Diffusion tensor imaging MR neurography in patients with acute or chronic plexopathy.

The nerve plexus is susceptible to various pathological processes. In addition to clinical and electrophysiological findings, magnetic resonance neurography (MRN) may contribute to characterize plexus involvement. Diffusion tensor imaging (DTI) was reported feasible for the nerve plexuses imaging but its value in the clinical practice remains uncertain. From 2014 to 2020, we routinely performed MRN including DTI at 3T in patients with acute or chronic plexopathy. DTI images were co-registered with conventional MRN images. MRN images including DTI were reviewed by consensus by two neuroradiologists and one neurologist. They retrospectively identified cases for whom the use of DTI had a potential impact on the diagnostic workup, seven of these clinical cases are presented here. Compared to conventional MRN, the added value of DTI consisted in: (i) improved detection of signal/morphological abnormalities of the plexus (due to removal of background structures, multiplanar reformatted views and large field of view), (ii) additional information regarding the microarchitecture of nerve fibers provided by DTI metrics, (iii) potential alternative for the use of gadolinium. This case series supports the implementation of DTI in MRN protocols.

Susceptibility Vessel Sign in Relation With Time From Onset to Magnetic Resonance Imaging.

Background and Purpose: In acute ischemic stroke, the susceptibility vessel sign (SVS) on T2* MR-sequence witnesses the red blood cell content of the clot. Although clot composition strongly depends on its age in vitro, the relationship between SVS and time has not been studied. In this study, we evaluated whether the presence of SVS was related to the time from symptom onset. Methods: We retrospectively analyzed our institutional registry of patients with acute stroke between November 2007 and June 2018. We included patients with an ischemic stroke confirmed by diffusion-weighted imaging magnetic resonance imaging within 8 hours from symptom onset caused by M1 or M2 occlusion and with interpretable T2*-weighted images. We compared clinical and imaging variables among SVS+ and SVS− patients. Time from onset was split into tertiles. Independent markers of SVS+ were identified using multivariable logistic regression. The probability of being SVS+ given time from symptoms onset was modeled using Probit regression. Results: Among the 608 patients included, 433 (71.2%) were SVS+. The odds of being SVS+ increased with time from symptom onset (P trend=0.005). In the multivariable analysis, factors independently associated with a SVS+ were symptom onset to magnetic resonance imaging ([130­180 min] odds ratio [OR], 1.62 [95% CI, 1.03­2.53]; [>180 min] OR, 3.14 [95% CI, 1.92­5.12]), type of magnetic resonance imaging-scanner (OR, 2.83 [95% CI, 1.82­4.41]), cardioembolic cause (OR, 1.51 [95% CI, 1.02­2.24]), and baseline National Institutes of Health Stroke Scale (OR, 1.05 [95% CI, 1.01­1.08]). The probability of being SVS+ increased with time from symptom onset (P=0.004): around 60% at 1 hour, 70% at 3 hours, 80% at 6 hours, and 90% at 8 hours. Conclusions: In acute ischemic stroke, the presence of SVS depends on time from onset to imaging.

Electrochemical skin conductance by Sudoscan®: a new tool to predict intradialytic hypotension.

BACKGROUND: Intradialytic hypotension (IDH), a common complication in haemodialysis (HD) patients, is associated with multiple risk factors including cardiac dysfunction and alterations of the peripheral autonomic nervous system. To what extent dysautonomia may contribute to the occurrence of IDH remains elusive. We sought to investigate the clinical utility of Sudocan®, a device that quantifies dysautonomia, in the prediction of IDH. METHODS: We conducted a prospective monocentric study in adult HD patients from July 2019 to February 2020. Dysautonomia was assessed by the measurements of hand and foot electrochemical skin conductance (ESC) using Sudocan®, before HD. The primary endpoint was the incidence of IDH (The National Kidney Foundation/Kidney-Dialysis Outcome Quality Initiative definition), according to the presence of a pathological hand and/or foot ESC value, during the 3-month study period. RESULTS: A total of 176 HD patients (64 ± 14 years old) were enrolled. Mean pre-dialysis HD hand and foot ESC was 45 ± 20 and 54 ± 22 µS, respectively. About 35% and 40% of patients had a pathological ESC at the hand and foot, respectively. IDH occurred in 46 patients. Logistic regression showed that pathologic pre-dialysis HD hand ESC was associated with an increased risk of IDH [odds ratio = 2.56, 95% CI (1.04-6.67), P = 0.04]. The cumulative risk incidence of IHD during the study was 5.65 [95% CI (2.04-15.71), P = 0.001] and 3.71 [95% CI (1.41-9.76), P = 0.008], with a pathological hand and foot ESC, respectively. CONCLUSIONS: A pathological hand ESC, as assessed by a non-invasive Sudoscan® test, is associated with an increased risk of IDH.

Patent Foramen Ovale Closure or Anticoagulation vs. Antiplatelets after Stroke.

BACKGROUND: Trials of patent foramen ovale (PFO) closure to prevent recurrent stroke have been inconclusive. We investigated whether patients with cryptogenic stroke and echocardiographic features representing risk of stroke would benefit from PFO closure or anticoagulation, as compared with antiplatelet therapy. METHODS: In a multicenter, randomized, open-label trial, we assigned, in a 1:1:1 ratio, patients 16 to 60 years of age who had had a recent stroke attributed to PFO, with an associated atrial septal aneurysm or large interatrial shunt, to transcatheter PFO closure plus long-term antiplatelet therapy (PFO closure group), antiplatelet therapy alone (antiplatelet-only group), or oral anticoagulation (anticoagulation group) (randomization group 1). Patients with contraindications to anticoagulants or to PFO closure were randomly assigned to the alternative noncontraindicated treatment or to antiplatelet therapy (randomization groups 2 and 3). The primary outcome was occurrence of stroke. The comparison of PFO closure plus antiplatelet therapy with antiplatelet therapy alone was performed with combined data from randomization groups 1 and 2, and the comparison of oral anticoagulation with antiplatelet therapy alone was performed with combined data from randomization groups 1 and 3. RESULTS: A total of 663 patients underwent randomization and were followed for a mean (±SD) of 5.3±2.0 years. In the analysis of randomization groups 1 and 2, no stroke occurred among the 238 patients in the PFO closure group, whereas stroke occurred in 14 of the 235 patients in the antiplatelet-only group (hazard ratio, 0.03; 95% confidence interval, 0 to 0.26; P<0.001). Procedural complications from PFO closure occurred in 14 patients (5.9%). The rate of atrial fibrillation was higher in the PFO closure group than in the antiplatelet-only group (4.6% vs. 0.9%, P=0.02). The number of serious adverse events did not differ significantly between the treatment groups (P=0.56). In the analysis of randomization groups 1 and 3, stroke occurred in 3 of 187 patients assigned to oral anticoagulants and in 7 of 174 patients assigned to antiplatelet therapy alone. CONCLUSIONS: Among patients who had had a recent cryptogenic stroke attributed to PFO with an associated atrial septal aneurysm or large interatrial shunt, the rate of stroke recurrence was lower among those assigned to PFO closure combined with antiplatelet therapy than among those assigned to antiplatelet therapy alone. PFO closure was associated with an increased risk of atrial fibrillation. (Funded by the French Ministry of Health; CLOSE ClinicalTrials.gov number, NCT00562289 .).

Diffusion-Weighted Imaging Hyperintensities in Subtypes of Acute Intracerebral Hemorrhage: Meta-Analysis.

Background and Purpose- Diffusion-weighted imaging (DWI) hyperintensities in intracerebral hemorrhage (ICH) are associated with increased risk of recurrent ICH, cognitive impairment, and death, but whether these lesions are specific to a subtype of ICH remains uncertain. We investigated the association between DWI lesions and ICH subtype and explored the risk factors for DWI lesions. Methods- In a systematic review of ICH studies, we identified those reporting prevalence of DWI lesions. Two reviewers independently assessed study eligibility and risk of bias and collected data. We determined the pooled prevalence of DWI lesions within 90 days after ICH onset for cerebral amyloid angiopathy- and hypertensive angiopathy-related ICH using random-effects meta-analysis. We calculated odds ratios to compare prevalence of DWI lesions by ICH subtype and to assess risk factors for DWI lesions. Results- Eleven studies (1910 patients) were included. The pooled prevalence of DWI lesions was 18.9% (95% CI, 11.1-26.7) in cerebral amyloid angiopathy- and 21.0% (95% CI, 15.3-26.6) in hypertensive angiopathy-related ICH. There was no difference in the prevalence of DWI lesions between cerebral amyloid angiopathy- (64/292 [21.9%]) and hypertensive angiopathy-related ICH (79/370 [21.4%]; odds ratio, 1.25; 95% CI, 0.73-2.15) in the 5 studies reporting data on both ICH pathogeneses. In all ICH, presence of DWI lesions was associated with neuroimaging features of microangiopathy (leukoaraiosis extension, previous ICH, and presence, and number of microbleeds) but not with vascular risk factors or the use of antithrombotic therapies. Conclusions- Prevalence of DWI lesions in acute ICH averages 20%, with no difference between cerebral amyloid angiopathy- and hypertensive angiopathy-related ICH. Detection of DWI lesions may add valuable information to assess the progression of the underlying microangiopathy.

Association of Blood Pressure During Thrombectomy for Acute Ischemic Stroke With Functional Outcome: A Systematic Review.

Background and Purpose- Optimal blood pressure (BP) targets during mechanical thrombectomy (MT) for acute ischemic stroke (AIS) are unknown, and randomized controlled trials addressing this issue are lacking. We aimed to perform a systematic review of studies evaluating the influence of periprocedural BP on functional outcome after MT. Methods- Studies assessing periprocedural BP effect on functional outcome published after January 1st, 2012 were included in the systematic review. The PRISMA checklist and flow diagram were followed for the design and reporting of this work. Results- Nine studies were included, for a total of 1037 patients. The heterogeneity in findings with respect to BP monitoring and studied parameters precluded a meta-analysis. Mean arterial pressure was the most frequently reported parameter to describe BP variability during MT, and systolic BP was the main parameter used to define periprocedural BP targets. Five studies suggested an association between 3 types of BP drops as predictors of poor functional outcome at 3 months: >40% drop in mean arterial pressure compared with baseline (odds ratio=2.8; [1.09-7.19]; P=0.032), lowest mean arterial pressure before recanalization (odds ratio=1.28; [1.01-1.62] per 10 mm Hg drop below 100 mm Hg; P=0.04), and MAP drops (odds ratio=4.38; [1.53-12.6] for drops >10%). Four studies did not show an association between BP during MT and functional outcome, including 3 studies with strict periprocedural systolic BP targets (within a 140-180 mm Hg). Conclusions- BP drops during MT may be associated with a worse functional outcome. When strict systolic BP targets are achieved, no association between BP and functional outcome was also noted. Both conclusions require further evaluation in randomized studies.

Treatment and Long-Term Outcomes of Primary Central Nervous System Vasculitis.

Background and Purpose- We aimed to analyze the long-term outcomes of patients with primary central nervous system vasculitis according to the different therapeutic strategies used to induce remission. Methods- We assessed the rate of prolonged remission (defined by the absence of relapse at ≥12 months after diagnosis) and the functional status at last follow-up in patients with primary central nervous system vasculitis included in the French cohort, who achieved a first remission according to the 3 main groups of treatments administered: glucocorticoids only (group 1); induction treatment with glucocorticoids and an immunosuppressant, but no maintenance (group 2); and combined treatment with glucocorticoids and an immunosuppressant for induction followed by maintenance therapy (group 3). Good functional status was defined as a modified Rankin Scale score ≤2 at the last follow-up. Results- Remission was achieved with the initial induction treatment in 106 (95%) of the 112. Prolonged remission without relapse was observed in 70 (66%) patients after 57 (12-198) months of follow-up. A good functional status at last follow-up (ie, modified Rankin Scale score ≤2) was observed in 63 (56%) patients. Overall mortality was 8%. The initial severity and the radiological presentations were comparable in the 3 treatment groups. More prolonged remissions ( P=0.003) and a better functional status at the last follow-up ( P=0.0004) were observed in group 3. In multivariate analysis, the use of maintenance therapy was associated with prolonged remission (odds ratio, 4.32 [1.67-12.19]; P=0.002) and better functional status (odds ratio, 8.09 [3.24-22.38]; P<0.0001). Conclusions- This study suggests that maintenance therapy with an immunosuppressant combined with glucocorticoids lead to the best long-term clinical and functional outcomes in patients with primary central nervous system vasculitis after having achieved remission with either glucocorticoids alone or in combination with another immunosuppressant.

Efficacy of Endovascular Therapy in Acute Ischemic Stroke Depends on Age and Clinical Severity.

BACKGROUND AND PURPOSE: Efficacy of endovascular treatment (EVT) for ischemic stroke because of large vessel occlusion may depend on patients' age and stroke severity; we, therefore, developed a prognosis score based on these variables and examined whether EVT efficacy differs between patients with good, intermediate, or poor prognostic score. METHODS: A total of 4079 patients with an acute ischemic stroke were identified from the Paris Stroke Consortium registry. We developed the stroke checkerboard (SC) score (SC score=1 point per decade ≥50 years of age and 2 points per 5 points on the National Institutes of Health Stroke Scale) to predict spontaneous outcome. The primary outcome was the adjusted common odds ratio for an improvement in the modified Rankin Scale at 90 days after EVT, in patients with low, intermediate, or high SC scores. To rule out potential selection biases, a nested case-control analysis, with individual matching for all major prognostic factors, was also performed, to compare patients with large vessel occlusion in the anterior circulation treated or not with EVT. RESULTS: In patients untreated with EVT, SC scores <8 were predictive of good outcomes (modified Rankin Scale score, 0-2; area under the curve, 0.87), whereas SC scores >12 were predictive of poor outcomes (modified Rankin Scale score, 4-6; area under the curve, 0.88). In the overall population, there was an interaction between EVT and prognosis group (P<0.001). EVT was associated with improved outcome in patients with SC scores >12 (common odds ratio, 1.70; 95% confidence interval, 1.13-2.56) and SC scores 8 to 12 (odds ratio, 1.37; 95% confidence interval, 1.11-1.69) but not in patients with SC scores <8 (odds ratio, 0.72; 95% confidence interval, 0.56-0.93). Similar results were obtained in the case-control analysis among 449 patients treated with EVT and 449 matched patients untreated with EVT. CONCLUSIONS: In patients stratified with the SC score, EVT was associated with improved functional outcome in older and more severe patients but not in younger and less severe patients.

Primary Angiitis of the Central Nervous System: Magnetic Resonance Imaging Spectrum of Parenchymal, Meningeal, and Vascular Lesions at Baseline.

BACKGROUND AND PURPOSE: Primary angiitis of the central nervous system remains challenging. To report an overview and pictorial review of brain magnetic resonance imaging findings in adult primary angiitis of the central nervous system and to determine the distribution of parenchymal, meningeal, and vascular lesions in a large multicentric cohort. METHODS: Adult patients from the French COVAC cohort (Cohort of Patients With Primary Vasculitis of the Central Nervous System), with biopsy or angiographically proven primary angiitis of the central nervous system and brain magnetic resonance imaging available at the time of diagnosis were included. A systematic imaging review was performed blinded to clinical data. RESULTS: Sixty patients met inclusion criteria. Mean age was 45 years (±12.9). Patients initially presented focal deficit(s) (83%), headaches (53%), cognitive disorder (40%), and seizures (38.3%). The most common magnetic resonance imaging finding observed in 42% of patients was multiterritorial, bilateral, distal acute stroke lesions after small to medium artery distribution, with a predominant carotid circulation distribution. Hemorrhagic infarctions and parenchymal hemorrhages were also frequently found in the cohort (55%). Acute convexity subarachnoid hemorrhage was found in 26% of patients and 42% demonstrated pre-eminent leptomeningeal enhancement, which is found to be significantly more prevalent in biopsy-proven patients (60% versus 28%; P=0.04). Seven patients had tumor-like presentations. Seventy-seven percent of magnetic resonance angiographic studies were abnormal, revealing proximal/distal stenoses in 57% and 61% of patients, respectively. CONCLUSIONS: Adult primary angiitis of the central nervous system is a heterogenous disease, with multiterritorial, distal, and bilateral acute stroke being the most common pattern of parenchymal lesions found on magnetic resonance imaging. Our findings suggest a higher than previously thought prevalence of hemorrhagic transformation and other hemorrhagic manifestations.

Adult primary angiitis of the central nervous system: isolated small-vessel vasculitis represents distinct disease pattern.

Objectives: We aimed to identify whether presentations and outcomes in adult patients with isolated small-vessel primary angiitis of the CNS (PACNS) would differ from other patients with large/medium-vessel involvement. Methods: In the French PACNS cohort, we compared the characteristics, treatments and outcomes of patients with isolated small-vessel disease (normal CT, MR and/or conventional angiograms, brain biopsy positive for vasculitis) with other patients who had large/medium-vessel involvement (vessel abnormalities on CT, MR or conventional angiograms). A good functional outcome was defined as a modified Rankin scale ⩽2 at last follow-up, regardless of the occurrence of relapse. Results: Among the 102 patients in the cohort, 26 (25%) had isolated small-vessel PACNS, whereas the 76 others demonstrated large/medium-vessel involvement. Patients with isolated small-vessel PACNS had more seizures (P < 0.0001), cognitive (P = 0.02) or consciousness impairment (P = 0.03) and more dyskinesias (P = 0.002) but less focal deficits (P = 0.0002) than other PACNS patients. They also had more abnormal cerebrospinal fluid analysis (P = 0.008) and gadolinium enhancements on MRI (P = 0.001) but less frequent acute ischaemic lesions (P < 0.0001) than patients with large/medium-vessel involvement. Treatments and modified Rankin scale at last follow-up did not differ between groups. Thirty-two (31%) patients relapsed; 14 (54%) with isolated small-vessel PACNS vs 18 (24%) with large/medium-vessel involvement (P = 0.004). Eight patients died, with no difference between the groups (P = 0.97). Conclusion: In our cohort, adult patients with isolated small-vessel PACNS presented some distinct disease features and relapsed more often than other PACNS patients who had large/medium-vessel involvement. Functional outcomes and mortality did not differ.

Maintenance therapy is associated with better long-term outcomes in adult patients with primary angiitis of the central nervous system.

Objective: We aimed to analyse the effect of maintenance therapy after induction on the outcomes of adult patients with primary angiitis of the CNS (PACNS). Methods: We analysed long-term outcomes (relapse, survival and functional status) of patients enrolled in the French multicentre PACNS cohort who achieved remission after induction treatment and with ⩾12 months' follow-up, according to whether or not they received maintenance therapy. Good outcome was defined as relapse-free survival and good functional status (modified Rankin scale ⩽ 2) at last follow-up. Results: Ninety-seven patients [46 (47%) female, median age: 46 (18-78) years at diagnosis] were followed up for a median of 55 (5-198) months. Induction treatment consisted of glucocorticoids in 95 (98%) patients, combined with an immunosuppressant in 80 (83%) patients, mostly CYC. Maintenance therapy was prescribed in 48 (49%) patients, following CYC in 42 of them. Maintenance therapy was started 4 (3-18) months after glucocorticoid initiation. At last follow-up, good outcomes were observed in 32 (67%) patients who had received maintenance therapy vs 10 (20%) who had not (P < 0.0001). Thirty-two (33%) patients experienced relapse [10 (22%) had received maintenance therapy while 22 (45%) had not, P = 0.01]; four subsequently died from relapse. In the multivariate analysis, maintenance therapy was the only independent predictor of good outcome [odds ratio (OR) = 7.8 (95% CI: 3.21, 20.36), P < 0.0001]. Conclusion: The results of this long-term follow-up study suggest that maintenance therapy in adults with PACNS is associated with better functional outcomes and lower relapse rates. Further studies are needed to confirm these findings.

Tumor-Like Presentation of Primary Angiitis of the Central Nervous System.

BACKGROUND AND PURPOSE: We aimed to describe the clinical and imaging features of patients with tumor-like presentation of primary angiitis of the central nervous system. METHODS: We retrospectively analyzed 10 patients enrolled in the French primary angiitis of the central nervous system cohort, who initially presented tumor-like brain lesions and compared them with other patients within the cohort. RESULTS: The 10 patients with tumor-like presentation in the cohort were younger and had more seizures at diagnosis than the other 75 patients (median of 37 [30-48] years versus 46 [18-79] years; P=0.008; 9 [90%] with seizures versus 22 [29%], P<0.001; respectively). All 10 patients had a biopsy (stereotactic procedure in 7 and open-wedge surgery in 3). Histological findings suggestive of vasculitis were observed in 9 patients in whom conventional cerebral angiography and magnetic resonance angiography were negative. In the remaining patient, vascular imaging demonstrated diffuse bilateral large- and medium-sized vessel involvement (biopsy did not reveal vasculitis). All patients with tumor-like presentation received glucocorticoids, combined with cyclophosphamide in 9 cases. With a median follow-up of 27 (12-130) months, 5 (50%) patients relapsed, but achieved remission again after treatment intensification. CONCLUSIONS: Patients with tumor-like presentation of primary angiitis of the central nervous system represent a subgroup characterized with mainly small-sized vessel disease that requires histological confirmation because vascular imaging is often normal. Although relapses are not uncommon, global outcomes are good under treatment with glucocorticoids and cyclophosphamide.

Changes in Activated Thrombin-Activatable Fibrinolysis Inhibitor Levels Following Thrombolytic Therapy in Ischemic Stroke Patients Correlate with Clinical Outcome.

BACKGROUND AND PURPOSE: Thrombin-activatable fibrinolysis inhibitor (TAFI) activation following thrombolysis may affect thrombolysis effectiveness in acute ischemic stroke (AIS). To support this hypothesis, we propose to study the relationship between TAFI consumption, activated/inactivated TAFI (TAFIa/ai) and stroke severity and outcome in 2 groups of AIS patients, one treated and one untreated with intravenous recombinant tissue type plasminogen activator (rt-PA). METHODS: In this prospective, longitudinal, multicenter, observational study, we aimed to study the association between TAFIa/ai and stroke outcome. TAFI levels were sequentially measured in patients treated with intravenous rt-PA thrombolysis (T), and in patients not given any thrombolytic therapy (NT). Baseline reference values were established in healthy subjects matched for age and gender. The National Institutes of Health Stroke Scale (NIHSS) score assessed at baseline and on day 2 was dichotomized into 2 severity groups (0-7 vs. >7). The modified Rankin Scale (mRS) score at day 90 was dichotomized for favorable (0-1) and unfavorable (2-6) outcomes. RESULTS: A total of 109 patients were included, with 41 receiving rt-PA. At admission, patients had higher TAFIa/ai levels than reference. A significant increase in TAFIa/ai levels was observed at the end of thrombolysis (mean change from baseline of 963%) and lasted up to 4 h (191%). Higher TAFIa/ai levels were associated with a more severe day 2 NIHSS score (p = 0.0098 at T2h post thrombolysis) and an unfavorable mRS score from T48h (p = 0.0417) to day 90 (p = 0.0046). In NT patients, higher TAFIa/ai levels at admission were associated with a more severe stroke, as assessed by day 2 NIHSS score (p = 0.0026) and mRS score (p = 0.0003). CONCLUSION: These data demonstrate a consistent relationship between TAFI levels and early clinical severity during rt-PA treatment.

Comparison of 3D multi-echo gradient-echo and 2D T2* MR sequences for the detection of arterial thrombus in patients with acute stroke.

OBJECTIVES: We compared a multi-echo gradient-echo magnetic resonance sequence (susceptibility-weighted angiography [SWAN]) with the T2* sequence for the detection of an arterial thrombus in acute ischaemic stroke. METHODS: Seventy-four consecutive patients with acute ischaemic stroke were included. Proximal arterial occlusions were diagnosed using time-of-flight (TOF) magnetic resonance angiography (MRA). Two-dimensional (2D) axial reformats from 3D SWAN were generated to match with 2D T2* images. For arterial thrombus detection, each set of MR images (T2*, 2D SWAN reformats and 3D multiplanar SWAN images) was examined independently and separately by three observers who assigned the images to one of three categories: (0) absence of thrombus, (1) uncertain thrombus, (2) certain thrombus. Agreement and diagnostic accuracy were calculated. RESULTS: Twenty-four proximal arterial occlusions involving the anterior (n = 20) or posterior (n = 4) circulation were found. Inter-observer agreement was moderate using T2* images (κ = 0.58), good using 2D SWAN reformats (κ = 0.83) and excellent using multiplanar SWAN images (κ = 0.90). For the diagnosis of thrombus, T2* images were 54% sensitive and 86% specific, 2D SWAN reformats were 83% sensitive and 94% specific and SWAN multiplanar analysis was 96% sensitive and 100% specific. CONCLUSIONS: Three-dimensional SWAN sequence improves the detection of arterial thrombus in patients with acute ischaemic stroke in comparison with the 2D T2* sequence. KEY POINTS: • Multi-echo gradient-echo MR (e.g. susceptibility-weighted angiograph, [SWAN]) is increasingly used in neuroradiology. • Compared with conventional T2* sequences, SWAN improves detection of arterial thrombus. • Multiplanar SWAN analysis had the best diagnostic performance for arterial thrombus detection. • Sensitivity was 96% and specificity 100%. • Findings support combination of time-of-flight and susceptibility effects in suspected acute stroke.

Aseptic meningitis and Fabry disease.

OBJECTIVE: Fabry disease is caused by enzymatic defects in alpha-galactosidase A that leads to the accumulation of glycosphingolipids throughout the body, resulting in a multisystemic disorder. The most common neurological manifestations are neuropathic pain, autonomic nervous system dysfunction and strokes, but some rarer neurological manifestations exist. Among these, aseptic meningitis is a possible complication. Our objectives were to measure the prevalence of this complication in a cohort of patients with Fabry disease, and to describe its clinical features. METHODS: We conducted a retrospective review of Fabry disease patients followed at our tertiary referral center between 1995 and September 2023 with at least one episode of meningitis, and performed a systematic review to identify similar published cases. RESULTS: Four patients out of 107 (3.7%) had at least one episode of aseptic meningitis. Our systematic review identified 25 other observations. The median age of these 29 patients was 29.0 years, the median cerebrospinal fluid leukocyte count was 24 cells/mm3 with a predominance of lymphocytes in 64.7% of cases. In 82.8% of the patients, the diagnosis of Fabry disease was unknown before the meningitis. Large artery stenosis was present in 17.2% of patients and 57.1% of patients had a recent stroke concomitant with the meningitis. Several differential diagnoses were evoked, such as multiple sclerosis or central nervous system vasculitis. INTERPRETATION: Our study suggests that Fabry disease should be considered as a cause of aseptic meningitis. The pathophysiological mechanisms underlying meningeal inflammation remain largely unknown but may reflect the dysregulation of pro-inflammatory signaling pathways.

Fluid-attenuated inversion recovery vascular hyperintensities are not visible using 3D CUBE FLAIR sequence.

OBJECTIVE: Fluid-attenuated inversion recovery (FLAIR) vascular hyperintensities (FVH), initially described on 2D FLAIR images, are a useful imaging marker in patients with acute ischaemic stroke. We aimed to compare the sensitivity of the 3D CUBE FLAIR sequence with 2D FLAIR for the detection of FVH. METHODS: Forty-seven consecutive patients admitted for a suspected stroke were explored by 2D and 3D CUBE FLAIR MR sequences at 1.5 and 3 T. Three blinded readers assessed FVH defined as hyperintensities within cerebral arteries. Location of FVH, acute brain infarct and arterial stenosis were also assessed. 2D images were compared with 3D images for the detection of FVH. Agreement between readers was assessed. RESULTS: Of the 47 patients, 21 FVHs were observed on 2D FLAIR images of 15 patients (11 with acute brain infarct and 11 with an arterial stenosis). No FVH was visualised on 3D CUBE FLAIR images for either proximal or distal locations. Agreement between readers was excellent. CONCLUSION: FVHs are not visible using 3D CUBE FLAIR images. This study suggests that, in suspected acute ischaemic stroke, the assessment of FVH should only be performed on conventional 2D FLAIR images. KEY POINTS: • Fluid-attenuated inversion recovery (FLAIR) vascular hyperintensities (FVH) are of neuroradiological importance. • FVHs are useful imaging markers in patients with an acute ischaemic stroke. • FVHs are not visible using 3D CUBE FLAIR images. • Assessment of FVH should be performed on conventional 2D FLAIR images.

Susceptibility-weighted angiography for the detection of high-flow intracranial vascular lesions: preliminary study.

OBJECTIVES: Susceptibility-weighted magnetic resonance imaging (MRI) sequences may demonstrate various signal intensities of draining veins in cases of high-flow vascular malformation (HFVM), including arteriovenous malformation (AVM) and dural arteriovenous fistula (dAVF). Our objective was to evaluate susceptibility-weighted angiography (SWAN) for the detection of HFVM. METHODS: Fifty-eight consecutive patients with a suspected intracranial vascular malformation were explored with SWAN and post-contrast MRI sequences at 3 T. The diagnosis of slow-flow vascular malformation (SFVM), including developmental venous anomaly (DVA) or brain capillary telangiectasia (BCT), was based on MRI. Patients with suspected HFVM underwent digital subtraction angiography (DSA). SWAN images were analysed by three blinded readers according to a three-point scale of the venous signal. RESULTS: Thirty-one patients presented 35 SFVM (26 DVA and 9 BCT) that systematically appeared hypointense on SWAN images. In patients with atypical MRI findings, DSA revealed one patient with an atypical DVA and 26 patients with HFVM (22 AVM and 4 dAVF). SWAN revealed at least one venous hyperintensity in all patients with HFVM. Agreement between readers was excellent. CONCLUSIONS: SWAN appears reliable for characterising blood flow dynamics in brain veins. In clinical practice, SWAN can routinely rule out HFVM in patients with atypical brain veins.

C-reactive protein and D-dimer in cerebral vein thrombosis: Relation to clinical and imaging characteristics as well as outcomes in a French cohort study.

Introduction: Cerebral venous sinus thrombosis (CVST) is a rare disease with highly variable clinical presentation and outcomes. Clinical studies suggest a role of inflammation and coagulation in CVST outcomes. The aim of this study was to investigate the association of inflammation and hypercoagulability biomarkers with CVST clinical manifestations and prognosis. Methods: This prospective multicenter study was conducted from July 2011 to September 2016. Consecutive patients referred to 21 French stroke units and who had a diagnosis of symptomatic CVST were included. High-sensitivity C-reactive protein (hs-CRP), neutrophil-to-lymphocyte ratio (NLR), D-dimer, and thrombin generation using calibrated automated thrombogram system were measured at different time points until 1 month after anticoagulant therapy discontinuation. Results: Two hundred thirty-one patients were included. Eight patients died, of whom 5 during hospitalization. The day 0 hs-CRP levels, NLR, and D-dimer were higher in patients with initial consciousness disturbance than in those without (hs-CRP: 10.2 mg/L [3.6-25.5] vs 23.7 mg/L [4.8-60.0], respectively; NLR: 3.51 [2.15-5.88] vs 4.78 [3.10-9.59], respectively; D-dimer: 950 µg/L [520-2075] vs 1220 µg/L [950-2445], respectively). Patients with ischemic parenchymal lesions (n = 31) had a higher endogenous thrombin potential5pM than those with hemorrhagic parenchymal lesions (n = 31): 2025 nM min (1646-2441) vs 1629 nM min (1371-2090), respectively (P = .0082). Using unadjusted logistic regression with values >75th percentile, day 0 hs-CRP levels of >29.7 mg/L (odds ratio, 10.76 [1.55-140.4]; P = .037) and day 5 D-dimer levels of >1060 mg/L (odds ratio, 14.63 [2.28-179.9]; P = .010) were associated with death occurrence. Conclusion: Two widely available biomarkers measured upon admission, especially hs-CRP, could help predict bad prognosis in CVST in addition to patient characteristics. These results need to be validated in other cohorts.

Neurological complications of varicella zoster virus reactivation: Prognosis, diagnosis, and treatment of 72 patients with positive PCR in the cerebrospinal fluid.

BACKGROUND: VZV infection can involve every level of the neurologic system: from the central nervous system (CNS) to the peripheral nervous system (PNS), including aseptic meningitis. Prognosis seems to differ between these neurological involvements. Prognostic factors remain unknown. METHODS: This is a retrospective multicenter study including all patients with a positive VZV polymerase chain reaction (PCR) in the cerebrospinal fluid (CSF) from eight centers in Paris (France) between 2011 and 2018. Unfavorable outcome was defined as mortality linked to VZV or incomplete recovery. Modified Rankin Scale (mRS) evaluated disability before and after the infection, with the difference designated as Rankin Delta. RESULTS: Seventy-two patients were included (53% male, median age 51 years, median mRS 0). Immunosuppression was reported in 42%. The clinical spectrum included 26 cases of meningitis, 27 instances of CNS involvement, 16 of PNS involvement, and 3 isolated replications (positive PCR but no criteria for neurological complications from VZV). Antiviral treatment was administered to 69 patients (96%). Sixty-two patients completed follow-up. Death linked to VZV occurred in eight cases. Unfavorable outcome (UO) occurred in 60% and was significantly associated with a higher prior mRS (Odd-ratio (OR) 3.1 [1.4-8.8] p = .012) and the presence of PNS or CNS manifestations (OR 22 [4-181] p = .001, OR 6.2 [1.3-33] p = .03, respectively, compared to meningitis). In the CSF, higher protein level (p < .0001) was also significantly associated with a higher Rankin Delta. CONCLUSIONS: Neurological complications of VZV with evidence of CSF viral replication are heterogeneous: aseptic meningitis has a good prognosis, whereas presence of CNS and PNS involvement is associated with a higher risk of mortality and of sequelae, respectively.

[Primary vasculitis of central nervous system]. / Vascularites primitives du système nerveux central.

"Primary vasculitis of central nervous system.Primary vasculitis of central nervous system (PVCNS) is an uncommon condition and diagnosis with an acceptable degree of certainty remains a difficult challenge. Parietal inflammation of the cerebral vessels appears in the absence of systemic disease. Publication of large cohorts of well-explored patients in USA and France over the 2 past decades significantly increased the experience. Headache is the most frequent symptom, generally associated with stroke or encephalopathy. Brain imaging including MRI and vascular imaging (MRA/CT angiography + conventional angiography) is now the cornerstone of diagnosis and cerebral biopsy seldom performed. Favorable prognostic factors have been identified in PVCNS, i.e. distal vessel involvement, no/very few ischemic events and meningeal enhancements after gadolinium injection. When these are present, prednisone is usually the only treatment proposed. Otherwise, cyclophosphamide is added to prednisone for 4 to 6 months, and maintenance immunosuppressive therapy is prescribed. Mortality of PVCNS is now 5-10% at 5 years, thanks to modern and rational medical care."

"Vascularites primitives du système nerveux central.Les vascularites primitives du système nerveux central représentent une entité rare et hétérogène de diagnostic difficile. L'atteinte inflammatoire de la paroi des vaisseaux cérébraux se développe en l'absence de toute affection systémique. Les céphalées dominent le tableau, associées à la survenue d'accidents vasculaires cérébraux (AVC) ou d'une encéphalopathie. Le diagnostic repose essentiellement sur l'imagerie cérébrale (imagerie par résonance magnétique [IRM] et imagerie vasculaire ­ ARM/angioscanner + angiographie conventionnelle), la biopsie cérébrale n'étant qu'exceptionnellement réalisée. Des facteurs de pronostic favorable ont été identifiés tels que l'atteinte des petits vaisseaux distaux, un nombre limité d'AVC et des prises de contraste méningées à l'IRM. La présence de ces facteurs fait envisager un traitement par corticothérapie seule. Dans le cas contraire, le cyclophosphamide est associé pendant 4 à 6 mois, avant un traitement immunosuppresseur d'entretien prolongé. La mortalité des patients avec vascularite primitive du système nerveux central ne dépasse pas aujourd'hui 5 à 10 % à 5 ans, grâce aux progrès récents de la prise en charge."