副肿瘤边缘叶性脑炎1例临床分析并文献回顾

发布时间：2018-06-24 18:01

本文选题：副肿瘤边缘叶性脑炎 + 未成熟畸胎瘤　；参考：《山东大学》2014年硕士论文

【摘要】：目的卵巢畸胎瘤相关性副肿瘤边缘叶脑炎(Paraneoplastic limbic encephalitis associated with ovarian teratoma)是一种由卵巢畸胎瘤引起的相对罕见的、远距离的、非转移性的边缘神经系统并发症,没有直接浸润和神经系统的压迫,是神经系统副肿瘤边缘叶性脑炎的一种特殊类型,可以引起严重的神经和精神症状,甚至导致死亡,在临床上较为罕见,症状较严重,且较难做出诊断,但是一旦确诊,通过手术切除肿瘤、免疫治疗、化学治疗等一项或多项联合治疗后,可以达到好转甚至完全治愈的良好效果,这也使副肿瘤边缘叶性脑炎在临床上越来越受到重视。因此,有必要分析、总结卵巢畸胎瘤相关性副肿瘤边缘叶性脑炎的发病特点、临床表现、诊断要点、鉴别诊断、临床治疗及预后,提高临床医生对于这类疾病的认识及重视程度,尽早确诊,以选择合适的治疗方案,将患者的痛苦减至最低,提高临床预后。方法 2013年7月,山东大学第二附属医院妇科收治了一例临床上罕见的卵巢未成熟畸胎瘤相关性抗NMDAR脑炎患者,结合本例诊断与治疗的成功案例,查找以往报道过的典型的卵巢畸胎瘤相关性副肿瘤边缘叶性脑炎的相关文献,总结和分析该病的发病特点、临床表现、相关辅助检查、诊疗方法及预后,提高认识,尽早发现原发肿瘤有助于临床诊疗。结果本例患者为30岁女性,首发症状为以意识障碍、癫痫发作、不自主运动为主的神经精神症状,查体欠合作,情绪不稳,言语尚清,无法交流,压眶反射存在,对光反射存在,出现口腔不自主咀嚼运动,有吞咽动作,存在痛觉感知,心肺查体无明显异常,腹部膨隆,可在右下腹部触及一巨大包块,活动度欠佳,脐周叩诊呈鼓音,移动性浊音(-),肢体运动可,肌力Ⅳ级,肌张力为Ⅲ级,颈软,双侧Babinski征(-),脑膜刺激征(-)。因患者无法配合,未进行双合诊检查。行腹部B超示：右侧腹腔内存在一混合回声团块,考虑为卵巢畸胎瘤恶性变。上腹部平扫+增强CT示：右侧腹腔内见一巨大混杂密度影,以恶性畸胎瘤首先考虑(右侧附件来源可能),脂肪肉瘤不能排除；颅脑MR平扫示：左侧脑室腔隙性脑梗塞；查血清CA19-9、 CA125、 CA242、 CA50均有不同程度的升高,查D-二聚体为697ug/L。后期查血清N-甲基-D-天冬氨酸受体(NMDAR)抗体(+),其他相关性检查均未见明显异常。入院后完善相关检查给予手术治疗,术后诊断为1、卵巢未成熟畸胎瘤Ⅲc期；2、抗N-甲基-D-天冬氨酸受体脑炎,术后辅助化疗、免疫治疗等综合治疗,最终达到良好的治疗效果,患者几乎痊愈出院。结论卵巢畸胎瘤相关性副肿瘤边缘叶性脑炎是一种罕见的多以神经精神症状为首发表现的副肿瘤综合征,主要症状包括：人格改变、烦躁、抑郁、癫痫发作、记忆丧失、痴呆、精神分裂症表现及睡眠障碍等等。如果未能及时确诊,患者可能进一步出现反应迟钝、中枢性通气不足、自主神经不稳定(温度、血压、心脏节律不稳定)、颜面部运动障碍、肢体及肌张力障碍等症状。这些症状通常在确诊肿瘤之前就出现,而且临床诊断指标缺乏特异性,早期往往很难与器质性脑炎做出鉴别,所以及早做出准确诊断较为困难。在临床上,尽早确诊行手术治疗是该病治疗的关键,虽然临床表现较为严重,甚至需要机械辅助通气,但是一旦确诊后,尽早手术切除肿瘤,同时联合免疫治疗(和)或化疗对患者临床症状的好转和最终康复具有重大意义。若临床上遇到因未知原因的精神、神经症状而就诊的女性患者,应该考虑副肿瘤相关性疾病的可能,仔细询问病史,筛查妇科肿瘤以及检测血清和(或)脑脊液中的相关特征性抗体,提高该病的确诊率,及早进行治疗,改善预后。
[Abstract]:objective
Ovarian teratoma associated paraneoplastic marginal lobar encephalitis (Paraneoplastic limbic encephalitis associated with ovarian teratoma) is a relatively rare, distant, non metastatic marginal neurologic complication caused by ovarian teratoma, without direct infiltration and compression of the nervous system, which is the edge of the nervous system paraneoplastic. A special type of leaf encephalitis that can cause serious neurological and mental symptoms and even lead to death. It is rare, severe, and difficult to make a diagnosis in clinical, but once a diagnosis is made, one or more combined treatment, such as surgical resection of tumors, immunotherapy, and chemical therapy, can be improved or even completely cured. Therefore, it is necessary to analyze the characteristics, clinical manifestations, diagnostic points, differential diagnosis, clinical treatment and preclinical treatment of the ovarian teratoma related paraneoplastic marginal leaf encephalitis, and to improve the understanding and attention of clinicians on this kind of disease. Diagnosis should be made as soon as possible, so as to choose the right treatment plan to minimize the pain of patients and improve the prognosis.
Method
In July 2013, a case of rare ovarian immature teratoma associated with anti NMDAR encephalitis was treated in the Second Affiliated Hospital of Shandong University. In combination with the successful cases of this case, the related literature on the typical ovarian teratoma associated marginal leaf encephalitis associated with ovarian teratoma was found, and the disease was summarized and analyzed. The characteristics of the disease, clinical manifestations, related auxiliary examinations, diagnosis and treatment methods and prognosis, raising awareness, early detection of primary tumors are helpful for clinical diagnosis and treatment.
Result
This case was a 30 year old woman. The first symptom was the neurological and mental symptoms of consciousness disorder, epileptic seizure and involuntary movement. The examination was not cooperative and emotional, the speech was still clear, it was unable to communicate, the orbital reflex existed, the light reflex existed, the oral involuntary masticatory movement, the swallowing movement, the pain perception, and the cardiopulmonary examination were not obvious. Abnormal, abdominal distention, can reach a huge mass in the lower right abdomen, poor activity, percussion in the umbilical cord, movement of voiced sound (-), limb movement, muscle strength IV, muscular tension of grade III, soft neck, bilateral Babinski sign (-), meningeal irritation (-).
Abdominal ultrasonography: a mixed echo mass in the right abdominal cavity was considered as a malignant transformation of the ovarian teratoma. The upper abdominal plain scan + enhanced CT showed a huge hybrid density in the right abdominal cavity, and the malignant teratoma was first considered (the source of the right appendage may) and the liposarcoma could not be excluded; the brain MR scan: the left ventricle lacunar brain The serum CA19-9, CA125, CA242 and CA50 were elevated in different degrees. The D- two polymer was found to be the serum N- methyl -D- aspartate receptor (NMDAR) antibody (+) in the later stage of 697ug/L., and the other correlation tests were not found to be abnormal.
After admission, the surgical treatment was perfected, the postoperative diagnosis was 1, the ovarian immature teratoma stage III C, 2, anti N- methyl -D- aspartic acid receptor encephalitis, postoperative adjuvant chemotherapy, immunotherapy and other comprehensive treatment, and finally achieved good therapeutic effect, and the patients almost healed out of the hospital.
conclusion
Ovarian teratoma associated paraneoplastic marginal lobar encephalitis is a rare paraneoplastic syndrome with multiple neuropsychiatric symptoms as the first manifestation. The main symptoms include personality change, irritability, depression, seizures, memory loss, dementia, schizophrenia performance and sleep disorders. Patients may be admitted if they are not diagnosed promptly. There are symptoms of slow reaction, insufficiency of central ventilation, autonomic nervous instability (temperature, blood pressure, cardiac rhythm instability), facial dyskinesia, and limb and dystonia. These symptoms usually occur before the diagnosis of tumor, and the clinical diagnosis is lack of specificity. Early diagnosis is often difficult to identify with organic encephalitis. It is difficult to make accurate diagnosis as early as possible. In clinical, early diagnosis of surgical treatment is the key to the treatment of the disease, although the clinical manifestations are more serious and even need mechanical ventilation, but once the diagnosis is made, the tumor is excised as soon as possible, combined with immunotherapy (and) or chemotherapy for the improvement of the patient's clinical symptoms and the final health. It is of great significance. If the female patients who are clinically diagnosed with an unknown cause and neurologic symptoms should consider the possibility of paraneoplastic related diseases, examine the history of the disease carefully, screen the gynecologic tumors and detect the related characteristic antibodies in the serum and / or cerebrospinal fluid, improve the diagnosis rate of the disease, make early treatment, improve Prognosis.
【学位授予单位】：山东大学
【学位级别】：硕士
【学位授予年份】：2014
【分类号】：R737.31;R739.4

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