Glucocorticoid-associated worsening in reversible cerebral vasoconstriction syndrome

Abstract

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

Reversible cerebral vasoconstriction syndrome in paediatric patients with systemic lupus erythematosus: implications for management

“…Calcium channel‐blocker therapy (nimodipine) may be discussed as a vasoactive drug, with cautious use and close monitoring because of systemic action, and hence the risk of precipitously dropping systemic blood pressure and causing cerebral ischaemic injury. A recent study showed that corticosteroids could worsen RCVS outcome . Therefore, in the setting of SLE or SLE flare‐up, acute neurological symptoms should lead to complete brain imaging, including cerebral angiography, and specific tests to rule out subarachnoid haemorrhage (CSF analysis with spectro‐photometry for xanthochromia and/or gradient echo magnetic resonance sequencing).…”

“…A recent study showed that corticosteroids could worsen RCVS outcome. 11 Therefore, in the setting of SLE or SLE flare-up, acute neurological symptoms should lead to complete brain imaging, including cerebral angiography, and specific tests to rule out subarachnoid haemorrhage (CSF analysis with spectrophotometry for xanthochromia and/or gradient echo magnetic resonance sequencing).…”

Cerebrospinal fluid findings in reversible cerebral vasoconstriction syndrome: a way to differentiate from cerebral vasculitis?

“…Nevertheless, the diagnostic workup showed a complete restitution of cerebral artery vasoconstriction within 4 weeks to 3 months which could be excluded to appear in PACNS without immunosuppressive therapy. Furthermore, RCVS patients are at risk of neurological worsening if treated with glucocorticoids, which will mainly be the firstline therapy in suspected cerebral vasculitis [9]. However, a conclusive interpretation is lacking in the literature, as well as the discussion of whether the diagnostic criterion of CSF leucocyte counts < 10/mm 3 should be maintained.…”

Corticosteroid therapy and severity of vasogenic edema in posterior reversible encephalopathy syndrome

“…[45] This possibility has also been raised for a related disorder – reversible cerebral vasoconstriction syndrome. [44] Alternatively, it is plausible that steroid administration simply reflects the presence of an underlying inflammatory or oncological disease, which may itself cause PRES through endothelial dysfunction [2]. However, were this the case, we would expect to find less vasogenic edema in patients receiving steroids.…”