospinal fluid (CSF) and serum samples, and pelvic computed tomography detected a large ovarian teratoma. The patient improved dramatically after removal of the teratoma and administration of corticosteroid therapy. When confronted with a young woman with uncontrolled fever and acute psychiatric symptoms, physicians should suspect anti-NMDAR encephalitis.
Adult ; Anti-N-Methyl-D-Aspartate Receptor Encephalitis* ; Antibodies ; Bipolar Disorder ; Blood Pressure ; Cerebrospinal Fluid ; Dermoid Cyst ; Diarrhea ; Encephalitis ; Female ; Fever ; Fever of Unknown Origin* ; Headache ; Humans ; N-Methylaspartate ; Nausea ; Prodromal Symptoms ; Skin ; Soft Tissue Infections ; Tachycardia ; Teratoma ; Vomiting

BACKGROUND AND PURPOSE: Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is the most common type of autoimmune synaptic encephalitis and it often responds to treatment. We analyzed the clinical characteristics of anti-NMDAR encephalitis in Korea. METHODS: Serum and/or cerebrospinal fluid (CSF) of adult patients (aged > or =18 years) with encephalitis of undetermined cause were screened for anti-NMDAR antibodies using a cell-based indirect immunofluorescence assay. The patients came from 41 university hospitals. RESULTS: Of the 721 patients screened, 40 were identified with anti-NMDAR antibodies and clinical details of 32 patients were obtained (median age, 41.5 years; 15 females). Twenty-two patients (68.8%) presented with psychiatric symptoms, 16 (50%) with seizures, 13 (40.6%) with movement disorders, 15 (46.9%) with dysautonomia, 11 (34.4%) with memory disturbance, and 11 (34.4%) with speech disturbance. Magnetic resonance imaging, electroencephalography, and CSF examinations yielded nonspecific findings. Tumor information was only available for 22 patients: 5 patients had tumors, and 2 of these patients had ovarian teratomas. Twenty-two patients received immunotherapy and/or surgery, and therapeutic responses were analyzed in 21 patients, of which 14 (66.7%) achieved favorable functional outcomes (score on the modified Rankin Scale of 0-2). CONCLUSIONS: This study investigated the clinical characteristics of adult anti-NMDAR encephalitis in Korea. Currently, elderly patients who do not have tumors are commonly diagnosed with this condition. Understanding the detailed clinical characteristics of this disease will improve the early detection of anti-NMDAR encephalitis in patients both young and old.
Adult ; Aged ; Anti-N-Methyl-D-Aspartate Receptor Encephalitis* ; Antibodies ; Cerebrospinal Fluid ; Electroencephalography ; Encephalitis ; Fluorescent Antibody Technique, Indirect ; Hospitals, University ; Humans ; Immunotherapy ; Korea ; Magnetic Resonance Imaging ; Memory ; Movement Disorders ; Primary Dysautonomias ; Seizures ; Teratoma

OBJECTIVETo study the clinical and laboratory features and diagnosis of the patient with anti-N-methyl-D-aspartate receptor(NMDAR)encephalitis in children.

METHODThe data of clinical feature, laboratory findings, and radiological manifestation were reviewed and analyzed.

RESULTOf the 7 patients, 4 were female and 3 were male. The age of onset was from 6.6 to 15.5 years (average 9.5 years). The onset of 4 cases started with convulsion. Six cases had seizures which was difficult to control by antiepileptic drugs. All patients had psychiatric symptoms and speech disorder. Six cases had different levels of decreased consciousness and dyskinesias. 6 cases had autonomic nerve instability, and 7 cases developed sleep disorders. The results of MRI examination were normal in all patients. The EEG of most patients showed focal or diffuse slow waves. Six cases had oligoclonal bands. All cases were confirmed to have the disease by detection of anti-NMDA receptor antibodies. No tumor was detected in any of the patients. All patients received immunotherapy.

CONCLUSIONAnti-NMDAR encephalitis is a severe but treatable disorder that frequently affects children and adolescents. Pediatric patients had clinical manifestations similar to those of adult patients. But children have a lower incidence of tumors and hypoventilation also occurs less frequently in children. Most of children had a good prognosis.

Adolescent ; Anti-N-Methyl-D-Aspartate Receptor Encephalitis ; complications ; diagnosis ; therapy ; Autoantibodies ; blood ; cerebrospinal fluid ; Autonomic Nervous System ; physiopathology ; Brain ; diagnostic imaging ; pathology ; Child ; Electroencephalography ; Female ; Humans ; Immunotherapy ; methods ; Magnetic Resonance Imaging ; Male ; Movement Disorders ; etiology ; Radiography ; Receptors, N-Methyl-D-Aspartate ; immunology ; Retrospective Studies ; Seizures ; etiology