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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