Haematogenous brain abscess complicating a case of Austrian syndrome

Austrian syndrome includes pneumococcal endocarditis, meningitis and rupture of the aortic valve. This study reports a case with a haematogenous brain abscess. Physicians should be aware of the risk factors and dramatic evolution of this rare disease, to avoid delays in diagnosis, and to prevent embolic complications and rupture of the aortic valve.     

Aneurysms of the sinus of Valsalva. Report of seven cases and review of the literature

The aneurysms of the sinuses of valsalva are relatively rare. The first case was described by Thurnam in 1840. Since then, more than 200 cases have been reported; in most surgery was performed. The lesions of the aortic sinuses are either congenital or the result of the syphilis or of the bacterial endocarditis.Between 1967 and 1976, seven patients (four men and three women) with this pathology were admitted to the Hospital Cantonal of Geneva (HCG). Their ages ranged from 13 to 51 years. Clinical symptoms consisted of chest pain, palpitations, dyspnea, cardiac insufficiency and fever. In one patient, a sudden cardiovascular shock was present. Physical examination had revealed a systolic and/or diastolic thrill in four patients, and a systolic and diastolic murmur on the left sternal edge in all. An increase in the differential arterial pressure was found in all patients and hepatomegaly in two. The electrocardiograms showed signs of left ventricular hypertrophy in five patients, right ventricular hypertrophy in four, incomplete right bundle branch block in one and signs of a subendocardial ischemic lesion in another. The chest films indicated a severe or moderate heart dilatation in all seven patients and an enlarged hilar area in six.All patients had been subject to cardiac catheterization. Rightsided pressures were increased in the majority of patients. Arterial and pulmonary capillary pressures were increased in all. A correct preoperative diagnosis had been made only twice.Cardiac surgery had been performed with cardiopulmonary bypass in all patients. The aneurysm was ruptured into the right ventricle in four patients and unruptured in the others. The lesion originated from the right coronary sinus in six patients and from both right and left coronary sinuses in one. Ventricular septal defects (three) and aortic lesions (five) were also found. The postoperative course was uneventful and all patients are still alive.     

Whole-Genome Analysis of Exserohilum rostratum from an Outbreak of Fungal Meningitis and Other Infections

Exserohilum rostratum was the cause of most cases of fungal meningitis and other infections associated with the injection of contaminated methylprednisolone acetate produced by the New England Compounding Center (NECC). Until this outbreak, very few human cases of Exserohilum infection had been reported, and very little was known about this dematiaceous fungus, which usually infects plants. Here, we report using whole-genome sequencing (WGS) for the detection of single nucleotide polymorphisms (SNPs) and phylogenetic analysis to investigate the molecular origin of the outbreak using 22 isolates of E. rostratum retrieved from 19 case patients with meningitis or epidural/spinal abscesses, 6 isolates from contaminated NECC vials, and 7 isolates unrelated to the outbreak. Our analysis indicates that all 28 isolates associated with the outbreak had nearly identical genomes of 33.8 Mb. A total of 8 SNPs were detected among the outbreak genomes, with no more than 2 SNPs separating any 2 of the 28 genomes. The outbreak genomes were separated from the next most closely related control strain by ∼136,000 SNPs. We also observed significant genomic variability among strains unrelated to the outbreak, which may suggest the possibility of cryptic speciation in E. rostratum.     

Movement- and task-related activations of motor cortical areas: A positron emission tomographic study

Using repeated measurements of regional cerebral blood flow with positron emission tomography, we investigated the regional cortical activations induced in 10 normal subjects, by two different finger motor tasks, i.e., a repeated flexion–extension of all fingers and a repeated flexion–extension of the middle finger. The all-finger movement only activated the primary sensorimotor cortex (SM) and the supplementary motor area (SMA) contralateral to the movement. However, the activation of the SMA was clearly task related during this motor task, because it was only observed when the movement was triggered by an auditory cue but not when it was self-paced. The middle finger movement was performed during self-paced conditions. It induced a much more complex pattern of activation than the all-finger movement, characterized by a high degree of SM and SMA activation contralateral to the side of the movement, as well as a slight ipsilateral activation of these areas. We suggest that this pattern of cortical activation may reflect the process of individuating finger movement or the early stages of motor learning of this unusual and technically difficult movement. Our data also confirm that the SM activation is closely linked to the intrinsic parameters of the movement; while the SMA may be activated by different aspects of the movement realization and preparation.     

Tolerance and short term efficacy of rituximab in 43 patients with systemic autoimmune diseases

OBJECTIVE: To assess the tolerance and efficacy of rituximab in patients with various autoimmune diseases seen in daily rheumatological practice. METHODS: 866 rheumatology and internal medicine practitioners were contacted by e-mail to obtain the files of patients treated with rituximab for systemic autoimmune diseases. Patients with lymphoma were analysed if the evolution of the autoimmune disease could be evaluated. RESULTS: In all, 43 of 49 cases could be analysed, including 14 with rheumatoid arthritis (RA), 13 with systemic lupus erythematosus (SLE), six with primary Sjogren's syndrome (pSS), five with systemic vasculitis, and five with other autoimmune diseases. Rituximab was prescribed for lymphoma in two patients with RA and two with pSS. In the 39 other cases, rituximab was given because of the refractory character of the autoimmune disease. The mean follow up period was 8.3 months (range 2 to 26). There were 11 adverse events in 10 patients and treatment had to be discontinued in six. Efficacy was observed in 30 patients (70%): RA 11, SLE 9, pSS 5, vasculitis 2, antisynthetase syndromes 2, sarcoidosis 1. The mean decrease in corticosteroid intake was 9.5 mg/d (range 0 to 50) in responders. Seven patients experienced relapse after mean 8.1 months (5 to 15). Three patients died because of refractory autoimmune disease. CONCLUSIONS: Despite absence of marketing authorisation, rituximab is used to treat various refractory autoimmune diseases in daily rheumatological practice. This study showed good tolerance and short term clinical efficacy, with marked corticosteroid reduction in patients with SLE, pSS, vasculitis, and polymyositis.     

Successful Long-Term Outcome of the First Combined Heart and Kidney Transplant in a Patient with Systemic AL Amyloidosis

Simultaneous cardiac and renal involvement is associated with a particularly poor prognosis in patients with AL amyloidosis (AL-A). We report the first case of a successful long-term outcome of combined heart and kidney transplantation not followed by autologous stem cell transplantation in a patient with systemic AL-A. The recipient was a 46-year-old man with end-stage renal failure associated with serious cardiac involvement in the context of AL-A. Before transplantation, two courses of oral melphalan plus prednisone induced partial hematologic remission, as shown by the decrease in circulating free light chain with no improvement of renal or heart function. The patient underwent combined heart and kidney transplantation as a rescue treatment. During the follow-up period (36 months), plasma cell dyscrasia remains in complete remission, with normal free lambda light chain levels and no recurrence of amyloid deposition on heart and kidney grafts. This case report demonstrates that combined heart and kidney transplantation not systematically associated with stem cell transplantation may be considered an additional therapeutic option in AL-A patients with severe organ dysfunction and partial hematologic remission.     

Thoracic splenosis: Case report of a symptomatic case

Thoracic splenosis is the autotransplantation of splenic tissue in the left thoracic cavity as a result of a splenic injury. This rare pathology is usually asymptomatic and may be discovered on incidental imaging, but the diagnosis often requires invasive procedures such as surgery in order to eliminate a neoplasic origin. We report a rare symptomatic case of a 39-year-old man presenting with chest pain and multiple nodules revealed on a computed tomography scan. The patient underwent a surgical exploration and the pathological studies concluded to a thoracic splenosis. Indeed, the previous medical history of the patient revealed a left thoraco-abdominal traumatism during childhood. The aim of this paper is to emphasize that the diagnosis can now be performed using only imaging techniques such as technetium-99 sulfur colloid or labelled heat-denatured red blood cell scintigraphy to avoid unnecessary invasive procedures including thoracotomy.     

Hand-assisted Laparoscopic Aortoiliac Surgery: Preliminary Report of 25 cases

Introduction: Since a few years vascular surgeons are developing laparoscopic vascular techniques. We report our preliminary results in this area, using the hand-assisted technique.

Materials and methods: Twenty-five patients were operated between February and December 2001 using the HandPort®-system. Indications were occlusive aortoiliac disease or infrarenal aortoiliac aneurysmal disease. Results: Mean operating time was 180’; mean aortic clamping time was 37’; mean blood loss was 521 mL. Mean laparotomy length was 7,9 cm. A conversion to a larger laparotomy was necessary in two patients. Mean hospital stay was seven days. Operative mortality was 4% (one postoperative death).

Conclusions: Hand-assisted laparoscopic aortoiliac surgery is feasible in community hospital settings.