Intracranial Stenosis Treated with Stenting in Patients with Suspected Cerebral Vasculitis: Two Case Reports.

Central nervous system vasculitis (CNSV) is an uncommon and poorly understood form of vasculitis. Early recognition is important because medical treatment might improve the outcome. However, randomized clinical trials on CNSV treatment do not exist. Endovascular treatment has been reported in few cases, but no data exist for intracranial stenting. We report 2 cases of patients with suspected CNSV and recurrent clinical episodes, treated with intracranial stenting. A 48-year-old man had relapsing episodes of right hemiparesis. Neuroradiological exams showed severe left carotid terminus stenosis. Despite immunosuppressive therapy, neuroradiological follow-up exams showed a worsening of the aforementioned stenosis with many transient episodes of weakness in the right limbs and aphasia. A 64-year-old woman had a sudden onset of dysarthria and transient aphasia. Neuroradiological exams showed a severe arterial stenosis involving the origin of left anterior cerebral artery and middle cerebral artery (MCA). Despite dual antiplatelet therapy, she presented an acute onset of severe aphasia, due to an occlusion of the left carotid terminus and proximal MCA. In both cases, endovascular procedure and intracranial stenting was performed, with marked improvement of cerebral blood flow. No more clinical episodes were reported. Intracranial stenting may be a valid therapeutic option in selected patients with CNSV and involvement of medium or large size vessels with clinical worsening despite best medical treatment.

Qualitative versus automatic evaluation of CT perfusion parameters in acute posterior circulation ischaemic stroke.

PURPOSE: To compare the diagnostic accuracy (ACC) in the detection of acute posterior circulation strokes between qualitative evaluation of software-generated colour maps and automatic assessment of CT perfusion (CTP) parameters. METHODS: Were retrospectively collected 50 patients suspected of acute posterior circulation stroke who underwent to CTP (GE "Lightspeed", 64 slices) within 24 h after symptom onset between January 2016 and December 2018. The Posterior circulation-Acute Stroke Prognosis Early CT Score (pc-ASPECTS) was used for quantifying the extent of ischaemic areas on non-contrast (NC)CT and colour-coded maps generated by CTP4 (GE) and RAPID (iSchemia View) software. Final pc-ASPECTS was calculated on follow-up NCCT and/or MRI (Philips Intera 3.0 T or Philips Achieva Ingenia 1.5 T). RAPID software also elaborated automatic quantitative mismatch maps. RESULTS: By qualitative evaluation of colour-coded maps, MTT-CTP4D and Tmax-RAPID showed the highest sensitivity (SE) (88.6% and 90.9%, respectively) and ACC (84% and 88%, respectively) compared with the other perfusion parameters (CBV, CBF). Baseline NCCT and CBF provided by RAPID quantitative perfusion mismatch maps had the lowest SE (29.6% and 6.8%, respectively) and ACC (38% and 18%, respectively). CBF and Tmax assessment provided by quantitative RAPID perfusion mismatch maps showed significant lower SE and ACC than qualitative evaluation. No significant differences were found between the pc-ASPECTSs assessed on colour-coded MTT and Tmax maps neither between the scores assessed on colour-coded CBV-CTP4D and CBF-RAPID maps. CONCLUSION: Qualitative analysis of colour-coded maps resulted more sensitive and accurate in the detection of ischaemic changes than automatic quantitative analysis.

Novel pathogenic TGFBR1 and SMAD3 variants identified after cerebrovascular events in adult patients with Loeys-dietz syndrome.

INTRODUCTION: Loeys-Dietz syndrome (LDS) is an autosomal dominant connective tissue disorder due to heterozygous pathogenic variants in transforming growth factor beta (TGFß) signaling-related genes. LDS types 1-6 are distinguished depending on the involved gene. LDS is characterized by multiple arterial aneurysms and dissections in addition to variable neurological and systemic manifestations. Patient 1: a 68-year-old man was admitted due to an aphasic transient ischemic attack (TIA). Brain CT-scan and CT angiography revealed a chronic and asymptomatic right vertebral artery dissection. Stroke diagnostic panel was unremarkable. His history showed mild stroke familiarity. At age of 49, he was treated for dissecting-aneurysm of the ascending aorta and started anticoagulation therapy. Seven years later, he underwent surgery for dissecting aneurysm involving aortic arch, descending-thoracic aorta, left subclavian artery, and both iliac arteries. Patient 2: a 47-year-old man presented a left hemiparesis due to right middle cerebral artery (MCA) and anterior cerebral artery (ACA) occlusion caused by right internal carotid artery (ICA) dissection after sport activity. Despite i.v. thrombolysis and mechanical thrombectomy, he developed malignant cerebral infarction and underwent decompressive hemicraniectomy. Digital subtraction angiography showed bilateral carotid and vertebral kinking, aneurysmatic dilatation on both common iliac arteries and proximal ectasia of the descending aorta. His father and his uncle died because of an ischemic stroke and a cerebral aneurysm rupture with a subarachnoid hemorrhage (SAH), respectively. DISCUSSION: in both cases, considering the family history and the multiple dissections and aneurysms, LDS molecular analysis was performed. In patient 1, the novel NM_005902.3 (SMAD3): c.840T > G; p.(Asn280Lys) likely pathogenic variant was identified, thus leading to a diagnosis of LDS type 3. In patient 2, the novel NM_004612.2 (TGFBR1): c.1225T > G; p.(Trp409Gly) likely pathogenic variant was found, allowing for a diagnosis of LDS type 1. CONCLUSION: LDS is characterized by genetic and clinical variability. Our report suggests that this genetically-determined connective tissue disorder is probably underestimated, as it might firstly show up with cerebrovascular events, although mild systemic manifestations. These findings could lead to identify people at risk of severe vascular complications (i.e., through genetic consult on asymptomatic relatives), in order to perform adequate vascular assessments and follow-up to prevent complications such as stroke.

CT perfusion and angiographic assessment of pial collateral reperfusion in acute ischemic stroke: the CAPRI study.

BACKGROUND: The purpose of this study was to evaluate the correlation between a novel angiographic score for collaterals and CT perfusion (CTP) parameters in patients undergoing endovascular treatment for acute ischemic stroke (AIS). METHODS: 103 patients (mean age 66.7±12.7; 48.5% men) with AIS in the anterior circulation territory, imaged with non-contrast CT, CT angiography, and CTP, admitted within 8 h from symptom onset and treated with any endovascular approach, were retrospectively included in the study. Clinical, neuroradiological data, and all time intervals were collected. Careggi Collateral Score (CCS) was used for angiographic assessment of collaterals and the Alberta Stroke Program Early CT Score (ASPECTS) for semiquantitative analysis of CTP maps. Two centralized core laboratories separately reviewed angiographic data, whereas CT findings were evaluated by an expert neuroradiologist. Univariate and multivariate analysis were performed considering CCS both as an ordinal and a dichotomous variable. RESULTS: 37/103 patients (35.9%) received intravenous tissue plasminogen activator. Median (IQR) ASPECTS was 9 (6-10) for admission CT, 9 (5-10) for cerebral blood volume (CBV) maps, 3 (2-3) for mean transit time maps, 3 (2-4), for cerebral blood flow maps, and 5 (3-7) for CTP mismatch. Univariate analysis showed a significant correlation between CCS and ASPECTS for all CTP parameters. Multivariate analysis confirmed an independent association only between CCS and CBV (p=0.020 when CCS was considered as a dichotomous variable, p=0.026 with ordinal CCS). CONCLUSIONS: A correlation between angiographic assessment of the collateral circulation and CTP seems to be present, suggesting that CCS may provide an indirect evaluation of the infarct core volume to consider for patient selection in AIS.

Intra-arterial therapy as a rescue strategy after clinically failed intravenous thrombolysis may increase the likelihood of a good outcome in patients with severe ischaemic stroke. A retrospective two centre study.

The purpose of this study was to evaluate the efficacy and safety of intra-arterial therapy as a rescue strategy after clinically failed intravenous thrombolysis (IVT) in acute ischaemic stroke patients. We conducted a retrospective analysis of consecutive acute ischaemic stroke patients treated with rescue therapy. The results from this study group were compared with those obtained from a control group consisting of 260 consecutive patients treated with IVT alone. The study group consisted of 52 patients with a mean age of 63 years and a median NIHSS score at admission of 17. Recanalization was achieved in 92% with a symptomatic haemorrhage rate of 9.6%. Rescue patients admitted with a severe stroke (NIHSS score >12) had a significantly better outcome at 90 days compared to patients with the same score but treated with IVT alone. No difference was seen for patients with a lower score at admission. This study indicates that rescue therapy may increase the proportion of patients with independent outcome if presenting with a severe stroke (NIHSS score >12) without increasing the rate of symptomatic haemorrhage.