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 ± 13 years; 78% of patients were women. Persistent clinical worsening occurred in 14% at 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.
Reversible segmental narrowing of the intracranial arteries has been described since several decades in numerous clinical settings, using variable nosology. Twenty-one years ago, we tentatively ...proposed the unifying concept that these entities, based on similar clinical-imaging features, represented a single cerebrovascular syndrome. This “reversible cerebral vasoconstriction syndrome” or RCVS has now come of age. A new International Classification of Diseases code, (ICD-10, I67.841) has been established, enabling larger-scale studies. The RCVS2 scoring system provides high accuracy in confirming RCVS diagnosis and excluding mimics such as primary angiitis of the central nervous system. Several groups have characterized its clinical-imaging features. RCVS predominantly affects women. Recurrent worst-ever (thunderclap) headaches are typical at onset. While initial brain imaging is often normal, approximately one-third to half develop complications such as convexity subarachnoid hemorrhages, lobar hemorrhages, ischemic strokes located in arterial “watershed” territories and reversible edema, alone or in combination. Vasoconstriction evolves over hours to days, first affecting distal and then the more proximal arteries. An overlap between RCVS and primary thunderclap headache, posterior reversible encephalopathy syndrome, Takotsubo cardiomyopathy, transient global amnesia, and other conditions has been recognized. The pathophysiology remains largely unknown. Management is mostly symptomatic: headache relief with analgesics and oral calcium-channel blockers, removal of vasoconstrictive factors, and avoidance of glucocorticoids that can significantly worsen outcome. Intra-arterial vasodilator infusions provide variable success. Overall, 90–95% of admitted patients achieve complete or major resolution of symptoms and clinical deficits within days to weeks. Recurrence is exceptional, although 5% can later develop isolated thunderclap headaches with or without mild cerebral vasoconstriction.
Reversible cerebral vasoconstriction syndromes (RCVS) and primary angiitis of the central nervous system (PACNS) are invariably considered in the differential diagnosis of new cerebral ...arteriopathies. However, prompt and accurate diagnosis remains challenging. Here we compared the features of 159 RCVS to 47 PACNS patients and developed criteria for prompt bedside diagnosis. Recurrent thunderclap headache (TCH), and single TCH combined with either normal neuroimaging, border zone infarcts, or vasogenic edema, have 100% positive predictive value for diagnosing RCVS or RCVS‐spectrum disorders. In patients without TCH and positive angiography, neuroimaging can discriminate RCVS (no lesion) from PACNS (deep/brainstem infarcts). Ann Neurol 2016;79:882–894
OBJECTIVETo develop a method to distinguish reversible cerebral vasoconstriction syndrome (RCVS) from other large/medium-vessel intracranial arteriopathies.
METHODSWe identified consecutive patients ...from our institutional databases admitted in 2013–2017 with newly diagnosed RCVS (n = 30) or non-RCVS arteriopathy (n = 80). Admission clinical and imaging features were compared. Multivariate logistic regression modeling was used to develop a discriminatory score. Score validity was tested in a separate cohort of patients with RCVS and its closest mimic, primary angiitis of the CNS (PACNS). In addition, key variables were used to develop a bedside approach to distinguish RCVS from non-RCVS arteriopathies.
RESULTSThe RCVS group had significantly more women, vasoconstrictive triggers, thunderclap headaches, normal brain imaging results, and better outcomes. Beta coefficients from the multivariate regression model yielding the best c-statistic (0.989) were used to develop the RCVS2 score (range −2 to +10; recurrent/single thunderclap headache; carotid artery involvement; vasoconstrictive trigger; sex; subarachnoid hemorrhage). Score ≥5 had 99% specificity and 90% sensitivity for diagnosing RCVS, and score ≤2 had 100% specificity and 85% sensitivity for excluding RCVS. Scores 3–4 had 86% specificity and 10% sensitivity for diagnosing RCVS. The score showed similar performance to distinguish RCVS from PACNS in the validation cohort. A clinical approach based on recurrent thunderclap headaches, trigger and normal brain scans, or convexity subarachnoid hemorrhage correctly diagnosed 25 of 37 patients with RCVS2 scores 3–4 across the derivation and validation cohorts.
CONCLUSIONRCVS can be accurately distinguished from other intracranial arteriopathies upon admission, using widely available clinical and imaging features.
CLASSIFICATION OF EVIDENCEThis study provides Class II evidence that the RCVS2 score accurately distinguishes patients with RCVS from those with other intracranial arteriopathies.
Summary Background In animal models of acute ischaemic stroke, blocking of the leukocyte-endothelium adhesion by antagonism of α4 integrin reduces infarct volumes and improves outcomes. We assessed ...the effect of one dose of natalizumab, an antibody against the leukocyte adhesion molecule α4 integrin, in patients with acute ischaemic stroke. Methods In this double-blind, phase 2 study, patients with acute ischaemic stroke (aged 18–85 years) from 30 US and European clinical sites were randomly assigned (1:1) to 300 mg intravenous natalizumab or placebo with stratification by treatment window and baseline infarct size. Patients, investigators, and study staff were masked to treatment assignments. The primary endpoint was the change in infarct volume from baseline to day 5 and was assessed in the modified intention-to-treat population. Secondary endpoints were the change in infarct volume from baseline to day 30, and from 24 h to days 5 and 30; the National Institute of Health Stroke Scale (NIHSS) at baseline, 24 h, and at days 5 (or discharge), 30, and 90; and modified Rankin Scale (mRS) and Barthel Index (BI) at days 5 (or discharge), 30, and 90. This trial is registered with ClinicalTrials.gov , number NCT01955707. Findings Between Dec 16, 2013, and April 9, 2015, 161 patients were randomly assigned to natalizumab (n=79) or placebo (n=82). Natalizumab did not reduce infarct volume growth from baseline to day 5 compared with placebo (median absolute growth 28 mL range −8 to 303 vs 22 mL −11 to 328; relative growth ratio 1·09 90% CI 0·91–1·30, p=0·78) or to day 30 (4 mL −43 to 121 vs 4 mL −28 to 180; 1·05 0·88–1·27, p=0·68), from 24 h to day 5 (8 mL −30 to 177 vs 7 mL −13 to 204; 1·00 0·89–1·12, p=0·49), and from 24 h to day 30 (−5 mL −93 to 81 vs −5 mL −48 to 48; 0·98 0·87–1·11, p=0·40). No difference was noted between the natalizumab and placebo groups in the NIHSS (score ≤1 or ≥8 point improvement) from baseline at 24 h, day 5 (or discharge), day 30 (27 35% vs 36 44%; odds ratio 0·69 90% CI 0·39–1·21, p=0·86), and day 90 (36 47% vs 37 46%; 1·10 0·63–1·93, p=0·39). More patients in the natalizumab group than in the placebo group had mRS scores of 0 or 1 at day 30 (13 18% vs seven 9%; odds ratio 2·88 90% CI 1·20–6·93, p=0·024) and day 90 (18 25% vs 16 21%; 1·48 0·74–2·98, p=0·18); and BI (score ≥95) at day 90 (34 44% vs 26 33%; 1·91 1·07–3·41, p=0·033) but not significantly at day 5 or day 30 (26 34% vs 26 32%; 1·13 0·63–2·00, p=0·37). Natalizumab and placebo groups had similar incidences of adverse events (77 99% of 78 patients vs 81 99% of 82 patients), serious adverse events (36 46% vs 38 46%), and deaths (14 18% vs 13 16%). Two patients in the natalizumab group died because of adverse events assessed as related to treatment by the investigator (pneumonia, and septic shock and multiorgan failure). Interpretation Natalizumab administered up to 9 h after stroke onset did not reduce infarct growth. Treatment-associated benefits on functional outcomes might warrant further investigation. Funding Biogen.
Approximately 10% to 14% of ischemic strokes occur in young adults.
To investigate the yield of diagnostic tests, neuroimaging findings, and treatment of ischemic strokes in young adults.
We ...retrospectively reviewed data from our Get with the Guidelines-Stroke database from 2005 through 2010.
University hospital tertiary stroke center.
A total of 215 consecutive inpatients aged 18 to 45 years with ischemic stroke/transient ischemic attack. The mean (SD) age was 37.5 (7) years; 51% were male.
There were high incidence rates of hypertension (20%), diabetes mellitus (11%), dyslipidemia (38%), and smoking (34%). Relevant abnormalities were shown on cerebral angiography in 136 of 203 patients, on cardiac ultrasonography in 100 of 195, on Holter monitoring in 2 of 192; and on hypercoagulable panel in 30 of 189 patients. Multiple infarcts were observed in 31% and were more prevalent in individuals younger than age 35 years. Relevant arterial lesions were frequently detected in the middle cerebral artery (23%), internal carotid artery (13%), and vertebrobasilar arteries (13%). Cardioembolic stroke occurred in 47% (including 17% with isolated patent foramen ovale), and 11% had undetermined stroke etiology. The median National Institutes of Health Stroke Scale score was 3 (interquartile range, 0-9) and 81% had good outcome at hospital discharge. Of the 29 patients receiving thrombolysis (median National Institutes of Health Stroke Scale score, 14; interquartile range, 9-17), 55% had good outcome at hospital discharge and none developed symptomatic brain hemorrhage.
This study shows the contemporary profile of ischemic stroke in young adults admitted to a tertiary stroke center. Stroke etiology can be determined in nearly 90% of patients with modern diagnostic tests. The causes are heterogeneous; however, young adults have a high rate of traditional vascular risk factors. Thrombolysis appears safe and short-term outcomes are favorable.
BACKGROUND AND PURPOSE—To compare hemorrhagic and nonhemorrhagic reversible cerebral vasoconstriction syndromes (RCVS) with a view to understand mechanisms.
METHODS—This single-center retrospective ...study included 162 patients with RCVS. Clinical, brain imaging, and angiography data were analyzed.
RESULTS—The mean age was 44±13 years, 78% women. Hemorrhages occurred in 43% including 21 patients with intracerebral hemorrhage (ICH) and 62 with convexal subarachnoid hemorrhage (cSAH). The frequency of triggers (eg, vasoconstrictive drugs) and risk factors (eg, migraine) were not significantly different between hemorrhagic and nonhemorrhagic RCVS or between subgroups (ICH versus non-ICH, isolated cSAH versus normal scan). Hemorrhagic lesions occurred within the first week, whereas infarcts and vasogenic edema accumulated during 2 to 3 weeks (P<0.001). Although all ICHs occurred before cSAH, their time course was not significantly different (P=0.11). ICH and cSAH occurred earlier than infarcts (P≤0.001), and ICH earlier than vasogenic edema (P=0.009). Angiogram analysis showed more severe vasoconstriction in distal versus proximal segments in all lesion types (ICH, cSAH, infarction, vasogenic edema, and normal scan). The isolated infarction group had more severe proximal vasoconstriction, and those with normal imaging had significantly less vasoconstriction. Multivariable analysis failed to uncover independent predictors of hemorrhagic RCVS; however, female sex predicted ICH (P=0.048), and angiographic severity predicted infarction (P=0.043).
CONCLUSIONS—ICH and cSAH are common complications of RCVS. Triggers and risk factors do not predict lesion subtype but may alter central vasomotor control mechanisms resulting in centripetal angiographic evolution. Early distal vasoconstriction is associated with lobar ICH and cSAH, and delayed proximal vasoconstriction with infarction.
A 64-year-old woman was evaluated because of a sudden onset of the worst headache of her life. Computed tomography of the head revealed a convexal subarachnoid hemorrhage. A diagnosis was made.
Endovascular thrombectomy (EVT) has revolutionized large vessel occlusion (LVO) stroke management, but often requires advanced imaging. The collateral pattern on CT angiograms may be an alternative ...because a symmetric collateral pattern correlates with a slowly growing, small ischemic core. We tested the hypothesis that such patients will have favorable outcomes after EVT. Consecutive patients (n = 74) with anterior LVOs who underwent EVT were retrospectively analyzed. Inclusion criteria were available CTA and 90-day modified Rankin Scale (mRS). CTA collateral patterns were symmetric in 36%, malignant in 24%, or other in 39%. Median NIHSS was 11 for symmetric, 18 for malignant, and 19 for other (p = 0.02). Ninety-day mRS ≤2, indicating independent living, was achieved in 67% of symmetric, 17% of malignant, and 38% of other patterns (p = 0.003). A symmetric collateral pattern was a significant determinant of 90-day mRS ≤2 (aOR = 6.62, 95%CI = 2.24,19.53; p = 0.001) in a multivariable model that included age, NIHSS, baseline mRS, thrombolysis, LVO location, and successful reperfusion. We conclude that a symmetric collateral pattern predicts favorable outcomes after EVT for LVO stroke. Because the pattern also marks slow ischemic core growth, patients with symmetric collaterals may be suitable for transfer for thrombectomy. A malignant collateral pattern is associated with poor clinical outcomes.
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DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK