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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。
Objective To investigate the characteristics of event-related potentials (P300) in patients with Spinocerebellar ataxia type 3 (SCA3) and to evaluate its evaluation of patients with cognitive dysfunction. Methods Totally 11 SCA3 patients (SCA3 group) diagnosed by genetic sequencing from July 2013 to July 2015 in inpatient department or outpatient department of neurology were selected as research object, and 13 healthy subjects were selected as control group. Auditory P300 was performed on all subjects and MMSE was used to assess cognitive function using the Montreal Cognitive Assessment Scale (MoCA). ICARS was used to assess cognitive function Economic disability severity score. Results (1) The scores of MMSE and Mo CA in SCA3 group were significantly lower than those in normal control group (all P <0.05). The scores of MMSE in SCA3 group showed that there were 5 cases (45.45%) of cognitive impairment in SCA3 group, MoCA scale score results showed that there were 6 cases (54.55%) of cognitive dysfunction in SCA3 group and no cognitive impairment in control group. (2) The incubation period of P300 in SCA3 group was (440.18 ± 43.34) ms and the amplitude was (14.95 ± 6.85) μV. The incubation period of P300 in control group was (321.08 ± 23.80) ms with amplitude of (15.26 ± 7.00) μV, There was significant difference in latency of P300 between two groups (P <0.01), but there was no significant difference in amplitude (P> 0.05). (3) Spearmans correlation analysis showed that the MMSE score of SCA3 group was negatively correlated with the latency of P300 (r = -0.690, P <0.05), while the MoCA score was negatively correlated with the latency of P300 (r = -0.828, P <0.01 ), P300 amplitude and ICARS score and cognitive impairment no significant correlation. Conclusion Some patients with SCA3 have cognitive impairment, and the latency of P300 can predict the degree of cognitive impairment in patients.