Dynamic changes in white matter hyperintensities in reversible cerebral vasoconstriction syndrome

Shih Pin Chen*, Kun Hsien Chou, Jong Ling Fuh, Yi Hua Huang, Chu Chung Huang, Jiing Feng Lirng, Yen Feng Wang, Ching Po Lin, Shuu Jiun Wang

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

28 Scopus citations


Importance: White matter hyperintense lesions (WMHs) are highly prevalent in patients with reversible cerebral vasoconstriction syndrome (RCVS); however, their characteristics and underlying pathophysiology are unclear. Objective: To investigate the spatiotemporal distribution and pathomechanisms of WMHs in patients with RCVS. Design, Setting, and Participants: We prospectively recruited patients with RCVS over a 3-year period from January 2010 through December 2012 from the headache center or emergency department of Taipei Veterans General Hospital, Taipei, Taiwan, a 2947-bed national medical center. In total, 85 patients with RCVS were approached, of whom 4 declined to participate, 5 declined follow-up scans, 6 were lost to follow-up, and 5 had suboptimal images. Patients received serial isotropic 3-dimension fluid-attenuated inversion recovery sequence imaging (1-mm slice thickness) with a 3-T magnetic resonance imaging machine as well as transcranial and extracranial color-coded sonography on registration and during follow-ups (at 1 and 2 months, with variations adapting to clinical condition). Data were analyzed from January 2015 to May 2017. Main Outcomes and Measures: The fluid-attenuated inversion recovery lesion segmentation toolbox was used to segment WMHs automatically. The WMHs were classified as periventricular or deep and were segmented into 13 anatomical locations. The neuroimaging scientists who executed the program were blinded to clinical information. Vascular parameters, including the Lindegaard index (vasoconstriction severity), pulsatility index, and resistance index of the internal carotid artery, were independently collected for comparison. Results: Sixty-five patients with RCVS completed the study and underwent a total of 162 magnetic resonance imaging examinations. Of the 65 included patients, 58 (89%) were women, and the mean (SD) age was 50.1 (8.9) years. The total mean (SD)WMHload peaked at 3.2 (4.4) cm3 in the third week postonset and fell to 0.8 (0.6) cm3 in the fourth week. White matter hyperintensities were predominantly frontal and periventricular. White matter hyperintensity load correlated strongly with Lindegaard index during the second week of the disease course (r = 0.908; P < .001) and also correlated with the pulsatility index and resistance index of the internal carotid artery. Conclusions and Relevance: White matter hyperintensities in patients with RCVS have a dynamic temporal evolution that parallels disease severity. The finding of partially reversible WMHs deserves attention and should be known by clinicians taking care of patients with RCVS. White matter hyperintensities in RCVS may be attributed, at least partially, to regional hypoperfusion and impaired dampening capacity to central pulsatile flow.

Original languageEnglish
Pages (from-to)1106-1113
Number of pages8
JournalJAMA Neurology
Issue number9
StatePublished - Sep 2018


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