Neuro-Behçet's disease (isolated cerebral thrombophlebitis excluded). Clinical pattern, prognostic factors, treatment and long term follow-up.
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to A Sbaï.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
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.
Explore the source record for details and available documents.
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.
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.
BACKGROUND: Vascular abnormalities are being reported with increasing frequency as a cause of major lower gastrointestinal hemorrhage in the elderly. They are occasionally very difficult to treat by conventional means. CASE REPORT: A 66-year-old white man with a history of type 2 diabetes mellitus, coronary artery disease, congestive heart failure, severe peripheral arterial occlusion disease and chronic renal insufficiency presented for five years recurrent major bleeding due to gastrointestinal angiodysplasia, requiring repeated transfusions. He was treated with efficacy using ethinyl-estradiol (30 micrograms) and norethisterone acetate (1 mg) given orally once daily. After six months of treatment, transfusion requirements fell to 0 unit and the patient's hemoglobin was stable at 13 g/dl. Attempts to stop hormone therapy (by the patient himself, without complaint of side effects) led to a fall in hemoglobin. CONCLUSION: Hormonal therapy should be considered when multiple degenerative mucosal vascular bleeding lesions are beyond the reach of therapeutic endoscopy leading to high transfusion needs and when surgical risk is unacceptably high.
INTRODUCTION: Behçet's disease is a systemic inflammatory disorder characterized by vasculitis. Its typical features are recurrent oral and genital ulcerations with uveitis. Although vascular lesions are not listed among the criteria for diagnosis of Behçet's disease, up to 25-35% of the patients develop complications in arterial and venous large vessels. EXEGESIS: We describe the case of a 45-year-old French man with Behçet-type vasculopathy. Though only one sign of Behçet's disease, i.e., oral ulcerations, was present, the patient had to undergo emergency surgery three times. The postoperative treatment combined corticosteroids, azathioprine, and oral anticoagulants. Three years later no therapy failure was observed. CONCLUSION: On the basis of 1) recurrent aortic aneurysms, 2) large arterial and venous occlusive lesions, 3) superficial phlebitis, and 4) ulcerations of the aorta in macro- and microscopic examination of resected aortic walls, we concluded that the patient's life threatening vasculopathy was of the Behçet's type, even if several of the diagnostic features of Behçet's disease were lacking. Variations in clinical features of Behçet's disease are observed that might be due to hereditary traits, particularly to the genetic expression of an incomplete phenotype that would lead to the lack of typical clinical features.
A 62-year-old white woman with chronic hepatitis C virus infection was diagnosed with systemic sarcoidosis during treatment with interferon-alpha. Seventeen cases of sarcoidosis associated with interferon treatment have been reported, with only 8 inpatients with chronic hepatitis C virus infection. Our patient, with no past history of sarcoidosis, developed subcutaneous sarcoid nodules, arthritis, uveitis, pneumonitis and heart involvement two months after she was started on treatment with interferon-alpha. Her symptoms resolved when she began corticosteroid therapy and interferon-alpha was stopped. This case report suggests that exogenous interferon-alpha may trigger or contribute to the development of this multisystem granulomatous disorder in which interferon-gamma and CD4 + T-lymphocyte are mainly involved. Patients should be monitored during and following interferon-alpha therapy, since the autoimmune diseases induced by interferon therapy do not always improve after cessation of treatment.
Spinal neurosarcoidosis is rare (0.43 p. 100 of all sarcoidosis) and can be the initial manifestation of the disease. A 43-year-old right handed African man developed a progressive dorsal neck pain, slowly worsened paresthesia weakness in the legs and a gait disturbance. Magnetic resonance imaging (MRI) scan of the cervical and thoracic spinal cord (sagittal T(1)- weighted image) revealed diffuse enlargement of the cord from C2 to T7 with intense intramedullary enhancement from C2 to T3 after administration of contrast material on sagittal T(1)- weighted image. Cranial MRI scan was normal. Radiographs of the chest revealed bilateral symmetric hilar mediastinal lymphadenopathy with no pulmonary infiltrates. Bronchial biopsy demonstrated non caseating granulomas with langerhans giant cells. The serum angiotensin converting enzyme level was elevated. The patient received corticosteroid with good progressive response. His neurologic symptoms improved markedly and twenty months post-treatment MRI showed no abnormality on enhanced T(1)- weighted images. Two years later he had no relapse. Only 6 to 10 p. 100 of patients with neurosarcoidosis have spinal cord involvement, which may account for the first clinical manifestation of the disease. Most patients with neurosarcoidosis have associated extraneurologic abnormalities. The diagnosis is supported usually by extraneurologic biopsies. Spinal cord biopsy needs to be considered on case-by-case basis.
Lymphocytic gastritis is characterized by intense lymphocytic infiltration of gastric epithelium. Excessive gastric protein loss is uncommon. We describe the case of a 49-year-old white woman suffering from generalized edema and abdominal pain. She had severe serum hypoproteinemia, hypoalbuminemia and hypogammaglobulinemia. There was no renal, cardiac or hepatic origin of protein loss, and no protein-losing enteropathy. Endoscopic examination showed diffuse varioliform gastritis and histology confirmed lymphocytic gastritis with > 30% intraepithelial lymphocytes without Helicobacter pylori. The protein loss stopped within two weeks of the beginning of omeprazole and extensive edema disappeared. Four years later, the patient was still free from edema. Inflammatory involvement of the gastric mucosa probably caused protein losing in this patient. Recognition of this exsudative gastropathy is important because long term remission is obtained with omeprazole.
Acute idiopathic pericarditis is complicated by recurrence in 15 to 30% of cases. The preventive treatment of recurrences is not well codified. Aspirin, non-steroidal anti-inflammatory drugs and corticoids are the commonest prescribed treatments. The objective of this study was to assess the value of colchicine in the prevention of recurrences of acute idiopathic pericarditis. Twenty-eight cases of recurrent acute pericarditis (2 episodes, 1 month between each episode) admitted to a department of internal medicine between 1989 and 1999 were reviewed. Analysis was concentrated on the 13 idiopathic forms. The subjects were 7 women and 6 men with an average age of 41 years (10-62) at the time of the first episode of acute pericarditis. These 13 patients were treated with colchicine (1 to 2 mg/day) after failure of conventional treatment (aspirin 13/13, NSAID 13/13, steroids 9/13 and pericardiocentesis 3/13). Ten patients were followed up regularly (6 months after starting colchicine) and were improved with respect to number, duration and intensity of their recurrences. The average duration of colchicine therapy was 17 +/- 14 months (6-48). Progressive withdrawal of NSAID and steroids was obtained in 8/10 cases. The authors conclude that colchicine is useful in the prevention of recurrence of acute pericarditis. It may be proposed as treatment of choice, especially in the idiopathic forms.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
UNLABELLED: In the most cases the causes of systemic vasculitis are unknown and treatment is symptomatic (corticosteroids often associated with immunosuppressive agents). We report three cases of systemic vasculitis associated with infections for which dramatic improvement was observed without cortico-therapy (in two patients). CASE REPORT 1: A previously overweight 72-year-old woman was admitted because of a one-year history of fever, fourteen kilogram weight loss, vascular purpura, and polyneuropathy. Abnormal laboratory values included inflammatory syndrome [erythrocyte sedimentation rate (ESR): 80mm/first hour, thrombocytosis: 500,000/microliter, hypereosinophilia (1200/microliter) and positive perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) [anti-myelo-peroxydase antibodies: 30 U (normal < 7)]. Neuromuscular biopsy showed necrotizing vasculitis involving small and medium arteries. Further studies revealed a florid diverticulosis of the colon and no other severe visceral involvement. Treatment was started then with sigmoidectomy. Within six weeks her general condition improved dramatically without corticotherapy: regression of all systemic manifestations, the ESR normalized, and p-ANCA became negative. Ten months later she was still asymptomatic. CASE REPORT 2: An 50-year-old-man developed a progressive fifteen-kilogram weight loss (within 2 months), severe polyneuropathy of all four limbs. His ESR was 120 mm/first hour, and C-reactive protein 200 mg/l. Neuromuscular biopsy showed necrotizing vasculitis affecting small vessels in the nerve and no immune deposits. Stomatologic examination revealed a multiple foci of dental infections. The extraction of all these foci of infections associated with antibiotics improved dramatically all systemic manifestations (within eight weeks), once more without corticotherapy. Eight months later the patient remained asymptomatic. CASE REPORT 3: A 30-year-old-woman was admitted because of five-week history of fever, myalgias, polyarthritis, and cutaneous nodules in her limbs. Abnormal laboratory values included inflammatory syndrome, proteinuria of 0.7 g/day, and a significant rise in Chlamydia trachomatis antibodies titres from 1/64 to 1/256 over a 5 week period. She had a previous history of genital condyloma. The prednisone initialed (0.5 mg/kg/day) twelve days prior to admission was gradually reduced (stopped within 2 months) and treatment with doxycycline (200 mg/day) was initiated. Within six weeks of antibacterial treatment we assisted to a total regression of the initial clinical manifestations and laboratory values became normalized. Ten months later she remained asymptomatic. CONCLUSION: In systemic vasculitis, investigations in a search of foci of infections are of dual interest: possible etiologic agent like our case reports (strong evidence for an infectious association) and, from a therapeutic perspective, we must identify the microbes behind vasculitis syndromes, since treatment with corticosteroids may have serious consequences if the patient has an active infectious disease.
BACKGROUND: D-penicillamine-induced muscle disorders are well-known, tiopronine-induced disorders are less often reported. CASE REPORT: A 62-year-old patient, given tiopronine for rheumatoid arthritis, developed severe polymyositis with characteristic clinical and pathology features. The course was favorable after tiopronine withdrawal and substitution with methotrexate. DISCUSSION: Clinicians should be aware of the side-effects of tiopronine, particularly muscle disorders, and implement careful surveillance to achieve early diagnosis and appropriate therapy.