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Previous studies reported cerebral hypoperfusion ocurred in patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL).1 2 As a cerebral small vessels disease, the reduction of blood flow velocity in cerebral small arteries may precede cerebral hypoperfusion and lesions formation. Due to the limitations of spatial resolution, conventional methods failed to detect the velocity of cerebral small vessels. Ultrahigh field MRI provided us a non-invasive way to investigate the velocity in lenticulostriate arteries (LSAs) of patients with CADASIL.3–5 Assessing blood flow velocity of small arteries can monitor the progress of the disease in long-term follow-up of patients.
Materials and methods
We enrolled 32 patients with CADASIL and 34 healthy controls from September 2019 to September 2020 at Peking University First Hospital. Details of subject selection, clinical assessment, MRI acquisition and analysis were described in online supplemental methods, online supplemental figure 1, online supplemental tables 1 and 2. The phase contrast magnetic resonance angiography (PC-MRA) was acquired at a 7 T MR scanner with high resolution (0.35×0.35×0.40 mm3) and low velocity-encoding (VENC) value (15.00 cm/s) was acquired to measure blood flow velocities of LSAs. The analysis pipeline of LSA velocities includes: (1) intensity bias corrections; (2) LSA region extraction on maximum intensity projection images; (3) threshold-based noise masking; (4) velocity components reconstruction from phase-difference data; (5) extracting dominant branches of the LSAs and calculating mean velocity. Test–retest experiments and Bland-Altman analysis were performed to validate the precision of the measurement method (online supplemental figure 2).5
Demographic and clinical features
One patient and three healthy controls were excluded for excessive head motion causing large artefacts and poor image quality. Ultimately, we included 31 patients from 24 pedigrees and 31 healthy controls in this study. The heterozygous mutations affecting the patients with CADASIL were listed in online supplemental table 3 …
CS and YW are joint first authors.
CS and YW contributed equally.
Contributors CS and YW drafted the manuscript. CS, CL, XF and SY acquired the clinical data. YW, ZL and QK acquired the MRI data. CS, YW, ZYX and ZHX analysed the data. ZZ, BW, YZ and JA provided MRI technical support. YY, ZW and WZ designed and conceptualized study. YY and ZZ revised the manuscript.
Funding This work was supported by the National Natural Science Foundation of China (81961128030 and 82001804), the Natural Science Foundation of Beijing Municipality (7191003), the Ministry of Science and Technology of China grant (2016YFC1300605 and 2019YFA0707103), the Capital’s Funds for Health Improvement and Research (CFH2020-2-5115), and the Strategic Priority Research Program of Chinese Academy of Science (XDB32010300).
Competing interests QK and JA were employed by Siemens and did technique support for the study.
Provenance and peer review Not commissioned; externally peer reviewed.
Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.
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