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@#N-methyl-D-aspartate receptor (NMDAR) antibody encephalitis is recognized as an autoimmune encephalitis, which is due to autoantibodies against synaptic NMDAR. This disorder affects individuals aged 23 months to 76 years and has a wide range of presentations. In Malaysia, more than 20 cases have been reported. Timely diagnosis and definitive immunotherapy are vital in optimizing functional recovery and prognosis. However, early diagnosis of the condition is often missed due to low awareness among clinicians in Malaysia. This article gathered the medical literature from Malaysia and highlights the aetio-pathophysiology, clinical presentation and management of the disease.
RESUMO
Objective@#To explore the clinical value of serum N-methyl-aspartate receptor (NMDAR) antibody level, brainstem auditory evoked potential (BAEP) and magnetic resonance imaging (MRI) in the differential diagnosis of viral encephalitis and anti-NMDAR encephalitis.@*Methods@#The clinical data of 68 children patients with encephalitis were retrospectively analyzed. The patients diagnosed with viral encephalitis were included in V group (n=52), and the patients diagnosed with anti-NMDAR encephalitis were included in N group (n=16). The clinical characteristics, serum NMDAR antibody level, and BAEP and MRI findings were compared between the two groups.@*Results@#The age, disease duration, abnormal behavior rate, sleep disorder rate and epileptic seizure rate in V group were significantly lower than those in N group [(6.62±1.20)Y/O vs.(8.46±1.85)Y/O, (3.53±0.71)d vs.(4.49±0.82)d, 30.77%(16/52)vs. 75.00%(12/16), 21.15%(11/52)vs. 62.50%(10/16), 26.92%(14/52)vs. 56.25%(9/16), t=4.681, t=4.560, χ2=9.882, χ2=7.958, χ2=4.701], while the abnormal rate of video EEG was significantly higher than that in N group [51.92(27/52)vs. 81.25%(13/16), χ2=4.345] (all P<0.05). There were no significant differences in gender, rates of prodromic infection symptoms, cognitive impairment, fever, headache, convulsion and incidence rate of meningeal irritation sign (P>0.05). The serum NMDAR antibody level in V group was significantly lower than that in N group [(3.40±0.69) ng/ml vs.(13.95±2.78) ng/ml t=25.319)] (P<0.05). There were no significant differences in the BAEP apparent involvement range and central auditory neurological damage between the two groups (P>0.05), but the peripheral auditory nerve damage and total BAEP abnormality rate in V group were significantly lower than those in N group [3.85%(4/104)vs. 21.88%(7/32), 6.73%(7/104)vs. 28.12%(9/32), 30.77%(16/52)vs. 62.50%(10/16), χ2=10.699, χ2=10.790, χ2=5.216] (all P<0.05). There were no significant differences in MRI signal intensity, lesion involvement range and total abnormal rate between the two groups (all P>0.05).@*Conclusions@#There were significant differences in serum NMDAR antibody level and BAEP test results among children patients with viral encephalitis or anti-NMDAR encephalitis, and they are helpful for early differential diagnosis.