[Neurological involvement of sarcoidosis: current diagnostic and therapeutic strategies]. / Atteinte neurologique de la Sarcoïdose : stratégies diagnostiques et thérapeutiques actuelles.

Neurosarcoidosis (NS) is a rare but severe form of sarcoidosis. NS is associated with significant morbidity and mortality. Mortality is about 10% at 10 years with more than 30% of patients who have a significant disability. The most frequent features are cranial neuropathy (the facial and optic nerve most commonly affected), cranial parenchymal lesions, meningitis, spinal corn abnormalities (20-30%) and more rarely peripheral neuropathy (approximately 10-15%). The challenge of diagnosis is to eliminate other diagnoses. Atypical presentations should make to discuss the need for cerebral biopsy in order to highlight the presence of granulomatous lesions while eliminating alternative diagnosis. Therapeutic management is based on corticosteroid therapy and immunomodulators. There are no comparative prospective study to allow us to define the first-line immunosuppressive treatment and the therapeutic strategy in refractory patients. Conventional immunosuppressants such as methotrexate, mycophenolate mofetil and cyclophosphamide are commonly used. Data on the efficacy of anti-TNFα (including infliximab) in refractory and/or severe forms are increasing during the last ten years. Additional data is necessary to assess their interest in first line in patients with severe involvement and a significant risk of relapse.

[Cardiac sarcoidosis: Diagnosis, prognosis and therapeutics]. / Sarcoïdose cardiaque : stratégies diagnostiques et thérapeutiques actuelles.

Sarcoidosis is a systemic granulomatous disease characterized by pulmonary involvement in most patients and more rarely by extrapulmonary involvement such as ocular, skin, salivary, lymph nodes and joints damages. Neurological and cardiac involvements are uncommon but are associated with increased morbidity and mortality. Cardiac sarcoidosis affects 5 to 20% of patients depending on the studies and autopsy studies even report cardiac involvement in 25% of sarcoidosis patients. This review aims to summarise main data on the diagnostic value of the different imaging techniques in cardiac sarcoidosis and to also detail the management of these patients who require a multidisciplinary approach.

Neurosarcoidosis: signs, course and treatment in 35 confirmed cases.

Thirty-five cases of biopsy-proven sarcoidosis with neurologic manifestations are reported. Neurosarcoidosis was the presenting symptom in 31% of cases and the only clinical manifestation in 17%. Mean follow-up time was 48 months. Central nervous system involvement was observed in 37% and meningitis in 40% of patients. Other manifestations were cranial nerve palsies (37%), peripheral neuropathy (40%), and myopathy (26%). Multiple neurologic manifestations were present in 51% of cases. All but 4 were treated with corticosteroids. Another immunosuppressive agent or cerebral irradiation was added in 6 and 2 patients, respectively. Complete recovery was observed in 46%, improvement in 46%, 4% remained stable, and 4% worsened. There were no deaths. We advocate treating neurosarcoidosis with corticosteroids as early as possible. If the patient's condition worsens, additional immunosuppressive agents or cerebral irradiation is warranted.

Diagnosis of primary cardiac sarcoma. Report of 4 cases and review of the literature.

The authors report the most extensive series of malignant primitive cardiac tumours. The patients presented with pericarditis in three cases and with a ventricular rhythm disorder in one patient. The diagnosis was made by non-invasive imaging techniques in three of the four. Angiography was carried out in three cases and was the determining diagnostic procedure in one. Diagnosis of the cardiac tumour was made prior to death in all cases. A histological diagnosis was obtained surgically in two cases. From the therapeutic viewpoint, surgical excision followed by chemotherapy and/or radiotherapy may prolong life. The average survival period of our patients was 11 months. The prognosis appears to be better after early surgical intervention. In view of the variability of the clinical features of cardiac sarcomas, cardiac computed tomography scanning is a valuable complement to cross-sectional echocardiography and, in our opinion, essential for early diagnosis.

[Surgery of endomyocardial fibrosis. Apropos of 32 cases]. / Chirurgie des fibroses endomyocardiques. A propos de 32 cas.

From 1971 to 1984, 32 patients with endomyocardial fibrosis (EMF) were treated by endocardial resection (decortication) and valve replacement. The population consisted in 20 men and 12 women (age ranged from 8 to 64); 19 patients were european and 13 african. All were symptomatic, 78 p. 100 in stages III or IV of the NYHA. Hypereosinophilia was detected in 21 patients and its cause was determined in 11 cases. Cardiac involvement was biventricular in 22 patients and monoventricular in 10 patients. Six patients died in the immediate postoperative period and 6 late deaths were observed, owing to extracardiac causes in 4. There were no recurrence of EMF. Despite a high mortality rate, the authors suggest that all symptomatic EMF should benefit from endocardial resection.

[Potential course of lesions of the ascending aorta in dystrophic aortic insufficiency]. / Potentiel évolutif des lésions de l'aorte ascendante dans les insuffisances aortiques dystrophiques.

Dystrophic aortic insufficiencies represent at present time an important etiology of operated on pure and voluminous aortic regurgitations (n = 95, i.e. 28.1% of cases followed by our working team). The valvular dysplasic lesions by which they are characterized were associated in 2 out of 3 cases with parietal lesions of the ascending aorta: annulo-ectasic disease (n = 42) or important non-aneurysmal dilatation of the ascending aorta (n = 20). However, these valvular dysplasias may also appear isolated in an aorta morphologically normal at peroperative examination (n = 33). Among the 42 patients suffering from, annulo-ectasic disease (group 1) the type of surgical correction varied with the period of operation. Valvular replacement isolated (n = 3, group 1 a) or associated with aortic supracoronary surgery (n = 17, group 1b) were the procedures before 1978. Since then, 22 patients (group 1c) had a total replacement of the ascending aorta were subjected to valvular interventions (19 replacements, 1 valvuloplasty). When performed, parietal biopsy of the aorta showed in 11 out of 15 cases signs of cystic medio-necrosis. After a delay of 53 +/- 40 months, 11 out of 20 patients of group 1a and 1b died, 5 of them from the evolution of lesions at the ascending aorta (3 ruptures and 2 dissections); one patient was reoperated 12 years after the placing of a supracoronary tube, because of the development of a voluminous aneurysm of the sinuses of Valvalva which initially were only simply dilated.(ABSTRACT TRUNCATED AT 250 WORDS)

[Traumatic aorto-right ventricular fistula. Apropos of a case]. / Fistule aorto-ventriculaire droite traumatique. A propos d'une observation.

The authors report the case of a 19 year old young man with an aorto-right ventricular fistula caused by a stab wound. The diagnosis was not made initially during surgery to control haemorrhage (left hemothorax due to a damaged internal mammary artery). However, three weeks later, a continuous murmur was detected and the diagnosis established by echocardiography and cardiac catheterisation. The lesions were repaired by open heart surgery under cardiopulmonary bypass. The authors describe the main features of this condition based on their own experience and a review of the literature.

[Nailfold capillaroscopy. Comparison of 100 subjects over 65 years of age and of 100 young adults]. / Capillaroscopie periungueale. Comparaison de 100 sujets âgés de plus de 65 ans et de 100 adultes jeunes.

Nailfold capillary microscopy patterns in 100 patients aged more than 65 years and free from inflammatory diseases were compared to those of 100 young healthy adults. A higher prevalence of arteriovenous sludge (36% vs 7%, p less than 10(-6)), increase in capillary loop length (12% vs. 0%, p less than 10(-3)) and especially prominent subpapillary plexus (63% vs 12%, p less than 10(-9)) was found in the geriatric group. Such capillary patterns cannot be considered as abnormal in patients aged more than 65 years. Enlargement of capillary loops and loss of capillaries were never encountered. Bushy capillary formations and hemorrhages were very uncommon in both groups.

[The advantages of histological samples in sarcoidosis. Retrospective multicenter analysis of 618 biopsies performed on 416 patients]. / Rentabilité des prélèvements histologiques au cours de la sarcoïdose. Analyse rétrospective multicentrique de 618 biopsies pratiquées chez 416 malades.

The diagnosis of sarcoidosis is based on the finding of an epithelioid granuloma at histological examination. A retrospective study of 618 biopsies obtained from 416 patients with sarcoid disease showed an average sensitivity of 69 p. 100. The sensitivity of guided biopsies, especially skin and lymph node biopsies, was almost 90 p. 100, whereas that of blind biopsies tended to increase with the degree of hazard involved, ranging from 49 p. 100 in proximal bronchial biopsy to 70 p. 100 in liver needle biopsy. When treatment is not urgently required, the Kveim test is safe, cheap and fairly sensitive (65 p. 100 positive results).

[Bacterial endocarditis on prosthetic valves]. / Endocardites infectieuses sur prothèses valvulaires.

Bacterial endocarditis is a rare, but often lethal, complication of cardiac valve replacement. The endocarditis is called "early" when it occurs within 2 months of the operation, and "late" when it develops after that period. Contamination of the prosthesis with bacteria may occur intra-operatively or post-operatively. The clinical diagnosis is often difficult in early endocarditis when another focus of infection is present and in late endocarditis in the absence of fever and positive blood cultures. Isolation of the pathogen from blood cultures is essential to the diagnosis and treatment. Therapeutic surgery now has wider indications than formerly. The incidence of this dangerous complication can only be reduced by well-planned and well executed prophylactic measures.

[Systemic vasculitis associated with non-streptococcal infection. Report of 4 cases]. / Vascularites systémiques satellites d'un foyer infectieux non streptococcique. A propos de 4 observations.

The responsibility of bacterial agents in the staring of systemic vasculitis is discussed on the basis of four cases: three men and one woman presenting with severe vasculitis which revealed or complicated a focus of infection without hematogenic dissemination. Two patients had gastrointestinal lesions and one had severe renal lesions. The vasculitis affected small caliber vessels, and in two out of four cases it involved arterioles of medium caliber with histological images resembling periarteritis nodosa. Antibiotic therapy alone was immediately effective against vasculitis. However, a purely cutaneous relapse occurred in three patients whose disease had regressed under an adjuvant treatment.

[Relapsing polychondritis and mesenteric panniculitis: apropos of 2 cases]. / Polychondrite atrophiante et panniculite mésentérique: à propos de 2 cas.

We report two cases of polychondritis associated with mesenteric panniculitis. Case 1. In February 1989, a woman born in 1949 presented with 40 degrees C fever accompanied by pain in the abdomen and pelvis. Eight days later, nodular skin lesions appeared on her lower limbs. The abdomen was swollen with gas and undepressible. An abdominal CT scan revealed partitioned peritoneal collections, and a guided needle aspiration produced a chylous fluid. Direct and indirect bacteriological examinations gave negative results. Histology showed intense inflammatory reaction with giant cells and lipophages, thereby confirming the presence of mesenteric panniculitis. Six months later, the development of chondritic lesions on the nose and the helix of the ear clinched the diagnosis of polychondritis. The patient was put on corticosteroid therapy for a few months, and in January this year (1993) she is durably asymptomatic. Case 2. In October 1977, a woman born in 1937 presented with polychondritis with prolonged fever, inflammatory syndrome and chondritic lesions of the nose, larynx and helix of the ear. In December 1978, she developed signs of abdominal obstruction. Laparotomy revealed infiltration by multiple nodular formations of the entire posterior line of attachment of the mesentery. Biopsies withdrew a puriform fluid. Histology showed a partly necrotic adipose tissue with giant cells and lipophages. High-dose corticosteroid therapy partially controlled the chondritic and abdominal manifestations. The occurrence of abdominal pain in patients with polychondritis may result from several disorders, such as iatrogenic complications, digestive tract vasculitis or ulcerative colitis, but also associated mesenteric panniculitis.

[Pancreatitis in systemic lupus erythematosus. Review of the literature apropos of 5 cases]. / Pancréatite au cours du lupus érythémateux disséminé. Revue de la littérature à propos de cinq observation.

We report five cases of pancreatitis in systemic lupus erythematosus. Three patients died. The cause of death was directly related to pancreatitis in two cases. Several causes of pancreatitis may be suspected: vasculitis, thrombosis when associated to antiphospholipid syndrome, biliary calculi, infection, metabolic abnormalities or adverse effects of therapy. About seventy cases of pancreatitis have been reported in systemic lupus erythematosus in the literature Pancreatitis was the inaugural sign of lupus in six cases. In 12 cases, besides lupus, no other cause of pancreatitis was found. However, pancreatic vasculitis or thrombosis was rarely demonstrated even in post-mortem examinations. The role of corticosteroids in pancreatitis in lupus is controversial since the evolution of pancreatitis in lupus is generally good with corticosteroids. We think that diagnosis of pancreatitis in lupus should not implicate discontinuation or decrease of corticosteroids dosage, unless their responsibility is absolutely demonstrated.

[Digital arteriography of the lower limbs by sequential, automatic table translation. Feasibility. Preliminary results]. / Artériographie numérique des membres inférieurs par avance séquentielle, mémorisée, du panneau de table. Faisabilité. Résultats préliminaires.

Digital subtraction angiography (DSA) as a method of evaluating lower limb arteries required examination of one limb at a time up to now. Moreover repeated injections of contrast material were necessary. This problem was mainly due to the relative small image intensifier field available and to the difficulty of repositioning the patient between mask and image acquisition. The authors report the preliminary results obtained with 24 patients studied on angiographic unit equipped with: A DSA system (DG 300-CGR) using 512 X 512 matrix size, an angiographic table (Angix M 200) allowing an automatic sequential translation, a removable image intensifier using a 16 inch field below the table. Thus, all those characteristics allow DSA of lower limb arteries. Only one injection of contrast material (Ioxaglate: 80 ml at 8-10 ml/second) was used per procedure. This, usually, allows good quality examination of lower limb arteries.

[The cardiologist and AIDS]. / Le cardiologue et le SIDA.

Since first individualized in Atlanta in 1981, the frequency of AIDS is constantly increasing and the risk groups (male homosexuals, heroin addicts, transfused hemophiliacs, Haitians and Africans) are no longer the only ones concerned. The progression of the disease in African heterosexuals foreshadows its probable course in the Western World. 8,000 AIDS cases were indexed in Europe as of June 1987; 45,000 are expected in 1989. Cardiologists are a priori much less concerned by this disease than infectious disease specialists, internists, dermatologists, respiratory diseases specialist or neurologists. However, under three circumstances they may have to become involved in this disease. 1) AIDS following blood transfusions: the greater the risk as the amount of blood transfused is important; patients undergoing ECC present an increased risk. As of June 30 1988, 320 cases of posttransfusion AIDS had been reported in France (7.82 p. cent of all reported AIDS cases). The mean incubation period, estimated at 54 months in 1986, would actually be much longer: distribution according to a Gauss curve with a period of 15 years +/- 5, which would lead to expect many cases in the years to come affecting patients who were transfused before August 1985, when systematic screening became mandatory. Since that time, the risk has markedly decreased but is not non-existent (pre-serology phase, contamination with HIV 2), resulting in a limitation of the indications of transfusions and restoring as often as possible to autotransfusion and normovolemic hemodilution.

[Retroperitoneal fibrosis]. / Les fibroses rétropéritonéales.

Neoplastic and non-neoplastic retroperitoneal fibrosis share the same macroscopic and histological features and the same clinical signs which mainly consist of abdominal pain followed by signs of compression. All carry a risk of extension to adjacent structures and may therefore produce serious complications. They may also be associated with a "systemic" syndrome with fever, altered general condition and high erythrocyte sedimentation rate. The necessary laboratory and radiological examinations are detailed. Non-neoplastic retroperitoneal fibrosis may be due to a variety of causes, the most frequent of which today are medications and aortic atheroma. Surgery is usually needed when the disease is life-threatening or of poor functional prognosis. Some authors have suggested that surgery should be combined with corticosteroid therapy. In some cases, regression of the fibrosis could only be obtained by giving steroids in high doses.

[Intrabronchial inhaled seed migration. Value of ultrasonography in the diagnosis of pleural-cutaneous fistula]. / Epis de graminée intrabronchiques migrateures. Apport de l'échographie dans le diagnostic de fistule pleuro-cutanée.

We report two cases of inhalation of seeds in infants aged 11 months and two years. In the first the syndrome of inhalation had not been apreciated. The seed blocked the left bronchus, provoking an obstructive emphysema with superinfection. The outcome was statisfactory after removal by bronchoscopy. In the second case although the aspiration of the particle was appreciated the endoscopy was performed too late to prevent the migration of the seed. After having produced a pneumonia it crossed the pleura and gave a subcutaneous swelling. This migration could in part be followed by ultrasound which enabled the appropriate moment for surgical exploration to be defined. These two cases show the two possible evolutions when the inhalation of a seed occurs and the contribution that can be made by ultrasound.

[Pulmonary hyalinizing granuloma. Apropos of 2 new cases]. / Les granulomes hyalinisants pulmonaires. A propos de 2 nouveaux cas.

Pulmonary hyalinising granuloma are nodular or localised fibrosing lesions of the pulmonary parenchyma and are single or multiple. We report two new cases of this disorder which is rare, as only 62 cases have been published in the literature. It is a pathology with few symptoms, sometimes revealed by general signs. Radiologically there are nodules which are most often round or oval, intra-parenchymal, well demarcated, single or multiple. The histological appearance is characteristic: the centre of the granuloma consists of a network of dense collagen fibres which are lamellar separated by clear spaces; the periphery is the seat of rich cellular infiltration of plasmocytes and lymphocytes in the peri-vascular region. Most often there is a spontaneous benign outcome. The frequent association of pulmonary hyalinising granuloma in fibrotic disorders and the similarity in the histological appearance leads to the hypothesis of a common pathogenesis in these disorders.

[Vitamin deficiency-induced pancytopenia mimicking leukemia. Three cases]. / Pancytopénie carentielle mimant une leucémie. Trois observations.

Folate and vitamin B12 are indispensable to normal cell division. High turnover tissues, therefore, are the first to be affected when these vitamins are deficient. Such deficiencies, which are known to result in megaloblastic anaemia at a late stage, may also influence the granulocyte and platelet lines, but pancytopenia rarely occurs. We report 3 cases of pancytopenia associated with folate and/or vitamin B12 deficiency in elderly patients. In two of these patients bone marrow examination showed a misleading "pseudo-leukaemia" due to a significant proportion of strongly dystrophic young cells. Cure was obtained within 30 to 45 days of vitamin therapy, with complete normalization of the haemogram.

[Chronic constrictive pericarditis responsible for an increase of CA 125 levels. Two cases]. / Péricardite chronique constrictive responsable d'une élévation du CA 125. Deux observations.

Despite its poor specificity, CA 125 is a widely used tumoral marker. Significant rises in serum CA 125 levels are found mainly in ovarian cancer, but they also occur in other malignant or benign pathologies such as chronic liver diseases. However, in benign pathologies the CA 125 concentrations hitherto reported did not exceed 850 U/ml. The authors report two cases of chronic constrictive pericarditis with ascites and pleural effusions in which a very high increase of serum CA 125 levels (above 1,200 U/ml) was directly related to the presence of pleural effusions.