To establish an intracranial vessel wall enhancement (VWE) scoring system and evaluate its potential as a protocol for monitoring systemic lupus erythematosus (SLE) disease activity and neuropsychiatric impairment.

In this retrospective study, fifty patients with SLE underwent conventional MRI and high-resolution magnetic resonance vascular wall imaging (HR-VWI) at three tertiary hospitals between August 2022 and December 2023. We analyzed VWE distribution in intracranial arteries, developed a scoring system based on enhancement patterns, and examined the relationship between VWE scores, disease activity, and cognitive function. Univariate analysis and logistic regression were utilized to identify clinical and imaging characteristics associated with sustained lupus low disease activity state (LLDAS) and central nervous system (CNS) neuropsychiatric impairment.

SLE patients exhibited multi-segmental intracranial vessel wall thickening with concentric or double-track enhancement. VWE scores positively correlated with disease activity and negatively with cognitive function (both P < 0.001). High VWE scores was negative predictors for achieving sustained LLDAS (OR, 1.52; 95 % CI: 1.01-2.28; P = 0.043). Logistic regression confirmed VWE scores as independent predictors of CNS neuropsychiatric impairment (OR, 1.92; 95 % CI: 1.03-3.60; P = 0.04). A VWE score > 4.0 demonstrated high sensitivity (96 %) and moderate specificity (65 %) for diagnosing CNS impairment (AUC = 0.82; P < 0.001).

The VWE scores system can serve as a valuable protocol for monitoring disease activity and detecting CNS neuropsychiatric impairment in SLE, offering a potential tool for early diagnosis and prognosis evaluation.

Autoimmune rheumatic diseases (AIRD) can cause intracranial artery stenosis (ICAS) and lead to stroke. This study aimed to characterize patients with ICAS associated with AIRD.

Using data from a high-resolution MR imaging database, we retrospectively reviewed patients with AIRD with ICAS. Stratification into vasculitis, atherosclerosis, and mixed atherovasculitis subtypes was based on imaging findings, followed by a comparative analysis of clinical characteristics and outcomes across these subgroups.

Among 139 patients (mean, 45.1 [SD, 17.3] years; 64.7% women), 56 (40.3%) were identified with vasculitis; 57 (41.0%), with atherosclerosis; and 26 (18.7%), with mixed atherovasculitis. The average interval from AIRD onset to high-resolution MRI was 5 years. Patients with vasculitis presented at a younger age of AIRD onset (mean, 34.5 [SD, 19.4] years), nearly 10 years earlier than other groups (P = .010), with a higher artery occlusion incidence (44.6% versus 21.1% and 26.9%, P = .021). Patients with atherosclerosis showed the highest cardiovascular risk factor prevalence (73.7% versus 48.2% and 61.5%, P = .021) but fewer intracranial artery wall enhancement instances (63.2% versus 100% in others, P < .001). The mixed atherovasculitis group, predominantly men (69.2% versus 30.4% and 24.6%, P < .001), exhibited the most arterial involvement (5 arteries per person versus 3 and 2, P = .001). Over an average 21-month follow-up, 23 (17.0%) patients experienced stroke events and 8 (5.9%) died, with the mixed atherovasculitis group facing the highest risk of stroke events (32.0%) and the highest mortality (12.0%).

Intracranial arteries are injured and lead to heterogeneous disease courses when exposed to AIRD and cardiovascular risk factors. While atherosclerosis acceleration is common, vasculitis may further contribute to the early development of occlusion and multiple artery involvement. Varied intracranial arteriopathies may result in different outcomes.

 Childhood stroke is a significant contributor to neurological morbidity often leading to debilitating outcomes. This study aimed to assess the clinical and radiological outcomes of angiographically proven central nervous system (CNS) arteriopathy in children.

 This prospective cohort study was conducted from June 2023 to May 2024 at The Children's Hospital, Lahore, Pakistan. The clinical outcomes were assessed using the Pediatric National Institute of Health Stroke Scale (PedNIHSS) at baseline and compared at six-month follow-up, while radiological outcomes were categorized as stable, regressive, or progressive disease. Clinical presentation, etiology, baseline PedNIHSS score, and arterial involvement were correlated with motor outcomes.

 Out of the 38 patients, 63.2% were female patients with a mean age of 4.27±2.43 years. The most common age group was 2-5 years (50.0%), followed by >5-10 years (31.6%). Global clinical presentation was seen in 60.5%, while 29.0% had a recurrence of stroke. Moyamoya disease (21.1%) was the most common etiological factor, followed by primary CNS angiitis (13.0%), infections (8.0%), and post-varicella focal cerebral arteriopathy (8.0%). Bilateral infarcts (55.3%) and anterior circulation involvement (68.4%) were the most affected areas, with the middle cerebral artery (MCA) being the most affected with complete involvement in 18.4% and partial involvement in 81.5% of cases. The mean baseline PedNIHSS score was 29±6.4, which improved to 19±4.8 at the six-month follow-up. Radiological outcomes showed in 42.2% of cases, with a progression of the disease on follow-up MRA; 31.6% had stable disease, while 21.0% had regressive disease. Significant factors associated with poor motor outcomes included global presentation (p=0.000008), etiological factors (p=0.047), bilateral infarcts (p=0.050), severe baseline PedNIHSS (p=0.000019), and progressive radiological disease (p=0.003).

 This study highlights the significant neurological impact of pediatric stroke. Early diagnosis, investigation of underlying causes, and identification of recurrence risk factors are crucial in preventing immediate and long-term complications.

Inflammatory causes of stroke are frequent and often pose diagnostic and therapeutic challenges due to the scarcity of randomized trials and the absence of clear guideline recommendations for many scenarios. Following the publication of the recommendations of the European Stroke Organization on primary angiitis of the central nervous system (PACNS) last year, the German Neurological Society (DGN) has issued very clear guidelines this year on the diagnostics and treatment of PACNS and updated the recommendations for systemic vasculitides; however, stroke often occurs not only as a result of primary vascular inflammation but also as a complication of another organ infection. Approximately 5% of all patients with sepsis, ca. 20% of patients with bacterial meningitis and up to 40% of patients with bacterial endocarditis suffer from a stroke as a complication. This article summarizes the key characteristics of these inflammatory causes of stroke and particularly focuses on the current recommendations for diagnostic and therapeutic management.

An 81-year-old man was admitted to our hospital with left hemiplegia after treatment for herpes zoster of the first branch of the right trigeminal nerve. CSF examination revealed an elevated varicella-zoster virus (VZV) antibody index. Brain MRI showed cerebral infarction in the right middle cerebral artery (MCA) territory and vessel wall thickening and enhancing effects at the ipsilateral MCA. Despite the standard treatment, the MCA stenosis progressed with recurrent infarcts. Percutaneous cerebral angioplasty was performed to the distal portion of the right MCA without deterioration. This case can provide a treatment option for refractory progressive VZV vasculopathy.

Without brain biopsy, there are limited diagnostic predictors to differentiate primary angiitis of the CNS (PACNS) from intracranial atherosclerotic disease (ICAD). We examined the utility of clinical, CSF, and quantitative vessel wall magnetic resonance imaging (VWMRI) variables in predicting PACNS from ICAD.

In this cross-sectional design, observational study, we reviewed electronic medical records to identify patients (18 years and older) who presented to our medical center between January 2015 and December 2021 for ischemic stroke due to intracranial vasculopathy. Patients with biopsy-proven PACNS, probable PACNS, or ICAD were included. Patients with secondary CNS vasculitis or no VWMRI data were excluded. On VWMRI, for each patient, a total of 20 vessel wall segments were analyzed for percent concentricity, percent irregularity, and concentricity to eccentricity (C/E) ratios. We also collected several clinical and CSF variables. Using logistic regression models, we assessed the diagnostic value of VWMRI, CSF, and clinical variables in predicting PACNS in patients with biopsy-proven disease. We then performed a sensitivity analysis to assess predictors of biopsy-proven and probable PACNS.

Thirty-two patients with ICAD (54.2%) and 27 patients with PACNS (45.8%) were included. Of the patients with PACNS, 21 (77.8%) were not biopsied and considered probable PACNS. Twenty-four patients with ICAD (75%) and 6 biopsy-proven patients with PACNS (22.2%) showed large vessel involvement and were included in the primary analysis. Encephalopathy (odds ratio [OR], 7.60; 95% CI 1.07-54.09) and seizure (OR 23.00; 95% CI 1.77-298.45) were significantly associated with PACNS. All patients were included in the sensitivity analysis, in which headache significantly predicted PACNS (OR 7.60; 95% CI 1.07-54.09). In the primary analysis, for every 1 white blood cell/µL increase in CSF, there was a 47% higher odds of PACNS (OR 1.47; 95% CI 1.04-2.07). On VWMRI, a C/E ratio >1 (OR 115.00; 95% CI 6.11-2165.95), percent concentricity ≥50% (OR 55.00; 95% CI 4.13-732.71), and percent irregularity <50% (OR 55.00; 95% CI 4.13-732.71) indicated significantly higher odds of PACNS compared with ICAD.

Our results suggest that quantitative VWMRI metrics, CSF pleocytosis, and clinical features of encephalopathy, seizure, and headache significantly predict a diagnosis of probable PACNS when compared with ICAD.

To identify the correlation between thrombosis and atherosclerosis in systemic lupus erythematosus (SLE) patients with antiphospholipid antibodies (aPLs) (SLE/aPLs) through high-resolution magnetic resonance imaging (HR-MRI) of the carotid artery.

A single-center, cross-sectional study was conducted. We collected consecutive patients with SLE/aPLs and healthy controls who underwent carotid HR-MRI examinations. The morphometric characteristics of the common carotid artery (CCA), internal carotid artery (ICA), external carotid artery (ECA), and carotid bulb (Sinus) were measured, and the differences in morphometric parameters between different groups were analyzed.

A total of 144 carotid arteries were analyzed. Compared with the control group, the wall area, wall thickness (WT and WTmax), and normalized wall index of CCA, ICA, ECA, and Sinus were increased in patients with SLE/aPLs, and the total vascular area (TVA) of CCA, ICA, and Sinus, and the bifurcation angle (BIFA) of ICA-ECA were also increased. A negative lupus anticoagulant (LAC) (with or without positive anticardiolipin antibody (aCL) or anti-β2glycoprotein antibody (aβ2GPI)) contributed to illustrating lower increased TVA and thickened vessel walls of CCA and ICA in SLE/aPLs patients without thrombotic events. Logistic regression analysis showed that WTmaxSinus and WTmaxGlobal were independent risk factors for thrombotic events in SLE/aPLs patients. The receiver operator characteristic curve showed that the cut-off value of WTmaxSinus was 2.855 mm, and WTmaxGlobal was 3.370 mm.

HR-MRI ensures the complete and accurate measurement of carotid morphometric parameters. Compared with the control group, the carotid artery in patients with SLE/aPLs is mainly characterized by diffusely thickened vessel walls, and the patients with thrombotic events showed additional higher vascular area of CCA and ICA, and BIFA of ICA-ECA without significant change in lumen area. The carotid arteries of SLE/aPLs patients with thrombotic events exhibited significant vessel wall thickening in all segments except ECA compared to those without thrombotic events. LAC-negative and non-thrombotic events distinguish relatively early atherosclerosis in the carotid arteries in patients with SLE/aPLs. Patients with SLE/aPLs that possess circumscribed thickened carotid vessel walls (>3.370 mm), particularly thickened at the Sinus (>2.855 mm), may require management strategies for the risk of thrombotic events.

To systematically analyse the time course of vessel wall enhancement and associated stenosis in patients with primary angiitis of the central nervous system (PACNS) following immunosuppressive therapy.

Two neuroradiologists retrospectively analysed MRIs of patients with PACNS seen at the Bern University Hospital and the St. Gallen Cantonal Hospital between 2015 and 2020. MRIs were examined for the presence of vessel wall enhancement, length of vessel wall enhancement (mm), circumferential extent of enhancement (degree) and degree of stenosis (%). Descriptive statistics and measurements of interobserver reliability were obtained. To investigate the temporal profiles of the variables following the commencement of immunosuppressant treatment, four series of Bayesian generalised multi-level models were generated.

A total of 23 patients with 43 affected vessels identified from 209 MRI exams were evaluated (mean follow-up: 715 days, standard deviation ± 487 days), leading to a complete dataset of 402 entries. Vessel wall enhancement and circumferential extent of enhancement decreased for approximately 1 year after the initiation of immunosuppressant therapy. Changes were more pronounced in younger patients. Disappearance of vessel wall enhancement (in at least one vessel) was seen in about half of patients after a median of 172 days interquartile range 113-244, minimum 54 days, maximum 627 days.

This study evaluated the typical time course of vessel wall enhancement in patients with PACNS. Our results could be a useful reference for radiologists and clinicians interpreting follow-up imaging in patients with PACNS.

Routine clinical exams can be interpreted with more confidence when radiologists are aware of the typical temporal evolution of vessel wall enhancement in patients with primary angiitis of the central nervous system after initiation of immunosuppressive therapy.

Few data exist for vessel wall imaging of primary angiitis of the central nervous system. Following immunosuppressant therapy, vessel wall enhancement decreases for approximately one year. These results may serve as a reference for radiologists performing follow-up imaging.

Central nervous system vasculitis (CNSV) is a rare disease. High-resolution vessel wall imaging (HR-VWI) enables the identification of inflammatory changes within the vessel wall. Few studies have applied HR-VWI to assess CNSV in children. This study delves into the utility of HR-VWI for diagnosing and treating CNSV in children, with the aim of enhancing clinical diagnosis and efficacy evaluation.

Imaging data were acquired from children who underwent HR-VWI examinations. The study meticulously analysed clinical data and laboratory tests to discern the characteristics and distribution patterns of diverse vasculitis forms.

In children, CNSV mainly involves medium vessels with grade 1 and 2 stenosis (grade 4 stenosis is rare), and the imaging features generally show centripetal and moderate enhancement, suggesting that this feature is specific for the diagnosis of CNSV. High-grade stenosis, concentric enhancement and strong enhancement of the vasculature indicate more severe disease activity. Remarkably, HR-VWI proved to be significantly more sensitive than magnetic resonance angiography in detecting CNSV. Among the 13 cases subjected to imaging review, 8 demonstrated a reduction or resolution of vessel wall inflammation. In contrast, five patients exhibited worsening inflammation in the vessel wall. HR-VWI demonstrated that changes in vessel wall inflammation were closely correlated with changes in brain parenchymal lesions and symptoms.

This study underscores the diagnostic value of HR-VWI in CNSV assessment and treatment monitoring, offering a quantitative evaluation of CNSV in children.

Intracranial arterial disease (ICAD) is a heterogeneous condition characterized by distinct pathologies, including atherosclerosis. Advances in magnetic resonance technology have enabled the visualization of intracranial arteries using high-resolution vessel wall imaging (HR-VWI). This review summarizes the anatomical, embryological, and histological differences between the intracranial and extracranial arteries. Next, we review the heterogeneous pathophysiology of ICAD, including atherosclerosis, moyamoya or RNF213 spectrum disease, intracranial dissection, and vasculitis. We also discuss how advances in HR-VWI can be used to differentiate ICAD etiologies. We emphasize that one should consider clinical presentation and timing of imaging in the absence of pathology-radiology correlation data. Future research should focus on understanding the temporal profile of HR-VWI findings and developing quantitative interpretative approaches to improve the decision-making and management of ICAD.

Systemic lupus erythematosus (SLE) is a heterogenous, devastating autoimmune inflammatory disease with multiorgan involvement. A variety of neurological and psychiatric symptoms may be caused by nervous system involvement, termed neuropsychiatric systemic lupus erythematosus.

We describe a young man newly diagnosed with SLE who had a stroke as an initial symptom and was found to have cerebral large-vessel vasculitis and Fahr syndrome.

The novelties of this report are the extensive cerebral calcification demonstrated on head computerized tomography in a patient with SLE, and the depiction of an underlying vasculitis on high-resolution magnetic resonance vessel wall imaging. It is our aim to describe this atypical form of neuropsychiatric systemic lupus erythematosus onset and to make known the usefulness of the new magnetic resonance imaging techniques for the diagnosis of cerebral large-vessel vasculitis.

Data regarding MR vessel-wall imaging (VWI) in patients with Moyamoya disease (MMD) is sparse, particularly in non-asian cohorts. We contribute data regarding the frequency of vessel wall contrast-enhancement (VW-CE) and its potential clinical significance in a European patient group.

Patients with a diagnosis of MMD who were examined by VWI were included in the study. VW-CE of stenoocclusive lesions of the terminal internal carotid artery and/or its proximal branches was rated qualitatively. Changes of VW-CE on available follow-up were recorded. VW-CE was correlated with diffusion-restricted lesions and magnetic resonance angiography (MRA) findings.

Eleven patients (eight female, three male) were included. Twenty-eight stenoocclusive lesions were analyzed, of which 16 showed VW-CE (57.1%). VW-CE was mostly concentric (n=15), rather than eccentric (n=1). In all three patients in whom follow-up VWI was available, changes of VW-CE were documented. Diffusion-restricted lesions were more frequently related to stenoocclusive lesions with VW-CE (n=9) than without VW-CE (n=2), bordering statistical significance. The affected arteries were assessed as stenotic and as occluded in 14 cases each and VW-CE was seen significantly more often in stenotic (n=12) than in occluded arteries (n=4). No correlation was found between the presence of VW-CE and moyamoya stages determined by MRA.

Our data suggest that concentric VW-CE is a relatively frequent finding in European MMD patients. VW-CE may change over time and occur in certain stages, possibly representing "active stenosing". Larger studies are needed to validate these findings and determine the clinical relevance of VW-CE in MMD.

Primary angiitis of the central nervous system (PACNS) is a rare disease, for which no validated guidelines exist. We report the findings of a survey on the clinical practice of physicians who manage adults with PACNS.

An online survey was distributed through neurology, internal medicine, and rheumatology societies in Canada and Europe. Participants who were directly involved as treating physicians for at least two adult patients with PACNS were eligible for the survey.

Ninety-six physicians completed the survey. Most participants were neurologists (n = 38, 40%), internists (n = 34, 35%) or rheumatologists (n = 22, 23%). Participants obtained a CNS biopsy in a median of 25% (IQR: 5-50%) of suspected PACNS cases. When determining the degree to which eight scenarios justified a CNS biopsy, participants achieved fair inter-rater agreement (Gwet's AC₂ 0.30, 95% CI 0.23-0.41). For induction therapy, 81 (84%) participants reported using glucocorticoids and cyclophosphamide in > 50% of patients. After obtaining remission, 85 (89%) participants systematically introduced or maintained immunosuppressive therapy. Glucocorticoids were prescribed for a median of 12 months. Maintenance therapy with another immunosuppressant was continued for a median of 24 months. In patients who achieved remission, we explored how eight scenarios with different imaging and CSF results supported an increase in treatment. Inter-rater agreement was substantial if the patient was symptomatic (0.66, 95% CI 0.58-0.80) and moderate (0.50, 95% CI 0.45-0.60) if asymptomatic.

This survey illustrates current real-world management of PACNS and emphasizes several areas for which physicians still lack study-based evidence and/or clinical practice guidelines.

To investigate the predictors of the improvement for patients with isolated intracranial vasculitis stenoses using high-resolution vessel wall magnetic resonance imaging (HR VW-MRI).

We retrospectively reviewed data from consecutive patients with confirmed intracranial vasculitis under the same conventional conservative treatment based on a prospectively established HR VW-MRI database between December 2016 and December 2020. According to the changes between the degree of stenosis at baseline compared to follow-up MR angiography, the patients were divided into an improvement group and a non-improvement group. A multivariate analysis was performed to identify the predictive factors associated with the improvement of stenoses secondary to intracranial vasculitis.

Overall, 41 patients (mean age 32.0 ± 10.1 years, 16 females) with isolated intracranial vasculitis stenoses were included (41.5 % [17/41] in the improvement group, and 58.5 % [24/41] were in the non-improvement group). The degree of wall enhancement on follow-up imaging was significantly reduced compared with that on the baseline imaging in the improvement group (P = 0.004). The multivariate analysis showed that the degree of enhancement (OR, 0.219, 95 % CI, 0.054 to 0.881; P = 0.033) at baseline was an independent predictive factor associated with the improvement in the intracranial vasculitis stenoses.

In patients with isolated intracranial vasculitis stenoses, the less enhancement the vessel wall was, the more likely the degree of stenosis would be reduced by conventional conservative therapy.

The clinical manifestations of central nervous system (CNS) vasculitis are highly variable. In the absence of a positive CNS biopsy, CNS vasculitis is particularly suspected when markers of both vascular disease and inflammation are present. To facilitate the clinical and therapeutic approach to this rare condition, CNS vasculitis can be classified according to the size of the involved vessels. Vascular imaging is used to identify medium vessel disease. Small vessel disease can only be diagnosed with a CNS biopsy. Medium vessel vasculitis usually presents with focal neurological signs, while small vessel vasculitis more often leads to cognitive deficits, altered level of consciousness and seizures. Markers of CNS inflammation include cerebrospinal fluid pleocytosis or elevated protein levels, and vessel wall, parenchymal or leptomeningeal enhancement. The broad range of differential diagnoses of CNS vasculitis can be narrowed based on the disease subtype. Common mimickers of medium vessel vasculitis include intracranial atherosclerosis and reversible cerebral vasoconstriction syndrome. The diagnostic workup aims to answer two questions: is the neurological presentation secondary to a vasculitic process, and if so, is the vasculitis primary (i.e., primary angiitis of the CNS) or secondary (e.g., to a systemic vasculitis, connective tissue disorder, infection, malignancy or drug use)? In primary angiitis of the CNS, glucocorticoids and cyclophosphamide are most often used for induction therapy, but rituximab may be an alternative. Based on the available evidence, all patients should receive maintenance immunosuppression. A multidisciplinary approach is necessary to ensure an accurate and timely diagnosis and to improve outcomes for patients with this potentially devastating condition.