Artériopathie et hétérogénéité artérielle

More and more clinical observations and trials support the concept of heterogeneity of atheroma according to the arterial bed. In a pilot study named “Étude Comparative des Lésions Athéromateuses” (ECLA), we have shown that carotid and femoral plaques possess different characteristics. Carotid arteries display increased lipid content compared to femoral arteries whereas femoral arteries are more prone to calcify and to develop osteoid metaplasia. These observations should lead the researcher and the clinician to look at the cellular and molecular mechanisms governing the heterogeneity of atheromas. At last, a better understanding of the characteristics of plaques should help us to determine plaque stability, to prevent cardiovascular events and to choose the best medical, endovascular or surgical option.     

Polyradiculonévrite aiguë révélant un lupus érythémateux systémique : une présentation inhabituelle d’évolution fatale

Introduction

Neurological manifestations of systemic lupus erythematosus are common and numerous. They mainly involve the central nervous system, peripheral involvement being rare. Acute polyradiculoneuropathy is very uncommon.

Case report

We report a 44-year-old man, who presented with acute polyradiculoneuropathy revealing systemic lupus erythematosus. Outcome was fatal despite treatment with corticosteroids and immunoglobulin.

Conclusion

Acute polyradiculoneuropathy is a very rare manifestation of systemic lupus erythematosus and can compromise functional and life prognosis. Early diagnosis and management are crucial.     

Les glomérulopathies associées aux néoplasies myéloprolifératives

Purpose

Myeloproliferative neoplasms (MPN) are hematological disorders characterized by clonal expansion of one or more medullary lines. Renal complications are rare, chiefly as acute renal failure. Glomerular involvement is exceptional

Methods

We report on a retrospective multicenter case series of eight patients who presented with a glomerulopathy (GP) associated with MPN

Results

All GP were revealed by a major proteinuria frequently associated with nephrotic syndrome and oedema. Histology was mainly characterized by lesions of focal segmental glomerulosclerosis associated with increased mesangial cellularity. The pathophysiology is still unclear but platelet-derived growth factor (PDGF) and transforming growth factor-β (TGF-β), which play a central role in the MPN may be involved. A majority of patients developed chronic renal failure despite of a therapy intended to block the renin-angiotensin system

Conclusion

Monitoring of proteinuria during the follow-up of MPN would allow earlier diagnosis of renal involvement. Further studies on a larger scale are needed to specify the pathophysiological mechanisms involved and the management of these complications     

Thrombophlébite cérébrale : une complication rare de l’infection aiguë à cytomégalovirus

Introduction

Acute cytomegalovirus (CMV) infection increases the risk of vascular thrombosis but reports of cerebral venous thrombosis are rare.

Case report

We report a 36-year-old woman who presented with a cerebral venous thrombosis and acute CMV infection heralded by a cytolytic hepatitis. Heterozygous factor V Leiden mutation was also identified. The patient was treated with anticoagulation for 1 year with favourable outcome.

Conclusion

Serologic tests for CMV infection should be performed in case of cerebral venous thrombosis with liver cytolysis or flu-like symptoms. CMV infection often triggers thrombosis in combination with other inherited or genetic predisposing risk factors that should always be searched.     

Péricardite récidivante : traquer le mésotheliome péricardique primitif

Introduction

Most of recurrent pericarditis are idiopathic and only 15 to 20% have a specific diagnosis. Primary pericardial mesothelioma is a rare cause of recurrent pericarditis. Diagnosis can be challenging and antedates patient's death in only 10 to 20% of cases. Histology of mesothelioma and immunohistochemistry are mandatory for the diagnosis. Median of survival before using pemetrexed was about 6 months after diagnosis.

Case report

We report the history of a 64-year-old woman for which repeated biopsy for recurrent pericarditis was necessary to diagnose a primary pericardial mesothelioma. The first biopsy had only found mesothelial hyperplasia.

Conclusion

This case report highlights the necessity of repeat pericardial biopsy in the case of adverse outcome.     

Dégénérescence cérébelleuse paranéoplasique révélant un cancer du sein : à propos d’un cas

Introduction

Paraneoplastic cerebellar degeneration may be a manifestation indicative of lung, gynecological or breast cancer. Nevertheless, breast cancer is rarely revealed by the occurrence of a paraneoplastic syndrome.

Case report

We report a 38-year-old patient who presented a paraneoplastic cerebellar degeneration with anti-Yo antibodies as the presenting manifestation of a breast cancer.

Conclusion

The diagnosis of a paraneoplastic neurological syndrome (PNS) should lead to urgent and comprehensive screening for cancer oriented by the type of PNS and the nature of the anti-neuronal antibody.     

Corrélation entre les dosages des dérivés methoxylés urinaires et l’index d’apnée-hypopnée dans une population d’hypertendus : étude pilote

Aims

Sleep disorders like obstructive sleep apnea in adults are associated with increased sympathetic activity, which induced high blood pressure and could be associated with resistant hypertension. Some studies have demonstrated that high urinary catecholamine levels in obstructive sleep apnea patients may be decreased with continuous positive airway pressure therapy. However, very few studies have demonstrated a correlation between apnea-hypopnea index and urinary catecholamine levels in hypertension patients.

Methods

In this pilot study, 20 hypertensive patients referred for hypertension work-up including night-time polygraphy and 24 h urinary catecholamine dosage were included.

Results

Mean age was 51 ± 11 years (30–76), 68% were males. Diagnosis of obstructive sleep apnea was confirmed in 13 patients at the end of the work-up. Mean apnea-hypopnea index was 14 ± 9 (2–32). The only urinary catecholamine parameter significantly increased in patients with obstructive sleep apnea was 24 h urinary normetanephrine (1931 ± 1285 vs 869 ± 293 nmol/24 h; P < 0.05). However, this difference was not significant when this parameter was adjusted to 24 h urinary creatinine. We observed a significant positive correlation between AHI and 24 h urinary normetanephine (r = 0.486; P = 0.035).

Conclusion

This pilot study confirms an isolated elevation of 24 h urinary normetanephrine in hypertensive patients with obstructive sleep apnea and shows a significant correlation between sleep disorders expressed by apnea-hypopnea index and urinary catecholamines excretion.     

Prise en charge d’un paragangliome rétropéritonéal

Paragangliomas are rare tumors arising from extraadrenal chromaffin cells. We report a 43-year-old man who presented with abdominal pain. An abdominal computed tomography scan revealed a large retroperitoneal mass. During an endoscopic biopsy of this tumor, the patient experienced marked hemodynamic fluctuations with tachycardia and high blood pressure, and an extraadrenal pheochromocytoma was suspected. Measurements of plasma and urinary catecholamines and urinary total metanephrines ruled in the diagnosis. Echocardiography disclosed acute myocardial dysfunction that returned to normal after surgical resection of the paraganglioma. This report also underlines the importance of the anesthetic preparation and monitoring around the surgical procedure and the need of a long-term follow-up to detect malignant paraganglioma in the absence of histological criteria of benign tumor.     

Phytostérols et athérosclérose

Phytosterols/stanols (PS) enriched food products have been shown to consistently lower plasma cholesterol levels. The intake of 2 g/d of PS decreases LDL-cholesterol by about 10%. With respect to the association of LDL-cholesterol lowering with reduction in the cardiovascular (CV) risk, it is likely that supplementation in PS reduces the incidence of CV disease. In addition, the vast majority of animal studies have shown that oral administration of PS reduces the progression atherosclerosis. However, it has been recently suggested that an increase in PS plasma concentrations may increase CV risk. Evidence to support this hypothesis come mainly from observations in sitosterolemic patients who hyperabsorb PS and cholesterol and display very high levels of PS, which may be associated with a premature atherosclerosis. Some epidemiological studies in non-sitosterolemic subjects have shown a positive correlation between PS plasma levels and coronary heart disease. However, these are observational studies and some of them present major methodological bias. In addition, recent studies with a larger number of subjects have indicated, either an absence or a negative relationship between PS and the incidence of CV disease. The guidelines of several French and international institutions recommend the use of PS enriched food in association with other classical recommendations in hypercholesterolemic subjects. However, further studies are highly encouraged to examine the CV benefit of PS enriched food.     

Syndrome de Tako Tsubo révélant une hémorragie méningée par rupture d’anévrisme

Cerebral hemorrhage is usually associated to many cardiac disorders, mimicking acute coronary syndrome. We relate a case of a postmenopausal woman presenting at emergency room for acute coronary syndrome and whose evaluation revealed a subarachnoidal hemorrhage, normal coronary arteries, and a typical Tako Tsubo aspect on echocardiography with apical ballooning and hyperkinesis with basal hypokinesis.     

Le syndrome de platydéoxie-orthopnée : à propos d’un cas

We report the case of an 80-year-old woman with symptomatic postural hypoxaemia caused by a right-to-left shunt through a patent foramen ovale. The hypoxaemia was enhanced by the supine position and disappeared in upright position. Potential mechanisms underlying postural variations of the shunt seemed to be similar to those describe in platypnea-orthodeoxia syndrome. Patient became asymptomatic after shunt resolution.