Reversible cerebral vasoconstriction syndrome identification of prognostic factors

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Abstract

Object

: Reversible cerebral vasoconstriction syndrome (RCVS) is described as a clinical and radiological entity characterized by thunderclap headaches, a reversible segmental or multifocal vasoconstriction of cerebral arteries with or without focal neurological deficits or seizures. The purpose of this study is to determine risk factors of poor outcome in patients presented a RCVS.

Methods

A retrospective multi-center review of invasive and non-invasive neurovascular imaging between January 2006 and January 2011 has identified 10 patients with criterion of reversible segmental vasoconstriction syndrome. Demographics data, vascular risks and evolution of each of these patients were analyzed.

Results

Seven of the ten patients were females with a mean age of 46 years. In four patients, we did not found any causative factors. Two cases presented RCVS in post-partum period between their first and their third week after delivery. The other three cases were drug-induced RCVS, mainly vaso-active drugs. Cannabis was found as the causative factor in two patient, Sumatriptan identified in one patient while cyclosporine was the causative agent in also one patient. The mean duration of clinical follow-up was 10.2 months (range: 0–28 months). Two patients had neurological sequelae: one patient kept a dysphasia and the other had a homonymous lateral hemianopia. We could not find any significant difference of the evolution between secondary RCVS and idiopathic RCVS. The only two factors, which could be correlated to the clinical outcome were the neurological status at admission and the presence of intraparenchymal abnormalities (ischemic stroke, hematoma) in brain imaging.

Conclusions

Fulminant vasoconstriction resulting in progressive symptoms or death has been reported in exceptional frequency. Physicians had to remember that such evolution could happen and predict them by identifying all factors of poor prognosis (neurological status at admission, the presence of intraparenchymal abnormalities).

Introduction

Reversible cerebral vasoconstriction syndrome (RCVS) has been proposed as a unifying term for a variety of similar syndromes [1], [2] including Call-Fleming syndrome [3], benign angiopathy of the central nervous system [4], postpartum cerebral angiopathy [5], migrainous vasospasm, thunderclap headaches with reversible vasospasm and drug-induced angiopathy [6]. RCVS is described as a clinical and radiological entity characterized by thunderclap headaches, a reversible segmental or multifocal vasoconstriction of cerebral arteries with or without focal neurological deficits or seizures [1], [6], [7], [8].

Approximately, 60% of cases are secondary to a known etiology [9]; the most frequently inferred ones are exposure to vasoactive drugs such as serotonergics [9], [10], [11], [12], [13], sympathomimetics [14], [15], [16] and chemotherapeutics [18], [19], some clinical states such as pregnancy [20], [21], [22], [23], [24] and puerperium [25]. RCVS has a middle-aged female preponderance [1], [3], [6], [7], [8], [9], [26], [27], [28], [29], [30], [31]. The most common clinical feature of RCVS is a severe acute headache [6], [9] presented as thunderclap headache, often accompanied by nausea, vomiting, photophobia, blurred vision and transient motor deficit [7], [32], [33], [34]. Neurovascular imaging (conventional cerebral angiography or non-invasive imaging) typically shows diffuse, multifocal, segmental narrowing involving large and medium-sized arteries with strings and bead appearance [6], [7], [8], [9], [35], [36], [37]. These radiological abnormalities disappeared spontaneously or in response to calcium-channel blockers in a delay of 2–3 weeks [1], [7], [38]. Despite the reversibility of angiographic signs, RCVS could be complicated by ischemic stroke [7], [8], [9], [38], [39], [40] or convexity subarachnoid hemorrhage [1], [7], [8]. These complications are rare and without identified precipitating factors.

Recent literature reflected the growing interest for RCVS. Approximately 75 isolated cases are described between 1992 and 2011. However, only two studies [6] including more than ten cases have been published. The combination of more frequent cerebrovascular imaging with non-invasive techniques and the use of vaso-active drugs put a growing number of patients within RCVS spectrum. Complications and prognostic factors of RCVS were not developed in published studies.

We present our experience on reversible cerebral vasoconstriction syndrome with ten consecutive cases of angiographically proven RCVS, which were diagnosed between the university hospital of Lausanne and the regional hospital of Sion. Two patients in our study developed fatal complications; we discuss prognostic factors of RCVS in light of a review of the literature.

Section snippets

Patients and methods

We retrospectively reviewed invasive and non-invasive neurovascular imaging performed in our medical centers between January 2006 and January 2011 in the radiological database. 318 patients were selected who presented cerebral vasospasm. We excluded all patients with a history of cerebral aneurysm and other cerebro-vascular malformations (282 patients). We also excluded patients without documentation of the reversibility of neurovascular abnormalities (287 patients). After the exclusion

Demographics and patients past medical histories

The demographic data of the 10 patients are indicated in Table 1. Seven of the ten patients were female; their ages ranged from 23 to 68 years with a mean age of 46 years. Six patients (60%) had a history of hypertension. Other chronic medical conditions included depression, diabetes mellitus and hypercholesterolemia. Five patients presented a history of primary headaches disorders or migraine. Two patients did not have any relevant past medical history.

One of the patients had a history of

Discussion

Reversible cerebral vasoconstriction syndrome is emerging as an important cause of stroke in patients younger than 60 years [1], [2], [3], [6], [7], [8], [9]. Vasoconstriction syndromes remain poorly characterized and under-recognized mainly because of the lack of specific diagnostic tests or diagnostic criteria [1], [2], [6], [7], [8], [9], [39]. Misdiagnosis is frequent because the clinical, radiologic and angiographic abnormalities are often indistinguishable from conditions that cause

Conclusion

Reversible cerebral vasoconstriction syndrome is more frequent than previously thought. Our results highlight that RCVS could present a catastrophic neurological evolution and also they emphasize the need of considering the presence of parenchymal abnormalities at admission as an independent prognostic factor. Our study is limited by its retrospective nature and by the small number of patients. Larger series, even if it is difficult to conduct regarding the rarity of the pathology, will permit

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