Reversible Vasoconstriction Syndrome Research Articles

Reversible cerebral vasoconstriction syndrome identification of prognostic factors

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. MethodsA 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. ResultsSeven 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. ConclusionsFulminant 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).

Abstract TP425: Clinical and Radiological Spectrum of Nontraumatic Cortical Subarachnoid Hemorrhages : A Consecutive Series of 62 Patients

Non-traumatic cortical subarachnoid hemorrhage (cSAH) is an unusual location of SAH not related to aneurysmal rupture. Several causes have been described in small cohorts. We report the characteristics and the follow-up of a large cohort of such patients. Methods: During 4 years, consecutive patients admitted in our institution with a cSAH revealed by neurological symptoms were included in a registry. Retrospective review of medical records, neuroimaging studies, and follow-up data was performed. Results: Sixty two patients (37 women; mean age ± SD : 66 ± 14 years) were included. The main clinical presentation was a transient deficit in 50 % of cases. Headache was present in only 39 % of cases. Cerebral amyloid angiopathy (CAA) accounted for 50 % of cases. The main other etiologies were reversible vasoconstriction syndrome (13 %), endocarditis (11 %), severe intracranial or cervical arterial stenoses (8 %) and cerebral venous thrombosis (5 %). Patients with CAA were older (p < 0.001) and suffered essentially from transient deficit (80 vs 18 %; p < 0.001) without headache (18 % vs 58 %; p = 0.002). Cortical hemosiderosis on MRI was strongly associated with CAA (77 % vs 0 %; p < 0.001). Follow up data were available in 55 patients (28 with CAA). Forty eight patients underwent an imaging follow up (25 with CAA). Recurrent hemorrhages (cSAH or hematoma) occurred in 52 % of CAA cases against 12 % in other origins (p = 0.01). Conclusions: Our data show that CCA is clearly the main etiology of cSAH with a specific pattern associating transient neurological deficit without headache and cortical hemosiderosis on MRI. Many origins can be found in younger patients to whom the diagnosis is guided by imaging findings and clinical background. The risk of recurrence is high in CAA contrary to other etiologies.

Abstract TP298: Acute Intracerebral Hemorrhage: Prospective Evaluation ofaA Pragmatic Stepwise Diagnostic Algorithm for Underlying Vascular Pathologies

BACKGROUND: Intracerebral hemorrhage (ICH) is one of the deadliest form of stroke, but the diagnostic work-up has only been insufficiently defined. We tested the unsefulness of a pragmatic step-wise diagnostic algorithm including CT/CTA, MRI/MRA, and digital subtraction angiography (DSA) on the detection of acutely treatment-relevant underlying pathologies. PATIENTS AND METHODS: We analyzed 114 consecutive patients (42% women, mean age 66 years +/-12) hospitalized for spontaneous ICH. All patients had initial CT with or without CTA. Those without evident underlying cause and no contraindication underwent MRI/MRA. Finally, diagnostic DSA was performed in cases without evident ICH etiology on prior imaging. RESULTS: Of the 114 ICH patients, 2 showed deep ICH with CT evidence of severe small vessel disease. In 2 cases, CTA revealed cerebral venous thrombosis. The remaining 107 (95%) patients without contraindication (n=2 had a pacemaker) underwent subsequent MRI/MRA allowing detection of underlying small vessel disease in 36 (32%) and 14 (12%) with acutely treatment relevant pathology: one cerebral venous thrombosis, six secondary hemorrhagic infarcts, three cavernous malformations, two brain tumors, and two patients had reversible acute vasoconstriction syndrome. Fifty-seven patients (50%) remained without diagnosis of any underlying pathology and underwent DSA revealing 2 dural arteriovenous fistula, 2 arteriovenous malformation, 1 moyamoya syndrome, 1 ruptured aneurysm, and 1 acute cerebral vasoconstriction syndrome. CONCLUSION: Our findings suggest the diagnostic sensitivity of brain CT alone is insufficient for the diagnostic work-up in ICH patients. A pragmatic, stepwise imaging algorithm based on additional CTA, MRI/MRA and DSA lead to the diagnosis of an underlying pathology in 52%, including the detection of acutely treatment-relevant pathologies in 19% (22 of 114 patients).

Stroke- and Pregnancy-Induced Hypertensive Syndromes

Pregnancy-induced hypertensive syndromes are an important cause of cerebrovascular diseases during pregnancy. Women with pregnancy-induced hypertensive syndromes are at an increased risk of ischemic and hemorrhagic strokes. Posterior reversible encephalopathy and reversible vasoconstriction syndromes are common. Cerebral venous sinus thrombosis may also occur. Preeclampsia and eclampsia reflect generalized endothelial dysfunction. Prompt diagnosis and identification of patients at risk allows for early therapeutic interventions and improved clinical outcomes.

Delayed angiographic changes in postpartum cerebral angiopathy

Postpartum cerebral angiopathy is a well-recognised subgroup of the reversible vasoconstriction syndromes. Increasingly described is a delay between clinical onset and angiographic changes. We report a patient who presented 19 days postpartum with severe thunderclap headaches, vertigo and a seizure. The clinical syndrome preceded evidence of extensive vasoconstriction on MRA imaging by 15 days; the changes were reversible at 3 months.

Angiopathie cérébrale aiguë réversible du post-partum : une cause particulière de céphalée aiguë du post-partum

We present the case of a 34-year-old woman who developed, in postpartum period of an uncomplicated pregnancy, a thunderclap headache with visual disturbance associated with a severe arterial hypertension. Both clinical evolution and cerebral imaging including angio-MR confirmed the diagnosis of postpartum reversible vasoconstriction syndrome. One of the leading causes of this syndrome is the use of vasoactive drugs as it was observed in the case of this patient. It is important to consider this syndrome in the differential diagnosis in patients presenting with headache in the postpartum period.

Segmental cerebral vasoconstriction: Successful treatment of secondary cerebral ischaemia with intravenous prostacyclin

We describe a 23-year-old male patient who presented with spontaneous intermittent and increasing attacks of severe, left-sided thunderclap headache combined with rapidly progressive muscle weakness and dysphasia, including gradual loss of consciousness. Subsequent CT, MRI and DSA showed progressive brain ischaemia and oedema within the left cerebral hemisphere with strict ipsilateral segmental arterial vasoconstriction. Despite extensive medical care, including steroids, the patient deteriorated rapidly. However, the clinical course changed dramatically within 15 h after the start of an intravenous infusion of prostacyclin at a dose of 0.9 ng/kg/min, with an almost complete recovery of consciousness and speech. In addition the pathophysiological alterations seen on magnetic resonance (imaging and digital) subtraction angiography including diffusion-weighted imaging and apparent diffusion coefficient maps shortly before prostacyclin treatment were clearly reduced when the patient was examined 3-4 days later and he continued to recover thereafter. Although not fully compatible, our case had several clinical characteristics and radiological findings reminiscent of those of the 'segmental reversible vasoconstriction syndrome', sometimes called the Call-Fleming syndrome.

Atraumatic convexal subarachnoid hemorrhage

To identify patterns of clinical presentation, imaging findings, and etiologies in a cohort of hospitalized patients with localized nontraumatic convexal subarachnoid hemorrhage. Twenty-nine consecutive patients with atraumatic convexal subarachnoid hemorrhage were identified using International Classification of Diseases-9 code from 460 patients with subarachnoid hemorrhage evaluated at our institution over a course of 5 years. Retrospective review of patient medical records, neuroimaging studies, and follow-up data was performed. There were 16 women and 13 men between the ages of 29 and 87 years. Two common patterns of presentations were observed. The most frequent presenting symptom in patients < or =60 years (n = 16) was a severe headache (n = 12; 75%) of abrupt onset (n = 9; 56%) with arterial narrowing on conventional angiograms in 4 patients; 10 (p = 0.003) were presumptively diagnosed with a primary vasoconstriction syndrome. Patients >60 years (n = 13) usually had temporary sensory or motor symptoms (n = 7; 54%); brain MRI scans in these patients showed evidence of leukoaraiosis and/or hemispheric microbleeds and superficial siderosis (n = 9; 69%), compatible with amyloid angiopathy (n = 10; p < 0.0001). In a small group of patients, the presentation was more varied and included lethargy, fever, and confusion. Four patients older than 60 years had recurrent intracerebral hemorrhages in the follow-up period with 2 fatalities. Convexal subarachnoid hemorrhage is an important subtype of nonaneurysmal subarachnoid bleeding with diverse etiologies, though a reversible vasoconstriction syndrome appears to be a common cause in patients 60 years or younger whereas amyloid angiopathy is frequent in patients over 60. These observations require confirmation in future studies.