[Role of cyclosporine in the occurrence of neuro-Behçet's disease?]. / Responsabilité de la ciclosporine dans la survenue d'un neuro-Behçet ?

Central neurological involvement in Behcet's disease (neuro-Behcet) occurs in 10 to 20% out of the patient. We report a 47-year-old man treated with cyclosporine who presented with neurologic manifestations of Behçet's disease. Relationship between cyclosporine and neuro-Behcet's disease are discussed.

[Recurrent aphthosis: safety of low dose thalidomide]. / L'aphtose récurrente: innocuité du thalidomide à faible dose.

PURPOSE: Thalidomide is an effective treatment for recurrent aphthosis but its effectiveness at low dose has been rarely assessed. METHODS: Single-centre non-randomized retrospective open study. RESULTS: Forty-seven patients were treated with thalidomide for recurrent aphthosis (41 patients) or Behçet disease (six patients). Remission was obtained with a dose of 25 mg daily. Discontinuation of treatment for side effects was not observed in this case series. CONCLUSION: Low dose thalidomide below 50 mg daily is an effective and well tolerated treatment of aphthosis.

[Thromboprophylaxis assessment in an internal medicine department]. / Evaluation de la thromboprophylaxie dans un service de médecine interne.

PURPOSE: There is a lack for French official guidelines for prophylaxis of venous thromboembolism. Therefore an internal referential based on MEDENOX study inclusion criteria was proposed in our internal medicine department. The main goal of this study was to assess the appropriateness of thromboprophylaxis prescriptions regarding the internal referential. METHODS: A retrospective and observational study included all the patients older than 18 years hospitalized in February and July 2007 in our internal medicine department for at least 48 hours and receiving no anticoagulant treatment. For each patient the following criteria were recorded: admission diagnosis, past medical history, current treatment, creatinine clearance, duration of hospitalisation, thromboprophylactic treatment and course during hospital stay. RESULTS: Eighty-nine patients were included. Forty-two (47.2%) patients had a thromboprophylaxis indication. Among them 40 (95.2%) actually received a thromboprophylaxis. Ten of the 47 patients (21.2%) without thromboprophylaxis indication also received a thromboprophylaxis. The most frequent indications for thromboprophylaxis were an infectious disease with an age greater than 75 years old and a cardiac failure stage III or IV of the NYHA. CONCLUSION: Most of the studies concerning medical thromboprophylaxis highlight its underutilization in the absence of a referential. In our study the referential use increased the percentage of patients with appropriate thromboprophylaxis but was associated with over-prescriptions in 21.2% of the cases. The use of electronic alerts may increase the number of patients receiving the appropriate treatment.

Rituximab treatment of stiff-person syndrome in a patient with thymoma, diabetes mellitus and autoimmune thyroiditis.

Stiff-person syndrome (SPS) is an autoimmune neurological disorder characterized by stiffness of the skeletal muscle with superimposed spasms and production of autoantibodies to glutamic acid decarboxylase (GAD) and amphiphysin. The disorder results from B cell-mediated clonal production of autoantibodies, requiring treatment with immunosuppressors; however, treatment results have been somewhat inconsistent. We report the results of rituximab treatment in a patient with SPS associated with a thymoma, diabetes mellitus, autoimmune thyroiditis and the presence of anti-GAD and anti-amphiphysin autoantibodies. The patient experienced a partial improvement following a thymectomy and the administration of prednisone, intravenous immunoglobulins and mycophenolate mofetil. Treatment with rituximab was followed by a complete sustained remission and the disappearance of serum anti-amphiphysin antibodies.

[Kappa light chain deposition disease, presenting as Sjögren's syndrome, successfully treated by high-dose melphalan and autologous blood stem transplantation]. / Maladie de Randall, simulant une maladie de Gougerot-Sjögren, traitée avec succès par chimiothérapie et autogreffe de moelle osseuse.

INTRODUCTION: Light chain deposition disease is a systemic disorder characterised by tissue deposition of monoclonal immunoglobulin light chains without tinctorial properties. It has been exceptionally reported with salivary involvement mimicking Sjögren's syndrome and peripheral neuropathy. CASE REPORT: We report a case of light chain deposition disease associated with plasma cell dyscrasia presenting as sicca syndrome with salivary glands hypertrophy and polyneuropathy successfully treated by high dose melphalan and autologous blood stem transplantation. CONCLUSION: Light chain deposition disease should be recognized as an aetiology of sicca syndrome and peripheral neuropathy. Further studies should assess the prevalence of sicca syndrome in light chain deposition disease and better characterise the neurological manifestations.

[Usefulness of antineutrophil cytoplamic antibodies in giant cell arteritis]. / Valeur des anticorps anticytoplasme des polynucléaires neutrophiles dans la maladie de Horton.

PURPOSE: The relevance of antineutrophil cytoplasmic antibody (ANCA) during giant cell arteritis is not established. The purpose of our study was to estimate the ANCA frequency and their impact on disease course of giant cell arteritis. METHODS: Thirty patients were followed-up for giant cell arteritis and had a detection of ANCA. RESULTS: Nine patient had positive ANCA (30%). These patients had no significant differences regarding clinical and biological data or number of relapses in comparison with patients having negative ANCA testing. However, the relapse occurred earlier in the presence of ANCA (six months versus 31.5 months). CONCLUSION: In giant cell arteritis, detection of ANCA seems predictive of a premature relapse and should be associated with an acute monitoring of ANCA positive patients.

[Stiff person syndrome associated with thymoma. Efficacy of thymectomy]. / Syndrome de la personne raide associé à un thymome: efficacité de la thymectomie.

INTRODUCTION: Stiff person syndrome is a rare autoimmune neurologic disorder characterized by axial muscular rigidity and intermittent painful spasms. Three distinct forms are described: auto-immune, paraneoplastic and idiopathic. EXEGESIS: We report the case of a 51-year-old man with an history of Stiff Person Syndrome with typical clinical, electrophysiological and immunological findings. Anti-glutamic acid decarboxylase antibodies were present, as well as anti-amphiphysin antibodies, commonly reported in the paraneoplastic syndrome. CT scan revealed a thymoma. Surgical resection was followed by reduction of the neurologic symptoms, without lowering the auto antibodies titer. CONCLUSIONS: The association between Stiff Person Syndrome and thymoma is exceptional. Thymectomy is an effective treatment and may act without change of the autoantibodies titer. Patients with Stiff Person Syndrome should be systematically tested for thymoma.

[Pseudo-myasthenic myopathy in a woman with anorexia nervosa]. / Pseudomyasthénie grave chez une anorexique.

Skeletal myopathy, although foreseeable in severe malnutrition, has been rarely reported in patients with anorexia nervosa. We report a case of oculo-oropharyngeal myopathy mimicking myasthenia in a 42-year-old woman with an history of anorexia nervosa, completely reversible after refeeding with carbohydrate diet, mainly chocolate. Nutritional myopathies are not well known and therefore rarely looked for in patients with anorexia nervosa. Classical presentation is a myogenic syndrome with severe type 2 fibre atrophy and biochemical characteristics similar to Mac Ardle's disease. A clinical form mimicking myasthenia gravis has never been reported. Refeeding programme leads to total recovery within a few days.

[Pronostic value of C-reactive protein measure in elderly patient with acquired pneumonia: correlation with Fine's score]. / Valeur pronostique du dosage de la protéine C réactive (CRP) dans les pneumopathies aiguës infectieuses du sujet âgé: corrélation avec le score de Fine 97.

UNLABELLED: The Fine's score, a predictive score of infectious pneumonia gravity, does not integrate inflammatory parameters, which are routinely used in the management of infectious pneumonia. The aim of our study was to establish a correlation between the Fine's score and C reactive protein. PATIENTS AND METHODS: One hundred patients hospitalized for infectious pneumonia, 57 men and 43 female, with an average age of 85 years were retrospectively recorded. RESULTS: The median level of C reactive protein was 157 mg/L. Global mortality rate was 26% and respectively 5, 16 and 45% in the grades III, IV, V of the Fine's score. Beyond 75,5 mg/L C reactive protein concentration, the mortality rate was contained between 28 and 32%. The comparison of the ROC curves of the Fine's score and C reactive protein did not showed any difference. CONCLUSION: C-Reactive protein is less precise than the Fine's score to assess infectious pneumonia gravity but seems to be an indicator of the potential gravity of the pneumonia.