| 摘要: |
| 目的:探讨卵巢畸胎瘤致抗N-甲基-D-天冬氨酸(NMDA)受体脑炎患者临床特点及诊治。方法:回顾性分析2013年1月至2017年7月就诊于我院因卵巢畸胎瘤致抗NMDA受体脑炎患者12例临床资料。结果:12例患者平均发病年龄27.4±8.4岁。9例以精神症状起病,3例以癫痫起病。在疾病发展过程中,所有患者均有精神行为异常,表现为言行紊乱、情感障碍、幻听、幻视、被害妄想等。10例(83.3%)出现不同程度意识障碍和运动障碍,9例(75.0%)在病程中出现癫痫样发作,6例(50.0%)出现自主神经功能紊乱,5例(41.7%)伴随中枢性低通气,2例(16.7%)出现言语障碍,2例(16.7%)出现近记忆减退。所有患者就诊前均无明显腹胀、腹痛等症状,仅1例(8.3%)出现月经周期延长。5例患者(41.7%)出现头部影像学改变,5例(41.7%)脑脊液有核细胞数增高,8例(66.7%)部分血清肿瘤标志物异常。12例患者脑脊液中抗NMDA受体抗体的检测均为阳性,其中6例患者同时进行了血中抗NMDA受体抗体的检测,5例(83.3%)阳性,1例(16.7%)阴性。经过免疫治疗及手术治疗,11例(91.7%)预后良好,1例拒绝手术死亡。术后病理检查示8例(72.7%)为成熟性畸胎瘤,2例(18.2%)为未成熟畸胎瘤,1例(9.1%)为交界性畸胎瘤。结论:临床上对于以精神行为异常或癫痫起病的年轻女性脑炎患者,应考虑到抗NMDA受体脑炎可能。同时应积极寻找是否存在畸胎瘤,尽早手术治疗有利于预后. |
| 关键词: 卵巢畸胎瘤 自身免疫性脑炎 抗N-甲基-D-天冬氨酸受体 |
| DOI： |
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| 基金项目: |
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| Analysis of Clinical Characteristics of 12 Autoimmune Encephalitis Patients Associated with Ovarian Teratoma |
| DU Xue;YANG Fan;BIAN Ce |
| (West China Second Hospital,Sichuan University;Key Laboratory of Birth Defects and Related Diseases of Women and Children,Ministry of Education) |
| Abstract: |
| Objective: To investigate the clinical characteristics,diagnosis and treatment of patients with anti-N-methyl-D-aspartate( NMDA)-receptor encephalitis associated with ovarian teratoma.Methods: The clinical data of patients with anti-NMDAR encephalitis associated with ovarian teratoma from Jau.2013 to Jul.2017 were analyzed retrospectively.Results: We ultimately included 12 patients and their average age was 27. 4±8. 4 years.9 patients presented with psychiatric symptoms first,and 3 patients developed with seizure.In the process of disease,all the patients displayed mental and behavioral abnormalities,including bizarre behavior,affective disorder,visual or auditory hallucinations,and persecutory delusion. Other symptoms including decreased level of consciousness( 10 cases,83. 3%),movement disorders( 10 cases,83. 3%),epilepsy( 9 cases,75. 0%),autonomic dysfunction( 6 cases,50. 0%),central hypoventilation( 5 cases,41. 7%),speech dysfunction( 2 cases,16. 7%)and recent memory deficits( 2 cases,16. 7%) were also observed.Only 1 patients complained prolonged menstrual cycle with no symptoms of abdominal distension or pain.The abnormal auxiliary examinations showed CSF lymphocytic pleocytosis in 5 patients( 41. 7%),abnormal brain CT/MRI change in 5 patients and elevated serum tumor markers in 8 patients( 66. 7%).As for the antibodies against NMDAR,all the patients were tested positive in CSF.While 6 patients also carried on the test for antibodies in serum,of which 5( 83. 3%) tested positive and 1( 16. 7%) negative.1 patient refused surgery and died finally.The others who received immunotherapy and operative treatment got a favorable prognosis.Postoperative pathological examination showed 8 cases( 72. 7%) of mature ovarian teratoma,2 cases( 18. 2%) of immature teratoma,and 1 case( 9. 1%) of borderline ovarian teratoma.Conclusions: For those young female patients who presented with psychiatric symptoms or seizure,anti-NMDAR encephalitis should be taken into consideration.And searching for any evidence of ovarian teratoma is necessary.Timely removal of tumor makes better prognosis. |
| Key words: Teratoma Autoimmuneencephalitis Anti-N-methyl-D-aspartatereceptor |
