中文摘要：

目的研究脊髓小脑共济失调3型（SCA3）患者事件相关电位P300的特点,并探讨其对患者认知功能障碍的评估价值。方法选取2013年7月至2015年7月在神经内科住院部或门诊就诊的经基因测序确诊的SCA3型患者11例（SCA3组）作为研究对象,并选择13例健康体检者为对照组。对所有受试者进行听觉P300检查,并采用简易智能状态量表（MMSE）、蒙特利尔认知评估量表（Mo CA）评估认知功能,采用国际协作共济失调评估量表（ICARS）进行共济失调严重程度评分。结果 （1）SCA3组MMSE和Mo CA评分均明显低于正常对照组（P均〈0.05）;MMSE量表评分结果显示,SCA3组存在认知功能障碍5例（45.45%）,对照组无认知功能障碍者;Mo CA量表评分结果显示,SCA3组存在认知功能障碍6例（54.55%）,对照组无认知功能障碍者。（2）SCA3组中P300的潜伏期为（440.18±43.34）ms,波幅为（14.95±6.85）μV;正常对照组P300的潜伏期为（321.08±23.80）ms,波幅为（15.26±7.00）μV,两组P300潜伏期差异有统计学意义（P〈0.01）,而波幅差异无统计学意义（P〉0.05）。（3）Spearmans相关性分析显示,SCA3组MMSE评分与P300的潜伏期呈负相关（r=-0.690,P〈0.05）,Mo CA评分与P300的潜伏期呈负相关（r=-0.828,P〈0.01）,P300波幅及ICARS评分与认知障碍无明显相关性。结论SCA3患者部分存在认知功能损害,P300的潜伏期可以预测患者的认知障碍程度。

英文摘要：

Objective To investigate the characteristics of event-related potentials（ ERPs） P300 and explore the value for assessing early cognitive impairment in patients with spinocerebellar ataxia type 3（ SCA3）. Methods A total of 11 SCA3 patients diagnosed by gene sequencing who visited the inpatients and / or admitted in neurology department between July2013 and July 2015 were selected（ SCA3 group）,and 13 health subjects were selected as the control group. The audition P300 examination was performed in all participants. Montreal cognitive assessment scale（ Mo CA） and mini mental state examination scale（ MMSE） were used to evaluate cognitive function,and international cooperative ataxia rating scale（ ICARS） was used for scoring of severity of ataxia. Results（ 1） The scores of MMSE and Mo CA in SCA3 group significantly decreased compared with control group（ all P 0. 05）. MMSE scoring showed that there were 5 patients with cognitive impairment（ 45. 45%） in SCA3 group,and there were no subjects with cognitive impairment in normal control group. Mo CA scoring showed that there were 6 patients with cognitive impairment（ 54. 55%） in SCA3 group,and there were no subjects with cognitive impairment in control group.（ 2） The results of P300 examination showed that there was significant difference in latency periods of P300 between SCA3 group and control group [（ 440. 18 ± 43. 34） ms vs（ 321. 08 ± 23. 80） ms,P 0. 01],and there was no significant difference in amplitudes of P300 between SCA3 group and control group [（ 14. 95 ±6. 85） μV vs（ 15. 26 ± 7. 00） μV,P 0. 05].（ 3） Spearmans correlation analysis showed that the MMSE score was negatively correlated with the latency period of P300（ r =- 0. 690,P 0. 05）,and the Mo CA scores was also negatively correlated with the latency period of P300（ r =- 0. 828,P 0. 01） in SCA3 group. The P300 amplitude and ICARS score were not correlated with cognitive impairment. Conclusions The cognitive impairment exists in par