Long-term clinical and ultrasound follow-up after transient perivascular inflammation of the carotid artery (TIPIC) syndrome: a multicenter study.

BACKGROUND: The present study aimed to describe the clinical and ultrasound (US) long-term follow-up of patients with transient perivascular inflammation of the carotid artery (TIPIC) syndrome and the risk of recurrence. METHODS: We enrolled patients with a definitive diagnosis of TIPIC syndrome who were included in a retrospective multicenter study. These patients were recontacted at least six months after the first TIPIC episode for a clinical and imaging follow-up. Each patient underwent a clinical evaluation through a tailored questionnaire as well as US imaging. RESULTS: Twenty-eight patients were enrolled with a median follow-up of 58.7 months (interquartile range = 8-121). Nineteen out of the 28 patients (67.8%) had residual pain, eight (28.6%) had experienced a clinical recurrence and 12 (42.9%) had a thickening of the carotid wall on US. No patients had neurological complication or other associated diseases. CONCLUSIONS: Patients with TIPIC syndrome have often residual pain and recurrence in about one quarter of cases but the long-term follow-up is in favor a benign self-limited pathology.Trial registration: ClinicalTrials.gov (identifier NCT03804112).

Reproducibility and accuracy of vessel wall MRI in diagnosing giant cell arteritis: a study with readers of varying expertise.

OBJECTIVE: To evaluate the reproducibility of vessel wall magnetic resonance imaging (VW-MRI) in diagnosing giant cell arteritis (GCA) among groups of radiologists with varying levels of expertise. METHODS: This institutional review board-approved retrospective single-center study recruited patients with suspected GCA between December 2014 and September 2021. Patients underwent 3 -T VW-MRI before temporal artery biopsy. Ten radiologists with varying levels of expertise, blinded to all data, evaluated several intracranial and extracranial arteries to assess GCA diagnosis. Interobserver reproducibility and diagnostic performance were evaluated. RESULTS: Fifty patients (27 women and 23 men) with a mean age of 75.9 ± 9 years were included. Thirty-one of 50 (62%) had a final diagnosis of GCA.VW-MRI had an almost perfect reproducibility among expert readers (kappa = 0.93; 95% CI 0.77-1) and substantial reproducibility among all readers, junior and non-expert senior readers (kappa = 0.7; 95% CI 0.66-0.73; kappa = 0.67 95% CI 0.59-0.74; kappa = 0.65; 95% CI 0.43-0.88 respectively) when diagnosing GCA. Substantial interobserver agreement was observed for the frontal branch of superficial temporal artery. Moderate interobserver agreement was observed for the superficial temporal artery and its parietal branch, as well as ophthalmic arteries in all groups of readers. Sensitivity, specificity, positive predictive value, negative predictive value, and accuracy varied depending on the group of readers. CONCLUSION: VW-MRI is a reproducible and accurate imaging modality for detecting GCA, even among less-experienced readers. This study advocates for the use of VW-MRI when diagnosing GCA even in less-experienced centers. CLINICAL RELEVANCE STATEMENT: VW-MRI is a reproducible and accurate imaging modality for detecting GCA, even among less-experienced readers, and it could be used as a first-line diagnostic tool for GCA in centers with limited expertise in GCA diagnosis. KEY POINTS: • Vessel wall magnetic resonance imaging (VW-MRI) is a reproducible and accurate imaging modality for detecting giant cell arteritis (GCA) in both extracranial and intracranial arteries. • The reproducibility of vessel wall magnetic resonance imaging for giant cell arteritis diagnosis was high among expert readers and moderate among less-experienced readers. • The use of vessel wall magnetic resonance imaging for giant cell arteritis diagnosis can be recommended even in centers with less-experienced readers.

Endovascular Therapy or Medical Management Alone for Isolated Posterior Cerebral Artery Occlusion: A Multicenter Study.

BACKGROUND: Whether endovascular therapy (EVT) added on best medical management (BMM), as compared to BMM alone, is beneficial in acute ischemic stroke with isolated posterior cerebral artery occlusion is unknown. METHODS: We conducted a multicenter international observational study of consecutive stroke patients admitted within 6 hours from symptoms onset in 26 stroke centers with isolated occlusion of the first (P1) or second (P2) segment of the posterior cerebral artery and treated either with BMM+EVT or BMM alone. Propensity score with inverse probability of treatment weighting was used to account for baseline between-groups differences. The primary outcome was 3-month good functional outcome (modified Rankin Scale [mRS] score 0-2 or return to baseline modified Rankin Scale). Secondary outcomes were 3-month excellent recovery (modified Rankin Scale score 0-1), symptomatic intracranial hemorrhage, and early neurological deterioration. RESULTS: Overall, 752 patients were included (167 and 585 patients in the BMM+EVT and BMM alone groups, respectively). Median age was 74 (interquartile range, 63-82) years, 329 (44%) patients were female, median National Institutes of Health Stroke Scale was 6 (interquartile range 4-10), and occlusion site was P1 in 188 (25%) and P2 in 564 (75%) patients. Baseline clinical and radiological data were similar between the 2 groups following propensity score weighting. EVT was associated with a trend towards lower odds of good functional outcome (odds ratio, 0.81 [95% CI, 0.66-1.01]; P=0.06) and was not associated with excellent functional outcome (odds ratio, 1.17 [95% CI, 0.95-1.43]; P=0.15). EVT was associated with a higher risk of symptomatic intracranial hemorrhage (odds ratio, 2.51 [95% CI, 1.35-4.67]; P=0.004) and early neurological deterioration (odds ratio, 2.51 [95% CI, 1.64-3.84]; P<0.0001). CONCLUSIONS: In this observational study of patients with proximal posterior cerebral artery occlusion, EVT was not associated with good or excellent functional outcome as compared to BMM alone. However, EVT was associated with higher rates of symptomatic intracranial hemorrhage and early neurological deterioration. EVT should not be routinely recommended in this population, but randomization into a clinical trial is highly warranted.

Early diffusion-weighted MRI at 3 Tesla detects ischemic changes of the optic nerve in anterior ischemic optic neuropathy.

OBJECTIVES: To assess the impact of timing from visual symptoms' onset to diffusion-weighted (DW) 3 T MRI completion to detect ischemic changes of the optic disc and optic nerve in AION patients. METHODS: This IRB-approved retrospective single-center study included 3 T MRI data from 126 patients with AION and 111 controls with optic neuritis treated between January 2015 and May 2020. Two radiologists blinded to all data individually analyzed imaging. A senior neuroradiologist resolved any discrepancies by consensus. The primary judgment criterion was the restricted diffusion of the optic disc and/or the optic nerve assessed subjectively on the ADC maps. ADC values were also measured. Spearman rank correlations were used to examine the relationships between timing from visual symptoms' onset to MRI completion and both the restricted diffusion and the ADC values. RESULTS: One hundred twenty-six patients (47/126 [37.3%] women and 79/126 [62.7%] men, mean age 69.1 ± 13.7 years) with AION were included. Restricted diffusion of the optic disc in AION eyes was more frequent in the early MRI group than in the late MRI group: 35/49 (71.4%) eyes versus 3/83 (3.6%) eyes, p < 0.001. ADC values of the pathological optic discs and optic nerves were lower in the early MRI group than in the late MRI group: 0.61 [0.52-0.94] × 10-3 mm2/s versus 1.28 [1.01-1.44] × 10-3 mm2/s, p < 0.001, and 0.74 [0.61-0.88] × 10-3 mm2/s versus 0.89 [0.72-1.10] × 10-3 mm2/s, p < 0.001, respectively. CONCLUSIONS: DWI MRI showed good diagnostic performance to detect AION when performed early after the onset of visual symptoms. KEY POINTS: • Restricted diffusion of the optic disc in eyes affected by AION was significantly more likely to be observed in patients who had undergone MRI within 5 days after onset of visual symptoms. • ADC values of the pathological optic discs and optic nerves were significantly lower in patients who had undergone MRI within 5 days after onset of visual symptoms of AION: 0.61 × 10-3 mm2/s versus 1.28 × 10-3 mm2/s, p < 0.001, and 0.74 × 10-3 mm2/s versus 0.89 × 10-3 mm2/s, p < 0.001, respectively. • The optimal threshold for timing from visual symptoms' onset to MRI completion to detect restricted diffusion of the optic disc and/or optic nerve was 5 days, with an AUC of 0.88 (CI95%: 0.82-0.94).

Use of Retinal Angiography and MRI in the Diagnosis of Giant Cell Arteritis With Early Ophthalmic Manifestations.

BACKGROUND: Giant cell arteritis (GCA) is a vasculitis often revealed by visual signs. Diagnosis is challenging and urgent. Retinal angiography (RA) and MRI allow effective diagnosis. We compared those and proposed an imaging-based approach to diagnose GCA in ophthalmological practice. METHODS: We conducted a retrospective study based on the data collected from patients suspected to have GCA on ophthalmological findings. Fluorescein (FA) and indocyanine green (ICG) RAs and MRI were performed and compared with final diagnosis. RESULTS: Among the 41 patients included, 25 were diagnosed with GCA. Sensitivities and specificities of FA and ICG were not different. MRI showed a higher sensitivity and specificity. The approach consisting in performing RA followed by MRI provided a better accuracy. CONCLUSION: Our study shows that RA can be supplemented by MRI in a specialized center to provide the most accurate diagnosis in GCA revealed by visual signs.

High-resolution MRI demonstrates signal abnormalities of the 3rd cranial nerve in giant cell arteritis patients with 3rd cranial nerve impairment.

OBJECTIVE: To determine the sensitivity and specificity of high-resolution (HR) MRI for detecting signal abnormalities of cranial nerves (CN) in giant cell arteritis (GCA) patients presenting with diplopia. METHODS: This IRB-approved retrospective single-center study included GCA patients who underwent 3-T HR MRI from December 2014 to January 2020. Two radiologists, blinded to all data, individually assessed for the presence of enhancement of the 3rd, 4th, and/or 6th CN on post-contrast HR imaging and high signal intensity on HR T2-WI, for signal abnormalities of extraocular muscles and the brainstem, and for inflammatory changes of the ophthalmic and extracranial arteries. A Fisher's exact test was used to compare patients with or without diplopia. RESULTS: In total, 64 patients (42/64 (66%) women and 22/64 (34%) men, mean age 76.3 ± 8 years) were included. Of the 64 patients, 14 (21.9%) presented with diplopia. Third CN enhancement was detected in 7/8 (87.5%) patients with 3rd CN impairment, as compared to no patients with 4th or 6th CN impairment or to patients without diplopia (p < 0.001). Third CN abnormal high signal intensity on HR T2-WI was detected in 4/5 patients (80%) with 3rd CN impairment versus none of other patients (p < 0.001). Sensitivity, specificity, positive predictive value, and negative predictive value for detecting 3rd CN signal abnormalities were of 0.88, 1, 1, and 0.99 and 0.8, 1, 1, and 0.98 for post-contrast HR imaging and HR T2-WI, respectively. CONCLUSIONS: HR MRI had excellent diagnostic sensitivity and specificity when detecting signal abnormalities of the 3rd CN in GCA patients presenting with 3rd CN impairment. KEY POINTS: • Third cranial nerve enhancement was detected in all patients with 3rd cranial nerve impairment except for one with transient diplopia. • The "check mark sign" might be useful to identify 3rd cranial nerve signal abnormalities in the orbital apex. • No signal abnormalities of the 4th or 6th cranial nerves could be detected on high-resolution MRI.

Dixon-T2WI magnetic resonance imaging at 3 tesla outperforms conventional imaging for thyroid eye disease.

OBJECTIVES: To determine the diagnostic performances of a single Dixon-T2-weighted imaging (WI) sequence compared to a conventional protocol including T1-, T2-, and fat-suppressed T2-weighted MRI at 3 T when assessing thyroid eye disease (TED). MATERIALS AND METHODS: This IRB-approved prospective single-center study enrolled participants presenting with confirmed TED from April 2015 to October 2019. They underwent an MRI, including a conventional protocol and a Dixon-T2WI sequence. Two neuroradiologists, blinded to all data, read both datasets independently and randomly. They assessed the presence of extraocular muscle (EOM) inflammation, enlargement, fatty degeneration, or fibrosis as well as the presence of artifacts. The Wilcoxon signed-rank test was used. RESULTS: Two hundred six participants were enrolled (135/206 [66%] women, 71/206 [34%] men, age 52.3 ± 13.2 years). Dixon-T2WI was significantly more likely to detect at least one inflamed EOM as compared to the conventional set (248/412 [60%] versus 228/412 [55%] eyes; (p = 0.02). Dixon-T2WI was more sensitive and specific than the conventional set for assessing muscular inflammation (100% versus 94.7% and 71.2% versus 68.5%, respectively). Dixon-T2WI was significantly less likely to show major or minor artifacts as compared to fat-suppressed T2WI (20/412 [5%] versus 109/412 [27%] eyes, p < 0.001, and 175/412 [42%] versus 257/412 [62%] eyes, p < 0.001). Confidence was significantly higher with Dixon-T2WI than with the conventional set (2.35 versus 2.24, p = 0.003). CONCLUSION: Dixon-T2WI showed higher sensitivity and specificity and showed fewer artifacts than a conventional protocol when assessing thyroid eye disease, in addition to higher self-reported confidence. KEY POINTS: • Dixon-T2WI has better sensitivity and specificity than a conventional protocol for assessing inflamed extraocular muscles in patients with thyroid eye disease. • Dixon-T2WI shows significantly fewer artifacts than fat-suppressed T2WI. • Dixon-T2WI is faster and is associated with significantly higher self-reported reader confidence as compared to a conventional protocol when assessing inflammatory extraocular muscles.

How can we combat multicenter variability in MR radiomics? Validation of a correction procedure.

OBJECTIVE: Test a practical realignment approach to compensate the technical variability of MR radiomic features. METHODS: T1 phantom images acquired on 2 scanners, FLAIR and contrast-enhanced T1-weighted (CE-T1w) images of 18 brain tumor patients scanned on both 1.5-T and 3-T scanners, and 36 T2-weighted (T2w) images of prostate cancer patients scanned in one of two centers were investigated. The ComBat procedure was used for harmonizing radiomic features. Differences in statistical distributions in feature values between 1.5- and 3-T images were tested before and after harmonization. The prostate studies were used to determine the impact of harmonization to distinguish between Gleason grades (GGs). RESULTS: In the phantom data, 40 out of 42 radiomic feature values were significantly different between the 2 scanners before harmonization and none after. In white matter regions, the statistical distributions of features were significantly different (p < 0.05) between the 1.5- and 3-T images for 37 out of 42 features in both FLAIR and CE-T1w images. After harmonization, no statistically significant differences were observed. In brain tumors, 41 (FLAIR) or 36 (CE-T1w) out of 42 features were significantly different between the 1.5- and 3-T images without harmonization, against 1 (FLAIR) or none (CE-T1w) with harmonization. In prostate studies, 636 radiomic features were significantly different between GGs after harmonization against 461 before. The ability to distinguish between GGs using radiomic features was increased after harmonization. CONCLUSION: ComBat harmonization efficiently removes inter-center technical inconsistencies in radiomic feature values and increases the sensitivity of studies using data from several scanners. KEY POINTS: • Radiomic feature values obtained using different MR scanners or imaging protocols can be harmonized by combining off-the-shelf image standardization and feature realignment procedures. • Harmonized radiomic features enable one to pool data from different scanners and centers without a substantial loss of statistical power caused by intra- and inter-center variability. • The proposed realignment method is applicable to radiomic features from different MR sequences and tumor types and does not rely on any phantom acquisition.

Increased diagnostic accuracy of giant cell arteritis using three-dimensional fat-saturated contrast-enhanced vessel-wall magnetic resonance imaging at 3 T.

OBJECTIVES: To compare the diagnostic accuracy of 3D versus 2D contrast-enhanced vessel-wall (CE-VW) MRI of extracranial and intracranial arteries in the diagnosis of GCA. METHODS: This prospective two-center study was approved by a national research ethics board and enrolled participants from December 2014 to October 2017. A protocol including both a 2D and a 3D CE-VW MRI at 3 T was performed in all patients. Two neuroradiologists, blinded to clinical data, individually analyzed separately and in random order 2D and 3D sequences in the axial plane only or with reformatting. The primary judgment criterion was the presence of GCA-related inflammatory changes of extracranial arteries. Secondary judgment criteria included inflammatory changes of intracranial arteries and the presence of artifacts. A McNemar's test was used to compare 2D to 3D CE-VW MRIs. RESULTS: Seventy-nine participants were included in the study (42 men and 37 women, mean age 75 (± 9.5 years)). Fifty-one had a final diagnosis of GCA. Reformatted 3D CE-VW was significantly more sensitive than axial-only 3D CE-VW or 2D CE-VW when showing inflammatory change of extracranial arteries: 41/51(80%) versus 37/51 (73%) (p = 0.046) and 35/50 (70%) (p = 0.03). Reformatted 3D CE-VW was significantly more specific than 2D CE-VW: 27/27 (100%) versus 22/26 (85%) (p = 0.04). 3D CE-VW showed higher sensitivity than 2D CE-VW when detecting inflammatory changes of intracranial arteries: 10/51(20%) versus 4/50(8%), p = 0.01. Interobserver agreement was excellent for both 2D and 3D CE-VW MRI: κ = 0.84 and 0.82 respectively. CONCLUSIONS: 3D CE-VW MRI supported more accurate diagnoses of GCA than 2D CE-VW. KEY POINTS: • 3D contrast-enhanced vessel-wall magnetic resonance imaging is a high accuracy, non-invasive diagnostic tool used to diagnose giant cell arteritis. • 3D contrast-enhanced vessel-wall imaging is feasible for clinicians to complete within a relatively short time, allowing immediate assessment of extra and intracranial arteries. • 3D contrast-enhanced vessel-wall magnetic resonance imaging might be considered a diagnostic tool when intracranial manifestation of GCA is suspected.

Remote brain microhaemorrhages may predict haematoma in glioma patients treated with radiation therapy.

OBJECTIVES: To evaluate the prevalence of cerebral remote microhaemorrhages (RMH) and remote haematomas (RH) using magnetic resonance susceptibility-weighted imaging (SWI) among patients treated for gliomas during follow-up. METHODS: We conducted a retrospective single centre longitudinal study on 58 consecutive patients treated for gliomas from January 2009 through December 2010. Our institutional review board approved this study. We evaluated the presence and number of RMH and RH found outside the brain tumour on follow-up MR imaging. We performed univariate and bivariate analyses to identify predictors for RMH and RH and Kaplan-Meier survival analysis techniques. RESULTS: Twenty-five (43%) and four patients (7%) developed at least one RMH or RH, respectively, during follow-up. The risk was significantly higher for patients who received radiation therapy (49% and 8% versus 0%) (p = 0.02). The risk of developing RH was significantly higher in patients with at least one RMH and a high burden of RMH. The mean age of those presenting with at least one RMH or RH was significantly lower. CONCLUSIONS: RMH were common in adult survivors of gliomas who received radiation therapy and may predict the onset of RH during follow-up, mainly in younger patients. KEY POINTS: • Brain RMH and RH are significantly more likely to occur after RT. • RMH occur in almost half of the patients treated with RT. • RMH and RH are significantly more frequent in younger patients. • RH occur only in patients with RMH.

Repeatability of apparent diffusion coefficient and intravoxel incoherent motion parameters at 3.0 Tesla in orbital lesions.

OBJECTIVES: To evaluate repeatability of intravoxel incoherent motion (IVIM) diffusion-weighted imaging (DWI) parameters in the orbit. METHODS: From December 2015 to March 2016, 22 patients were scanned twice using an IVIM sequence with 15b values (0-2,000 s/mm2) at 3.0T. Two readers independently delineated regions of interest in an orbital mass and in different intra-orbital and extra-orbital structures. Short-term test-retest repeatability and inter-observer agreement were assessed using the intra-class correlation coefficient (ICC), the coefficient of variation (CV) and Bland-Altman limits of agreements (BA-LA). RESULTS: Test-retest repeatability of IVIM parameters in the orbital mass was satisfactory for ADC and D (mean CV 12% and 14%, ICC 95% and 93%), poor for f and D*(means CV 43% and 110%, ICC 90% and 65%). Inter-observer repeatability agreement was almost perfect in the orbital mass for all the IVIM parameters (ICC = 95%, 93%, 94% and 90% for ADC, D, f and D*, respectively). CONCLUSIONS: IVIM appeared to be a robust tool to measure D in orbital lesions with good repeatability, but this approach showed a poor repeatability of f and D*. KEY POINTS: • IVIM technique is feasible in the orbit. • IVIM has a good-acceptable repeatability of D (CV range 12-25 %). • IVIM interobserver repeatability agreement is excellent (ICC range 90-95 %). • f or D* provide higher test-retest and interobserver variabilities.

Comparison of a Whole-Brain Contrast-Enhanced 3D TSE T1WI versus Orbits Contrast-Enhanced 2D Coronal T1WI at 3T MRI for the Detection of Optic Nerve Enhancement in Patients with Acute Loss of Visual Acuity.

BACKGROUND AND PURPOSE: MR imaging is the technique of choice for patients presenting with acute loss of visual acuity with no obvious ophthalmologic cause. The goal of our study was to compare orbits contrast-enhanced 2D coronal T1WI with a whole-brain contrast-enhanced 3D (WBCE-3D) TSE T1WI at 3T for the detection of optic nerve enhancement. MATERIALS AND METHODS: This institutional review board-approved retrospective single-center study included patients presenting with acute loss of vision who underwent 3T MR imaging from November 2014 to February 2020. Two radiologists, blinded to all data, individually assessed the presence of enhancement of the optic nerve on orbits contrast-enhanced 2D T1WI and WBCE-3D T1WI separately and in random order. A McNemar test and a Cohen κ method were used for comparing the 2 MR imaging sequences. RESULTS: One thousand twenty-three patients (638 women and 385 men; mean age, 42 [SD, 18.3] years) were included. There was a strong concordance between WBCE-3D T1WI and orbits contrast-enhanced 2D T1WI when detecting enhancement of the optic nerve: κ = 0.87 (95% CI, 0.84-0.90). WBCE-3D T1WI was significantly more likely to detect canalicular enhancement compared with orbits contrast-enhanced 2D T1WI: 178/1023 (17.4%) versus 138/1023 (13.5%) (P < .001) and 108/1023 (10.6%) versus 90/1023 (8.8%) (P = .04), respectively. The WBCE-3D T1WI sequence detected 27/1023 (3%) instances of optic disc enhancement versus 0/1023 (0%) on orbits contrast-enhanced 2D T1WI. There were significantly fewer severe artifacts on WBCE-3D T1WI compared with orbits contrast-enhanced 2D T1WI: 68/1023 (6.6%) versus 101/1023 (9.8%) (P < .001). The median reader-reported confidence was significantly higher with coronal T1WI compared with 3D TSE T1WI: 5 (95% CI, 4-5) versus 3 (95% CI, 1-4; P < .001). CONCLUSIONS: Our study showed that there was a strong concordance between WBCE-3D T1WI and orbits contrast-enhanced 2D T1WI when detecting enhancement of the optic nerve in patients with acute loss of visual acuity with no obvious ophthalmologic cause. WBCE-3D T1WI demonstrated higher sensitivity and specificity in diagnosing optic neuritis, particularly in cases involving the canalicular segments.

TARGET: A phase I/II open-label multicenter study to assess safety and efficacy of fexagratinib in patients with relapsed/refractory FGFR fusion-positive glioma.

Background: Oncogenic FGFR-TACC fusions are present in 3-5% of high-grade gliomas (HGGs). Fexagratinib (AZD4547) is an oral FGFR1-3 inhibitor with preclinical activity in FGFR-TACC+ gliomas. We tested its safety and efficacy in patients with recurrent FGFR-TACC + HGGs. Patients and Methods: TARGET (NCT02824133) is a phase I/II open-label multicenter study that included adult patients with FGFR-TACC + HGGs relapsing after ≥1 line of standard chemoradiation. Patients received fexagratinib 80 mg bd on a continuous schedule until disease progression or unacceptable toxicity. The primary endpoint was the 6-month progression-free survival rate (PFS6). Results: Twelve patients with recurrent IDH wildtype FGFR-TACC + HGGs (all FGFR3-TACC3+) were included in the efficacy cohort (male/female ratio = 1.4, median age = 61.5 years). Most patients (67%) were included at the first relapse. The PFS6 was 25% (95% confidence interval 5-57%), with a median PFS of 1.4 months. All patients without progression at 6 months (n = 3) were treated at first recurrence (versus 56% of those in progression) and remained progression-free for 14-23 months. The best response was RANO partial response in 1 patient (8%), stable disease in 5 (42%), and progressive disease in 6 (50%). Median survival was 17.5 months from inclusion. Grade 3 toxicities included lymphopenia, hyperglycaemia, stomatitis, nail changes, and alanine aminotransferase increase (n = 1 each). No grade 4-5 toxicities were seen. A 32-gene signature was associated with the benefit of FGFR inhibition in FGFR3-TACC3 + HGGs. Conclusions: Fexagratinib exhibited acceptable toxicity but limited efficacy in recurrent FGFR3-TACC3 + HGGs. Patients treated at first recurrence appeared more likely to benefit, yet additional evidence is required.

Validation of a multimodal algorithm for diagnosing giant cell arteritis with imaging.

PURPOSE: The purpose of this study was to identify which combination of imaging modalities should be used to obtain the best diagnostic performance for the non-invasive diagnosis of giant cell arteritis (GCA). MATERIALS AND METHODS: This IRB-approved prospective single-center study enrolled participants presenting with a suspected diagnosis of GCA from December 2014 to October 2017. Participants underwent high-resolution 3T magnetic resonance imaging (MRI), temporal and extra-cranial arteries ultrasound and retinal angiography (RA), prior to temporal artery biopsy (TAB). Diagnostic accuracy of each imaging modality alone, then a combination of several imaging modalities, was evaluated. Several algorithms were constructed to test optimal combinations using McNemar test. RESULTS: Forty-five participants (24 women, 21 men) with mean age of 75.4 ± 16 (SD) years (range: 59-94 years) were enrolled; of these 43/45 (96%) had ophthalmological symptoms. Diagnosis of GCA was confirmed in 25/45 (56%) patients. Sensitivity and specificity of MRI, ultrasound and RA alone were 100% (25/25; 95% CI: 86-100) and 86% (19/22; 95% CI: 65-97), 88% (22/25; 95% CI: 69-97) and 84% (16/19; 95% CI: 60-97), 94% (15/16; 95% CI: 70-100) and 74% (14/19; 95% CI: 49-91), respectively. Sensitivity, specificity, positive predictive and negative predictive values ranged from 95 to 100% (95% CI: 77-100), 67 to 100% (95% CI: 38-100), 81 to 100% (95% CI: 61-100) and 91 to 100% (95% CI: 59-100) when combining several imaging tests, respectively. The diagnostic algorithm with the overall best diagnostic performance was the one starting with MRI, followed either by ultrasound or RA, yielding 100% sensitivity (22/22; 95% CI: 85-100%) 100% (15/15; 95% CI: 78-100) and 100% accuracy (37/37; 95% CI: 91-100). CONCLUSION: The use of MRI as the first imaging examination followed by either ultrasound or RA reaches high degrees of performance for the diagnosis of GCA and is recommended in daily practice.

A Magnetic Resonance Imaging Radiomics Signature to Distinguish Benign From Malignant Orbital Lesions.

OBJECTIVES: Distinguishing benign from malignant orbital lesions remains challenging both clinically and with imaging, leading to risky biopsies. The objective was to differentiate benign from malignant orbital lesions using radiomics on 3 T magnetic resonance imaging (MRI) examinations. MATERIALS AND METHODS: This institutional review board-approved prospective single-center study enrolled consecutive patients presenting with an orbital lesion undergoing a 3 T MRI prior to surgery from December 2015 to July 2019. Radiomics features were extracted from 6 MRI sequences (T1-weighted images [WIs], DIXON-T2-WI, diffusion-WI, postcontrast DIXON-T1-WI) using the Pyradiomics software. Features were selected based on their intraobserver and interobserver reproducibility, nonredundancy, and with a sequential step forward feature selection method. Selected features were used to train and optimize a Random Forest algorithm on the training set (75%) with 5-fold cross-validation. Performance metrics were computed on a held-out test set (25%) with bootstrap 95% confidence intervals (95% CIs). Five residents, 4 general radiologists, and 3 expert neuroradiologists were evaluated on their ability to visually distinguish benign from malignant lesions on the test set. Performance comparisons between reader groups and the model were performed using McNemar test. The impact of clinical and categorizable imaging data on algorithm performance was also assessed. RESULTS: A total of 200 patients (116 [58%] women and 84 [42%] men; mean age, 53.0 ± 17.9 years) with 126 of 200 (63%) benign and 74 of 200 (37%) malignant orbital lesions were included in the study. A total of 606 radiomics features were extracted. The best performing model on the training set was composed of 8 features including apparent diffusion coefficient mean value, maximum diameter on T1-WIs, and texture features. Area under the receiver operating characteristic curve, accuracy, sensitivity, and specificity on the test set were respectively 0.869 (95% CI, 0.834-0.898), 0.840 (95% CI, 0.806-0.874), 0.684 (95% CI, 0.615-0.751), and 0.935 (95% CI, 0.905-0.961). The radiomics model outperformed all reader groups, including expert neuroradiologists (P < 0.01). Adding clinical and categorizable imaging data did not significantly impact the algorithm performance (P = 0.49). CONCLUSIONS: An MRI radiomics signature is helpful in differentiating benign from malignant orbital lesions and may outperform expert radiologists.

Three Tesla 3D High-Resolution Vessel Wall MRI of the Orbit may Differentiate Arteritic From Nonarteritic Anterior Ischemic Optic Neuropathy.

BACKGROUND: Anterior ischemic optic neuropathy (AION) is the most common cause of acute optic neuropathy in older patients. Distinguishing between arteritic AION (A-AION) and nonarteritic (NA-AION) is paramount for improved patient management. PURPOSE: The aim of this study was to evaluate 3-dimensional high-resolution vessel wall (HR-VW) magnetic resonance imaging (MRI) at 3 T to discriminate A-AION from NA-AION. MATERIALS AND METHODS: This prospective single-center study was approved by a national research ethics board and included 27 patients (17 A-AION and 10 NA-AION) with 36 AIONs from December 2014 to August 2017 who underwent 3 T HR-VW MRI. Two radiologists blinded to clinical data individually analyzed the imaging separately and in random order. Discrepancies were resolved by consensus with a third neuroradiologist. The primary diagnostic criterion was the presence of inflammatory changes of the ophthalmic artery. Secondary diagnostic criteria included the presence of an enhancement of the optic nerve or its sheath, the optic disc, or inflammatory changes of posterior ciliary or extracranial arteries. A Fisher exact test was used to compare A-AION from NA-AION patients. RESULTS: Inflammatory changes of the ophthalmic artery were present in all patients with A-AION but in none of NA-AION (P < 0.0001). Its sensitivity, specificity, positive predictive value, and negative predictive value were 100%. Inflammatory changes of posterior ciliary arteries were significantly more likely in A-AOIN (82% vs 0%, P < 0.0001). Interreader and intrareader agreements were almost perfect (κ = 0.82-1). CONCLUSIONS: High-resolution vessel wall MRI seems highly accurate when distinguishing A-AION from NA-AION and might be useful to improve patient management.

Hyperfrontality and hypoconnectivity during refreshing in schizophrenia.

Anomalous activations of the prefrontal cortex (PFC) and posterior cerebral areas have been reported in previous studies of working memory in schizophrenia. Several interpretations have been reported: e.g., neural inefficiency, the use of different strategies and differences in the functional organization of the cerebral cortex. To better understand these abnormal activations, we investigated the cerebral bases of a working memory component process, namely refreshing (i.e., thinking briefly of a just-activated representation). Fifteen patients with schizophrenia and 15 control subjects participated in this functional magnetic resonance imaging (fMRI) study. Participants were told that whenever they saw a word on the screen, they had to read it silently to themselves (read and repeat conditions), and when they saw a dot, they had to think of the just-previous word (refresh condition). The refresh condition (in comparison with the read condition) was associated with significantly increased activation in the left inferior frontal gyrus and significantly decreased connectivity within the prefrontal cortex and between the prefrontal and parietal cortices in patients with schizophrenia in comparison with control subjects. These results suggest that prefrontal dysfunctions in schizophrenia might be related to a defective ability to initiate (rather than to execute) specific cognitive processes.

Cerebral vasculopathy is associated with severe vascular manifestations in systemic sclerosis.

OBJECTIVE: To investigate brain involvement in patients with systemic sclerosis (SSc). METHODS: Sixty-three patients with SSc fulfilling the American College of Rheumatology and/or Leroy and Medsger criteria were retrospectively studied, including 30 (47.6%) with limited cutaneous and 27 (42.9%) with diffuse cutaneous SSc. Forty-one patients underwent computed tomography (CT) scan and magnetic resonance imaging (MRI) of the brain, 11 patients only CT scan, and the remaining 11 patients only MRI. Cerebral vasculopathy on MRI and CT scan was defined as absent or mild (score < 1), moderate (1 or= 2) on a 4-point scale (0 to 3). RESULTS: Cerebral vasculopathy was identified on CT scan in 22 patients (moderate in 12 and severe in 10) and on MRI in 38 patients (moderate in 28 and severe in 10). Patients with severe cerebral vasculopathy seen on MRI were more likely to have pulmonary arterial hypertension (PAH; p = 0.003) and showed a tendency to have scleroderma renal crisis (SRC; p = 0.25, test for trend p = 0.097). A similar association was found between severe cerebral vasculopathy seen on CT scan and PAH (p = 0.026) or SRC (p = 0.04). After adjusting for age and hypertension, severe cerebral vasculopathy was still associated with increased risk of severe vascular manifestations [odds ratio (OR) 32, 95% confidence interval (CI) 3.45-297, p = 0.002 for CT scan; OR 26, 95% CI 1.71-394, p = 0.019 for MRI]. CONCLUSION: Severe cerebral vasculopathy is associated with severe vascular manifestations in SSc patients. SSc patients with severe vascular complications should undergo neuroradiological imaging assessment of brain involvement.