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anti-gamma-aminobutyric acid-B  receptor and        Examination
          leucine-rich, glioma-inactivated1. [19-21]          Half of the patients with anti-NMDAR encephalitis had
                                                              no abnormalities in magnetic resonance imaging (MRI),
          Here, we reviewed all the cases reported with details and   while others had obvious changes in hippocampus,
          made a profile about anti-NMDAR encephalitis in China.  cerebellum, cortex, frontal lobe, insular lobe, basal
                                                              ganglia, brain stem, and medulla. Multifocal metabolic
          MANIFESTATION, DIAGNOSIS, TREATMENT,                abnormalities were found in the cortex or beneath the
          AND PROGNOSIS OF ANTI-NMDA RECEPTOR                 cortex in positron emission tomography or single-photon
          ENCEPHALITIS                                        emission computed tomography tests. Low perfusion in
                                                              frontal lobe and brain atrophy could be seen in some
          Epidemiological features                            patients, which reversed in follow-ups, 5–7 years later. [18,39]
          The precise data on the incidence of anti-NMDAR
          encephalitis is still unknown. However, it is believed   Majority of the patients had slow waves in
          to be the most common paraneoplastic syndrome.      electroencephalography  (EEG),  and  continuous
          A  retrospective analysis found that anti-NMDAR     δ-θ rhythms predominated the tension episode of
          encephalitis accounts for 1% of all encephalitis cases   anti-NMDAR encephalitis. These slow waves were
          of unknown origin. [22]  A prospective study found that   not accompanied by abnormal movements, and did
          anti-NMDAR encephalitis accounts for 4% of the      not react to anti-epileptic drugs. Video EEG should be
          reasons leading to encephalitis in Britain, and is the   involved in diagnosis and treatment. [18,40]
          second most common immune-related encephalitis
          after acute disseminated encephalomyelitis, and     Cerebrospinal fluid samples of all patients were
          the most common antibody-related encephalitis. [23]    abnormal. Lymphocytes and quantity of protein in
          Moreover, the incidence of anti-NMDAR encephalitis   CSF increased in 80% of the patients at an early stage.
          is increasing. [23]                                 Oligoclonal bands appeared in 60% of patients, and
                                                              intrathecal synthesis of anti-NMDAR antibody could be
          In 2008, Dalmau et al. [16]  analyzed 100 consecutive   detected in most patients. In the long-term follow-up
          patients and found that 91 of them were females with a   patients, the titer of anti-NMDAR antibody in serum was
          mean age of 23 years. About 59% (58/98) of patients were   still high, while it could not be detected in CSF. Because
          paraneoplastic, and teratoma was the most common    of the linear correlation between the loss of NMDAR
          cancer (54/58, 93%). The data were updated 2 years later   in synapse and the titer of anti-NMDAR antibody in
          after the sample size had reached 400. Eighty percent of   CSF, the latter is considered to be one of the definitive
          the patients were females, and the younger the patients   diagnostic criteria. [41,42]
          were the lower was the percentage of paraneoplastic
          syndrome. About 60% of the patients above 18 years   All the reported Chinese patients showed positive
          had cancer. [18]                                    results for antibody detection. Some of the patients
                                                              had a high signal in fluid-attenuated inversion recovery
          Incidence  of  anti-NMDAR  encephalitis  in  China  is   and T2 of MRI, while others showed extreme δ brush wave.
          unknown, too. From all the 32 cases reported with   Similar findings were observed in patients abroad. [29]
          details and 3 patients of ours, we found 80% (28/35) of
          them are females, and the mean age is 19.8 ± 9.7 years.   Diagnostic criteria and methods
          About 40% (14/35) are reported with teratoma [Table 1].   The emerging neuropsychiatric symptoms and
          Maybe, the gender ratio and age pattern of this disease   anti-NMDAR antibodies in serum or CSF are
          in China described by Ren et al. [37]  are more precise   accepted as  diagnostic criteria for anti-NMDAR
          because of  their bigger pool of patients.          encephalitis. [16]  Detection of anti-NMDAR antibody
                                                              includes two necessary methods: immunochemistry
          Symptoms and signs                                  with rat hippocampus (and cerebellum) neurons,
          The most common symptoms include mental             and genetically engineered HEK 293 cells expressing
          disorders, memory loss, decrease in consciousness,   NMDAR on the surface. [14]  Antibody in CSF has higher
          movement  disorders,  seizures,  autonomic  nerve   sensitivity for diagnosis as compared to blood. [41]
          symptoms, and hypoventilation.  [38]  Most of these
          symptoms are associated with pathological changes   Assay kits for detecting anti-NMDAR antibody are
          in brain function.                                  provided by EUROIMMUN Corporation. According to



          Neuroimmunol Neuroinflammation | Volume 1 | Issue 1 | June 2014                                   19
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