【摘要】 目的 研究不同亞型多系統萎縮（multiple system atrophy，MSA）患者的臨床特點。 方法 回顧分析2009年1月—2011年1月收治的105例“很可能的”MSA患者的臨床資料，包括發病年齡、首發癥狀、臨床表現、治療反應性等。 結果 105例MSA患者中，男57例，女48例，發病年齡58歲。以小腦性共濟失調為主要特點的MSA（MSA with predominant cerebellar ataxia，MSA-C）患者76例，以帕金森綜合征為主要特點的多系統萎縮（MSA with predominant parkinsonism，MSA-P）患者29例。39例患者僅以小腦功能障礙為首發癥狀；29例患者僅以帕金森綜合征為首發癥狀，23例患者僅以自主神經功能障礙為首發癥狀，其余14例患者的首發表現至少包括2種癥狀組合。至最后一次隨訪時，54例患者同時存在小腦功能障礙、帕金森綜合征、自主神經功能障礙和錐體束征，51例患者表現為自主神經功能障礙與小腦功能障礙和（或）帕金森綜合征的不同形式的組合。 結論 MSA患者以MSA-C為主。由于在病程早期，MSA與其他帕金森綜合征或小腦性共濟失調疾病的鑒別較為困難，因此，仔細動態觀察患者臨床特點的演變情況，對MSA的診斷至關重要。【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 比較5種新一代抗癲癇藥物對成人全面強直陣攣發作單藥治療的保留率。 方法 選擇2010年7月－2011年6月354例確診為癲癇全面強直陣攣發作患者，分別采用拉莫三嗪、左乙拉西坦、奧卡西平、托吡酯、加巴噴丁5種藥物進行單藥治療，對其5種藥物的6、12個月保留率進行比較。 結果 5種藥物的6、12個月保留率分別為：拉莫三嗪90.8%、79.8%，左乙拉西坦88.0%、66.7%，奧卡西平82.1%、58.2%，托吡酯81.2%、58.0%，加巴噴丁26.5%、20.6%。6個月保留率加巴噴丁與其他4種藥物比較差異有統計學意義（P＜0.001），其他藥物之間差異無統計學意義。12個月保留率拉莫三嗪與其他4種藥物比較差異有統計學意義（P＜0.005），其他藥物之間差異無統計學意義。 結論 拉莫三嗪對成人全面強直陣攣發作單藥治療12個月保留率最高。通過對5種新一代抗癲癇藥物12個月保留率比較研究，可以對臨床單藥治療癲癇藥物選擇提供一定參考。
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.