Syndrome d’encéphalopathie postérieure réversible induit par un décongestionnant nasal contenant de la pseudo-éphédrine

Introduction

Posterior reversible encephalopathy syndrome is a clinico-radiological entity characterized by neurologic symptoms in association with usually reversible bilateral posterior hemispheric oedema on neuroimaging. Many pathological conditions and treatments have been associated with this syndrome.

Case report

We report a 19-year-old woman, followed-up for hypocomplementemic urticarial vasculitis, who presented with a posterior reversible encephalopathy syndrome induced by the intake of an over-the-counter cold remedy containing pseudoephedrine. Clinical manifestations and radiological abnormalities resolved after anti-hypertensive therapy and withdrawal of sympathomimetic drug.

Conclusion

The diagnosis of posterior reversible encephalopathy syndrome should be considered in patients with compatible clinical and radiological presentation because of its potential reversibility with an appropriate management. Intake of drugs, including over-the-counter cough and cold drugs, should be looked for in the history as well as autoimmune disorders.     

Déficit en cuivre et neuropathie périphérique compliquant une gastrectomie

Introduction

Bariatric surgery may be indicated in patients with morbid obesity. Peripheral and central neurological complications can occur after bariatric surgery.

Observation

We report a 47-year-old woman who presented with a tetraparesis related to copper deficiency after sleeve gastrectomy for morbid obesity.

Conclusion

Neurological complications related to copper deficiency can occur after bariatric surgery.     

La maladie de Behçet chez la femme

Purpose

Behçet's disease (BD) is a multisystemic inflammatory disease characterized, on the one hand, by the frequency of benign mucocutaneous and articular manifestations, and on the other hand by the severity of ocular, neurological, vascular and digestive manifestations which may threaten functional or vital prognosis. The male BD predominance is obvious in many retrospective studies and a few studies only have focused on BD in women.

Methods

In this study, we report the epidemiological, clinical, outcome and therapeutic data of a series of 71 female BD compared to a series of 111 male BD. This retrospective study was conducted in the internal medicine department of Monastir university hospital over a 19-year period (1990 to 2008), including all patients followed-up and treated for BD.

Results

The comparison of clinical manifestations of BD between men and women showed the predominance of erythema nodosum (P = 0.016), necrotic pseudofolliculitis (P = 0.001), and joint involvement (P = 0.009) in women, while genital ulcers (P = 0.039), ocular involvement (P = 0.003) and vascular (P = 0.002) involvement were more common in men.

Conclusion

Our results confirmed the results of previous studies regarding the benign nature of BD in women compared to men.     

Atteintes ORL de la maladie de Fabry : à propos de 25 observations

Purpose

Fabry disease (FD, OMIM 301500) is an X-linked lysosomal storage disorder due to deficient activity of the enzyme alpha-galactosidase A. Males and females exhibit severe organ involvement. The high incidence of otological symptoms was recently reported.

Patients and methods

Monocentric and retrospective study of twenty-five patients with FD (13 families; seven males and 18 females). The patients underwent audiological assessment before initiation and during enzyme replacement therapy. We also analysed neurologic heart and kidney status.

Results

Twenty patients (80%; 13 females and seven males) complained of otologic symptoms. Audiological evaluation showed a sensorineural hearing loss in 17 patients, bilateral in 16 out of them. Vestibular examination showed a functional impairment in two patients (one female, one male). Correlations were found between hearing loss and either kidney disease (73,3%), neurological complications (100%) and cardiomyopathy (80%). Fourteen patients (56%; seven females, seven males) received enzyme replacement therapy. Improvement or stabilization of the audiological evaluation was reported in seven patients, whereas worsening was observed in three patients.

Conclusion

This study confirms the high frequency of audiological involvements in females and males with FD. Our analysis suggests that the frequency of hearing loss is increased in the presence of renal or neurologic involvement or cardiomyopathy. There is no clinically significant efficacy of enzyme replacement therapy on hearing function. Although the pathophysiology remains unknown, a vascular mechanism responsible of the inner ear involvement seems to be privileged.     

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     

Complications vasculaires de l’abord artériel fémoral : impact du système de fermeture percutanée Perclose

Introduction

Vascular complications at the femoral access site is an important factor of morbidity. The aims of this study were to evaluate the efficacy and safety of the percutaneous closure device (Perclose) during interventional cardiology procedures.

Patients and methods

All patients with percutaneous closure of the femoral access site by the Perclose system in 2010 were included. We evaluated the indications of the procedures, the success rate of implantation and the bleeding complications according to antithrombotic therapy used.

Results

Three hundred and seventy five patients underwent a percutaneous closure by the Perclose system. Acute coronary syndromes with or without elevation of ST segment were the main indications of procedures (74.9%). The success rate of percutaneous closure of the femoral access site was 97.3%. The rate of minor, moderate, and severe bleeding of the entire cohort according to the GUSTO classification was respectively 4.2%, 0.5% and 1%. Bleeding complications were similar in both groups of patients (2.1% vs 7% P = 0.122). In case of unsuccessful deployment of femoral closure devices, the risk of bleeding complications range from 4.3% to 60% (P = 0.0000036).

Conclusion

The use of the Perclose system is associated with a low rate of severe bleeding at the femoral access site. However, the failures of percutaneous closure increase the risk of bleeding complications.     

Analyse des complications cardiovasculaires survenant lors des transferts inter-hospitaliers de patients ayant un syndrome coronarien sans élévation du segment ST non compliqué

Introduction

Managing patients with non-ST elevation myocardial infarction (NSTEMI) require their transfer to an interventional cardiology center. The inter-hospital transfer (IHT) of these patients is managed by the Emergency Medical Service (EMS) which often requests a medicalised transport of such patients due to the risk of complications.

Aim of the study

Evaluating the incidence of cardiovascular complications that require medical intervention, occurring during IHT of patients with uncomplicated NSTEMI.

Methods

We conducted a retrospective study for one year (August 2010–July 2011) in the SAMU-85 unit using the regulation software Centaure^®. All patients with NSTEMI who had been transferred from one of the peripheral hospitals in the region of Vendee were included.

Results

The study population group is composed of one hundred and fifty-nine patients. No cardiovascular complications requiring medical intervention occurred during the transport. Seventeen patients (10.7%) had cardiovascular complications: repolarization disorder (7), chest pain (3), dyspnea (3), arrhythmia (2), and bradycardia (2). These complications did not require action or medical intervention.

Conclusion

The medicalization of IHT of patients with NSTEMI who do not show complications before transfer, is probably questionable. Indeed, according to this study made on 159 patients, no cardiovascular event requiring medical intervention was reported during transport.     

Nouveautés dans la prise en charge de l’infarctus cérébral en 2014 : ce que le cardiologue doit savoir

Introduction

Interventional cardiology procedures are regularly exposed to ischemic neurological complications. IV fibrinolysis is the only approved treatment in ischemic stroke but is very often contraindicated in these situations. Many techniques of interventional neuroradiology (mechanical thrombectomy) have been developed over the past years and are used to treat these patients.

Observation

We report the case of two patients who were admitted in emergency for ischemic stroke with contraindication to IV fibrinolysis (cardioversion for atrial fibrillation under anticoagulation; 24 hours after carotid surgery). These patients were treated by endovascular thrombectomy procedure.

Discussion

After validation of IV fibrinolysis within 4.5 hours after stroke onset, techniques of mechanical thrombectomy have gradually been developed, either as a complementary treatment or as an alternative in the case of CI to fibrinolysis. These endovascular thrombectomy devices currently allow recanalization of proximal cerebral occlusions, which correlates with a favorable clinical prognosis. A review of the literature is provided, along with a discussion about the techniques currently being improved, their advantages and disadvantages and the selection of patients that can benefit from endovascular procedures.

Conclusion

In the case of a sudden occurrence of a neurological deficit during a cardiovascular procedure, a “thrombolysis alert” should be triggered. This will permit the rapid establishment of a clinico-radiological report for selecting stroke patients eligible for a procedure of recanalization by thrombolysis and/or mechanical thrombectomy.     

Myélopathie à Schistosoma mansoni

Introduction

Neurological complications of schistosomiasis remain exceptional even in hyperendemic area.

Case report

We report a 26-year-old Senegalese man, without past medical history, who was admitted for spastic paraplegia, acute retention of urine, and pain in low back and lower limbs. The final diagnosis was spinal cord schistosomiasis. Diagnosis was based on the endemic context, MRI medullar conus imaging, schistosoma serology in cerebrospinal fluid and blood, and the absence of other cause of myelopathy. Treatment was based on praziquantel, corticosteroids and physiotherapy. The outcome was favorable after a 2-year follow-up.

Conclusion

Schistosomiasis should be included in the differential diagnosis of myelopathy in patients living actually, or even traveled in the past, in endemic tropical areas.     

Méningite à éosinophiles

Introduction

Diagnosis of Angiostrongylus cantonensis meningitis is not always obvious, even in endemic areas.

Case report

We report a 51-year-old Polynesian man, farmer without past medical history, who was admitted for mild fever, headache, neurological subjective symptoms, and severe muscle pain impeding walking. The diagnosis of A. cantonensis meningitis, guided by hypereosinophilia was confirmed by lumbar puncture and positive specific serology. Lumbar puncture and administration of corticosteroids rapidly reduced headache whereas paraesthesias declined more progressively.

Conclusion

Diagnosis of A. cantonensis meningitis should be considered in patients living or returning from endemic areas with headache associated with subjective neurological symptoms and hypereosinophilia.     

Efficacité clinique et biologique du tocilizumab au cours de la maladie de Horton : à propos de trois observations et revue de la littérature

Introduction

Treatment of giant cell arteritis is based on prolonged corticosteroid therapy but adverse side effects are common especially in the elderly.

Case reports

We report three patients with giant cell vasculitis treated by tocilizumab, an interleukin-6 receptor antibody, owing to resistance or intolerance to corticosteroid therapy. A favorable outcome was rapidly observed both on clinical and biological data allowing a corticoid therapy sparing.

Conclusion

Tocilizumab is a promising treatment of giant cell arteritis but controlled trials are needed to confirm its efficacy.     

Complications infectieuses induites par le mésusage de la buprénorphine haut dosage (Subutex) : analyse rétrospective de 42 observations

Purpose

Misuse of high-dose buprenorphine (HDB), mainly by injection, is responsible of frequent infectious adverse events.

Methods

This is a retrospective study of infectious complications occurring in patients using HDB by injection. Forty-two cases were identified (29 men and ten women) and the data were collected between March 1999 and December 2008.

Results

The infectious complications included cutaneous infections (27 cases), endocarditis (nine cases), osteoarticular infections (four spondylodiscitis and one sacroiliitis), and a vascular embolism with decrease in visual acuity.

Conclusion

The results of HDB maintenance treatment must be improved, both from the point of view of substitution and to limit its misuse by intravenous route injection. Health professionals have to play an important role in drug addict patients’ education and supervision, to prevent buprenorphine injection and related infectious complications.     

Cholangite sclérosante de réanimation : évolution favorable sous acide ursodésoxycholique et fénofibrate

Introduction

Ischemic cholangitis in intensive care unit is a recently reported liver disease in patients who have had a prolonged mechanical ventilation and vasopressive drug support for multiple organ deficiency. Prognosis is usually poor and the only life-saving therapy is liver transplantation despite ursodesoxycholic acid treatment.

Case report

We report a 63-year-old man who presented with a sclerosis cholangitis after a month in intensive care unit, effectively treated with fenofibrate and ursodesoxycholic acid. Recent reports underline fenofibrate efficacy in the treatment of primary biliary cirrhosis, especially in association with ursodesoxycholic acid. This treatment has prevented liver transplantation for our patient with a correct quality of life.

Conclusion

The addition of fibrate to ursodesoxycholic acid improves persistent cholestasis in sclerosing cholangitis.     

Utilidad de la broncoscopia flexible en la evaluación de infiltrados pulmonares en la población con trasplante de células madre hemopoyéticas: experiencia de 14 años en un solo centro

Introduction

Pulmonary infiltrates are common within the hematopoietic stem cell transplant (HSCT) population and unfortunately portend an increased mortality. Bronchoscopy is often utilized as an initial diagnostic tool, but the literature supporting its diagnostic utility and effect on clinical management varies significantly. The aim of this study was to investigate the diagnostic ability, complication rate, and clinical impact of flexible bronchoscopy (FB) in evaluating pulmonary infiltrates in a large HSCT population.

Patients and method

Retrospective review of all patients undergoing FB after HSCT in the Bone Marrow Transplant Unit from 1996 to 2009.

Results

FB was performed 162 times in 144 patients with pulmonary infiltrates yielding positive results in 52.5%. The most common positive results were bacterial pneumonia (31%), fungal pneumonia (15%), and alveolar hemorrhage (11%). Treatment changes occurred in 44% of patients after FB. Treatment changes included antibiotic modification (59%), addition of corticosteroids (21%), antifungal modification (12%), and antiviral modification (7%). The overall complication rate associated with FB was 30%, although 84% of these complications were considered minor.

Conclusions

FB in patients with pulmonary infiltrates after HSCT should still be considered a valuable tool in the evaluation and management of pulmonary infiltrates in the HSCT population. Future prospective, multicenter randomized studies are needed to evaluate the overall clinical impact that bronchoscopic results and management changes have in this unique population.     

TRAPS : influence du génotype sur l’expression de la maladie. À propos de deux observations

Introduction

Tumor necrosis factor receptor associated periodic fever syndrome (TRAPS) is defined as recurrent attacks of generalized inflammation for which no infectious or auto-immune cause can be identified; it is caused by dominantly inherited mutations in the gene encoding the first TNF receptor. We report two additional cases of patients with TRAPS, suggesting that mutation pattern of TNFRSF 1A gene may influence the TRAPS phenotype.

Case reports

The first patient, with a C30S mutation, exhibited severe digestive clinical manifestations; because the patient required high-dose corticosteroids regimen to improve TRAPS manifestations, he was further given successfully etanercept. The second patient, with a R92Q mutation of TNFRSF 1A gene, presented with moderate symptoms; TRAPS outcome was favourable after corticosteroid therapy initiation.

Conclusion

Therefore, R92Q may be associated with a mild disease phenotype. On the other hand, C30S mutation appears to be associated with a severe phenotype, leading to an increased risk of amyloidosis. These findings suggest that these latter patients may require a closer follow-up.     

Anévrismes infectieux de l’aorte thoracique : présentation de 7 cas et revue de la littérature

Purpose

Infectious aortic aneurysms are rare conditions, being responsible of 2% of aortic aneurysms. Most published results are surgical case series concerning infected abdominal aorta. In this retrospective study, we assessed clinical features and outcome of patients presenting infectious thoracic aortic aneurysms.

Patients and methods

Diagnosis was based upon a combination of imaging evidence for thoracic aorta aneurysm and evidence for an infective aetiology including a culture of a causative pathogen, or a favourable outcome with anti-infective therapy. Retrospective case series.

Results

Six men and one woman were included, with a mean age of 66 years. All the patient presented at least one cardiovascular risk factor or atherosclerosis localisation. Fever (71%) and chest pain (42%) were the most common clinical presenting manifestations. The causative pathogens were: Staphylococcus aureus (N = 1), Salmonella enteritidis (N = 3) and Candida albicans (N = 1). The contrast-enhanced computed-tomography disclosed an aneurysm whose diameter reached more than 50 mm (N = 5), that increased rapidly in size (N = 5), or presented an inflammatory aspect of the aortic wall (N = 4). Management was both medical and interventional: surgery (N = 3) or endoluminal repair (N = 4). Outcome was favourable in six patients; one patient died from aneurysm-related complications.

Conclusion

Clinical manifestations revealing an infectious thoracic aneurysm are variable. Diagnosis should be considered in patients presenting a rapidly-growing aneurysm, especially in the presence of elevated acute phase reactants. Endoluminal repair constitutes a treatment option. The role of FDG-PET for diagnosis and follow-up remains to be defined.     

Rétinopathie dysorique à l’interféron au cours de l’hépatite chronique virale C. À propos d’un cas et revue de la littérature

Introduction

The ophthalmic complications following interferon therapy in chronic hepatitis C are rare. The most common adverse ophthalmic outcome is the dysoric retinopathy characterized by the presence at the fundus examination of cotton wool spots and retinal hemorrhages particularly around the optic disc.

Case report

A 63-year-old man presented to the hepatology department with a compensated cirrhosis C. His medical history was positive for hypertension controlled by medical treatment. A combined treatment with pegylated interferon α2a plus ribavirin was initiated. Three months later, the patient reported a sudden decreased vision in both eyes. Fundus examination revealed cotton wool spots with retinal hemorrhage. The diagnosis of dysoric retinopathy was established. The antiviral treatment was discontinued. One month later, the patient was asymptomatic and the ocular lesions have disappeared.

Conclusion

Dysoric retinopathy is a non-specific complication of interferon therapy in chronic hepatitis C. Despite its good prognosis, a careful fundus examination is required before and during the treatment especially for the patients with risk factors for this adverse event (advanced age, diabetes and high blood pressure).