Reversible cerebral vasoconstriction syndrome identification of prognostic factors

Robert, T.N.; Marchini, A.; Oumarou, Gaston; Uské, A

doi:10.1016/j.clineuro.2013.08.014

Cited by 45 publications

(63 citation statements)

References 37 publications

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“…However, a minority of patients will demonstrate delayed clinical worsening in the first few weeks following symptoms onset, most often due to the development of an ischemic infarct. 39,42,43,77 A more fulminant course of RCVS leading to permanent disability or death can be encountered in 5%-10% of patients. 1,2,6,12,43,60,77 Recurrence of RCVS appears to be rare, though some patients may have chronic mild headaches and fatigue on follow-up.…”

Section: Prognosis and Clinical Coursementioning

confidence: 99%

“…39,42,43,77 A more fulminant course of RCVS leading to permanent disability or death can be encountered in 5%-10% of patients. 1,2,6,12,43,60,77 Recurrence of RCVS appears to be rare, though some patients may have chronic mild headaches and fatigue on follow-up. 2,42,43,78 RCVS encountered in the postpartum period deserves special attention because it has been reported to be more likely to follow a fulminant course, with multifocal infarct, intracranial hemorrhage, extensive vasogenic edema, and death.…”

Section: Prognosis and Clinical Coursementioning

confidence: 99%

“…The syndrome typically follows a self-limiting, monophasic course, with resolution of symptoms by 3 weeks, and no new symptoms after 1 month. 1,13,17,23,39,57,77 By definition, resolution of vasoconstriction should occur by 3 months. However, a minority of patients will demonstrate delayed clinical worsening in the first few weeks following symptoms onset, most often due to the development of an ischemic infarct.…”

Section: Prognosis and Clinical Coursementioning

confidence: 99%

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Reversible Cerebral Vasoconstriction Syndrome, Part 1: Epidemiology, Pathogenesis, and Clinical Course

Miller

Shivashankar

Mossa‐Basha

et al. 2015

AJNR Am J Neuroradiol

205

194

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SUMMARY:Reversible cerebral vasoconstriction syndrome is a clinical and radiologic syndrome that represents a common presentation of a diverse group of disorders. The syndrome is characterized by thunderclap headache and reversible vasoconstriction of cerebral arteries, which can either be spontaneous or related to an exogenous trigger. The pathophysiology of reversible cerebral vasoconstriction syndrome is unknown, though alterations in cerebral vascular tone are thought to be a key underlying mechanism. The syndrome typically follows a benign course; however, reversible cerebral vasoconstriction syndrome may result in permanent disability or death in a small minority of patients secondary to complications such as ischemic stroke or intracranial hemorrhage.ABBREVIATIONS: RCVS ϭ reversible cerebral vasoconstriction syndrome; PRES ϭ posterior reversible encephalopathy syndrome

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Section: Prognosis and Clinical Coursementioning

confidence: 99%

Section: Prognosis and Clinical Coursementioning

confidence: 99%

Section: Prognosis and Clinical Coursementioning

confidence: 99%

See 1 more Smart Citation

Reversible Cerebral Vasoconstriction Syndrome, Part 1: Epidemiology, Pathogenesis, and Clinical Course

Miller

Shivashankar

Mossa‐Basha

et al. 2015

AJNR Am J Neuroradiol

205

194

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“…Severe headache (often thunderclap) is the prominent clinical manifestation but stroke occurs in up to 60% of cases, including small cortical subarachnoid haemorrhage (22-34%), intracerebral haemorrhage (6-20%) and IS (4-54%) [4,5,6,7,8,9]. Although RCVS is recognised as a potential cause of stroke [4,7,9,10,11,12,13,14,15], its frequency as a cause of IS has been evaluated only in 2 retrospective studies [16,17]. The three aims of our study were to determine the frequency of RV in a prospective series of 159 young IS patients, to describe the trigger factors and the detailed clinical and radiological characteristics in patients with IS and RV, and to compare these characteristics with those described in the four main published series of RCVS.…”

Section: Introductionmentioning

confidence: 99%

Ischaemic Strokes with Reversible Vasoconstriction and without Thunderclap Headache: A Variant of the Reversible Cerebral Vasoconstriction Syndrome?

Wolff¹,

Armspach

Lauer

et al. 2014

Cerebrovasc Dis

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Background: Reversible vasoconstriction (RV) may cause ischaemic stroke (IS) in the absence of any other defined stroke aetiology. The three objectives of our study were to evaluate the frequency of RV in a prospective series of young IS patients, to describe the detailed clinical-radiological features in the patients with RV and IS, and to compare these characteristics with those of reversible cerebral vasoconstriction syndrome (RCVS). Methods: We identified between October 2005 and December 2010, 159 consecutive young patients (<45 years) hospitalized for an acute IS confirmed by cerebral magnetic resonance imaging. An extensive diagnostic work-up was performed including toxicological urinary screening for cannabis, cocaine and amphetamines, and the usual biological, cardiac and vascular investigations for an IS in the young. We specifically studied patients with IS and RV, which was defined as multifocal intracranial arterial stenoses confirmed by intracranial arterial imaging that resolved within 3-6 months. Results: Out of 159 patients with IS, 21 (13%, 12 males, 9 females; mean age 32 years) had multifocal cerebral arterial stenoses that were fully reversible at 3-6 months, and no other cause for stroke. IS were located on posterior territory in 71% of cases, and vasoconstriction predominated on posterior cerebral and superior cerebellar arteries. Precipitating factors of IS and RV were the use of cannabis resin (n = 14), nasal decongestants (n = 2) and triptan (n = 1). Most cases (74%) had unusual severe headache, but none had thunderclap headache. None of 21 cases had reversible posterior leukoencephalopathy, cortical subarachnoid or intracerebral haemorrhage. Conclusion: RV was the sole identified cause of IS in 13% of our cohort. These young patients with IS and RV may have a variant of RCVS, related to an increased susceptibility to vasoactive agents in some individuals. RV in our patients differs from the classical characteristics of RCVS by the absence of thunderclap headache, reversible brain oedema and subarachnoid or intracranial haemorrhage. Intracranial arteries should be looked for, by appropriate vascular imaging, in young patients with IS at the acute stage and during the follow-up period.

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“…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 immunosuppressive treatment.…”

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confidence: 99%

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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Reversible cerebral vasoconstriction syndrome identification of prognostic factors

Cited by 45 publications

References 37 publications

Reversible Cerebral Vasoconstriction Syndrome, Part 1: Epidemiology, Pathogenesis, and Clinical Course

Reversible Cerebral Vasoconstriction Syndrome, Part 1: Epidemiology, Pathogenesis, and Clinical Course

Ischaemic Strokes with Reversible Vasoconstriction and without Thunderclap Headache: A Variant of the Reversible Cerebral Vasoconstriction Syndrome?

Glucocorticoid-associated worsening in reversible cerebral vasoconstriction syndrome

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