A 35-year history of recurrent multiple cranial neuropathy due to primary Sjögren's syndrome.
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Biomedical subjects
Publications and source records attributed to M Zuber.
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OBJECTIVE: Differentiation between normal and abnormal physical state of the myocardium, not possible with conventional echocardiography, so far could be done with integrated backscatter (IBS) as a research tool only. METHODS: This study investigates myocardial texture analysis with new commercially available real time IBS in 12 normal individuals and in 18 patients with severe left ventricular dysfunction due to coronary artery disease (CAD) in 8 and dilated cardiomyopathy (DCM) in 10 patients. Analysis of IBS amplitude and cyclic variation (dB) in the parasternal long and short axis view of the septum and the posterior wall were measured and corrected with IBS curve of the blood to get absolute values. RESULTS: Compared to normal individuals patients with left ventricular dysfunction had a reduced myocardial cyclic variation (P<0.0001), which correlated to regional systolic wall thickening (r=0.64, P=0.001) and global shortening fraction (r=0.62, P<0.01). Although systolic wall thickening in the posterior wall was lower in CAD patients (% thickening, 11.9+/-10 vs. 21.9+/-8, P=0.004), absolute cyclic variation was reduced in both, CAD and DCM patients in the same order of magnitude. However, the higher maximal IBS amplitude in the posterior wall observed in CAD when compared to DCM patients (13.2+/-4.4 vs. 9.2+/-2.4 dB; P=0.002) indicate fibrosis or scar. The dissociation between cyclic variation and systolic wall thickening could implicate hybernating myocardium. CONCLUSION: Real-time IBS has progressed from research to routine as a tool to obtain additional and valuable information to conventional echocardiography in daily practice.
Patients undergoing mitral valve surgery are at risk of left ventricular failure in the immediate post-operative period. In order to understand better the mechanisms of post-operative haemodynamic instability, we used transoesophageal echocardiography to assess the immediate response of the left ventricle to mitral valve replacement for mitral regurgitation or stenosis. A decrease in left ventricular preload, despite adequate filling pressures, was common to both groups and suggests the presence of diastolic dysfunction. A marked impairment in global systolic pump function was observed only in the regurgitation group and correlated with the left ventricular afterload. Transoesophageal echocardiography provides valuable information on the individual changes in left ventricular function and its determinants after mitral valve replacement that are not reflected by haemodynamic measurements.
Replacement of the aortic valve exposes the left ventricle to myocardial ischaemia and imposes on it abrupt changes in loading conditions and geometry. We studied the immediate changes in the left ventricular function of patients undergoing aortic valve replacement by transoesophageal echocardiography. Patients with aortic regurgitation responded to surgery with a decrease in global systolic function associated with a fall in preload. Patients with aortic stenosis showed an impairment in myocardial contractility and ventricular filling, and their global systolic function did not improve despite the marked reduction in afterload. This can be a consequence of inadequate myocardial protection and intra-operative ischaemic injury of the hypertrophic myocardium. Information provided by transoesophageal echocardiography contributes to optimal and individualized management of the period immediately after aortic valve replacement.
OBJECTIVE: To evaluate the outcome of pregnancy in women with congenital shunt lesions. SETTING: Retrospective study in a tertiary care centre. METHODS: Pregnancy history was obtained by a standardised questionnaire and medical records were reviewed. PATIENTS: 175 women were identified, at a mean (SD) age of 42 (14) years. Pregnancies occurred in 126 women: 50 with an atrial septal defect, 22 with a ventricular septal defect, 22 with an atrioventricular septal defect, 19 with tetralogy of Fallot, and 13 with other complex shunt lesions. RESULTS: 309 pregnancies were reported by 126 woman (2.5 (1.6) pregnancies per woman). The shortening fraction of the systemic ventricle was 40 (8)%, and 98% were in New York Heart Association class I-II at last follow up. Spontaneous abortions occurred in 17% of pregnancies (abortion rate, 0.4 (0.9) per woman). Gestational age of the 241 newborn infants was 8.8 (0.8) months. There were no maternal deaths related to pregnancy. Pre-eclampsia and embolic events were observed in 1.3% and 0.6%, respectively of all pregnancies. Women with complex shunt lesions more often underwent caesarean section (70% v 15-30%, p = 0.005) and gave birth to smaller babies for equivalent gestation (2577 (671) g v 3016 (572) to 3207 (610) g, p < 0.05). The recurrence risk of congenital heart disease was 2.5%. CONCLUSIONS: The outcome of pregnancy is favourable in women with congenital shunt lesions if their functional class and their systolic ventricular function are good. Such patients can be reassured.
UNLABELLED: Meningeal metastatic disease usually occurs as a complication of a brain tumor and is exceptionally isolated in patients with solid tumors. We report the case of a 74-year-old woman admitted for mechanical S1 sciatica refractory to drug therapy. She had been treated for breast cancer three years earlier. Physical findings were pain upon hyperextension of the lumbar spine and absence of the ankle jerks. Analysis of cerebrospinal fluid sampled during an intrathecal glucocorticoid injection showed 1 g/L of protein and 11 normal cells per mm3. Grade 3 L5-S1 spondylolisthesis was seen on plain radiographs, computed tomography scans, and magnetic resonance imaging scans. At that point, the patient developed sphincter dysfunction and motor loss in the left lower limb in the distribution of several nerve roots. Findings were normal from a myelogram and a magnetic resonance imaging study of the brain. A repeat cerebrospinal fluid analysis showed 1.1 g/L of protein and 5 cells/mm3. Because of the discrepancy between the clinical and imaging study findings, the patient was transferred to a neurology department. A third cerebrospinal fluid study showed numerous adenocarcinoma cells, and a repeat magnetic resonance imaging demonstrated a mass in the dural sac opposite L2. A program of monthly intrathecal methotrexate injections was started. A fatal meningeal relapse occurred eight months later. CONCLUSION: This case shows that a leptomeningeal metastasis can cause isolated nerve root pain, and demonstrates the diagnostic value of magnetic resonance imaging and cerebrospinal fluid cytology in patients with atypical symptoms, particularly when there is a history of malignant disease.
We have described recently that TNBS-induced colitis, an animal model of chronic inflammatory bowel disease (IBD), can be cured by treatment with anti-CD44v7. This finding led us to evaluate whether CD44v7 may be of functional importance in patients with IBD. Expression of CD44 variant isoforms (CD44v) has been evaluated on PBMC of 46 patients with IBD, 43 patients with autoimmune diseases not affecting the gastrointestinal tract, 26 patients with nonautoimmune disease, and 24 healthy donors. In all groups, expression of CD44v on freshly harvested PBMC was not above or was borderline above background levels. After in vitro stimulation, expression of CD44 standard (CD44s) and CD44v6 was strongly up-regulated. Exclusively on PBMC of patients with autoimmune disease, high expression of CD44v3 and CD44v7 was observed. CD44v3 and CD44v7 were mainly expressed on subsets of CD4+ lymphocytes, B cells, and monocytes; CD44v6 was predominantly detected on CD4+ and CD8+ cells. Considering functional activity, CD44v7 apparently exerted a dual effect. After culturing PBMC in the presence of anti-CD44v7, a higher percentage of cells produced IL-10. This was irrespective of whether the PBMC were derived from healthy donors or from patients with autoimmune disease or IBD. On the other hand, PBMC of all donors proliferated upon cross-linking of CD3 and CD44s or CD3 and CD44v6. Instead, costimulatory activity of CD44v7 was seen only in PBMC of patients with autoimmune disease and IBD. Because expression and function of CD44v7 in patients with systemic autoimmune disease and IBD have been much like the ones in mice suffering of TNBS-induced colitis, it is tempting to speculate that blockade of CD44v7 could also be of therapeutic relevance in the human diseases.
Angiitis of the central nervous system is a rare disease which may result from numerous causes responsible for the presence of inflammatory lesions of the vascular wall. These inflammatory lesions may sometimes be associated with necrosis. Cerebral vessels of all sizes may be involved. The clinical presentation is highly variable, with focal to diffuse manifestations and acute to chronic evolution. Angiography is the cornerstone diagnostic procedure, showing multiple segmental stenoses of the cerebral arteries sometimes separated by fusiform dilatations. Although suggestive, this angiographical pattern is not unequivocal and other causes must be carefully ruled out. Only cerebral and/or leptomeningeal biopsy can provide a definite diagnosis of cerebral angiitis but this invasive diagnostic procedure is not performed in the majority of cases. Among the numerous causes of cerebral angiitis, one can individualize infectious diseases, primary systemic angiitis with cerebral involvement, angiitis secondary to various systemic diseases and other miscellaneous causes such as drug abuse or neoplasm.
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INTRODUCTION: The optimal inguinal hernia repair technique is a matter of debate. The Shouldice operation has long been regarded as the gold standard. While specialized hernia clinics have reproduced good results, a recurrence rate for trainees and non-specialized surgeons of between 10% and 15% seems to be more common. Laparoscopic hernia surgery has brought decreasing postoperative morbidity, low post-operative disability and early return to work. These advantages of a tension-free technique are due to the use of a prosthetic mesh. It is largely the efforts of Gilbert, Lichtenstein and Rutkow/Robbins which have popularized the use of prosthetic mesh by an open anterior approach in the USA. METHODS: Since January 1997 we have prospectively evaluated the open mesh plug repair described by Rutkow/Robbins in Basel and Lugano. We have operated on 110 patients (103 male, 7 female). All patients were interviewed 4 weeks postoperatively at our clinic. RESULTS: There were no major complications. Two patients underwent reoperation for drainage of a haematoma. There was no early recurrence or infection. After 4 weeks 82% of all patients were back to normal activity. An early return to work has been noted (10 days on average for sedentary workers). CONCLUSION: The main advantages of this technique are simplicity, low cost, reduced post-operative disability and early return to work. It is much too early to say anything concerning recurrence in this series.
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Advanced Breast Biopsy Instrumentation (ABBI) allows radiologically guided stereotactic excision of non-palpable radiodense lesions with high accuracy. Tissue cylinders of 5, 10, 15 or 20 mm diameter and of variable lengths can be removed very accurately under local anaesthesia and on an outpatient basis. Thirty-six patients with suspicious clusters of microcalcifications (n = 29) and with round lesions (n = 7) of the breast were qualified for ABBI. We were able to perform the excisional biopsy in a total of 34 patients. The breast of one woman was too small to safely fit into the system and in another woman the lesion could not be visualized by the system. In 2/34 cases (6%), the excision was imprecise due to slight dislocation of the breast parenchyma by the advancing cylinder knife. In one case (3%), ABBI missed the target within a dense mastopathic breast. In all cases the excisions were well tolerated. No wound complications occurred and the cosmetic result was excellent. Histology revealed 28 benign (82%) and 6 malignant (18%) lesions. Among the 27 small microcalcifications there were 3 invasive carcinomas, 3 ductal carcinomas in situ (DCIS), 1 lobular hyperplasia, 14 mastopathies, 1 fibroadenoma, 1 duct papilloma and 4 calcifications in scars. Four of the 7 round-shaped lesions were found to be fibroadenomas, 1 lobular hyperplasia, and 2 mastopathies. With the ABBI system, non-palpable breast lesions can be precisely localized and excised.
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