Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is the most prevalent type of encephalitis. Investigating the pathogenesis of anti-NMDAR encephalitis will enhance our understanding of this disease and play a central part in providing reasonable treatment for the patients. The pathogenesis is elucidated as follows: (1) the findings of the relationship between anti-NMDAR encephalitis and tumors; (2) further research on the relationship between anti-NMDAR encephalitis and tumors; (3) NMDAR epitopes and the autoimmunity of patients; (4) the interaction between antibody and NMDAR; (5) the pathogenesis of anti-NMDAR encephalitis without tumors. This review gives a brief introduction to the methodology and way of finding out the valuable clinical problems and making a clear and explicit explanation of them by exhibiting the process of discovering the disease, disclosing its relationship with tumors, and investigating its pathological and molecular mechanism. Current studies have demonstrated that anti-NMDAR encephalitis is an autoimmune disease of the nervous system that is closely associated with tumors, particularly ovarian teratoma.
Anti-N-Methyl-D-Aspartate Receptor Encephalitis ; etiology ; Antibodies ; immunology ; Autoimmunity ; Humans ; Neoplasms ; complications ; Receptors, N-Methyl-D-Aspartate ; immunology

Autoimmune Diseases ; diagnosis ; etiology ; therapy ; Child, Preschool ; Encephalitis ; diagnosis ; etiology ; therapy ; Female ; Humans ; Receptors, N-Methyl-D-Aspartate ; immunology

Adult ; Female ; Humans ; Limbic Encephalitis ; etiology ; Ovarian Neoplasms ; complications ; Paraneoplastic Syndromes, Nervous System ; etiology ; Receptors, N-Methyl-D-Aspartate ; immunology ; Seizures ; etiology ; Teratoma ; complications

Inflammation, a self-defensive reaction against various pathogenic stimuli, may become harmful self-damaging process. Increasing evidence has linked chronic inflammation to a number of neurodegenerative disorders including Alzheimer's disease (AD), Parkinson's disease (PD), and multiple sclerosis. In the central nervous system, microglia, the resident innate immune cells play major role in the inflammatory process. Although they form the first line of defense for the neural parenchyma, uncontrolled activation of microglia may directly toxic to neurons by releasing various substances such as inflammatory cytokines (IL-1beta, TNF-alpha, IL-6), NO, PGE
alpha-Synuclein/physiology ; Signal Transduction ; Parkinson Disease/*etiology/immunology ; Multiple Sclerosis/etiology ; Models, Biological ; Microglia/immunology/metabolism/*physiology ; Metalloproteases/physiology ; Melanins/physiology ; Matrix Metalloproteinase 3 ; Inflammation Mediators/metabolism ; Humans ; Encephalitis/*etiology/immunology ; Cytokines/secretion ; Animals ; Alzheimer Disease/etiology ; AIDS Dementia Complex/etiology

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