【摘要】：目的：總結分析Bickerstaff腦干腦炎(BBE)和Miller Fisher綜合征(MFS)患者的臨床特點,以探討兩者的癥狀學特征、疾病分類學關系及預后,同時研究各免疫調節(jié)治療措施在BBE和MFS患者中的療效及兩組對免疫調節(jié)治療反應的差異。 方法：對符合BBE和MFS診斷標準的患者(分別32例和67例)的病歷資料進行回顧性研究,收集患者各種臨床表現(xiàn)、輔助檢查結果、治療方案及預后情況等,歸納及比較BBE和MFS患者臨床特征的異同點；采用Kaplan-Meier生存曲線分析法比較各免疫調節(jié)治療方案對BBE和MFS病程預后的影響；同時通過Logistic回歸分析確定BBE患者不良預后的危險因素。 結果： 1.BBE和MFS患者均存在以上呼吸道感染為主的前驅感染病史,具有眼外肌麻痹和共濟失調的共同癥狀,頭痛、眼內肌麻痹、延髓麻痹、面癱、眼瞼下垂、淺表感覺障礙等癥狀在兩組患者中均較常見且發(fā)生率相近。且均存在腦脊液蛋白-細胞分離現(xiàn)象及神經電生理、頭部影像學檢查結果異常等周圍神經系統(tǒng)及中樞神經系統(tǒng)受累表現(xiàn)。BBE患者的中樞神經系統(tǒng)受累表現(xiàn)如意識障礙、腱反射亢進、Babinski征陽性、頭部MRI腦干部位異常信號及腦電圖異常結果等較MFS常見。 2.治療上,Kaplan-Meier生存曲線分析提示與對照組對比,IVIg聯(lián)合激素治療組在BBE患者意識障礙癥狀的改善和治愈時間上差異有統(tǒng)計學意義(p0.05)；IVIg、激素單獨治療在BBE患者意識障礙、眼外肌麻痹、共濟失調癥狀的改善和治愈時間上差異無統(tǒng)計學意義(p0.05)；IVIg治療組分別與激素組、聯(lián)合治療組對比,在BBE患者意識障礙、眼外肌麻痹及共濟失調癥狀改善和治愈的時間上差異無統(tǒng)計學意義(p0.05)。與對照組相比,IVIg、PE、激素及IVIg聯(lián)合激素治療組在MFS患者眼外肌麻痹和共濟失調癥狀的改善及治愈時間上無明顯差異(p0.05)；IVIg治療組分別與PE組、激素組、聯(lián)合治療組對比,在MFS患者眼外肌麻痹和共濟失調癥狀開始改善的時間上差異無統(tǒng)計學意義(p0.05)。且兩組患者對免疫調節(jié)治療反應無明顯差異。 3.兩組患者平均隨訪時間均1年,未見復發(fā)病例。雖存在9例BBE患者死亡,但絕大多數(shù)的BBE(66%)和MFS(97%)患者癥狀完全消失,預后良好。單變量分析結果顯示急性期出現(xiàn)高熱、意識障礙和需機械輔助通氣與否,在BBE患者近期預后上的差異有統(tǒng)計學意義(p0.05)。多因素Logistic回歸分析結果表明,需機械輔助通氣可能為影響B(tài)BE患者預后的獨立危險因素(p0.05)。 結論： 1.BBE與MFS患者的臨床特點相似且存在交叉重疊,中樞及周圍神經系統(tǒng)均有受累表現(xiàn),提示兩者可能形成中樞神經系統(tǒng)和周圍神經系統(tǒng)受累程度不同的同一連續(xù)性自身免疫性疾病譜。 2. IVIg聯(lián)合激素治療可加快BBE患者意識障礙的恢復,改善早期出現(xiàn)意識障礙患者的預后。 3.急性期需機械輔助通氣可能是影響B(tài)BE患者近期預后的獨立危險因素。
[Abstract]:Objective: to summarize and analyze the clinical features of patients with Bickerstaff encephalococcal encephalitis (BBE) and Miller Fisher syndrome (MFS) in order to explore their symptom characteristics, disease taxonomic relationship and prognosis. At the same time, the effect of immunomodulatory therapy in patients with BBE and MFS and the difference of response to immunomodulatory therapy between the two groups were studied. Methods: the medical records of 32 cases and 67 cases of BBE and MFS were retrospectively studied, and the clinical manifestations, auxiliary examination results, treatment plan and prognosis of the patients were collected. The clinical features of BBE and MFS were summarized and compared. Kaplan-Meier survival curve analysis was used to compare the effects of different immunomodulatory therapy regimens on the prognosis of BBE and MFS, and Logistic regression analysis was used to determine the risk factors of poor prognosis in BBE patients. Results: both 1.BBE and MFS patients had a history of prodromal infection with upper respiratory tract infection, common symptoms of extraocular muscle paralysis and ataxia, headache, intraocular paralysis, bulbar palsy, facial paralysis, blepharoptosis. Symptoms such as superficial sensory disorders were more common in both groups and the incidence was similar. All of them had the phenomenon of cerebrospinal fluid protein-cell separation, neuroelectrophysiology, abnormal head imaging and other manifestations of involvement of peripheral nervous system and central nervous system. The involvement of central nervous system in patients with BBE, such as disturbance of consciousness, was found in all patients. Hyperreflexia, positive Babinski sign, abnormal brain stem signal and abnormal EEG of head MRI were more common than MFS. 2. In treatment, Kaplan-Meier survival curve analysis showed that compared with the control group, IVIg combined with hormone treatment group in the improvement of BBE patients with symptoms of consciousness disorders and cure time difference was statistically significant (p0.05); There was no significant difference in the improvement of symptoms of consciousness, extraocular muscle paralysis and ataxia in patients with BBE treated with IVIg, alone (p0.05). There was no significant difference in the improvement and cure time of the symptoms of BBE patients with consciousness disturbance, extraocular muscle paralysis and ataxia in the IVIg treatment group and the hormone group and the combined treatment group (p0.05). Compared with the control group, there was no significant difference in the improvement and cure time of extraocular muscle paralysis and ataxia in the treatment group of IVIg,PE, and IVIg combined with hormone (p0.05). There was no significant difference between IVIg group and PE group, hormone group and combined treatment group in the time when the symptoms of extraocular muscle paralysis and ataxia began to improve in MFS patients (p0.05). There was no significant difference in response to immunomodulatory therapy between the two groups. 3. The average follow-up time of the two groups was 1 year, and no recurrent cases were found. Although 9 BBE patients died, the majority of patients with BBE (66%) and MFS (97%) had completely disappeared symptoms and had a good prognosis. Univariate analysis showed that there were significant differences in the short-term prognosis of BBE patients with acute hyperthermia, disturbance of consciousness and need of mechanical assisted ventilation (p0.05). Multivariate Logistic regression analysis showed that the need for mechanical assisted ventilation may be an independent risk factor for the prognosis of patients with BBE (p0.05). Conclusion: the clinical features of patients with 1.BBE and MFS are similar and overlap. The central nervous system and peripheral nervous system are involved. These results suggest that both of them may form the same continuous autoimmune disease spectrum with different degrees of involvement in the central nervous system and peripheral nervous system. 2. IVIg combined with hormone therapy can accelerate the recovery of consciousness disorders in BBE patients and improve the prognosis of patients with early consciousness disorders. 3. Mechanical-assisted ventilation may be an independent risk factor for the short-term prognosis of BBE patients.
【學位授予單位】：中南大學
【學位級別】：碩士
【學位授予年份】：2014
【分類號】：R744.5

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