Cerebral dural arteriovenous fistulas in patients with PTEN-related hamartoma tumor syndrome.

Central nervous system (CNS) dural arteriovenous fistulas (DAVF) have been reported in PTEN-related hamartoma tumor syndrome (PHTS). However, PHTS-associated DAVF remain an underexplored field of the PHTS clinical landscape. Here, we studied cases with a PTEN pathogenic variant identified between 2007 and 2020 in our laboratory (n = 58), and for whom brain imaging was available. Two patients had DAVF (2/58, 3.4%), both presenting at advanced stages: a 34-year-old man with a left lateral sinus DAVF at immediate risk of hemorrhage, and a 21-year-old woman with acute intracranial hypertension due to a torcular DAVF. Interestingly, not all patients had 3D TOF/MRA, the optimal sequences to detect DAVF. Early diagnosis of DAVF can be lifesaving, and is easier to treat compared to developed, proliferative, or complex lesions. As a result, one should consider brain MRI with 3D TOF/MRA in PHTS patients at genetic diagnosis, with subsequent surveillance on a case-by-case basis.

Deep Learning for Detecting BRCA Mutations in High-Grade Ovarian Cancer Based on an Innovative Tumor Segmentation Method From Whole Slide Images.

BRCA1 and BRCA2 genes play a crucial role in repairing DNA double-strand breaks through homologous recombination. Their mutations represent a significant proportion of homologous recombination deficiency and are a reliable effective predictor of sensitivity of high-grade ovarian cancer (HGOC) to poly(ADP-ribose) polymerase inhibitors. However, their testing by next-generation sequencing is costly and time-consuming and can be affected by various preanalytical factors. In this study, we present a deep learning classifier for BRCA mutational status prediction from hematoxylin-eosin-safran-stained whole slide images (WSI) of HGOC. We constituted the OvarIA cohort composed of 867 patients with HGOC with known BRCA somatic mutational status from 2 different pathology departments. We first developed a tumor segmentation model according to dynamic sampling and then trained a visual representation encoder with momentum contrastive learning on the predicted tumor tiles. We finally trained a BRCA classifier on more than a million tumor tiles in multiple instance learning with an attention-based mechanism. The tumor segmentation model trained on 8 WSI obtained a dice score of 0.915 and an intersection-over-union score of 0.847 on a test set of 50 WSI, while the BRCA classifier achieved the state-of-the-art area under the receiver operating characteristic curve of 0.739 in 5-fold cross-validation and 0.681 on the testing set. An additional multiscale approach indicates that the relevant information for predicting BRCA mutations is located more in the tumor context than in the cell morphology. Our results suggest that BRCA somatic mutations have a discernible phenotypic effect that could be detected by deep learning and could be used as a prescreening tool in the future.

Heart and systemic sclerosis-findings from a national cohort study.

OBJECTIVES: Heart involvement is one of the leading causes of death in systemic sclerosis (SSc). The prevalence of SSc-related cardiac involvement is poorly known. Our objective was to investigate the prevalence and prognosis burden of different heart diseases in a nationwide cohort of patients with SSc. METHODS: We used data from a multicentric prospective study using the French SSc national database. Focusing on SSc-related cardiac involvement, we aimed to determine its incidence and risk factors. RESULTS: Over the 3528 patients with SSc 312 (10.9%) had SSc-related cardiac involvement at baseline. They tended to have a diffuse SSc subtype more frequently, more severe clinical features, and presented more cardiovascular risk factors. From the 1646 patients available for follow-up analysis, SSc-related cardiac involvement was associated with an increased risk of death. There was no significant difference in overall survival between SSc-related cardiac involvement, ischaemic heart disease or pulmonary arterial hypertension. Regarding survival analysis, 98 patients developed SSc-related cardiac involvement at five years (5-year event rate: 11.15%). Regarding reduced LVEF < 50% and left ventricular diastolic dysfunction, the 5-year event rate was 2.49% and 5.84% respectively. Pericarditis cumulative incidence at five years was 3%. Diffuse SSc subtype was a risk factor for SSc-related cardiac involvement and pericarditis. Female sex was associated with less left ventricular diastolic dysfunction incidence. CONCLUSIONS: Our results describe the incidence and prognostic burden of SSc-related cardiac involvement at a large scale, with gender and diffuse SSc subtype as risk factors. Further analyses should assess the potential impact of treatment on these various cardiac outcomes.

"National survey for preliminary evaluation of neuroradiological protocols in patients with infective endocarditis".

OBJECTIVES: The aim of this study was to identify imaging protocols in patients with infective endocarditis through a nationwide survey. METHODS: An electronic evolutionary survey was sent to interventional Neuroradiologists among neuroradiological centers, under the aegis of the Société Française de Neuroradiologie. Among 33 contacted centers, 25 completed the survey (21 universitary hospitals and 4 peripheric hospitals). RESULTS: Most of the centers (88%) used systematic imaging screening in IE patients. MRI was the first imaging method used in 66% of cases, while CT was used in 44%. When no IIA was detectable in CT-scan screening, 6 (54,54%) stopped investigations, while 9 (81,81%) continued with MRI exploration in case of hemorrhage, ischemia or enhancement. Sulcal hemorrhage on MRI was an indication of complementary DSA in 25 centers (100%). Regarding IIA characterization, 12 centers (48%) used systematic DSA, whereas for 10 centers (40%), DSA was conditioned by hemorrhage or patient status. CONCLUSION: We highlighted large variations in Neuroimaging exploration and follow-up of IE patients in real-world practices. Expert guidelines able to standardize practices are warranted to improve the management of this serious and often misdiagnosed pathology.

Descriptive and functional anatomy of the Heschl Gyrus: historical review, manual labelling and current perspectives.

PURPOSE: The Heschl Gyrus (HG), which includes the Primary Auditory Cortex (PAC), lies on the upper surface of the superior temporal gyrus (T1). It has been the subject of growing interest in the fields of neuroscience over the past decade. Given the considerable interhemispheric and interindividual variability of its morphology, manual labelling remains the gold standard for its radio-anatomical study. The aim of this study was to revisit the original work of Richard L. Heschl, to provide a broad overview of the available anatomical knowledge and to propose a manually labelled 3D digital model. METHODS: We reviewed existing works on the HG, from Heschl's original publication of 1878, Dejerine neuroanatomical atlas of 1895 to the most recent digital atlases (Julich-Brain Cytoarchitectonic Atlas, the Human Connectome Project). Our segmentation work was based on data from the BigBrain Project and used the MRIcron 2019 software. RESULTS: The original publication by Heschl has been translated into French and English. We propose a correspondence of previous nomenclatures with the most recent ones, including the Terminologia Neuroanatomica. Finally, despite the notable anatomical variability of the HG, clear and coherent segmentation criteria allowed us to generate a 3D digital model of the HG. DISCUSSION AND CONCLUSION: Heschl's work is still relevant and could impulse further anatomical and functional studies. The segmentation criteria could serve as a reference for manual labelling of the HG. Furthermore, a thorough, and historically based understanding of the morphological, microstructural and functional characteristics of the HG could be useful for manual segmentation.

Homologies of spinal ascending nociceptive pathways between rats and macaques: can we transpose to human? A review and analysis of the literature.

PURPOSE: Due to the difficulty of using neural tracers in humans, knowledge of the nociceptive system's anatomy is mainly derived from studies in animals and mainly in rats. The aim of this study was to investigate the morphological differences of the ascending spinal nociceptive pathways between the rat and the macaque monkey; in order to evaluate the variability of this anatomy during phylogenesis, and thus to know if the anatomical description of these pathways can be transposed from the rat to the human. METHODS: A review and analysis of the literature were performed. The criteria used for comparison were: origins, pathways, their terminations in target structures, and projections from target structures of ascending spinal nociceptive pathways. The monkey was used as an intermediate species for comparison because of the lack of data in humans. The hypothesis of transposition of anatomy between rat and human was considered rejected if differences were found between rat and monkey. RESULTS: An anatomical difference in termination was found for the spino-annular or spino-periaqueductal grey (spino-PAG) pathway and transposition of its anatomy from rat to human was rejected. No difference was found in other pathways and the transposition of their anatomy from rat to human was therefore, not rejected. CONCLUSION: This work highlights the conservation of most of the ascending spinal nociceptive pathways' anatomy between rat and monkey. Thus, the possibility for a transposition of their anatomy between rat and human is not rejected.

Antiphospholipid syndrome in patients over 65 years: A comparative study of clinical and biological features and thrombotic relapses.

OBJECTIVE: The aim of the study was to describe clinical and biological characteristics and thrombotic relapses of patients diagnosed with antiphospholipid syndrome (APS) after the age of 65 years, in comparison with patients diagnosed with APS before 65. METHODS: This retrospective multicenter study was performed to 2005 from 2017 and included patients diagnosed with APS after the age of 65 years, in accordance with Sydney criteria. We compared these patients with APS patients diagnosed before the age of 65 years, and with control thrombotic patients older than 65 years. RESULTS: Fifty-eight APS patients over the age of 65 years were compared to 127 APS patients aged less than 65 and to 58 controls. In elderly APS versus younger APS, there was a male predominance (58.6% vs 36.2% p = .001); myocardial infarction and lower limb deep vein thrombosis (LLDVT) were more frequent in elderly, respectively, 12.1% versus 1.6% (p = .005), and 44.8% versus 29.9% (p = .048). Anticardiolipin antibody (aCL) IgM was more frequently found in old patients compared to younger patients (33.9% vs 18.1%, p = .02), contrary to lupus anticoagulant (LAC) (52.8% vs 66.9%, p = .02). Older patients were more often diagnosed with single positive APS (82.8% vs 59.8% p = .002). The thrombotic relapse free survival was lower in elderly APS patients (p = .044) compared to younger APS. Elderly APS patients had more recurrent arterial and venous thrombosis (p = .03) and had poorer overall survival (p = .004) than elderly controls. CONCLUSION: In this study, APS was different in patients aged more than 65 years, with a male predominance and more myocardial infarctions and LLDVT at diagnosis. Single antiphopholipid positivity and aCL IgM were more frequent in older patients. Older patient with APS had more thrombotic recurrence during follow-up. Compared to elderly controls, elderly APS patients had more thrombosis recurrences and poorer survival.

Endovascular treatment of infectious intracranial aneurysms complicating infective endocarditis: a series of 31 patients with 55 aneurysms.

PURPOSE: Endovascular treatment (EVT) has become a major option in management of infectious intracranial aneurysms (IIAs) complicating infective endocarditis. We report a retrospective, single-center series of consecutive patients with IIAs treated by EVT. METHODS: Patients were included from January 2009 to July 2020. IIAs were diagnosed on DSA. Each patient underwent a neurological assessment before and after EVT and was followed up by imaging within 15 days of EVT. Safety was assessed on the evolution of NIHSS score. A minor stroke was defined as a worsening of NIHSS < 4 points. Efficacy was defined as the absence of hemorrhagic event during cardiac surgery and the exclusion of the IIA on control imaging. RESULTS: Sixty-two IIAs (30 ruptured) were diagnosed in 31 patients. Fifty-six IIAs were diagnosed on the first DSA and 6 on the early control exploration. EVT was achieved in 55 IIAs by parent artery occlusion with glue in 52 distal IIAs and coils in 3 proximal IIAs. IIAs were located in 90.9% of cases on a fourth-division branch of a cerebral artery. The neurological examination remained unchanged in 29 patients (93.5%), and 2 patients suffered minor stroke. EVT was performed before cardiac surgery in 20/22 patients. All treated IIAs were excluded on follow-up imaging. No hemorrhage was observed during cardiac surgery or in the aftermath. Seven (11.3%) unruptured IIAs were not embolized. CONCLUSION: EVT of IIAs by occlusion of the parent artery is effective in preventing rupture and carries no significant neurological risk.

Semi-Quantitative [18F]FDG-PET/CT ROC-Analysis-Based Cut-Offs for Aortitis Definition in Giant Cell Arteritis.

[18F]fluorodeoxyglucose-positron emission tomography/computed tomography ([18F]FDG-PET/CT) is used to diagnose large vessel vasculitis in giant cell arteritis (GCA). We aimed to define a semi-quantitative threshold for identifying GCA aortitis from aortic atheroma or the control. Contrast enhanced computed tomography (CECT) was used as the reference imaging for aortic evaluation and to define aortitis, aortic atheroma and control aortas. [18F]FDG-PET/CT was performed on 35 GCA patients and in two different control groups (aortic atheroma (n = 70) and normal control (n = 35)). Aortic semi-quantitative features were compared between the three groups. GCA patients without aortitis on CECT were excluded. Of the GCA patients, 19 (54.3%) were not on glucocorticoids (GC) prior to [18F]FDG-PET/CT. The SUVmax, TBRblood and TBRliver aortic values were significantly higher in the GCA aortitis group than in the aortic atheroma and control groups (p < 0.001). Receiver operating characteristic curve analyses brought to light quantitative cut-off values allowing GCA aortitis diagnosis with optimal sensitivity and specificity versus control or aortic atheroma patients for each PET-based feature analyzed. Considering the overall aorta, a SUVmax threshold of 3.25 and a TBRblood threshold of 1.75 had a specificity of 83% and 75%, respectively, a sensitivity of 81% and 81%, respectively, and the area under the ROC curve (AUC) was 0.86 and 0.83, respectively, for aortitis detection compared to control groups in GCA cases with GC. A SUVmax threshold of 3.45 and a TBRblood threshold of 1.97 had a specificity of 90% and 93%, respectively, a sensitivity of 89% and 89%, respectively, with an AUC of 0.89 and 0.96, respectively, for aortitis detection compared to the control in GC-free GCA cases. Discriminative thresholds of SUVmax and TBRblood for the diagnosis of GCA aortitis were established using CECT as the reference imaging.

Venous sinus stenting after repair of skull base spontaneous cerebrospinal fluid leaks: A single-center retrospective cohort series with case-control analysis.

BACKGROUND: Management of idiopathic intracranial hypertension (IIH) is recommended after surgical repair of spontaneous cerebrospinal fluid leaks (sCSF-leaks) of the skull base for prevention of recurrence. PURPOSE: To assess the feasibility of venous sinus stenting, a treatment commonly used for the treatment of IIH associated with intracranial venous sinus stenosis (VSS), after sCSF-leaks closure. MATERIALS AND METHODS: A single-center cohort series of consecutive patients who underwent sCSF-leak closure was retrospectively analyzed. Stenting was considered either for leak recurrence or in prophylactic manner after repair in patients with VSS as confirmed by cerebral venous imaging. Leak recurrence, need for new repair or adjunctive treatment of IIH, meningitis, and stenting complications were determined at the last follow-up. Cases who had prophylactic stenting were compared to historical controls before stenting option. RESULTS: Twenty-two patients had intracranial venous stenting after sCSF-leak closure. Their median age was 58 years (Q1=45; Q3=68), BMI=31 kg.m-2 (Q1=27; Q3=36), and female rate=85%. The overall rate of successful repair after stenting was 95% (95% CI = 87-100%) at a median follow-up of 2.4 years (Q1=1.2; Q3=3.3). Adjunctive treatment for IIH was needed in 4 patients (4/22, 18%) including 2 patients without leak recurrence. No meningitis, permanent morbidity or mortality was observed after stenting. Compared to 18 controls, cases had significantly less recurrence (P = 0.03), and a trend for less adjunctive treatment for IIH (P = 0.06). CONCLUSIONS: Our study suggests that stenting might be a valid option for prevention of sCSF-leak recurrences after repair in patients with intracranial venous sinus stenosis.

European Multicenter Study of ET-COVID-19.

BACKGROUND AND PURPOSE: Acute ischemic stroke and large vessel occlusion can be concurrent with the coronavirus disease 2019 (COVID-19) infection. Outcomes after mechanical thrombectomy (MT) for large vessel occlusion in patients with COVID-19 are substantially unknown. Our aim was to study early outcomes after MT in patients with COVID-19. METHODS: Multicenter, European, cohort study involving 34 stroke centers in France, Italy, Spain, and Belgium. Data were collected between March 1, 2020 and May 5, 2020. Consecutive laboratory-confirmed COVID-19 cases with large vessel occlusion, who were treated with MT, were included. Primary investigated outcome: 30-day mortality. SECONDARY OUTCOMES: early neurological improvement (National Institutes of Health Stroke Scale improvement ≥8 points or 24 hours National Institutes of Health Stroke Scale 0-1), successful reperfusion (modified Thrombolysis in Cerebral Infarction grade ≥2b), and symptomatic intracranial hemorrhage. RESULTS: We evaluated 93 patients with COVID-19 with large vessel occlusion who underwent MT (median age, 71 years [interquartile range, 59-79]; 63 men [67.7%]). Median pretreatment National Institutes of Health Stroke Scale and Alberta Stroke Program Early CT Score were 17 (interquartile range, 11-21) and 8 (interquartile range, 7-9), respectively. Anterior circulation acute ischemic stroke represented 93.5% of cases. The rate modified Thrombolysis in Cerebral Infarction 2b to 3 was 79.6% (74 patients [95% CI, 71.3-87.8]). Thirty-day mortality was 29% (27 patients [95% CI, 20-39.4]). Early neurological improvement was 19.5% (17 patients [95% CI, 11.8-29.5]), and symptomatic intracranial hemorrhage was 5.4% (5 patients [95% CI, 1.7-12.1]). Patients who died at 30 days exhibited significantly lower lymphocyte count, higher levels of aspartate, and LDH (lactate dehydrogenase). After adjustment for age, initial National Institutes of Health Stroke Scale, Alberta Stroke Program Early CT Score, and successful reperfusion, these biological markers remained associated with increased odds of 30-day mortality (adjusted odds ratio of 2.70 [95% CI, 1.21-5.98] per SD-log decrease in lymphocyte count, 2.66 [95% CI, 1.22-5.77] per SD-log increase in aspartate, and 4.30 [95% CI, 1.43-12.91] per SD-log increase in LDH). CONCLUSIONS: The 29% rate of 30-day mortality after MT among patients with COVID-19 is not negligible. Abnormalities of lymphocyte count, LDH and aspartate may depict a patient's profiles with poorer outcomes after MT. Registration: URL: https://www.clinicaltrials.gov. Unique identifier: NCT04406090.

Navigability of a long sheath in the lateral dural sinuses facilitated by the pilot balloon technique: technical note.

Dural sinus stenting is an increasingly recognized intervention for the treatment of lateral sinus stenosis. This procedure can be challenging in tortuous anatomy and in the presence of intraluminal septa because of poor trackability and crossability of long sheath commonly used for stenting. We report a technique using a pilot angioplasty balloon positioned at the distal end of the long sheath that improves its navigability in dural sinuses and facilitated the intervention.

Primary conus medullaris arteriovenous shunt and secondary lumbo-sacral epidural arteriovenous fistula: One malformation can hide another.

We report an anatomical-based association between conus medullaris pial arteriovenous shunt that drain caudally towards the lumbosacral area with very delayed onset of an acquired lumbar epidural shunt, draining secondarily towards intradural veins and responsible for a venous congestive myelopathy with identical clinical symptoms. These patients require close clinical and imaging follow-ups in order to propose adequate treatments before onset of irreversible neurological deficits. MRA should include the lumbo-sacral area in its field of view.

Mechanical Thrombectomy for Acute Ischemic Stroke Amid the COVID-19 Outbreak: Decreased Activity, and Increased Care Delays.

BACKGROUND AND PURPOSE: The efficiency of prehospital care chain response and the adequacy of hospital resources are challenged amid the coronavirus disease 2019 (COVID-19) outbreak, with suspected consequences for patients with ischemic stroke eligible for mechanical thrombectomy (MT). METHODS: We conducted a prospective national-level data collection of patients treated with MT, ranging 45 days across epidemic containment measures instatement, and of patients treated during the same calendar period in 2019. The primary end point was the variation of patients receiving MT during the epidemic period. Secondary end points included care delays between onset, imaging, and groin puncture. To analyze the primary end point, we used a Poisson regression model. We then analyzed the correlation between the number of MTs and the number of COVID-19 cases hospitalizations, using the Pearson correlation coefficient (compared with the null value). RESULTS: A total of 1513 patients were included at 32 centers, in all French administrative regions. There was a 21% significant decrease (0.79; [95%CI, 0.76-0.82]; P<0.001) in MT case volumes during the epidemic period, and a significant increase in delays between imaging and groin puncture, overall (mean 144.9±SD 86.8 minutes versus 126.2±70.9; P<0.001 in 2019) and in transferred patients (mean 182.6±SD 82.0 minutes versus 153.25±67; P<0.001). After the instatement of strict epidemic mitigation measures, there was a significant negative correlation between the number of hospitalizations for COVID and the number of MT cases (R2 -0.51; P=0.04). Patients treated during the COVID outbreak were less likely to receive intravenous thrombolysis and to have unwitnessed strokes (both P<0.05). CONCLUSIONS: Our study showed a significant decrease in patients treated with MTs during the first stages of the COVID epidemic in France and alarming indicators of lengthened care delays. These findings prompt immediate consideration of local and regional stroke networks preparedness in the varying contexts of COVID-19 pandemic evolution.

Acute Stroke Management During the COVID-19 Pandemic: Does Confinement Impact Eligibility for Endovascular Therapy?

During the coronavirus disease 2019 (COVID-19) pandemic, the World Health Organization recommended measures to mitigate the outbreak such as social distancing and confinement. Since these measures have been put in place, anecdotal reports describe a decrease in the number of endovascular therapy (EVT) treatments for acute ischemic stroke due to large vessel occlusion. The purpose of our study was to determine the effect on EVT for patients with acute ischemic stroke during the COVID-19 confinement. In this retrospective, observational study, data were collected from November 1, 2019, to April 15, 2020, at 17 stroke centers in countries where confinement measures have been in place since March 2020 for the COVID-19 pandemic (Switzerland, Italy, France, Spain, Portugal, Germany, Canada, and United States). This study included 1600 patients treated by EVT for acute ischemic stroke. Date of EVT and symptom onset-to-groin puncture time were collected. Mean number of EVTs performed per hospital per 2-week interval and mean stroke onset-to-groin puncture time were calculated before confinement measures and after confinement measures. Distributions (non-normal) between the 2 groups (before COVID-19 confinement versus after COVID-19 confinement) were compared using 2-sample Wilcoxon rank-sum test. The results show a significant decrease in mean number of EVTs performed per hospital per 2-week interval between before COVID-19 confinement (9.0 [95% CI, 7.8-10.1]) and after COVID-19 confinement (6.1 [95% CI, 4.5-7.7]), (P<0.001). In addition, there is a significant increase in mean stroke onset-to-groin puncture time (P<0.001), between before COVID-19 confinement (300.3 minutes [95% CI, 285.3-315.4]) and after COVID-19 confinement (354.5 minutes [95% CI, 316.2-392.7]). Our preliminary analysis indicates a 32% reduction in EVT procedures and an estimated 54-minute increase in symptom onset-to-groin puncture time after confinement measures for COVID-19 pandemic were put into place.

Ischemic stroke in giant-cell arteritis: French retrospective study.

Acute cerebrovascular ischemic events are a rare and severe complication of giant cell arteritis (GCA). We aimed to determine the prevalence of GCA-related stroke, the overall survival and the relapse-free survival in patients with GCA. A multicentric retrospective analysis was performed on 129 patients with GCA diagnosed between September 2010 and October 2018 in two University Hospitals. Among 129 GCA patients, 18 (16%) presented an acute ischemic cerebrovascular event. Patients with stroke were older (83 [67-96] years versus 76 [58-96]; p = 0.014) and more frequently males (61% versus 30%; p = 0.014) than those without stroke. The frequency of anterior ischemic optic neuropathy was higher in patients with stroke (n = 6, 33%) than patients without stroke (n = 12, 11%)(p = 0.02). Overall survival was significantly decreased in GCA patients with stroke (4.4 months), comparatively to patients without stroke (221.7 months; log rank test = 0.006). The 3-years relapse-free survival was decreased in patients with stroke (8.42 versus78.0 months; log rank = 0.0001), as well as the time with sustained remission (78 versus 139 months; log rank test = 0.0004). This study shows the prevalence and risk factors of ischemic stroke in GCA.

Inflammatory disorders associated with trisomy 8-myelodysplastic syndromes: French retrospective case-control study.

OBJECTIVE: We report cases of myelodysplastic syndrome/myeloproliferative neoplasms (MDS/MPN) with trisomy 8 associated with inflammatory and autoimmune diseases (IADs). METHOD: Data for 21 patients with trisomy 8-MDS/MPN and IADs were analyzed and compared to 103 patients with trisomy 8-MDS/MPN without IADs. RESULTS: The median age of MDS/MPN patients with IADs was 67 [59-80]. The IADs were Behçet's-like disease in 11 (52%) patients, inflammatory arthritis in 4 (19%) and Sjögren's syndrome, autoimmune hemolytic anemia, aseptic abscess, periarteritis nodosa, Sweet's syndrome and unclassified vasculitis in one patient each. Overall, 17/21 (81%) patients with IADs received treatment (88% with steroids), with complete and partial response in 7/17 (35%) and 8/17 (47%), respectively. The effect of MDS treatment on IADs could be assessed in seven patients receiving azacytidine: five achieved remission and two partial response. As compared with the 103 trisomy 8-MDS/MPN cases without IADs, those with IADs were more often non-European (P = 0.005) and had poor karyotype (P < 0.001). We found no difference in overall survival or acute myeloid leukemia progression between trisomy 8-associated MDS/MPN with and without IADs. CONCLUSION: The spectrum of IADs associated with trisomy 8-positive MDS/MPN is dominated by Behçet's-like disease. Steroid therapy is effective, but mostly sparing therapies are necessary. Azacytidine could be an effective alternative.

Morphology of the lateral fossa of the brain (sylvian valley): anatomo-radiological aspects and surgical application.

INTRODUCTION: The knowledge acquired on the lateral fossa of the brain (LFB) is heterogeneous and incomplete. Our goal was to provide a morphological description of the LFB and analyze the impact of these descriptions on the surgical approach of the region. METHODS: The morphology of LFB was studied on 40 cerebral hemispheres of 20 right-handed subjects aged 18-55 years with an MRI of 1.5 T. The anatomo-radiological identification of the two section levels preceded the description of the shapes of the LFB. From these landmarks, the forms presented by the LFB were identified and described on each of the transverse, sagittal and frontal planes. The comparison of the proportion of shapes made it possible to identify the typical shapes at each section level and on each section plane. RESULTS: The average age of the subjects was 33 years with extremes of 19 and 54 years including 7 women and 13 right-handed men. According to the plane and the level of section, 6 typical morphologies of the LFB have been described, 2 of which were identical. The forms did not vary according to the cerebral hemisphere or the sex of the subject. The set of typical morphologies made it possible to determine a reference subject called NSK which presented the greatest number of typical morphological characteristics. CONCLUSION: Knowledge of LFB anatomical imaging is of paramount importance in the pre-surgical evaluation of pathologies in this region. The reference subject will be used for our future biometric and three-dimensional manual reconstruction work in this region.

Circumferential Thick Enhancement at Vessel Wall MRI Has High Specificity for Intracranial Aneurysm Instability.

Purpose To identify wall enhancement patterns on vessel wall MRI that discriminate between stable and unstable unruptured intracranial aneurysm (UIA). Materials and Methods Patients were included from November 2012 through January 2016. Vessel wall MR images were acquired at 3 T in patients with stable (incidental and nonchanging over 6 months) or unstable (symptomatic or changing over 6 months) UIA. Each aneurysm was evaluated by using a four-grade classification of enhancement: 0, none; 1, focal; 2, thin circumferential; and 3, thick (>1 mm) circumferential. Inter- and intrareader agreement for the presence and the grade of enhancement were assessed by using κ statistics and 95% confidence interval (CI). The sensitivity, specificity, and negative and positive predictive values of each enhancement grade for differentiating stable from unstable aneurysms was compared. Results The study included 263 patients with 333 aneurysms. Inter- and intrareader agreement was excellent for both the presence of enhancement (κ values, 0.82 [95% CI: 0.67, 0.99] and 0.87 [95% CI: 0.7, 1.0], respectively) and enhancement grade (κ = 0.92 [95% CI: 0.87, 0.95]). In unruptured aneurysms (n = 307), grade 3 enhancement exhibited the highest specificity (84.4%; 233 of 276; 95% CI: 80.1%, 88.7%; P = .02) and negative predictive value (94.3%; 233 of 247) for differentiating between stable and unstable lesions. There was a significant association between grade 3 enhancement and aneurysm instability (P < .0001). Conclusion In patients with intracranial aneurysm, a thick (>1 mm) circumferential pattern of wall enhancement demonstrated the highest specificity for differentiating between stable and unstable aneurysms. © RSNA, 2018.