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.
Humans ; Cytokines ; Anti-N-Methyl-D-Aspartate Receptor Encephalitis/therapy* ; Biomarkers

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.
Male ; Female ; Humans ; Child ; Infant, Newborn ; Anti-N-Methyl-D-Aspartate Receptor Encephalitis ; Retrospective Studies ; Hashimoto Disease/therapy* ; Encephalitis, Arbovirus

Adult ; Anesthesia ; methods ; Anti-N-Methyl-D-Aspartate Receptor Encephalitis ; etiology ; therapy ; Female ; Humans ; Ovarian Neoplasms ; therapy ; Teratoma ; therapy

Adult ; Anesthesia ; methods ; Anti-N-Methyl-D-Aspartate Receptor Encephalitis ; drug therapy ; Cystectomy ; Female ; Humans ; Immunotherapy ; methods ; Young Adult

Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a new category of severe, potentially treatable autoimmune encephalitis and can appear in patients of all ages, but more frequently in children. It is a highly characteristic syndrome evolving in five stages: the prodromal phase (viral infection-like symptoms), psychotic phase, unresponsive phase, hyperkinetic phase, and gradual recovery phase. The treatment for this disorder includes first-line immunotherapy (steroids, intravenous immunoglobulin, plasmapheresis), second-line immunotherapy (rituximab, cyclophosphamide), and tumor removal. Hereby the progresses, selections and shortcomings of the treatment protocols for this disease are introduced.
Anti-N-Methyl-D-Aspartate Receptor Encephalitis ; therapy ; Antibodies, Monoclonal, Murine-Derived ; therapeutic use ; Child ; Humans ; Immunoglobulins, Intravenous ; therapeutic use ; Immunosuppressive Agents ; therapeutic use ; Immunotherapy ; Plasmapheresis ; Prognosis ; Rituximab

OBJECTIVETo evaluate the efficacy and safety of cyclophosphamide as a second-line drug in the treatment of children with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis.

METHODSSix children with anti-NMDAR encephalitis, who showed poor response to steroids and intravenous immunoglobulin, were given cyclophosphamide as a second-line immunotherapy. Follow-up was performed to evaluate the efficacy and safety of cyclophosphamide.

RESULTSAfter first-line immunotherapy for 1-4 weeks, the six patients had reduced psychiatric symptoms, seizures, and involuntary movements; three patients had an improved level of consciousness and were able to make simple conversations. However, all the patients still showed slow response, as well as cortical dysfunction symptoms such as aphasia, alexia, agraphia, acalculia, apraxia, and movement disorders. The six patients continued to receive cyclophosphamide as a sequential therapy. They were able to answer simple questions 7 days after treatment. Three school-aged patients were able to make simple calculation, had greatly improved reading and writing ability, and almost recovered self-care ability 2-3 weeks later. The cognitive function of the six patients was almost restored to the level before the onset of disease, and their living ability returned to normal 2-3 months later. During the treatment period, there were no adverse reactions or abnormal results of routine blood test and liver and kidney function tests.

CONCLUSIONSChildren with anti-NMDAR encephalitis should be given appropriate immunotherapy as soon as possible. Cyclophosphamide as a sequential therapy has good efficacy and safety.

Adolescent ; Anti-N-Methyl-D-Aspartate Receptor Encephalitis ; drug therapy ; physiopathology ; psychology ; Child ; Child, Preschool ; Cognition ; Cyclophosphamide ; therapeutic use ; Female ; Humans ; Immunotherapy ; Male

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

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