Reversible cerebral vasoconstriction syndromes and primary angiitis of the central nervous system: clinical, imaging, and angiographic comparison

Singhal, Aneesh B.; Topçuoğlu, Mehmet Akif; Fok, Joshua; Kurşun, Oğuzhan; Nogueira, Raul G; Frosch, Matthew P.; Caviness, Verne S.

doi:10.1002/ana.24652

Cited by 168 publications

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References 36 publications

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“…Over the last decade, the clinical and imaging features of reversible cerebral vasoconstriction syndrome (RCVS) have been extensively characterized [1][2][3][4][5][6][7] and distinguished from its historic mimic, primary angiitis of the CNS (PACNS). [7][8][9] Patients with RCVS invariably present with recurrent severe thunderclap headaches.…”

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“…[7][8][9] Patients with RCVS invariably present with recurrent severe thunderclap headaches. One-third to half develop ischemic and hemorrhagic brain lesions, either alone or in combination.…”

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“…Other RCVS studies have shown an association between transfemoral cerebral angiography and transient worsening, 2 or the development of infarcts and transient/permanent worsening. 10 Vasoconstrictive drugs such as serotonergic antidepressants have been consistently identified as triggers of RCVS 2,6,7,12 and may worsen vasospasm in the setting of aneurysmal subarachnoid hemorrhage 13,14 ; however, their contribution to RCVS-related worsening is uncertain. On the other hand, case reports have suggested a therapeutic role for calcium channel blockers and intra-arterial vasodilator infusions.…”

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“…The clinical imaging features of the first 159 patients have been published. 6,7 The diagnosis was established as follows: vasoconstriction reversibility was documented in 128 patients. An additional 30 patients developed typical thunderclap headaches, had evidence for segmental cerebral artery narrowing, and had a self-limited clinical course with no recurrences during follow-up.…”

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“…Angiographic categorization was completed without knowledge of treatment and prior to data analysis; moreover, the unbiased real-time clinical neuroradiology interpretations were considered for this categorization. Vasoconstriction severity scores, 7 though useful for quantifying distribution and overall severity, were not used to assess progression since they are insensitive to change (e.g., caliber reduction along a greater length of the same arterial segment would not score differently). Categorical data were analyzed using the x 2 , Fisher exact test, Student t test, or Mann-Whitney U test.…”

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Glucocorticoid-associated worsening in reversible cerebral vasoconstriction syndrome

2017

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Objective: Factors predicting poor outcome in patients with the reversible cerebral vasoconstriction syndrome (RCVS) have not been identified. Methods:In this single-center retrospective study, we analyzed the clinical, brain imaging, and angiography data in 162 patients with RCVS. Univariable and multivariable regression analysis were performed to identify predictors of persistent (nontransient) clinical worsening, radiologic worsening, early angiographic progression, and poor discharge outcome (modified Rankin Scale score 4-6).Results: The mean age was 44 6 13 years; 78% of patients were women. Persistent clinical worsening occurred in 14% at 6.6 6 4.1 days after symptom onset, radiologic worsening in 27% (mainly new infarcts), and angiographic progression in 15%. Clinical worsening correlated with angiographic progression and new nonhemorrhagic lesions. Age and sex did not independently predict any type of worsening. Infarction on baseline imaging predicted poor outcome. Prior serotonergic antidepressant use predicted clinical and angiographic worsening but not poor outcome. Intra-arterial vasodilator therapy independently predicted clinical worsening and poor discharge outcome but was offered to more severe cases. Glucocorticoid treatment proved to be an independent predictor of clinical, imaging, and angiographic worsening and poor outcome. Of the 23 patients with clinical worsening, 17 received glucocorticoids (15 within the preceding 2 days). There were no significant differences in baseline brain lesions and angiographic abnormalities between glucocorticoid-treated and untreated patients. Conclusion:Patients with RCVS at risk for worsening can be identified on basis of baseline features. Iatrogenic factors such as glucocorticoid exposure may contribute to worsening. Over the last decade, the clinical and imaging features of reversible cerebral vasoconstriction syndrome (RCVS) have been extensively characterized 1-7 and distinguished from its historic mimic, primary angiitis of the CNS (PACNS). [7][8][9] Patients with RCVS invariably present with recurrent severe thunderclap headaches. One-third to half develop ischemic and hemorrhagic brain lesions, either alone or in combination. Despite the dramatic onset and frequently ominous cerebral angiographic appearance, over 90% have excellent clinical outcome. 2,6 Yet studies that included a high proportion of inpatients have reported poor outcome (discharge modified Rankin Scale [mRS] score .3, including death) in 5%-14% of patients. 6,10 Factors predicting clinical outcome in RCVS have not been adequately identified. 10,11 We have reported an association between poor clinical outcome (mRS . 3) and glucocorticoid treatment, 6,7 which if confirmed has major clinical implications since glucocorticoids are frequently offered due to consideration of PACNS, a condition that warrants prompt initiation of long-term

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Glucocorticoid-associated worsening in reversible cerebral vasoconstriction syndrome

2017

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Dynamic Changes in White Matter Hyperintensities in Reversible Cerebral Vasoconstriction Syndrome

Chen

Chou²,

Fuh

et al. 2018

JAMA Neurol

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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) WMH load peaked at 3.2 (4.4) cm 3 in the third week postonset and fell to 0.8 (0.6) cm 3 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.

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Unusual causes of ischemic stroke and transient ischemic attack

Caprio

Lin

2019

Warlow's Stroke

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Reversible cerebral vasoconstriction syndromes and primary angiitis of the central nervous system: clinical, imaging, and angiographic comparison

Cited by 168 publications

References 36 publications

Glucocorticoid-associated worsening in reversible cerebral vasoconstriction syndrome

Glucocorticoid-associated worsening in reversible cerebral vasoconstriction syndrome

Dynamic Changes in White Matter Hyperintensities in Reversible Cerebral Vasoconstriction Syndrome

Unusual causes of ischemic stroke and transient ischemic attack

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