本文選題：副腫瘤邊緣葉性腦炎 + 未成熟畸胎瘤　； 參考：《山東大學(xué)》2014年碩士論文

【摘要】：目的 卵巢畸胎瘤相關(guān)性副腫瘤邊緣葉腦炎(Paraneoplastic limbic encephalitis associated with ovarian teratoma)是一種由卵巢畸胎瘤引起的相對(duì)罕見(jiàn)的、遠(yuǎn)距離的、非轉(zhuǎn)移性的邊緣神經(jīng)系統(tǒng)并發(fā)癥,沒(méi)有直接浸潤(rùn)和神經(jīng)系統(tǒng)的壓迫,是神經(jīng)系統(tǒng)副腫瘤邊緣葉性腦炎的一種特殊類(lèi)型,可以引起嚴(yán)重的神經(jīng)和精神癥狀,甚至導(dǎo)致死亡,在臨床上較為罕見(jiàn),癥狀較嚴(yán)重,且較難做出診斷,但是一旦確診,通過(guò)手術(shù)切除腫瘤、免疫治療、化學(xué)治療等一項(xiàng)或多項(xiàng)聯(lián)合治療后,可以達(dá)到好轉(zhuǎn)甚至完全治愈的良好效果,這也使副腫瘤邊緣葉性腦炎在臨床上越來(lái)越受到重視。因此,有必要分析、總結(jié)卵巢畸胎瘤相關(guān)性副腫瘤邊緣葉性腦炎的發(fā)病特點(diǎn)、臨床表現(xiàn)、診斷要點(diǎn)、鑒別診斷、臨床治療及預(yù)后,提高臨床醫(yī)生對(duì)于這類(lèi)疾病的認(rèn)識(shí)及重視程度,盡早確診,以選擇合適的治療方案,將患者的痛苦減至最低,提高臨床預(yù)后。 方法 2013年7月,山東大學(xué)第二附屬醫(yī)院婦科收治了一例臨床上罕見(jiàn)的卵巢未成熟畸胎瘤相關(guān)性抗NMDAR腦炎患者,結(jié)合本例診斷與治療的成功案例,查找以往報(bào)道過(guò)的典型的卵巢畸胎瘤相關(guān)性副腫瘤邊緣葉性腦炎的相關(guān)文獻(xiàn),總結(jié)和分析該病的發(fā)病特點(diǎn)、臨床表現(xiàn)、相關(guān)輔助檢查、診療方法及預(yù)后,提高認(rèn)識(shí),盡早發(fā)現(xiàn)原發(fā)腫瘤有助于臨床診療。 結(jié)果 本例患者為30歲女性,首發(fā)癥狀為以意識(shí)障礙、癲癇發(fā)作、不自主運(yùn)動(dòng)為主的神經(jīng)精神癥狀,查體欠合作,情緒不穩(wěn),言語(yǔ)尚清,無(wú)法交流,壓眶反射存在,對(duì)光反射存在,出現(xiàn)口腔不自主咀嚼運(yùn)動(dòng),有吞咽動(dòng)作,存在痛覺(jué)感知,心肺查體無(wú)明顯異常,腹部膨隆,可在右下腹部觸及一巨大包塊,活動(dòng)度欠佳,臍周叩診呈鼓音,移動(dòng)性濁音(-),肢體運(yùn)動(dòng)可,肌力Ⅳ級(jí),肌張力為Ⅲ級(jí),頸軟,雙側(cè)Babinski征(-),腦膜刺激征(-)。因患者無(wú)法配合,未進(jìn)行雙合診檢查。 行腹部B超示：右側(cè)腹腔內(nèi)存在一混合回聲團(tuán)塊,考慮為卵巢畸胎瘤惡性變。上腹部平掃+增強(qiáng)CT示：右側(cè)腹腔內(nèi)見(jiàn)一巨大混雜密度影,以惡性畸胎瘤首先考慮(右側(cè)附件來(lái)源可能),脂肪肉瘤不能排除；顱腦MR平掃示：左側(cè)腦室腔隙性腦梗塞；查血清CA19-9、 CA125、 CA242、 CA50均有不同程度的升高,查D-二聚體為697ug/L。后期查血清N-甲基-D-天冬氨酸受體(NMDAR)抗體(+),其他相關(guān)性檢查均未見(jiàn)明顯異常。 入院后完善相關(guān)檢查給予手術(shù)治療,術(shù)后診斷為1、卵巢未成熟畸胎瘤Ⅲc期；2、抗N-甲基-D-天冬氨酸受體腦炎,術(shù)后輔助化療、免疫治療等綜合治療,最終達(dá)到良好的治療效果,患者幾乎痊愈出院。 結(jié)論 卵巢畸胎瘤相關(guān)性副腫瘤邊緣葉性腦炎是一種罕見(jiàn)的多以神經(jīng)精神癥狀為首發(fā)表現(xiàn)的副腫瘤綜合征,主要癥狀包括：人格改變、煩躁、抑郁、癲癇發(fā)作、記憶喪失、癡呆、精神分裂癥表現(xiàn)及睡眠障礙等等。如果未能及時(shí)確診,患者可能進(jìn)一步出現(xiàn)反應(yīng)遲鈍、中樞性通氣不足、自主神經(jīng)不穩(wěn)定(溫度、血壓、心臟節(jié)律不穩(wěn)定)、顏面部運(yùn)動(dòng)障礙、肢體及肌張力障礙等癥狀。這些癥狀通常在確診腫瘤之前就出現(xiàn),而且臨床診斷指標(biāo)缺乏特異性,早期往往很難與器質(zhì)性腦炎做出鑒別,所以及早做出準(zhǔn)確診斷較為困難。在臨床上,盡早確診行手術(shù)治療是該病治療的關(guān)鍵,雖然臨床表現(xiàn)較為嚴(yán)重,甚至需要機(jī)械輔助通氣,但是一旦確診后,盡早手術(shù)切除腫瘤,同時(shí)聯(lián)合免疫治療(和)或化療對(duì)患者臨床癥狀的好轉(zhuǎn)和最終康復(fù)具有重大意義。若臨床上遇到因未知原因的精神、神經(jīng)癥狀而就診的女性患者,應(yīng)該考慮副腫瘤相關(guān)性疾病的可能,仔細(xì)詢(xún)問(wèn)病史,篩查婦科腫瘤以及檢測(cè)血清和(或)腦脊液中的相關(guān)特征性抗體,提高該病的確診率,及早進(jìn)行治療,改善預(yù)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.
【學(xué)位授予單位】：山東大學(xué)
【學(xué)位級(jí)別】：碩士
【學(xué)位授予年份】：2014
【分類(lèi)號(hào)】：R737.31;R739.4

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