Bickerstaff脑干脑炎和Miller Fisher综合征的临床对比性研究
发布时间：2018-11-25 10:20
【摘要】：目的：总结分析Bickerstaff脑干脑炎(BBE)和Miller Fisher综合征(MFS)患者的临床特点,以探讨两者的症状学特征、疾病分类学关系及预后,同时研究各免疫调节治疗措施在BBE和MFS患者中的疗效及两组对免疫调节治疗反应的差异。 方法：对符合BBE和MFS诊断标准的患者(分别32例和67例)的病历资料进行回顾性研究,收集患者各种临床表现、辅助检查结果、治疗方案及预后情况等,归纳及比较BBE和MFS患者临床特征的异同点；采用Kaplan-Meier生存曲线分析法比较各免疫调节治疗方案对BBE和MFS病程预后的影响；同时通过Logistic回归分析确定BBE患者不良预后的危险因素。 结果： 1.BBE和MFS患者均存在以上呼吸道感染为主的前驱感染病史,具有眼外肌麻痹和共济失调的共同症状,头痛、眼内肌麻痹、延髓麻痹、面瘫、眼睑下垂、浅表感觉障碍等症状在两组患者中均较常见且发生率相近。且均存在脑脊液蛋白-细胞分离现象及神经电生理、头部影像学检查结果异常等周围神经系统及中枢神经系统受累表现。BBE患者的中枢神经系统受累表现如意识障碍、腱反射亢进、Babinski征阳性、头部MRI脑干部位异常信号及脑电图异常结果等较MFS常见。 2.治疗上,Kaplan-Meier生存曲线分析提示与对照组对比,IVIg联合激素治疗组在BBE患者意识障碍症状的改善和治愈时间上差异有统计学意义(p0.05)；IVIg、激素单独治疗在BBE患者意识障碍、眼外肌麻痹、共济失调症状的改善和治愈时间上差异无统计学意义(p0.05)；IVIg治疗组分别与激素组、联合治疗组对比,在BBE患者意识障碍、眼外肌麻痹及共济失调症状改善和治愈的时间上差异无统计学意义(p0.05)。与对照组相比,IVIg、PE、激素及IVIg联合激素治疗组在MFS患者眼外肌麻痹和共济失调症状的改善及治愈时间上无明显差异(p0.05)；IVIg治疗组分别与PE组、激素组、联合治疗组对比,在MFS患者眼外肌麻痹和共济失调症状开始改善的时间上差异无统计学意义(p0.05)。且两组患者对免疫调节治疗反应无明显差异。 3.两组患者平均随访时间均1年,未见复发病例。虽存在9例BBE患者死亡,但绝大多数的BBE(66%)和MFS(97%)患者症状完全消失,预后良好。单变量分析结果显示急性期出现高热、意识障碍和需机械辅助通气与否,在BBE患者近期预后上的差异有统计学意义(p0.05)。多因素Logistic回归分析结果表明,需机械辅助通气可能为影响BBE患者预后的独立危险因素(p0.05)。 结论： 1.BBE与MFS患者的临床特点相似且存在交叉重叠,中枢及周围神经系统均有受累表现,提示两者可能形成中枢神经系统和周围神经系统受累程度不同的同一连续性自身免疫性疾病谱。 2. IVIg联合激素治疗可加快BBE患者意识障碍的恢复,改善早期出现意识障碍患者的预后。 3.急性期需机械辅助通气可能是影响BBE患者近期预后的独立危险因素。
[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.
【学位授予单位】：中南大学
【学位级别】：硕士
【学位授予年份】：2014
【分类号】：R744.5
本文编号：2355749
[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.
【学位授予单位】：中南大学
【学位级别】：硕士
【学位授予年份】：2014
【分类号】：R744.5
【参考文献】
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1 吴磊;吴卫平;黄德晖;徐全刚;蒲传强;;Miller-Fisher综合征和Bickerstaff脑干脑炎的临床特点及鉴别诊断[J];临床神经病学杂志;2007年02期
，本文编号：2355749
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