[Characteristics of ocular involvement in Behçet disease in Tunisia: Monocentric study and review of literature]. / Caractéristiques de la maladie de Behçet avec atteinte oculaire en Tunisie: étude monocentrique et revue de la littérature.

BACKGROUND: Behçet's disease is a multisystemic inflammatory disease characterized by recurrent oral and genital ulcers, skin lesions and uveitis. The diagnosis of Behçet's disease is based on clinical criteria. The etiology of the disease is unknown but the wide variations of ethnic prevalence and of the prevalence in the same ethnic group in different geographic areas indicate environmental triggering of a genetically determined disorder. PATIENTS AND METHODS: A retrospective analysis of the medical charts of 150 Behçet's disease patients seen in our internal medicine department between 1995 and 2010 was undertaken. Patients with confirmed ocular involvement were analyzed and compared with those without ocular involvement. RESULTS: Among the 150 medical charts studied, 85 patients were included in the study. Thirty-three patients (38.5%) had ocular involvement. Mean age at ocular BD diagnosis onset were 35.3. Male to female ratio was 5.6. Ocular involvement was bilateral in 26 patients (78.8%). Uveitis was the most common ocular lesion (n=31 patients, 93.9%). Panuveitis was the most common anatomical location (n=21, 63.6%). The comparison of patients treated for BD with or without ocular involvement showed a statistically significant association between ocular and neurological manifestations (p=0.03). All patients with ocular involvement were treated with corticosteroids. Immunosuppressive (IS) treatments were used in 28 patients (84.8%). Cyclophosphamide was the most used as first-line treatment (71.4%). Cyclophosphamide relayed by azathioprine was the most adopted protocol (28.5%). In case of resistance or relapse and depending on the other manifestations of the BD, the IS used in first intention was replaced by another one. Seven of the 33 patients had received treatment with infliximab (IFX) after failure of other therapeutic lines. CONCLUSION: Ocular prognosis in the BD can be improved by early treatment and regular monitoring. It is important to adjust the therapeutic protocol to the anatomic form, to the severity of uveitis and to the extra-ocular manifestations associated.

[Central nervous system involvement in systemic sclerosis]. / Une atteinte du système nerveux central au cours de la sclérodermie systémique.

Systemic sclerosis is a connective disease usually known to spare the central nervous system. This has been much debated by recent imaging studies. We report a 56-year-old woman followed-up for systemic sclerosis since 2005. Four years later, she presented with cerebellar and pyramidal syndrome. Magnetic resonance imaging showed signs of cerebral vasculitis. The patient was treated by corticosteroids and cyclophosphamide pulses followed by azathioprine for maintenance therapy. Clinical and radiological outcomes were favourable. In patients with systemic sclerosis and neurological symptoms, abnormalities in the cerebral magnetic resonance imaging may, in the absence of another obvious etiology, indicate a central nervous system involvement associated with this systemic disorder.

[Pleural amyloidosis attesting to generalised amyloidosis]. / L'amylose pleurale: manifestation révélatrice d'une amylose généralisée.

During the course of generalised amyloidosis, pulmonary diseases are mainly represented by tracheobronchial involvement and diffuse or nodular parenchymal localizations. The authors report the case of a 61-year-old woman presenting exudative pleural effusion. The diagnosis of generalised amyloidosis in association with multiple myeloma with cardiac and renal involvement was established. A thoracoscopy performed during the investigation of the pleural effusion and the biopsy revealed amyloid deposits in the pleura. The patient died although melphalan/prednisone treatment was initiated. Pleural involvement in amyloidosis is without doubt under-estimated and plays a central role in the creation and persistence of pleural effusion.

[Cytomegalovirus myelitis in immunocompetent adult]. / Myélite à cytomégalovirus chez un adulte immunocompétent.

INTRODUCTION: Cytomegalovirus (CMV) infection occurs in 40 to 100% of general population. It is often asymptomatic in immunocompetent subject but may induce neurological syndromes such as encephalitis and myelitis. CASE RECORD: We reported a case of a 64-years-old woman immunocompetent, with acute proximal upper and lower limb weakness, paresthesias and two episodes of urinary retention. MRI of the spine showed abnormal enhancement from cervical to lumbar spine indicative for myelitis. Diagnosis of CMV associated myelitis was confirmed by a positive CMV serology. Administration of ganciclovir was followed by a partial improvement in five months. DISCUSSION: Few cases of CMV acute myelitis in immunocompetent patients have been reported in the literature. The pathogenesis is not well known, however, immune-mediated central nervous system damage may be attributed to the pathogenesis of the disease. Early diagnosis and treatment improves the prognosis.