【摘要】 目的 研究不同亚型多系统萎缩（multiple system atrophy，MSA）患者的临床特点。 方法 回顾分析2009年1月—2011年1月收治的105例“很可能的”MSA患者的临床资料，包括发病年龄、首发症状、临床表现、治疗反应性等。 结果 105例MSA患者中，男57例，女48例，发病年龄58岁。以小脑性共济失调为主要特点的MSA（MSA with predominant cerebellar ataxia，MSA-C）患者76例，以帕金森综合征为主要特点的多系统萎缩（MSA with predominant parkinsonism，MSA-P）患者29例。39例患者仅以小脑功能障碍为首发症状；29例患者仅以帕金森综合征为首发症状，23例患者仅以自主神经功能障碍为首发症状，其余14例患者的首发表现至少包括2种症状组合。至最后一次随访时，54例患者同时存在小脑功能障碍、帕金森综合征、自主神经功能障碍和锥体束征，51例患者表现为自主神经功能障碍与小脑功能障碍和（或）帕金森综合征的不同形式的组合。 结论 MSA患者以MSA-C为主。由于在病程早期，MSA与其他帕金森综合征或小脑性共济失调疾病的鉴别较为困难，因此，仔细动态观察患者临床特点的演变情况，对MSA的诊断至关重要。【Abstract】 Objective To investigate subtypes and clinical features of multiple system atrophy (MSA). Methods The clinical data of 105 probable MSA patients treated in our hospital from January 2009 to January 2011 were analyzed, including the age at onset, initial symptoms, clinical manifestations and responsivity to levodopa. Results The 105 probable MSA patients consisted of 57 males and 48 females, including 76 patients (72.4%) of MSA with predominant cerebellar ataxia (MSA-C) and 29 patients (27.6%) of MSA with predominant parkinsonism (MSA-P). The mean age at onset was 58 years. The initial symptom of 39 patients was pure cerebellar dysfunction. Twenty-nine patients presented pure parkinsonism as the initial symptom. The initial symptom of 23 patients was pure dysautonomia. By the last clinical visit, 54 patients had cerebellar dysfunction, parkinsonism, autonomic failure and pyramidal signs. Conclusion The study revealed a predominance of MSA-C patients. The differentiation of MSA and other forms of parkinsonism or cerebellar ataxia may be difficult at the early stage. For more accurate diagnosis, it is important to carefully observe the clinical progression.
目的 比较5种新一代抗癫痫药物对成人全面强直阵挛发作单药治疗的保留率。 方法 选择2010年7月－2011年6月354例确诊为癫痫全面强直阵挛发作患者，分别采用拉莫三嗪、左乙拉西坦、奥卡西平、托吡酯、加巴喷丁5种药物进行单药治疗，对其5种药物的6、12个月保留率进行比较。 结果 5种药物的6、12个月保留率分别为：拉莫三嗪90.8%、79.8%，左乙拉西坦88.0%、66.7%，奥卡西平82.1%、58.2%，托吡酯81.2%、58.0%，加巴喷丁26.5%、20.6%。6个月保留率加巴喷丁与其他4种药物比较差异有统计学意义（P＜0.001），其他药物之间差异无统计学意义。12个月保留率拉莫三嗪与其他4种药物比较差异有统计学意义（P＜0.005），其他药物之间差异无统计学意义。 结论 拉莫三嗪对成人全面强直阵挛发作单药治疗12个月保留率最高。通过对5种新一代抗癫痫药物12个月保留率比较研究，可以对临床单药治疗癫痫药物选择提供一定参考。
ObjectiveTo explore the regulation of nuclear factorκB (NFκB) on tumor necrosis factorα (TNFα) expression in the liver and its role in liver injury in rats with acute pancreatitis.MethodsSeventytwo Wistar rats were randomly divided into three groups: acute pancreatitis group (AP), acute pancreatitis treated with pyrrolidine dithiocarbamate (PDTC) group (APP) and sham operation group (SO). The hepatic NFκB activities were determined with electrophoretic mobility shift assays. The expressions of hepatic TNFα mRNA were detected with RTPCR. The levels of serum alanine aminotransferase (ALT) were also measured.ResultsThe NFκB activities were significantly higher in AP and APP groups than those in SO group 3-6 hours after operation. The expressions of TNFα mRNA were ber in AP and APP groups than those in SO group 3-24 hours after operation. The levels of serum ALT were also significantly higher in these two groups than those in SO group 3-24 hours after operation. However, compared with AP group, the activities of NFκB, the expressions of TNFα mRNA and the levels of ALT significantly decreased in APP group.ConclusionThe activation of hepatic NFκB is associated with the liver injury by regulating TNFα mRNA expression in acute pancreatitis.