L’aspergillome sphenoïdal : a propos d’un cas sphenoidalaspergilloma : a case report

Introduction : L’aspergillose sphénoïdale isolée est une pathologie rare potentiellement grave vu le risque d’extension neuro-méningée et orbitaire. malgré qu’elle est plus fréquente chez les immuno-déprimés, l’atteinte de l’immunocompétent a été décrite. a travers une observation, nous rapportons les caractéristiques clinico-radiologiques, thérapeutiques et évolutives de l’aspergillome sphénoïdal.Observutin : Homme de 35 ans a consulté pour des céphalées rétro-orbitaires évoluant depuis 3 mois. L’examen était normal à part une rhinorrhée purulente postérieure. Le scanner a montré un processus hétérogène comblant le sinus sphénoïdal associé à une lyse osseuse. Une sphénoïdotomiepar voie endonasale a permis de confirmer le diagnostic et de nettoyer les lésions aspergillaires. Un traitement par voriconazol a été associé avec évolution favorable après un recul de 18 mois.Conclusion : L’aspergillome sphénoïdale est l’un des diagnostics différentiel des tumeurs sphénoïdales chez l’immunocompétent. Le traitement est essentiellement chirurgical avec de très bons résultats. L’adjonction d’un antifongique systémique est controversée chez l’immunocompétent.Mots- clefs : aspergillome, sphénoïde, imagerie, Tumeur, chirurgieObjective : Isolated sphenoidalaspergillosis is rare but potentially grave because of the risk of neuro-meningeal and orbitalextension. althoughitis more common in immuno-compromised, the achievement of immunocompetent has been described. Through an observation, we report the clinic-radiological, therapeutic and evolutionary featuresof sphenoidalaspergilloma.Case-report : male 35 years consulted for retro-orbital headache lasting for 3 months. The exam was normal except a purulent rhinorrhea. The CT scan showed a heterogeneous process filling the sphenoid sinus associated with bone loss. Sphenoidotomy by an endonasal approach confirmed the diagnosis of aspergillosis lesions. Treatment with voriconazole was associated with favorable outcome after 18 months.Conclusion : Sphenoidal as pergilloma is a differential diagnosis of tumors in the immuno-competent. Treatment is mainly surgical with good results. The addition of a systemic antifungal agent is controversial in the immunocompetent.Keywords : aspergilloma, sphenoïd, imagery, tumor, surger

Budd-Chiari syndrome in a 25-year-old woman with Behçet's disease: a case report and review of the literature

<p>Abstract</p> <p>Introduction</p> <p>The risk that patients with Behçet's disease will develop thrombotic complications has been previously described. Although it is distributed worldwide, Behçet's disease is rare in the Americas and Europe. Even though the pathogenic mechanisms of vascular complications of Budd-Chiari syndrome in patients with Behçet's disease are unknown, severe vascular complications of Budd-Chiari syndrome associated with Behçet's disease seem to affect mainly young men.</p> <p>Case presentation</p> <p>We report a case of Budd-Chiari syndrome, a severe vascular complication that developed in a 25-year-old Afro-Brazilian woman with Behçet's disease.</p> <p>Conclusion</p> <p>Severe vascular complications of Budd-Chiari syndrome in patients with Behçet's disease are much more common in young adult male patients; we present a rare case of Budd-Chiari syndrome in a young Afro-Brazilian woman with Behçet's disease.</p

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

Amyloidosis in Behcet′s disease

Behcet′s disease (BD) is a multisystem vasculitis with protean manifestations. It is characterized by a heightened state of inflammation, although the factors that initiate and sustain this inflammation are not clear. We report some cases of BD-associated amyloidosis and have similar features. The patients developed nephrotic syndrome due to secondary amyloidosis, which was refractory to the immunosuppressive agents. Two patients expired and the third was lost to follow-up during the course. The BD complicated with amyloidosis is associated with high mortality despite the current aggressive therapy

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