中南大学学报(医学版)
中南大學學報(醫學版)
중남대학학보(의학판)
JOURNAL OF CENTRAL SOUTH UNIVERSITY (MEDICAL SCIENCES)
2011年
6期
498-503
,共6页
周洁%雷立芳%廖鑫鑫%王俊岭%江泓%唐北沙%沈璐
週潔%雷立芳%廖鑫鑫%王俊嶺%江泓%唐北沙%瀋璐
주길%뢰립방%료흠흠%왕준령%강홍%당북사%침로
脊髓小脑性共济失调3型%马查多-约瑟夫病%国际协作共济失调评估量表%共济失调等级量表%临床评估
脊髓小腦性共濟失調3型%馬查多-約瑟伕病%國際協作共濟失調評估量錶%共濟失調等級量錶%臨床評估
척수소뇌성공제실조3형%마사다-약슬부병%국제협작공제실조평고량표%공제실조등급량표%림상평고
spinocerebellar ataxia type 3%Machado-Joseph disease%international cooperative ataxia rating scale (ICARS)%scale for the assessment and rating of ataxia (SARA)%clinical assessment
目的:探讨国际协作共济失调评估量表(ICARS)与共济失调等级量表(SARA)评估脊髓小脑性共济失调3型/ Machado-Joseph 病患者的相关因素.方法:应用ICARS与SARA对126例基因检测明确诊断为脊髓小脑性共济失调3型患者进行评估,分析ICARS评分、SARA评分与发病年龄、病程、CAG重复次数的相关性.结果:ICARS总评分(Y1)、SARA总评分(Y2)与病程(X2)呈正相关(r=0.586,P<0.05;r=0.643,P<0.05),回归方程分别为Y1=13.072+2.388 X2(F=68.874,P<0.05),Y2=4.403+0.961 X2(F=87.254,P<0.05).ICARS总评分、SARA总评分与年龄的比值分别与CAG重复次数呈正相关(r=0.328,P<0.05;r=0.335,P<0.05).ICARS各项评分、SARA各项评分与病程均呈正相关(r=0.257~0.589,P<0.05;r=0.432~0.623,P<0.05).ICARS各项评分、SARA各项评分与年龄比值分别与CAG重复次数呈正相关(r=0.263~0.403,P<0.05;r=0.189~0.366,P<0.05).ICARS总评分、SARA总评分均随着病情严重程度增加而增加.结论:ICARS与SARA评分量表均能够有效地反映共济失调患者的病情严重程度,研究者可根据实际需要选用合适的量表.
目的:探討國際協作共濟失調評估量錶(ICARS)與共濟失調等級量錶(SARA)評估脊髓小腦性共濟失調3型/ Machado-Joseph 病患者的相關因素.方法:應用ICARS與SARA對126例基因檢測明確診斷為脊髓小腦性共濟失調3型患者進行評估,分析ICARS評分、SARA評分與髮病年齡、病程、CAG重複次數的相關性.結果:ICARS總評分(Y1)、SARA總評分(Y2)與病程(X2)呈正相關(r=0.586,P<0.05;r=0.643,P<0.05),迴歸方程分彆為Y1=13.072+2.388 X2(F=68.874,P<0.05),Y2=4.403+0.961 X2(F=87.254,P<0.05).ICARS總評分、SARA總評分與年齡的比值分彆與CAG重複次數呈正相關(r=0.328,P<0.05;r=0.335,P<0.05).ICARS各項評分、SARA各項評分與病程均呈正相關(r=0.257~0.589,P<0.05;r=0.432~0.623,P<0.05).ICARS各項評分、SARA各項評分與年齡比值分彆與CAG重複次數呈正相關(r=0.263~0.403,P<0.05;r=0.189~0.366,P<0.05).ICARS總評分、SARA總評分均隨著病情嚴重程度增加而增加.結論:ICARS與SARA評分量錶均能夠有效地反映共濟失調患者的病情嚴重程度,研究者可根據實際需要選用閤適的量錶.
목적:탐토국제협작공제실조평고량표(ICARS)여공제실조등급량표(SARA)평고척수소뇌성공제실조3형/ Machado-Joseph 병환자적상관인소.방법:응용ICARS여SARA대126례기인검측명학진단위척수소뇌성공제실조3형환자진행평고,분석ICARS평분、SARA평분여발병년령、병정、CAG중복차수적상관성.결과:ICARS총평분(Y1)、SARA총평분(Y2)여병정(X2)정정상관(r=0.586,P<0.05;r=0.643,P<0.05),회귀방정분별위Y1=13.072+2.388 X2(F=68.874,P<0.05),Y2=4.403+0.961 X2(F=87.254,P<0.05).ICARS총평분、SARA총평분여년령적비치분별여CAG중복차수정정상관(r=0.328,P<0.05;r=0.335,P<0.05).ICARS각항평분、SARA각항평분여병정균정정상관(r=0.257~0.589,P<0.05;r=0.432~0.623,P<0.05).ICARS각항평분、SARA각항평분여년령비치분별여CAG중복차수정정상관(r=0.263~0.403,P<0.05;r=0.189~0.366,P<0.05).ICARS총평분、SARA총평분균수착병정엄중정도증가이증가.결론:ICARS여SARA평분량표균능구유효지반영공제실조환자적병정엄중정도,연구자가근거실제수요선용합괄적량표.
Objective To investigate the related factors of international cooperative ataxia rating scale (ICARS) and scale for the assessment and rating of ataxia scores (SARA) in patients with spinocerebellar ataxia type 3/Machado-Joseph disease. Methods A total of 126 SCA3/MJD patients were assessed by ICARS and SARA. The relation between ICARS or SARA scores and age of onset, disease duration and CAG repeat size was analyzed. Results Either the total ICARS or the total SARA score was positively related with the disease duration(r=0.586,P<0.05;r=0.643,P<0.05). Simple linear regression equations were: Y1(total ICARS score)=13.072+2.388 X2(disease duration)(F=68.874,P<0.05); Y2(total SARA score)=4.403+ 0.961 X2(disease duration)(F=87.254, P<0.05). Either age adjusted the total ICARS score or age adjusted the total SARA score was positively related with CAG repeat size(r=0.328, P<0.05; r=0.335, P<0.05). Both the ICARS subscores and the SARA subscores were positively related with the disease duration(r=0.257-0.589, P<0.05; r=0.432-0.623, P<0.05). Both age adjusted ICARS subscores and age adjusted SARA subscores were positively related with CAG repeat size(r=0.263-0.403, P<0.05; r=0.189-0.366, P<0.05). Analysis of variance showed that the total ICARS score and the total SARA score increased with the disease stage. Conclusion ICARS and SARA are both reliable and effective scales in assessing the severity of ataxia in patients with SCA3/MJD, and researchers can choose the most suitable scale according to specific requirement.