Diagnosis of Reversible Cerebral Vasoconstriction Syndrome
Muehlschlegel S, Kursun O, Topcuoglu MA, Fok J, Singhal AB
JAMA Neurol. 2013;70:1254-1260
Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by clinical features of recurrent thunderclap headaches and angiographic findings of reversible segmental multifocal cerebral artery narrowing. Subarachnoid hemorrhage (SAH) occurs in more than 30% of patients with RCVS. Because of overlapping clinical and imaging characteristics, patients with RCVS with SAH (RCVS-SAH) may be misdiagnosed with aneurysmal SAH (aSAH), cryptogenic "angiogram-negative" SAH (cSAH), or other potentially life-threatening conditions.
The goal of this retrospective analysis was to identify predictors that could help differentiate patients presenting with RCVS-SAH from those presenting with aSAH and cSAH. At an academic hospital and tertiary referral center, standard criteria were used to diagnose 38 consecutive patients with RCVS-SAH (from 1998 to 2009), 515 with aSAH (from 1995 to 2003), and 93 with cSAH (from 1995 to 2003).
On the basis of multivariate logistic regression analysis, predictors distinguishing RCVS-SAH from aSAH were younger age, chronic headache disorder, previous depression, previous chronic obstructive pulmonary disease, lower Hunt-Hess grade, lower Fisher SAH group, higher number of affected arteries, and the presence of bilateral arterial narrowing.
Younger age, female sex, previous hypertension, chronic headache disorder, lower Hunt-Hess grade, lower Fisher SAH group, and the presence of bilateral arterial narrowing helped to differentiate RCVS-SAH from cSAH.
The limitations of this study include small sample sizes; retrospective design creating potential biases; data collection spanning several years; and lack of aSAH patients with SAH from arteriovenous malformations, dural arteriovenous fistulas, or other vascular lesions.
Nonetheless, the investigators identified important clinical and imaging characteristics that differentiate RCVS-SAH from aSAH and cSAH. These may facilitate diagnostic accuracy, clinical management, and resource utilization. Furthermore, the findings suggest that the diffuse, multifocal, prolonged vasoconstriction in RCVS is not related to the presence or location of subarachnoid blood but may instead result from the activity and sensitivity of vascular receptors.
Additional research is needed to determine whether use of these predictors could improve bedside diagnosis, reduce unnecessary testing, and shorten hospital length of stay and to elucidate the pathophysiology of vasoconstriction in RCVS.
Abstract
Differentiating Reversible Cerebral Vasoconstriction Syndrome With Subarachnoid Hemorrhage From Other Causes of Subarachnoid Hemorrhage
Muehlschlegel S, Kursun O, Topcuoglu MA, Fok J, Singhal AB
JAMA Neurol. 2013;70:1254-1260
Study Summary
Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by clinical features of recurrent thunderclap headaches and angiographic findings of reversible segmental multifocal cerebral artery narrowing. Subarachnoid hemorrhage (SAH) occurs in more than 30% of patients with RCVS. Because of overlapping clinical and imaging characteristics, patients with RCVS with SAH (RCVS-SAH) may be misdiagnosed with aneurysmal SAH (aSAH), cryptogenic "angiogram-negative" SAH (cSAH), or other potentially life-threatening conditions.
The goal of this retrospective analysis was to identify predictors that could help differentiate patients presenting with RCVS-SAH from those presenting with aSAH and cSAH. At an academic hospital and tertiary referral center, standard criteria were used to diagnose 38 consecutive patients with RCVS-SAH (from 1998 to 2009), 515 with aSAH (from 1995 to 2003), and 93 with cSAH (from 1995 to 2003).
On the basis of multivariate logistic regression analysis, predictors distinguishing RCVS-SAH from aSAH were younger age, chronic headache disorder, previous depression, previous chronic obstructive pulmonary disease, lower Hunt-Hess grade, lower Fisher SAH group, higher number of affected arteries, and the presence of bilateral arterial narrowing.
Younger age, female sex, previous hypertension, chronic headache disorder, lower Hunt-Hess grade, lower Fisher SAH group, and the presence of bilateral arterial narrowing helped to differentiate RCVS-SAH from cSAH.
Viewpoint
The limitations of this study include small sample sizes; retrospective design creating potential biases; data collection spanning several years; and lack of aSAH patients with SAH from arteriovenous malformations, dural arteriovenous fistulas, or other vascular lesions.
Nonetheless, the investigators identified important clinical and imaging characteristics that differentiate RCVS-SAH from aSAH and cSAH. These may facilitate diagnostic accuracy, clinical management, and resource utilization. Furthermore, the findings suggest that the diffuse, multifocal, prolonged vasoconstriction in RCVS is not related to the presence or location of subarachnoid blood but may instead result from the activity and sensitivity of vascular receptors.
Additional research is needed to determine whether use of these predictors could improve bedside diagnosis, reduce unnecessary testing, and shorten hospital length of stay and to elucidate the pathophysiology of vasoconstriction in RCVS.
Abstract
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