Schwannoma of the external auditory canal: An exceptional site.

INTRODUCTION: Schwannomas are benign solitary neural tumours that are only exceptional located in the external auditory canal, as only a few cases have been reported in the literature. CASE REPORT: We report a case of schwannoma of the external auditory canal in an 18-year-old man admitted for an isolated mass of the initial segment of the right external auditory canal visible to the naked eye, obstructing all of the external auditory meatus. Computed tomography of the temporal bone showed an isolated mass of the external auditory canal. Management of this patient consisted of biopsy-excision of the mass, histological examination of which confirmed a schwannoma of the external auditory canal. DISCUSSION: Although rare, the possibility of a nerve tumour of the external auditory canal should always be considered. These tumours may be isolated or may occur in the context of von Recklinghausen's disease. The clinical presentation in the external auditory canal may correspond to recurrent otitis externa secondary to obstruction of the canal by the tumour, as in the case reported here. The definitive diagnosis must be based on the results of histological and immunohistochemical examination.

[Thyroid function after hypofractionated adjuvant radiotherapy for localized breast cancer]. / Fonction thyroïdienne après une radiothérapie adjuvante hypofractionnée pour un cancer du sein localisé.

PURPOSE: We aimed to evaluate the impact on the thyroid function of hypofractionated adjuvant radiotherapy of localized breast cancer. MATERIAL AND METHOD: This was a prospective study, including 50 patients with breast cancer treated by hypofractionated adjuvant radiation, only half of whom received radiotherapy on the supraclavicular region. The analysis focused on clinical, dosimetric and biological data collected through periodic dosing of thyroid hormones. RESULTS: The incidence of hypothyroidism was 4% of the patients. The multivariate correlation analysis showed a significant association between initial thyroid volume, volume receiving 50Gy, 40Gy and 30Gy and the incidence of hypothyroidism, no statistical link was found between the bilateral breast, chemotherapy, hormone therapy and the type of surgery and the incidence of radiation-induced hypothyroidism. CONCLUSION: Although hypofractionation theoretically exposes the thyroid gland to late radiotherapy complications, radiation-induced dysthyroidism remains a complication underestimated by clinicians, and in the absence of prevention and treatment guidelines, it is necessary to delineate systematically the thyroid gland, to try to minimize as much as possible the doses received by this organ, and to monitor the thyroid function by periodic serum assays.

Acute celiac trunk thrombosis revealed by biliary peritonitis.

Acute thrombosis of the celiac trunk is a very uncommon condition, which is a life-threatening emergency. The clinical presentation is highly variable depending on the extent of the ischemic territory. We report a case of biliary peritonitis related to an acute thrombosis of the celiac trunk. This case highlights the role of abdominal computed tomography in the diagnosis of acute upper abdominal pain.

[Intraprostatic calcifications as natural fiducial markers in image-guided radiotherapy for prostate cancer]. / Calcifications intraprostatiques : marqueurs fiduciels naturels dans la radiothérapie guidée par l'image du cancer de la prostate.

PURPOSE: To establish whether intraprostatic calcifications can serve as natural fiducials for image-guided radiotherapy (IGRT), replacing the implantation of intraprostatic fiducial markers. PATIENTS AND METHODS: Patients with prostate cancer, having intraprostatic calcifications visible on CT scan were selected and underwent intensity-modulated radiotherapy/3D conformal radiotherapy with IGRT in the department of radiotherapy of Henri-Mondor Hospital. All cone-beam computed tomographies (CBCT) were repositioned on intraprostatic calcifications. For each acquired image, displacements of intraprostatic calcifications were calculated with reference to position on planning CT in three directions: lateral, longitudinal and vertical. RESULTS: Between 2011 and 2013, nine patients had 183 CBCT. For each image, three displacements and space coordinates were calculated using a single reference (intraprostatic calcification). Mean lateral, longitudinal and vertical movements were 0.26±5.7 mm, -1±4.6 mm and 0.42±3.5 mm, respectively. CONCLUSION: Studies exploring prostatic movements with fiducial markers as reference and ours with natural fiducials yield similar results. Our data confirm previous studies that have suggested that intraprostatic calcifications can be used as natural fiducials with potential reduction of iatrogenic risks and costs associated with the implantation of fiducial markers.

[Non-targeted effects (bystander, abscopal) of external beam radiation therapy: an overview for the clinician]. / Effets non ciblés (bystander, abscopal) de la radiothérapie externe : potentielles implications pour le clinicien ?

Radiotherapy is advocated in the treatment of cancer of over 50 % of patients. It has long been considered as a focal treatment only. However, the observation of effects, such as fatigue and lymphopenia, suggests that systemic effects may also occur. The description of bystander and abscopal effects suggests that irradiated cells may exert an action on nearby or distant unirradiated cells, respectively. A third type of effect that involves feedback interactions between irradiated cells was more recently described (cohort effect). This new field of radiation therapy is yet poorly understood and the definitions suffer from a lack of reproducibility in part due to the variety of experimental models. The bystander effect might induce genomic instability in non-irradiated cells and is thus extensively studied for a potential risk of radiation-induced cancer. From a therapeutic perspective, reproducing an abscopal effect by using a synergy between ionizing radiation and immunomodulatory agents to elicit or boost anticancer immune responses is an interesting area of research. Many applications are being developed in particular in the field of hypofractionated stereotactic irradiation of metastatic disease.

[Hepatitis B prevalence and risk factors in Morocco]. / Prévalence de l'infection par le virus de l'hépatite B et l'évaluation des facteurs de risque au Maroc.

AIM: The aim of this study was to determine the prevalence of hepatitis B and the risk factors in Morocco. STUDY DESIGN: A total number of 16,634 individuals were screened for HBsAg using the Murex HBsAg Version 3 assay and were interviewed using a structured standard questionnaire to collect information about risk factor. RESULTS: Two hundred seventy-six subjects were positive for HBsAg, the prevalence of HBV infection was 1.66%. Using a structured standard questionnaire we reported that sexual behaviours (43.84%) are among the main risk factors for HBV transmission. CONCLUSION: This study indicates that the prevalence of HBsAg in Morocco is currently estimated at 1.66% in the active population. The risk factors for HBV infection identified here indicate that prevention is the most cost-effective method for successfully controlling HBV infection, so vaccination remains the best way to control this infection and its related complications.

Range-reference determination of lymphocyte subsets in Moroccan blood donors.

BACKGROUND: Information on lymphocyte populations (T, B, and Natural killer cells) and subpopulations (CD4 and CD8) in Morocco is scarce if not inexistent. OBJECTIVE: To establish a reference value of these cells in 242 Moroccan young adult blood donors by flow cytometry. RESULTS: Smokers had significantly higher total leukocyte count (p < 0.001), total lymphocyte count (p < 0.0001) and higher CD3+CD4+ cells (p < 0.0001). The percentage of CD3-CD56+ subsets was affected by smoking (p < 0.01). Our analysis positively correlate with previous observations of an increase of absolute CD4+ T cells, with no changes in other lymphocyte subset cells in smokers. The lymphocyte subpopulation distributions for all antigens were found to be similar to those reported in Saudi and Italian adults, while higher levels were reported for the same gender in other countries, especially Ghana and Kuwait. CONCLUSION: The international classification standards of the HIV-infected subjects according to their rates of CD4 are applicable to the present study's population.

Cerebral venous thrombosis in Behçet's disease.

OBJECTIVE: To analyze the clinical findings, treatment, outcome, and prevalence of cerebral venous thrombosis (CVT) in a large cohort of patients with Behçet's disease (BD) from a single center. METHODS: We reported a series of 64 consecutive patients with CVT who fulfilled the international criteria for BD. Multivariate analysis was performed to define factors that affect prognosis. RESULTS: Among a cohort of 820 patients with BD, CVT was present in 64 (7.8%). Compared with BD patients without CVT, those with CVT had lower parenchymal central nervous system involvement (4.7% versus 28.7%; P = 0.0001) and higher extraneurologic vascular lesions (62.5% versus 38.8%; P = 0.03). Up to 90% of patients responded to anticoagulation therapy without severe hemorrhagic complications. Neither steroid nor immunosuppressant use provided better outcome. Severe visual loss due to optic atrophy was the main complication of CVT, being found in 15% of patients. In multivariate analysis, papilledema (odds ratio [OR] 7.1, 95% confidence interval [95% CI] 1.6-31.9) and concurrent prothrombotic risk factors (OR 4.6, 95% CI 1.1-20.2) were independently associated with the occurrence of sequelae. Factors associated with relapse of thrombosis were concurrent prothrombotic risk factors (hazard ratio [HR] 4.9, 95% CI 1.5-15.4) and a peripheral venous thrombosis (HR 2.8, 95% CI 0.7-10.5). After a mean +/- SD followup of 8.2 +/- 6.9 years, 4 deaths unrelated to CVT were noted. CONCLUSION: CVT in patients with BD may result in serious neurologic outcomes. Anticoagulation represents a safe and effective therapy. Extensive investigation of prothrombotic disorders should be considered.

Subacute bacterial endocarditis presenting as polymyalgia rheumatica or giant cell arteritis.

OBJECTIVE: To report on several patients with subacute bacterial endocarditis who were initially presumed, incorrectly, to have polymyalgia rheumatica or giant cell arteritis. METHODS: We report 3 cases of subacute streptococcal endocarditis mimicking giant cell arteritis in 2 cases and polymyalgia rheumatica in one. We reviewed the literature through Medline search of French and English-language articles published between 1966 and 2005 and found 5 similar cases. RESULTS: Shoulder and/or pelvic girdle pain was associated with neck or back pain in all patients. Scalp tenderness, bilateral jaw pain, amaurosis fugax were present in 2 patients. One patient had no fever. Two patients were treated with corticosteroids with initial good clinical response in one. Appropriate antibiotic therapy resulted in the rapid disappearance of rheumatic complaints in 2 patients and achieved a definitive cure of endocarditis in all cases. CONCLUSION: Rheumatologic symptoms may hinder the correct diagnosis of infective endocarditis in patients who present with a clinical picture suggesting polymyalgia rheumatica or giant cell arteritis. In such cases, blood cultures should be systematically drawn.

[Neurological manifestations of Behçet's disease]. / Manifestations neurologiques de la maladie de Behçet.

The neurological complications of Behçet's Disease are observed in 5 to 35 percent of cases depending of series. They included vascular and central nervous system manifestations. Vascular involvement is dominated by cerebral venous thrombosis marked by benign intracerebral hypertension. Prognosis is favourable with steroids and anticoagulation. Arterial involvement (thrombosis or aneurysm) are seldomly observed but increasingly with angio-MRI. Parenchymal involvement is dominated by meningoencephalitis eventually associated with fever. Myelitis and cranial nerve palsy are seldomly encountered. Cerebrospinal fluid is abnormal with lymphocytosis or pleiocytosis. MRI has a high sensitivity with hypersignals in the brainstem, basal ganglia, internal capsule and thalamus. Prognosis of central nervous system involvement is severe. Initially recovery is frequent, but severe impairment occurs due to relapse. In our experience, therapy including steroids, immunosuppressive drugs and colchicine improve short term and long term prognosis due to significant reduction of relapse in patients with good observance.

Increased soluble p55 and p75 tumour necrosis factor-alpha receptors in patients with hepatitis C-associated mixed cryoglobulinaemia.

To investigate whether tumour necrosis factor alpha (TNFalpha) plays a role in the pathogenesis of hepatitis C virus-associated mixed cryoglobulinaemia (HCV-MC), we measured soluble TNFalpha and its soluble p55 (sTNFR1) and p75 (sTNFR2) receptors in the serum of patients with HCV-MC. TNFalpha, sTNFR1 and sTNFR2 were measured in the serum of 32 patients with HCV-MC, 18 patients with hepatitis C without MC (HCV) and 18 healthy volunteers, using specific immunoassays. Correlations between clinical and biological parameters and the concentrations of TNFalpha and sTNFRs were established by studying detailed clinical records of the 32 HCV-MC patients. Although higher, TNFalpha levels were not significantly different in HCV-MC patients compared with healthy or HCV controls. sTNFR1 and sTNFR2, however, were significantly higher in HCV-MC compared with controls or with HCV patients, and higher concentrations of sTNFR1 and sTNFR2 were observed in patients with severe visceral vasculitis, compared with patients with limited purpura. sTNFR1 concentrations positively correlated with fibrinogen levels but TNFalpha, sTNFR1 and sTNFR2 did not correlate with other biological parameters such as rheumatoid factor concentrations, CH50 or C4 values. These data suggest a role for TNFalpha in the pathogenesis of the immune complex-mediated vasculitis associated with HCV-MC.

Hyperhomocysteinemia and arterial aneurysm.

Hyperhomocysteinemia (HCY) is an independent risk factor for atherosclerosis. Arterial aneurysm has rarely been described in association with heterozygous HCY. Here we report two cases of this association. Case 1 was 32-Year-old man who presented with distal trophic manifestations of the lower extremities. Upon investigation, occlusive arterial disease with fusiform aneurysm of both popliteal arteries and occlusion of the left cubital artery were found. Laboratory findings indicated HCY due to homozygous methylene tetrahydrofolate reductase (MTHFR) deficiency. Case 2 was 38-year-old man with no history of trauma who presented with repeated ischemic events involving the right hand in association with isolated aneurysm of the right cubital artery. Histological study demonstrated extensive dystrophic changes in the aneurysmal vessel wall, including sclerohyalin deposits. The only abnormality was homozygous MTHFR deficiency. Pathologic changes induced by HCY in vessel walls may be implicated in early arterial aneurysm. The association of anatomic lesions, young age, and absence of other causes suggests that the relationship between HCY and arterial aneurysm observed in these two patients was not coincidental.

[Collagenous colitis. A study of 11 cases]. / La colite collagène. A propos d'une série de 11 cas.

BACKGROUND: Collagenous colitis (CC) is a rare large bowel inflammatory disorder. The aim of this retrospective study was to analyze extra-digestive manifestations of CC, associated systemic diseases, clinical course and response to therapeutic regimens. PATIENTS AND METHODS: Between 1989 and 1999, eleven patients were diagnosed as having CC, in two Departments of Internal Medicine and Gastroenterology, on the basis of usual criteria: chronic watery diarrhea, endoscopic examination showing normal findings and subepithelial collagen band thicker than 10 micrometer. RESULTS: Ten women and one man, age 39 to 88 years (mean: 66.6), had had their diarrhea for 1 to 240 months before diagnosis (mean: 48). Associated extra-digestive manifestations were: weight loss (3 cases out of 11), inflammatory arthralgia (4 cases), fever (2 cases), pelvispondylitis (2 cases), autoimmune thyroiditis (2 cases), and Sharp syndrome (1 cases). Laboratory abnormalities were: inflammatory syndrome (3 cases), anemia (5 cases), hypogammaglobulinemia (3 cases), and hypoalbuminemia (4 cases). Stool cultures and parasite examination were negative in all patients. Therapy used successively for CC until resolution of chronic diarrhea (uncontrolled with symptomatic treatment) was: sulfasalazine in 7 patients (beneficial (B) in 5 out of 7), 5-aminosalicylic acid in 6 (B: 2/6), corticosteroids in 2 (B: 2/2), azathioprine in 1 (B: 1/1). After a mean follow-up of 18 months (range: 1-72), resolution of CC was observed in 8 out of 11 patients. DISCUSSION: Mean characteristics of CC were: female predominance (91%), presence of extra-digestive manifestations (36%); mucosal inflammation with subepithelial collagen band and frequent association to rheumatic or autoimmune diseases suggest an immune basis for CC. Usefulness of systematic colon biopsies is underlined. Clinical course is often favorable with sulfasalazine and 5-aminosalicylate acid. Corticosteroids are rarely used.

[Intramedullary spinal cord sarcoidosis. Case report and review of the literature]. / Neurosarcoïdose intramédullaire inaugurale d'une sarcoïdose systémique. A propos d'un cas et revue de la littérature.

Sarcoidosis is a multisystemic granulomatosis disease of unknown etiology which commonly involves hilary nodes and lungs. Involvement of the nervous system represents 5 to 15% of the cases. We report on a rare case of cervicothoracic spinal cord sarcoidosis in an African man and review the literature on the subject. His only symptoms were those of a subacute myelopathy. On MR-imaging, coexistence of abnormal signal of cervicothoracic spinal cord with mediastinal lymph nodes was suggestive of sarcoidosis, which was confirmed by bronchial biopsies making unnecessary biopsies of the spinal cord lesion. Under corticotherapy both symptoms and radiological abnormalities rapidly regressed. We emphasize the rarity of sarcoidosis exclusively revealed by a myelopathy. We stress the importance of searching other localizations which are easier and safer to biopsy than the spinal cord lesion itself and illustrate the MR appearances of the lesion which are suggestive, however not specific of the disease.