CAJ | 학술논문

目的：研究脊髓小脑共济失调3型（spinocerebellar ataxia type 3，SCA3）患者体重指数（body mass index ，BMI）与胰岛素/胰岛素样生长因子1（IIS）系统、临床和生化指标的相关性。方法比较21例SCA3型患者和性别年龄与之相匹配的22例健康对照者的BMI、血清胰岛素（Ins）、胰岛素样生长因子1（IGF‐1）及其结合蛋白3（IGFBP‐3）的差异，并分析BMI和Ins、IGF‐1、IGFBP‐3、CAG重复序列、年龄、发病年龄、病程及国际协作共济失调评估量表（ICARS）总评分及各分项评分间的相关性。结果（1）与健康对照组相比，SCA3型患者BMI以及Ins、IGF‐1、IGFBP‐3水平降低（均 P＜0.05），血清IGF‐1/IGFBP‐3比值在两组间比较差异无统计学意义（P＞0.05）。（2）Spearman相关分析显示，BMI与血清Ins水平呈正相关（r＝0.504，P＝0.020），与CAG重复序列、IC A RS总评分及眼球运动评分呈负相关（ r＝－0.566，P＝0.018；r＝－0.503，P＝0.047；r＝－0.617，P＝0.011），与其他临床及生化指标无明显相关性（P＞0.05）；姿势和步态障碍评分与中、重度SCA3患者组BMI值呈负相关（r＝－0.824，P＝0.006），与轻度SCA3患者组BMI值无明显相关性（r＝－0.404，P＝0.171）。结论体质量减轻可能是SCA3型患者的早期症状，可协助临床诊断及疾病分期。
목적：연구척수소뇌공제실조3형（spinocerebellar ataxia type 3，SCA3）환자체중지수（body mass index ，BMI）여이도소/이도소양생장인자1（IIS）계통、림상화생화지표적상관성。방법비교21례SCA3형환자화성별년령여지상필배적22례건강대조자적BMI、혈청이도소（Ins）、이도소양생장인자1（IGF‐1）급기결합단백3（IGFBP‐3）적차이，병분석BMI화Ins、IGF‐1、IGFBP‐3、CAG중복서렬、년령、발병년령、병정급국제협작공제실조평고량표（ICARS）총평분급각분항평분간적상관성。결과（1）여건강대조조상비，SCA3형환자BMI이급Ins、IGF‐1、IGFBP‐3수평강저（균 P＜0.05），혈청IGF‐1/IGFBP‐3비치재량조간비교차이무통계학의의（P＞0.05）。（2）Spearman상관분석현시，BMI여혈청Ins수평정정상관（r＝0.504，P＝0.020），여CAG중복서렬、IC A RS총평분급안구운동평분정부상관（ r＝－0.566，P＝0.018；r＝－0.503，P＝0.047；r＝－0.617，P＝0.011），여기타림상급생화지표무명현상관성（P＞0.05）；자세화보태장애평분여중、중도SCA3환자조BMI치정부상관（r＝－0.824，P＝0.006），여경도SCA3환자조BMI치무명현상관성（r＝－0.404，P＝0.171）。결론체질량감경가능시SCA3형환자적조기증상，가협조림상진단급질병분기。
Objective To evaluate the body mass index (BMI) of patients with spinocerebellar ataxia type 3 (SCA3) and to assess its correlations with insulin/insulin like grow th factor 1 (IIS) system ,clinical and biochemical findings .Methods A case‐control study of the differences of serum insulin ,insulin like growth factor 1 (IGF‐1) ,and insulin‐like growth factor binding protein 3 (IGFBP‐3) between 21 SCA3 patients and 22 healthy subjects matched by sex and age was performed .The correlations between BMI and insulin ,IGF‐1 and IGFBP‐3 ,age ,age of onset ,disease duration ,CAG repeat size , total and clausal scores of the international cooperative ataxia rating scale (ICARS) were analyzed .Results (1) Comparing with the control subjects , BMI ,serum levels of insulin ,IGF‐1 ,and IGFBP‐3 were lower in SCA3 patients (P< 0.05) ,whereas IGF‐1/IGFBP‐3 were not statistically different between control and SCA 3 subjects ( P> 0.05 ) . (2 ) By Spearman analysis ,BMI presented a negative correlation with CAG repeat size ,total scores of ICARS ,and scores of eyeball movement (r= -0.566 , P= 0.018 ;r= -0.503 , P= 0.047 ;r= -0.617 , P= 0.011) ,whereas it has a positive correlation with serum insulin levels (r=0.504 , P=0.020);It has no correlation with other clinical or biochemical index (P>0.05) .ICARS posture gait score was negatively related with BMI in moderate and severe SCA3 patients (r= -0.824 , P=0.006) ,but not correlated with mild SCA3 patients .Conclusions Weight loss might be a primary disturbance of SCA3 ,which can help to make diagnosis and assess the severity of the disease .