An extensive hemimelic polyostotic fibrous dysplasia: a case report.

We report a new case of polyostotic fibrous dysplasia (FD). A 26-year-old woman was referred to our department complaining of pain in her left arm. She had suffered for tow fractures in left leg and arm previously. Plain radiographs showed osteolytics lesions at the left humerus and radius. Histological examination of the surgical specimens showed FD. She has beneficed with zoledronic acid perfusion.

[Sciatica in a Tunisian teenager: pelvic hydatid cyst]. / Lombosciatique chez un adolescent tunisien. Kyste hydatique pelvien.

The purpose of this report is to describe the case of a 13-year-old boy presenting typical sciatica leading to the discovery of primary pelvic hydatid cyst extending to the ischiatic bone. Diagnosis was suspected based on echography and magnetic resonance imaging and confirmed by surgical exploration.

[Chronic hydarthrosis of the knee in a Tunisian patient: tabetic arthropathy]. / Epanchement articulaire récidivant du genou chez un tunisien.

Tabetic arthropathy (TA) is a type of neuropathic arthropathy that has become rare. The purpose of this report is to describe a case of tabetic arthropathy involving the knee. A 53-year-old man was hospitalized for painless right knee arthropathy. Clinical examination demonstrated hydarthrosis of the right knee, peripheral neuropathic syndrome of the lower limbs, and paralysis of the IXth and Xth cranial pairs. Plain radiography showed the presence of lytic lesions in the internal femoral condyle and tibial plateau. Syphilitic serology tests were positive in the blood and cerebrospinal fluid.

MHC class I and class II genes in Tunisian patients with reactive and undifferentiated arthritis.

OBJECTIVE: To study HLA class I and class II association in Tunisian patients with reactive (ReA) and undifferentiated arthritis (UA). METHODS: The study included 17 patients with ReA defined according to the European Spondylarthropathy Study Group criteria for spondylarthropathy (SpA), 11 patients classified as having undifferentiated arthritis and 100 unrelated healthy controls. HLA class I antigens were typed serologically and HLA class II alleles were genotyped molecularly by the polymerase chain reaction with sequence-specific primers technique. RESULTS: There was a major difference between HLA alleles in ReA and UA patients when compared separately with controls. Increased frequencies of HLA-B27 (p=7.76 10-12, OR=59.30), HLA-B51 (p=0.015, OR=4.91) and HLA-DRB1*04 (p=0.033, OR=2.90) alleles were found in patients with ReA but not in patients with UA. HLA-B27 was not expressed totally in our cohort of UA patients. A significant increase of HLA-B15 (p=0.002, OR=18.40) and a moderate increase of HLA-B7 (p=0.043, OR=5.15) was found in patients with UA, but not in patients with ReA. In the B27 negative patients, HLA-DRB1*04 association with ReA was found independently of B27. CONCLUSION: Our data confirmed a significant association of HLA-B27 with ReA in the Tunisian population. Our results also suggested that some of the additional HLA antigens were associated with ReA including HLA-B51 and HLA-DRB1*04 alleles. UA seemed to have a genetic background different from ReA in Tunisian patients.

[Erasmus syndrome: case report]. / Syndrome d'Erasmus: à propos d'une observation.

Erasmus' syndrome involves the association of systemic scleroderma and exposure to silica particles with or without silicosis. The authors report the observation of a miner with a history of professional silicosis resulting in pulmonary fibrosis. Twenty-five years later, the patient consulted for dysphagia associated with inflammatory arthralgia, cutaneous sclerosis extended to the face and limbs, sclerodactyly and Raynaud's phenomenon. The diagnosis of scleroderma was confirmed and Erasmus' syndrome was retained.

[Pericardial tamponade complicating rheumatoid arthritis: a case report]. / Tamponnade compliquant une polyarthrite rhumatoïde : à propos d'une observation.

BACKGROUND: Cardiovascular involvement in rheumatoid arthritis (RA) is increasingly observed and may be associated with the severity of rheumatoid arthritis. It is dominated by heart ischemic diseases related to atherosclerosis. Specific rheumatoid heart disease is commonly asymptomatic and found at autopsy or by echocardiography. Pericarditis is the commonest cardiac complication of RA. It is rarely clinically apparent and pericardial tamponade is exceptional. CASE REPORT: Herein, we report an unusual case of a 53-year-old female patient with a six-year history of seropositive and erosive rheumatoid arthritis who had developed a pericarditis complicated with tamponade resolved by pericardiocenthesis and high dose systemic steroids. Histopathology showed chronic inflammation and fibrosis. Under 1mg/day of colchicine, there were no recurrences at 10 months. CONCLUSION: Pericarditis is uncommon in rheumatoid arthritis. Forms with constriction or tamponade may have a fatal outcome. Pericardectomy usually recommended in constrictive forms, is sometimes indicated for tamponade. Some observations and randomised studies of idiopathic pericarditis suggest that colchicine may be interesting for the treatment and prevention of recurrences of rheumatoid arthritis-associated pericarditis.

[Primary synovialosarcoma: a case of unusual location]. / Le synovialosarcome primitif: a propos d'une localisation inhabituelle.

AIM: Report a new case of primary Synovialosarcoma. CASE: We report a case of a 16 year old girl, who presented with a tumefaction on the right clavicle evolving for 3 months. Various explorations carried out, in particular thoracic scanner and imagery by magnetic resonance, showed a multilocular cystic formation of the seat and the clavicle reaching the mediastinum. A pathological examination of the part (piece) highlighted a biphasic synovialosarcoma of grade III. This diagnosis was validated by the immunohistochemistry study and the detection of transcribed specific fusion of the synovialosarcoma, SYT-SSX1. CONCLUSION: The unusual locations of the primitive SS must be known because the diversity of their microscopic aspects may cause confusion in the diagnosis. The immunohistochemistry, and more recently the cytogenetic studies, helps to solve these problems of differential diagnosis.

[Prevalence of osteoporotic fractures in Tunisian women]. / Prévalence des fractures ostéoporotiques chez la femme tunisienne.

PURPOSE OF THE STUDY: Osteoporotic fractures are an important public health problem due to significant morbidity and mortality and the socioeconomic burden. In Tunisia, the impact of such fractures is often underestimated due to lack of epidemiological data. We evaluated the prevalence of these fractures in a sample population at risk in order to better ascertain the amplitude of this problem in our country. MATERIAL AND METHODS: This prospective study involved 2,000 menopaused women aged 50 years or more. The sample was randomly selected among the population of Manouba, an urban administrative district of the capital of Tunisia. One thousand three hundred eleven women agreed to participate in this survey. Women with a risk of secondary osteoporosis were excluded. We retained for study fractures related to primary osteoporosis. History taking recorded anthropometric data and medical history, particularly gyn-ob history. All fractures occurring after minor trauma, in women aged 50 years or more, and involving the femoral neck, the wrist, or the proximal humerus were noted. Lateral x-rays of the thoracolumbar spine were studied to search for unrecognized vertebral fractures. RESULTS: The study population included 1,311 women, mean age 64.07 +/- 9.31 years, menopaused at mean 48.29 +/- 3.84 years. Among these women, 212 (16.2%) had presented a fracture at one of the localizations retained for study. Vertebral wedge fractures accounted for 59.83% of all fractures, wrist fractures 31.96%, proximal femoral fractures 4.51% and proximal humeral fractures 3.69%. DISCUSSION: Osteoporotic fractures are not uncommon in women in Tunisia. Increasing life expectancy in our country suggests this public health problem will grow in the years to come, pointing out the importance of better management of osteoporosis to prevent fractures.

Hereditary chondrocalcinosis in a Tunisian family.

A clinical and radiological survey of 77 members of a Tunisian family with hereditary chondrocalcinosis was performed. Articular chondrocalcinosis was documented by X-rays in 7 living members of 3 generations. No associated or secondary forms of the disease were found. Clinical features of the disease appeared early in life and radiologic involvement was extensive. The mode of inheritance appeared to be autosomal dominant with incomplete penetrance. Electron microscopy study of synovium and cartilage biopsies from one patient demonstrated calcium pyrophosphate dihydrate crystals. HLA typing revealed that all affected subjects bore the haplotype A1 B12 DR3.

[Current aspects of Pott's disease in Tunisia. 29 cases]. / Aspects actuels du mal de Pott en Tunisie. A propos d'une série de 29 cas.

It is a retrospective study, reviewing all cases of vertebral tuberculosis recorded between 1989 and 1999 in La Rabta Hospital (Tunis). 29 patients have been included whose average age was 49 years. The main symptoms which appeared within a average period of six months were vertebral pain, associated with fever in half of the cases. The diagnosis of infectious spondylitis was made according to the results of Computer tomography in 26 cases. The diagnosis of infection was confirmed by characteristic histological pattern and/or culture biopsy. 27 patients were cured without complications except for 2 of them who developed a spinal cord compression. This complication was quickly stemmed by steroid therapy.

[Hydatid cyst of the adductor muscles. MRI aspect, Apropos of 1 case]. / Kyste hydatique des adducteurs de la cuisse. Aspect I.R.M, à propos d'un cas.

Muscular involvement is rare, representing less than 1% of hydrated cyst localisation's. Clinical symptoms are insidious and non-specific causing a frequent delay in diagnosis. We report the observation of a 50 year-old woman consulting for inguinal tumefaction with radiological exploration suggestive of hydrated cyst of adductors muscles. Magnetic resonance imaging is helpful in diagnosis, since it reveals a very suggestive aspect. Treatment is based on surgery, which cures patients but incurs a low risk of local relapse.

[Light chain multiple myeloma revealed by an amyloid arthropathy. A report of two cases]. / Myélome à chaînes légères révélé par une arthropathie amyloïde. A propos de deux observations.

INTRODUCTION: Amyloid arthropathy occurs in 5-15% of patients with multiple myeloma. It is rarely inaugural. Some cases are reported in which the articular manifestations are present many months before the diagnosis of myeloma. We report two unusual cases where the amyloid arthropathy reveals the multiple myeloma. EXEGESIS: Case 1: A 54-year-old man take medical advice for a polyarthritis evolving for 6 months. He has no evidence of multiple myeloma except a hypercalcemia and a hypoalbuminemia. The diagnosis of multiple myeloma is based on an immunobinding of plasmatic proteins. It is a light chain multiple myeloma, lambda type. Amylosis is revealed by biopsy specimens from the synovial tissue of the knee. The patient died rapidly because of an intestinal hemorrhage. Case 2: A 78-year-old woman complains from a polyarthritis evolving for 4 months. She has besides an elevated erythrocyte sedimentation rate, an anemia and a renal insufficiency, a profound hypogammaglobulinemia. There is no radiological abnormalities. The diagnosis of multiple myeloma is based on serum and urine immunoelectrophoresis. It is a light chain multiple myeloma, lambda type. The patient is at her sixth monthly course of cyclophosphamid and prednisone. As far as amyloid arthropathy is concerned, it is relatively improved by corticoid intra-articular injections. CONCLUSION: These are two case reports of a light chain multiple myeloma of lambda type, associated to an amyloid arthropathy in its polyarticular presentation, mimicking a rheumatoïd arthritis. The revelatory nature of the amyloid arthropathy is interesting to report.

[Patelar metastasis of primary lung cancer]. / Métastase rotulienne d'un cancer broncho-pulmonaire primitif.

Patellar metastasis is uncommon. The clinical presentation is often misleading, suggestive of septic arthritis or meniscal disorder after knee trauma. We report the case of a 71-year-old patient who developed knee pain aggravated by movement with pseudo-blockage which was found to be related to a secondary localization of a primary lung cancer. Low blood flow in the patella probably explains why patellar metastasis is so rare.

Sweet's syndrome in a patient with rheumatoid arthritis, Sjögren's syndrome and lymph node tuberculosis.

Sweet's syndrome (SS) is an acute neutrophilic dermatosis characterised by abrupt onset of fever, leukocytosis and cutaneous eruption, with dermal neutrophilia on skin biopsy. Most cases are idiopathic but SS can be associated with various affections, especially neoplastic, inflammatory and infectious diseases. The authors report the case of an SS occurring in a patient with a known rheumatoid arthritis associated with a secondary Sjögren's syndrome, with incidental finding of concurrent lymph node tuberculosis. In case of SS, an associated disease (malignant, inflammatory or infectious diseases) must imperatively be searched for, knowing that two or more of these affections can coexist.

Back pain caused by a pseudo-tumorous vertebral collapse: atypical presentation of primary vertebral hydatidosis.

Hydatidosis, also known as echinococcosis, is a rare but serious parasitic disease in endemic areas. Primary spinal location is extremely rare. This case report describes a rare instance of hydatid cyst that caused severe and progressive low-back pain and neurologic dysfunction. Spine MRI showed a unique vertebral collapse of Th12 body with multicystic lesions filling the spinal canal. In addition, hydatidosis serodiagnostic test was positive at 1/725. Treatment depended on the actual surgical removal of the cysts. Surgery consisted in excision and extirpation of the cysts, associated with decompressive laminectomy. The diagnosis was confirmed on the basis of histological results. No coincidental hydatid visceral involvement was found. Antihelminthic drugs (Albendazole) were promptly given before surgery for a long period. The outcome was satisfactorily marked by total regression of the motor deficit and sphincter disorders.

Brucellar spondylodiscitis affecting non-contiguous spine levels.

Brucellosis is a zoonosis that affects several organs. The spine is the most common site of musculoskeletal involvement. However, multiple-level spinal involvement is rare in brucella spondylodiscitis. The authors report a case of a 56-year-old male shepherd who had developed a spondylodiscitis affecting simultaneously the cervical, thoracic and lumbar regions. The diagnosis was established by using MRI after the brucella-agglutination test was found to be positive. A high degree of suspicion in the diagnosis of brucellar spondylodiscitis is essential to reduce the delay for the treatment. Thus, it should be essentially included in the differential diagnosis of longstanding cervical, thoracic or back pain, particularly in regions where brucellosis is endemic. Screening serological tests for brucella should be used more widely in cases with low index of suspicion, especially in endemic areas.

Spine fracture in patient with ankylosing spondylitis: A case report.

Spinal fractures in patients with ankylosing spondylitis may be the result of minor trauma. These fractures may lead to severe neurological deficits, and they are difficult to detect using standard radiography. Often, CT-scans and MRI are required for diagnosis.