In a recent study,1 glucocorticoid exposure was found to be an independent predictor of poor outcomes, as well as clinical, imaging, and angiographic worsening in reversible cerebral vasoconstriction syndrome (RCVS).
Although excellent clinical outcomes occur in over 90% of patients with RCVS,1 previous research, which included a high ratio of inpatients, has recorded poor outcomes.2 Aneesh Singhal, MD and Mehmet Topcuoglu, MD, both from Harvard Medical School, sought to identify predictors of poor outcomes in RCVS.
The retrospective study included 162 Massachusetts General Hospital patients with RCSV (mean age= 44 years; 78% female). Persistent clinical worsening occurred in 14%, radiological worsening in 27%, and angiographic progression in 15%. Of the 23 patients with clinical worsening, 74% were prescribed glucocorticoids (65% within the preceding 2 days). Baseline brain lesions and angiographic abnormalities did not differ between the glucocorticoid-treated and untreated patients.
In exploratory multivariate models, glucocorticoids were strong independent predictors for clinical worsening (Odds Ratio[OR]: 10.2; P <.001), radiological worsening (OR: 4.36; P =.001), angiographic worsening (OR: 3.83; P =.004), and poor outcomes (OR: 4.23; P =.025).
Notably, serotonergic antidepressant use was associated with clinical (OR: 3.06; P =.022) and angiographic (OR: 2.59; P =.041) worsening.
The authors reported that, “Of the 46 patients who received steroids, 17 (37%) showed persistent clinical worsening, 2 showed clinical improvement, and 27 showed no clinical change.” However, out of 116 patients not treated with steroids, only 6 (5%) showed worsening (P <.001).
Despite recent research differentiating the clinical and imaging features of RCVS from its mimic, primary angiitis of the CNS (PACNS),3 glucocorticoids are often prescribed to RCVS patients either due to a misdiagnosis of PACNS, or fear of missing PACNS.1 The authors demonstrated in a previous study3 that the diagnosis of RCVS “can be made with alacrity based on bedside clinical and brain imaging features alone and that our criteria have nearly 100% specificity in distinguishing RCVS from PACNS.”
The authors emphasized the importance of withholding glucocorticoids in RCVS patients, those with an uncertain diagnosis, and in cases of clinical or radiological progression. “Symptomatic pain management and removal of vasoconstrictive precipitants and strategies to avoid the Valsalva maneuver are typically adequate,” the authors wrote.
- Singhal AB, Topcuoglu MA. Glucocorticoid-associated worsening in reversible cerebral vasoconstriction syndrome. Neurology. 2016 Dec 9; doi:10.1212/WNL.0000000000003510 [Epub ahead of print]
- Katz BS, Fugate JE, Ameriso SF, et al. Clinical worsening in reversible cerebral vasoconstriction syndrome. JAMA Neurol. 2014;71:68-73.
- Singhal AB, Topcuoglu MA, Fok JW, et al. Reversible cerebral vasoconstriction syndromes and primary angiitis of the central nervous system: clinical, imaging, and angiographic comparison. Ann Neurol. 2016;79:882-94.