| Background and Objectives:Anti-NMDA receptors (N-methyl-D-aspartate--receptor) encephalitis is an acute or subacute onset, with NMDA receptor antibody correlation limbic encephalitis, can affect the edge of the hippocampus, amygdala and insular cortex. The clinical manifestations is the central nervous system autoimmune encephalitis which characterized by psychological and behavioral abnormalities, seizures and consciousness disorders. It is generally caused by the tumor, infection [1]. Granerod study[2] shows the disease accounted for about 4% encephalitis in the UK, domestic Chunling Xu et al. reported the first confirmed cases in 2010 and case reports have increased since then, the specific incidence has not been reported. In recent years, with the upgrading of the level of anti-NMDA receptor antibodies, it can be found that the anti-NMDA receptor encephalitis is second only to the acute disseminated encephalomyelitis in immune-mediated encephalitis, which has aroused public concernd. The disease is characterized by insidious onset, complex and diverse clinical manifestations, diagnosis and treatment difficulty, rapid progression, a higher disability and recurrence rate and poor prognosis. After the treatment methods improvement and new methods application of anti-NMDA receptors encephalitis, the patient’s condition and the cure rate has been greatly improved than that in the past. At present, according to the patient’s symptoms and condition, it already has anti-viral therapy, hormone therapy, immune modulation therapy, surgery, rehabilitation therapy, antiepileptic treatment and antipsychotic treatments. However, in clinical treatment, each method has advantages and disadvantages, there is a lot of controversy in the process of selection, pending the formation of a recognized, unified treatment plan. Anti-NMDA receptor encephalitis prognosis analysis is important to the prognosis judgement, treatment selection, treatment planning and rational allocation of medical resources. For the science discussion about its prognostic judgment, we can only find a few case reports in current domestic and yet in-depth study. This study was a retrospective analysis of the immediate and long-term efficacy of 44 patients in our hospital with anti-NMDA receptor encephalitis. It systematically evaluated prognostic factors of anti-NMDA receptor encephalitis, and provided important support for clinical treatment.Materials and methods:44 cases with anti-NMDA receptor encephalitis between January 2010 and March 2015 from the Guangdong 999 Brain Hospital and Zhujiang Hospital are analyzed. There is no unified international clear diagnostic criteria of NMDA receptor encephalitis. All patients included in this study are in line with California Encephalitis Project (California Encephalitis Project, CEP) specified in encephalitis diagnostic criteria, and by serum and (or) in cerebrospinal fluid to detect anti-NMDA receptor antibodies [4]. While virus detection to exclude herpes simplex virus encephalitis and other viral encephalitis. And analyzed retrospectively observed demographic information, medical history, clinical manifestations, laboratory tests, imaging studies and treatment and other information.Results:Between the 44 cases,23 patients were female (52%), and 21 males (48%). The main clinical manifestations is psychological and behavioral abnormalities (41/44), seizures (33/44) consciousness disorders (19/44), involuntary movement(17/44) and central dysfunction ventilation (14/44). According to the state of consciousness,25 patients were diveded into unconscious disturbance,11 patients with consciousness disorders in 2 weeks and 8 patients with coma for more than 2 weeks. Laboratory examination:cerebrospinal NMDA receptor antibody positive rate was 100%,64%(28/44) of the NMDA receptor antibodies in serum and cerebrospinal fluid were both positive, only 16 cases of cerebrospinal fluid of NMDA receptor antibodies were positive.30 cases’s cerebrospinal fluid anti-NMDA receptor IgG were positive (titer 1:1),8 cases(1:10),3 cases (1:32) and 3 cases (1:100). EEG of 15 patients showed frontotemporal slow wave activity (34%),8 cases showed temporal frontoparietal region sharp spikes or sharp spike and wave distribution (18%),6 cases of slow-wave activity associated with frontotemporal shark spikes, some spikes (14%), 15 cases were normal (34%).17 cases Cranial MRI+enhanced with no obvious abnormality (39%),5 cases of diffuse brain atrophy (11%),22 cases (soft) meningeal enhancement (50%), including 11 cases of limbic enhancement (25%); abdominal ultrasonography or CT, and 38 cases were normal (86%),6 cases with tumors (14%). Given IVIG, methylprednisolone, control seizures, improve psychiatric symptoms for comprehensive treatment,34 patients achieved the desired effect (77%),6 patients achieved better results (14%),4 patients with poor efficacy (9%). It is found that tumors were all female (13%),5 cases of adnexal tumor and 1 of mediastinal tumor. The 4 cases were surgical excision downlink in relatively stable condition. Between the 25 unconscious disturbance group,11 cases of immunoglobulin combined hormone therapy, only 14 cases of hormone therapy, significant efficiency (92%), efficiency (8%).11 cases suffered consciousness disorders in 2 weeks and 10 cases of hormone therapy combined with immune globulin, significant efficiency (37%), efficiency (27%), inefficient (36%).8 cases were in a coma more than 2 weeks,6 cases immunoglobulin combined hormone therapy, significant efficiency (88%), efficiency (12%). In the unconscious disturbance group, the Rankin score of the<2 weeks in a coma group and> 2 weeks in a coma after treatment [0.80±0.58,2.73± 1.49,0.88±0.64] is significantly lower than that before treatment [3.40±0.65,4.91 ±0.32,4.75±0.46] (p<0.001], the Rankin score difference before and after treatment, respectively [-2.60±0.64,-2.18±1.40,-3.87±0.99], MRS scores were significantly improved.Meanwhile, compared the group unconscious disturbance with the group that over 2 weeks in a coma, the difference was statistically significant (p= 0.005); while compared the group into 2 weeks in a coma with the group that over 2 weeks in a coma, the difference was statistically significant (p= 0.001). But the uric acid, serum cystatin C of the three groups was not statistically significant difference (p= 0.84, p= 0.19). Cerebrospinal fluid anti-NMDA receptor-positive (titer 1:1) 30 patients with a mean length of stay (58.7±67.86) d; cerebrospinal fluid anti-NMDA receptor IgG (1:10) 8 patients with a mean length of stay (80.5±28.07) d; cerebrospinal fluid anti-NMDA receptor IgG (1:32) 3 patients with a mean length of stay (59.00±29.20) d; cerebrospinal fluid anti-NMDA receptor IgG (1:100) 3 patients with a mean length of stay (43.33±17.09) d.The titer of anti-NMDA receptor in cerebrospinal fluid has no relation with the average hospital stay. The Rankin score has no relation with gender, lesion site, uric acid was not statistically significant (p=0.93 p=0.63, p= 0.623) before and afer the anti-NMDA receptor encephalitis treatment. Among the unconscious disturbance, two weeks in a coma, more than two weeks in a coma, age, GSC score, Rankin score before treatment, Rankin score after treatment, the index hospitalization days, cerebrospinal fluid white blood cells, CSF protein, uric acid, serum cystatin C, the difference of GSC score, Rankin score before treatment, Rankin score and hospital days after treatment was statistically significant, which GSC Rating:,unconscious disturbance group comapare with the one that over 2 weeks in a coma, p<0.001; group unconscious disturbance compare with that into 2 weeks in a coma, p<0.001.The group into 2 weeks in a coma compare that over 2 weeks in a coma, p= 0.11. The average hospitalized days of the unconscious disturbance, into two weeks in a coma and more than two weeks in a coma was 45.28±33.63,97.18± 73.02,63.88±17.57 respectively, p=0.01, where the unconscious disturbance compare the group into 2 weeks in a coma, p= 0.007. This shows that the average hospitalized days of the unconscious disturbance group is shortest, while the average hospitalized days of the into two weeks in a coma is longest. Rankin score before treatment:unconscious disturbance compare the over 2 weeks in a coma, p<0.001; unconscious disturbance compare the group over 2 week in a coma, p<0.001. Rankin score after treatment:unconscious disturbance compare the group into 2 weeks in a coma, p<0.001; the group into 2 weeks in a coma compare that over 2 weeks in a coma, p<0.001. The difference between Uric acid and serum cystatin C was not statistically significant (p= 0.84, p= 0.19). And the difference of age, gender, whether located on the edge of the leaf lesions, cerebrospinal fluid and the severity was not statistically significant.Conclusions:1.Anti-NMDA receptor encephalitis occurs in patients 10-29 years of age, with women slightly more common. Its usually clinical manifestations is psychological and behavioral abnormalities, seizures and consciousness disorders, nvoluntary movement and central dysfunction ventilation, which may be associated with tumor, and the associated tumor advised surgery as soon as possible.2. Anti-NMDA receptor encephalitis prognosis relates to the period from onset to the coma, the shorter period, the worse prognosis. And the unconscious disturbance have high cure rate.3. The prognosis and the titers of anti-NMDA have no linear relationship.4. Anti-NMDA receptor encephalitis recommend stratification of treatment, in 2 weeks and more than 2 weeks in a coma caught recommend hormone therapy combined with immune globulin, unconscious disturbance may choose hormone therapy alone.5. Anti-NMDA receptor encephalitis coma group cystatin C blood levels were higher than unconscious disturbance, indicating that serum cystatin C was related to the severity of anti-NMDA receptor encephalitis. |
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