Intracardiac thrombus in Behçet’s disease

Behçet’s disease (BD) is a multisystem inflammatory disorder. Intracardiac thrombus (ICT) formation is an uncommon but important complication of BD. Of the cases of Behçet’s disease, we selected those with ICT. All patients fulfilled the diagnostic criteria of the International Study Group of Behçet’s disease. The ICT in each case was confirmed by ultrasonography, computed tomography and MRI. Clinical features and laboratory parameters were determined. Among our 518 patients with BD, 8 were diagnosed as having intracardiac thrombus (ICT). All were male; the mean age at the time of the ICT diagnosis was 30.8 years. The main presenting symptoms were hemoptysis, chest pain, and dyspnea. It was associated with pulmonary artery aneurysm and vena cava thrombosis in 3 cases each, pulmonary embolism, and lower limbs deep venous thrombosis in 1 case each. The coexistence of other cardiac complications was as follows: pericarditis in 2 cases, myocarditis, endomyocardial fibrosis, and coronary arteritis with consequent myocardial infarction in one case each. In all cases, echocardiography was sufficient to reach the diagnosis. Chest computed tomography performed in all cases led to the diagnosis of associated pulmonary vasculo-Behçet lesions in 4 cases. All patients received colchicine, anticoagulation, and corticosteroids. Seven patients were on immunosuppressant agents (2 patients received azathioprine and 5 cyclophosphamide). Clinical remission with ICT resolution was observed in 5 cases. Combined immunosuppressive therapy with prednisone and cyclophosphamide might be needed to treat ICT due to BD

Automated Analysis of Pedestrian Crossing Speed Behavior at Scramble-phase Signalized Intersections Using Computer Vision Techniques

This study investigates changes in pedestrian speed behavior following the implementation of a scramble phase. The aim is to demonstrate the feasibility of automatic collection of pedestrian data; and to study pedestrian speed variations with respect to design changes to intersection crossings. The results show that the average crossing speed is higher after the implementation of the scramble phase. Within the scramble, the average crossing speed is higher for diagonal crossing than side crosswalk crossing. The average crossing speed is lower for pedestrians crossing during the Walk interval. Pedestrians have higher speed through the first half of the crosswalk

Les manifestations neurologiques de la maladie de Behçet : analyse d’une série de 27 patients

International audiencePurpose. – To describe epidemiological and clinical characteristics of neurological involvement in Behçet’s disease (BD) and to determine a subgroup of patients at high risk for this complication.Patients and methods. – The medical notes of 105 patients with BD fulfilling the criteria of the international Study Group for Behçet’s disease were retrospectively reviewed. Patients were divided into two groups according to the presence (group 1) or not (group 2) of neurological and/or psychiatric involvement attributable to BD. The epidemiological, clinical and genetic (HLA B51 and MICA 6 frequency) features in the two groups were analysed and compared using the Kruskall-Wallis and the chi-square tests.Results. – Twenty-seven patients (25.7%) had clinical evidence of neurological involvement. They were 20 men and 7 women. The mean age at neurological onset was 34.26 ± 8.79 years. Nineteen patients (70.3%) had meningoparenchymal “MP” central nervous system involvement (brainstem: 9, hemispheric involvement: 6, spinal cord: 4, psychiatric involvement: 2, isolated pyramidal signs: 1, aseptic meningitis:1). Seven patients (25.9%) had cerebral large vessels involvement that is cerebral angio-Behçet “CAB” (intracranial hypertension: 5 cases due to cerebral venous thrombosis: 3 and pseudotumor cerebri: 2, cerebral haemorrhage: 1, cerebral arterial thrombosis: 1). One patient (3.7%) had both “MP” and “CAB” involvement. Headache was significantly more frequent in non-parenchymal patients. In group 1, complete recovery or improvement with mild neurological impairment was seen in 13 cases, improvement with severe disability in 3 cases, worsening in 1case, the course was stationary in 1 case and 3 patients died (11.2%). Arterial aneurysms were significantly more frequent in “CAB” subgroups than in subgroup 2.Conclusion. – Frequency of neurological involvements in BD was high in our study. Arterial aneurysms seem to be a risk factor to these complications. Cerebral angio-Behçet appears to be a protector factor against uveitis.Propos. – Analyser les caractéristiques cliniques de l’atteinte neurologique au cours de la maladie de Behçet (MB) et déterminer un sous-groupe de patients à haut risque pour cette complication.Patients et méthodes. – Cent cinq dossiers de patients atteints d’une MB répondant aux critères diagnostiques du groupe international d’étude sur la MB ont été rétrospectivement revus. Les patients ont été divisés en 2 groupes selon l’existence (groupe 1) ou non (groupe 2) de manifestations neurologiques et/ou psychiatriques attribuées à la MB. Les différentes caractéristiques cliniques et génétiques (HLA-B51 et MICA A6) ont été comparées au moyen des test de Kruskall Wallis et du χ2.Résultats. – Vingt-sept patients (25,7 %) ont présenté des manifestations neurologiques. Il s’agissait de 20 hommes et 7 femmes ayant un âge moyen de 34,26 ± 8,79 ans. Dix-neuf patients (70,3 %) avaient une atteinte centrale méningoparenchymateuse « MP » (tronc cérébral : 9, hémisphères cérébraux : 6, moelle : 4, atteinte psychiatrique : 2, syndrome pyramidal isolé : 1, méningite aseptique isolée : 1). Sept patients (25,9 %) avaient une atteinte des gros vaisseaux cérébraux qualifiée d’angio-Behçet cérébral (ABC) (hypertension intracrânienne : 5 cas dont 3 par thromboses veineuses cérébrales et 2 « idiopathiques », 1 thrombose artérielle cérébrale et 1 hémorragie cérébroméningée). Un patient (3,7 %) avait les 2 types d’atteinte. Parmi les signes cliniques les céphalées étaient significativement plus fréquentes dans le groupe « MP ». Dans le groupe 1, l’évolution des manifestations neurologiques (précisée dans 21 cas) s’est faite vers la rémission complète ou avec des séquelles minimes dans 13 cas, la persistance de séquelles importantes dans 3 cas, l’aggravation dans 1 cas et la non-amélioration dans 1 cas ; trois patients sont décédés. Aucun élément clinique ou paraclinique n’avait de valeur pronostique significative dans le groupe 1. Par comparaison au groupe 2, les anévrismes étaient significativement plus fréquents dans le sous-groupe « ABC ». L’uvéite était plus fréquente dans le sous-groupe « ABC » que dans le sous-groupe « MP » de façon significative et que le groupe 2 mais avec une différence à la limite de la signifiance.Conclusion. – La fréquence de l’atteinte neurologique dans la MB est élevée dans notre série (25,7 %). Les anévrismes artériels semblent constituer un facteur de risque pour l’angio-Behçet cérébral. L’angio-Behçet cérébral paraît protéger les patients contre l’uvéite

Successful treatment of refractory adult onset Still’s disease with rituximab

Adult-onset Still’s disease (AOSD) is an uncommon inflammatory condition of unknown origin. In chronic disease, joint involvement is often predominant and erosions are noted in one third of patients. Therapeutic strategies derive from observational data. Corticosteroids are usually the first-line treatment. With inadequate response to corticosteroids, methotrexate appears the best choice to control disease activity and allow for tapering of steroid use. For refractory disease, biological therapy seems the most promising. We report here the case of a 38-year-old female patient with AOSD refractory to cytotoxic agents, treated by rituximab infusion therapy with favorable outcome

EULAR recommendations for the management of Behçet disease

Objectives: To develop evidence-based European League Against Rheumatism (EULAR) recommendations for the management of Behcet disease (BD) supplemented where necessary by expert opinion. Methods: The multidisciplinary expert committee, a task force of the EULAR Standing Committee for Clinical Affairs (ESCCA), consisted of nine rheumatologists (one who was also a clinical epidemiologist and one also a Rehabilitation Medicine doctor), three ophthalmologists, one internist, one dermatologist and one neurologist, representing six European countries plus Tunisia and Korea. A patient representative was also present. Problem areas and related keywords for systematic literature research were identified. Systematic literature research was performed using Medline and the Cochrane Library databases from 1966 through to December 2006. A total of 40 initial statements were generated based on the systematic literature research. These yielded the final recommendations developed from two blind Delphi rounds of voting. Results: Nine recommendations were developed for the management of different aspects of BD. The strength of each recommendation was determined by the level of evidence and the experts' opinions. The level of agreement for each recommendation was determined using a visual analogue scale for the whole committee and for each individual aspect by the subgroups, who consider themselves experts in that field of BD. There was excellent concordance between the level of agreement of the whole group and the "experts in the field". Conclusion: Recommendations related to the eye, skin-mucosa disease and arthritis are mainly evidence based, but recommendations on vascular disease, neurological and gastrointestinal involvement are based largely on expert opinion and uncontrolled evidence from open trials and observational studies. The need for further properly designed controlled clinical trials is apparent