常染色体显性遗传性脑动脉病伴皮层下梗死和白质脑病患者的MR定量指标及其与临床的关系

目的 对常染色体显性遗传性脑动脉病伴皮层下梗死和白质脑病(CADASIL)患者的白质高信号和脑体积进行定量分析,并探讨其与临床的关系.方法 15例通过病理检查确诊为CADASIL的患者进行常规MR扫描,统计脑半卵圆中心、内囊后肢、外囊、胼胝体和颞极白质受病变累及情况.利用计算机后处理软件计算标准化颅脑体积和白质高信号占颅脑体积的百分比,并与年龄、美国国立卫生研究院卒中量表(NIHSS)和简易精神状态检查(MMSE)量表评分进行Spearman相关性分析.结果 CADASIL患者的白质病变累及部位依次为:半卵圆中心(13/15)、颞极白质(10/15)、外囊(8/15)、内囊后肢(5/15)、胼胝体(4/15).白质高信号占颅脑体积的百分比为(5.7±1.4)%,标准化颅脑体积为(1602±58)×103mm3.年龄与标准化颅脑体积呈负相关(r=-0.555,P＜0.05);白质高信号百分比与NIHSS、MMSE量表评分分别呈正、负相关(r=0.522,P＜0.05;r=-0.679,P＜0.01);标准化颅脑体积与NIHSS评分呈负相关(r=-0.624,P＜0.05).结论 CADASIL患者的白质高信号和脑体积可以定量测量,这两种影像学指标可以在一定程度上反映患者的病情.白质高信号的发展可能预示患者认知功能的下降.

Quantitative MR measures in patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy and their relationships with clinical scales

Objective To analyze white matter hyperintensities(WMH)and brain volumes quantitatively in patients with Cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy(CADASIL)on conventional MRI and correlate them with clinical parameters.Methods Fifteen patients with CADASIL confirmed by pathological investigation underwent conventional MRI examinations.Involvement of centrum semiovale,posterior limbs of the internal capsule,corpus callosum,external capsules and white matter of the temporal poles was determined by an experienced radiologist.Normalized brain volume(NBV)and the percentage of WMH to brain volume were calculated with softwares and they were correlated with NIHSS and MMSE statistically.Results Involvement of white matter in the brains of patients with CADASIL included:semiovale centrum in 13 cases,white matter of the temporal poles in 10 cases,external capsules in 8 cases,posterior limbs of the internal capsule in 5 cases,corpus callosum in 4 cases.The percentage of WMH to brain volume was(5.7±1.4)%,and the NBV was(1602±58)×103mm3.Spearman test showed a significant relationship between age and NBV(r=-0.555,P＜0.05).There was a significant relationship between NBV and NIHSS(r=-0.624.P＜0.05).The percentage of WMH to brain volume correlated significantly with NIHSS and MMSE(r=0.522,P＜0.05;r=-0.679,P＜0.01).Conclusions The white matter hvperintensities and brain volume in patients with CADASIL can be assessed quantitatively,which showed correlation with severity of the clinical scale.The development of WMH may refleet the degree of cognitive impairment in CADASIL.