CAJ | 학술논문

目的总结常染色体显性遗传性脑动脉病伴皮质下梗死和白质脑病(CADASIL)的眼底动脉改变规律,探讨其与临床表现以及头颅MRI改变的关系.方法 7个家系的16例患者(男4例,女12例)经过我院病理和基因检查证实为CADASIL,平均年龄(43.4±8.1)岁,平均病程(4.7±3.4)年.对所有患者进行简易精神状态量表(MMSE)评分、改良Rankin评分、眼底动脉检查和头颅MRI检查,把眼底动脉狭窄程度分为0～Ⅲ级,对头颅MRI改变进行Coulthard评分.计算患者眼底动脉狭窄分级与头颅MRI评分、MMSE、改良Rankin评分以及年龄的相关系数.选择16名健康人(在性别、年龄、视力等方面匹配)作为对照进行眼底血管检查.结果 16例患者中15例出现眼底动脉狭窄,其中4例存在轻度动脉-静脉交叉压迫.出现0级、Ⅰ级、Ⅱ级和Ⅲ级眼底动脉狭窄的患者分别为1例、4例、7例和4例,相应头颅MRI的Coulthard评分平均分按眼底动脉狭窄程度从0到Ⅲ级分别为6.0、19.0、25.1和29.8分.眼底动脉狭窄分级和头颅MRI的Coulthard评分、MMSE、改良Rankin评分、年龄的相关系数分别为0.743(P＜0.001)、-0.429(P＜0.05)、0.437(P＞0.05)和0.299(P＞0.05).对照组有2例出现Ⅰ级眼底动脉狭窄.结论眼底动脉狭窄是CADASIL常见的视网膜改变;眼底动脉狭窄和头颅MRI改变以及痴呆可能有一定的统计学相关性.
목적 총결상염색체현성유전성뇌동맥병반피질하경사화백질뇌병(CADASIL)적안저동맥개변규률,탐토기여림상표현이급두로MRI개변적관계.방법 7개가계적16례환자(남4례,녀12례)경과아원병리화기인검사증실위CADASIL,평균년령(43.4±8.1)세,평균병정(4.7±3.4)년.대소유환자진행간역정신상태량표(MMSE)평분、개량Rankin평분、안저동맥검사화두로MRI검사,파안저동맥협착정도분위0～Ⅲ급,대두로MRI개변진행Coulthard평분.계산환자안저동맥협착분급여두로MRI평분、MMSE、개량Rankin평분이급년령적상관계수.선택16명건강인(재성별、년령、시력등방면필배)작위대조진행안저혈관검사.결과 16례환자중15례출현안저동맥협착,기중4례존재경도동맥-정맥교차압박.출현0급、Ⅰ급、Ⅱ급화Ⅲ급안저동맥협착적환자분별위1례、4례、7례화4례,상응두로MRI적Coulthard평분평균분안안저동맥협착정도종0도Ⅲ급분별위6.0、19.0、25.1화29.8분.안저동맥협착분급화두로MRI적Coulthard평분、MMSE、개량Rankin평분、년령적상관계수분별위0.743(P＜0.001)、-0.429(P＜0.05)、0.437(P＞0.05)화0.299(P＞0.05).대조조유2례출현Ⅰ급안저동맥협착.결론 안저동맥협착시CADASIL상견적시망막개변;안저동맥협착화두로MRI개변이급치태가능유일정적통계학상관성.
Objective To assess retinal arteriole stenosis in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) and to evaluate the relationship between retinal arteriole stenosis with cranial MRI changes and clinical features. Methods Sixteen CADASIL patients (mean age was (43.4±8.1)years, mean duration was(4.7±3.4)years)and sixteen age matched healthy individuals were enrolled. Mini-Mental State Examination (MMSE) and modified Rankin scale scoring were performed in 16 patients. Cranial MRl with Coulthard scores was assessed on CADASIL patients as well. Retinal examinations for arteriole stenosis were done for each subject. Bivariate correlations (Kendall's tau-b) were used to assess the relationship among the grade of retinal stenosis, MMSE, modified Rankin seale, cranial MRI with Coulthard scores and age. Results Retinal arteriole stenosis presented in 15 cases. in whom 4 cases showed mild arterio-venous nicking. while it only presented in 2 controls. Grade 0.Ⅰ, and Ⅲ of retinal arteriole stenosis are noted in 1/16,4/16,7/16 and 4/16cases respectively. Coulthard scores of cerebral MRl were 6.0.19.0.25.1 and 29.8 respectively from Grade 0 toⅢ of retinal arteriole stenosis. The correlation coefficient of retinal arteriole stenosis and cranial MRI scores was 0.743(P＜0.001＝,MMSE-0.429(P＜0.05＝,modified Rankin scale 0.437(P＞0.05).and age 0.299(P＞0.05).Conclusion Retinal arteriole stenosis is significantly correlated with lesion in cranial MRI and dementia.