From the *Department of Nuclear Medicine, †Neurology, ‡Internal Medicine, Cardiology Division, §Physical Medicine and Rehabilitation, and ¶Radiology, Wonkwang University School of Medicine and Institute of Wonkwang Medical Science, Iksan, Republic of Korea.
Clin Nucl Med. 2013 Nov;38(11):e426-8. doi: 10.1097/RLU.0b013e3182815cbf.
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a rare hereditary small vessel disease. Although the symptoms are exclusively neurological, arteriopathy is generalized. We performed cardiac evaluation using myocardial perfusion single photon emission computed tomography (SPECT), dual-source 128-channel multidetector computed tomography (MDCT) angiogram, echocardiogram, and electrocardiogram (ECG) in a 46-year-old woman with CADASIL. No abnormal findings were observed on MDCT angiogram, echocardiogram, or ECG. However, SPECT demonstrated reversible perfusion defects in the left anterior descending artery territory. We suggest that myocardial perfusion SPECT is a valuable tool to identify risk from cardiovascular accident in CADASIL patients.
伴有皮质下梗死和白质脑病的脑常染色体显性动脉病（CADASIL）是一种罕见的遗传性小血管疾病。尽管症状完全是神经方面的，但动脉病是全身性的。我们对一位 46 岁的 CADASIL 女性患者进行了心脏评估，包括心肌灌注单光子发射计算机断层扫描（SPECT）、双源 128 通道多排螺旋 CT（MDCT）血管造影、超声心动图和心电图（ECG）。MDCT 血管造影、超声心动图或 ECG 均未见异常。然而，SPECT 显示左前降支区域有可逆性灌注缺陷。我们建议心肌灌注 SPECT 是识别 CADASIL 患者心血管意外风险的一种有价值的工具。
