VEXAS syndrome: expanding the clinical and molecular spectrum

International audienc

Bypass grafting to the third segment of the vertebral artery for symptomatic extensive vertebrobasilar atherosclerotic disease

Symptomatic vertebrobasilar atherosclerotic disease is rarely encountered but represents a high-risk factor for recurrent transient ischemic attack or stroke. Posterior strokes are usually associated with embolism or hemodynamic impairment. Extensive disease involving the V3 and V4 segments of the vertebral artery (VA) remains infrequent, and optimal management is limited owing to the infrequency of this disease. We illustrate the case of a 65-year-old man who presented with recurrent transient episodes of dizziness with acute onset of instability, nausea, and left visual blurring. Magnetic resonance imaging findings of the head were normal, and computed tomography angiography revealed severe atherosclerotic disease of both VAs, with proximal occlusion of the right VA and multiple tight stenoses of the left VA at the V1 and V4 segments. Duplex ultrasound found markedly reduced anterograde flow in the VAs and basilar arteries and nonsignificant stenosis of the internal carotid arteries. Optimal medical treatment led to a decrease of transient symptoms. However, the patient developed a cerebellar infarction in the left posteroinferior cerebellar artery territory with left VA V4 segment occlusion. Surgical revascularization of the right VA was decided by the multidisciplinary team. Through an anterolateral approach of the right VA V3 segment, revascularization was performed using a common carotid artery-to-right VA bypass using a reversed saphenous vein graft. The patient fully recovered and was free of symptoms during the next 14 months of follow-up. In the case of extensive VA occlusive disease, surgical reconstruction of the distal VA using a bypass from the common carotid artery represents an option to improve hemodynamics and/or eliminate an embolic source of posterior stroke on a case-by-case basis

Covid-19 and dysregulated cerebral perfusion: observations with multimodal MRI

SummaryThe pathogenesis of encephalopathy-associated Covid-19 is still unclear. Multimodal brain MRI in 25 Covid-19 patients with neurological symptoms revealed angiographic and brain perfusion changes suggesting an under-recognized dysregulated brain perfusion not identified by morphological neuroimaging alone. Endothelial dysfunction, a key pathomechanism of dysregulated brain perfusion, may contribute to central-nervous-system disturbances in Covid-19.</jats:p

Cerebrovascular complications and outcomes of critically ill adult patients with infective endocarditis

Abstract Background Neurological complications are associated with poor outcome in patients with infective endocarditis (IE). Although guidelines recommend systematic brain imaging in the evaluation of IE patients, the association between early brain imaging findings and outcomes has never been evaluated in critically ill patients. We aimed to assess the association of CT-defined neurological complications with functional outcomes of critically ill IE patients. Methods This retrospective cohort study included consecutive patients with severe, left-sided IE hospitalized in the medical ICU of a tertiary care hospital. Patients with no baseline brain CT were excluded. Baseline CT-scans were classified in five mutually exclusive categories (normal, moderate-to-severe ischemic stroke, minor ischemic stroke, intracranial hemorrhage, other abnormal CT). The primary endpoint was 1-year favorable outcome, defined by a modified Rankin Scale score of 0–3. Results Between 06/01/2011 and 07/31/2018, 156 patients were included. Among them, 87/156 (56%) had a CT-defined neurological complication, including moderate-to-severe ischemic stroke (n = 33/156, 21%), intracranial hemorrhage (n = 24/156, 15%), minor ischemic stroke (n = 29/156, 19%), other (n = 3/156, 2%). At one year, 69 (45%) patients had a favorable outcome. Factors negatively associated with favorable outcome in multivariable analysis were moderate-to-severe ischemic stroke (OR 0.37, 95%CI 0.14 − 0.95) and age (OR 0.94, 95%CI 0.91–0.97). By contrast, the score on the Glasgow Coma Scale was positively associated with favorable outcome (per 1-point increment, OR 1.23, 95%CI 1.08–1.42). Sensitivity analyses conducted in operated patients revealed similar findings. Compared to normal CT, only moderate-to-severe ischemic stroke was associated with more frequent post-operative neurological complications (n = 8/23 (35%) vs n = 1/46 (2%), p &lt; 0.01). Conclusion Moderate-to-severe ischemic stroke had an independent negative impact on 1-year functional outcome in critically ill IE patients; whereas other complications, including intracranial hemorrhage, had no such impact. </jats:sec

Cerebrovascular complications and outcomes of critically ill adult patients with infective endocarditis

Abstract Background Neurological complications are associated with poor outcome in patients with infective endocarditis (IE). Although guidelines recommend systematic brain imaging in the evaluation of IE patients, the association between early brain imaging findings and outcomes has never been evaluated in critically ill patients. We aimed to assess the association of CT-defined neurological complications with functional outcomes of critically ill IE patients. Methods This retrospective cohort study included consecutive patients with severe, left-sided IE hospitalized in the medical ICU of a tertiary care hospital. Patients with no baseline brain CT were excluded. Baseline CT-scans were classified in five mutually exclusive categories (normal, moderate-to-severe ischemic stroke, minor ischemic stroke, intracranial hemorrhage, other abnormal CT). The primary endpoint was 1-year favorable outcome, defined by a modified Rankin Scale score of 0–3. Results Between 06/01/2011 and 07/31/2018, 156 patients were included. Among them, 87/156 (56%) had a CT-defined neurological complication, including moderate-to-severe ischemic stroke (n = 33/156, 21%), intracranial hemorrhage (n = 24/156, 15%), minor ischemic stroke (n = 29/156, 19%), other (n = 3/156, 2%). At one year, 69 (45%) patients had a favorable outcome. Factors negatively associated with favorable outcome in multivariable analysis were moderate-to-severe ischemic stroke (OR 0.37, 95%CI 0.14 − 0.95) and age (OR 0.94, 95%CI 0.91–0.97). By contrast, the score on the Glasgow Coma Scale was positively associated with favorable outcome (per 1-point increment, OR 1.23, 95%CI 1.08–1.42). Sensitivity analyses conducted in operated patients revealed similar findings. Compared to normal CT, only moderate-to-severe ischemic stroke was associated with more frequent post-operative neurological complications (n = 8/23 (35%) vs n = 1/46 (2%), p < 0.01). Conclusion Moderate-to-severe ischemic stroke had an independent negative impact on 1-year functional outcome in critically ill IE patients; whereas other complications, including intracranial hemorrhage, had no such impact

Additional file 5 of Cerebrovascular complications and outcomes of critically ill adult patients with infective endocarditis

Additional file 5: Figure S1. Details of baseline brain CT classification IS= Ischemic stroke HT=hemorrhagic transformation of an ischemic strok

Additional file 3 of Cerebrovascular complications and outcomes of critically ill adult patients with infective endocarditis

Additional file 3: Table S3. Multivariate analysis of factors associated with favorable outcome (mRS 0-3) in the whole cohort at one year for brain CT categories others than “moderate-to-severe ischemic stroke”

Additional file 4 of Cerebrovascular complications and outcomes of critically ill adult patients with infective endocarditis

Additional file 4: Table S4. Sensitivity analysis including the variable “severe regurgitation” of factors associated with favorable functional outcome (mRS 0-3) at one year

Additional file 6 of Cerebrovascular complications and outcomes of critically ill adult patients with infective endocarditis

Additional file 6: Figure S2. Brain CT of four different patients from mentioned groups A. minor stroke of a distal branch of the left middle cerebral artery (ASPECTS 9/10, minor ischemic stroke group), B. large stroke involving the right middle cerebral artery with midline shift due to edema and mass effect (ASPECTS 3/10, moderate-to-severe ischemic stroke), C. intraparenchymal in left centrum semiovale with swirl sign (cerebral hemorrhage group) and D. hemorrhagic transformation of a left posterior cerebral artery stroke (also cerebral hemorrhage group)

Additional file 2 of Cerebrovascular complications and outcomes of critically ill adult patients with infective endocarditis

Additional file 2: Table S2. Univariable analysis of baseline characteristics associated with outcome