1/19. Systemic lupus erythematosus-associated catastrophic antiphospholipid syndrome occurring after typhoid fever: a possible role of Salmonella lipopolysaccharide in the occurrence of diffuse vasculopathy-coagulopathy.We report a case of well-documented typhoid fever in a 30-year-old woman with inactive systemic lupus erythematosus with asymptomatic lupus anticoagulant and high-titer anticardiolipin antibody (aCL). Despite prompt eradication of the salmonella typhi obtained with appropriate antibiotic therapy, multiple organ system dysfunction occurred. The central nervous system was involved, with ischemic infarcts in the occipital lobes. High-dose corticosteroid therapy failed to improve the neurologic manifestations, which responded to repeated plasmapheresis. A sharp fall in aCL and anti-beta2-glycoprotein I antibody titers was recorded before the start of plasmapheresis. At the same time, IgM and IgG antibodies to Salmonella group O:9 lipopolysaccharide became detectable; the IgM antibodies disappeared within 4 months, whereas the IgG antibodies remained detectable during the next 13 months. Despite treatment with high-dose corticosteroids and cyclophosphamide, rapidly progressive glomerulonephritis developed, leading to chronic renal failure. There is convincing evidence of a link between the S. typhi infection and the ensuing catastrophic syndrome in this patient, probably precipitated by bacterial antigens.- - - - - - - - - - ranking = 1keywords = lobe (Clic here for more details about this article) |
2/19. Primary antiphospholipid syndrome and cerebral atrophy: a rare association?Neurologic complications are common in patients with antiphospholipid syndrome. In this article, we report the case of a young woman with neurologic disorders, a history of hypertension and transient ischemic attacks, and cerebral atrophy associated with primary antiphospholipid syndrome (PAPS). magnetic resonance imaging of the brain showed multiple ischemic lesions and remarkable atrophy of frontal and parietal lobes. Cerebral atrophy in patients with PAPS can be considered as a feature of this disease. The case is discussed on the basis of relevant past literature. Although there are few reports on neuroradiologic findings in patients with PAPS, cerebral atrophy has been described. Because PAPS is more frequently recognized today than in the past, this condition should be included in the differential diagnosis of cerebral atrophy, particularly in young patients.- - - - - - - - - - ranking = 1keywords = lobe (Clic here for more details about this article) |
3/19. chorea in juvenile primary antiphospholipid syndrome. Reversible decreased circulation in the basal ganglia visualised by single photon emission computed tomography.chorea was observed in a 12-year-old girl with primary antiphospholipid syndrome (APS). She developed severe chorea in a few weeks. On immunosuppressive treatment, including high doses of glucocorticoids and cyclophosphamide, she had a rapid clinical recovery. Single photon emission computed tomography (SPECT) of the brain showed decreased circulation in the basal ganglia and in the medial parts of both temporal lobes. One month after treatment, SPECT was completely normalised. APS in children has a variety of clinical manifestations, and should be suspected in cases of unexplained thromboembolic disease or obscure neurological symptoms.- - - - - - - - - - ranking = 1keywords = lobe (Clic here for more details about this article) |
4/19. antiphospholipid syndrome with cortical blindness resulting from infarction around the posterior cerebral artery in an elderly woman.An 87-year-old woman with antiphospholipid syndrome accompanied by cortical blindness and thalamic syndrome resulting from infarction of the posterior cerebral artery is reported. She was hospitalized because of laceration of the head. Two months later, she complained of loss of visual acuity, sharp pain and numbness involving the left half of the body except her face. New right posterior lobe infarction and the existence of old left infarctions were confirmed by serial CT scans. Helical CT scan revealed embolization of the posterior cerebral artery with atherosclerotic stenosis. Serological examination showed biologically false-positive and positive findings for lupus anticoagulant. She was treated with warfarin potassium and clonazepam.- - - - - - - - - - ranking = 1keywords = lobe (Clic here for more details about this article) |
5/19. Catastrophic antiphospholipid antibody syndrome manifesting as an orbital ischemic syndrome.Painful bilateral ophthalmoparesis, marked proptosis, increased intraocular pressure, and blindness developed in a 29-year-old woman with protein c deficiency and catastrophic antiphospholipid syndrome. magnetic resonance imaging of the orbits showed bilateral proptosis, globe tenting, and tethering of the optic nerves consistent with an orbital ischemic syndrome. Despite aggressive therapy for antiphospholipid syndrome, the patient died. The autopsy showed necrosis of orbital tissues. This is the first report of orbital ischemic syndrome from protein c deficiency and antiphospholipid syndrome.- - - - - - - - - - ranking = 1keywords = lobe (Clic here for more details about this article) |
6/19. Primary antiphospholipid syndrome: an unusual cause of adrenal insufficiency.We describe a boy, 10 years and 5 months old, who developed acute adrenal gland insufficiency which was confirmed by hormonal investigation. Abdominal magnetic resonance imaging showed unilateral enlargement of the right adrenal gland, whereas the other gland seemed normal - no cause was apparent. Three months later the patient presented with thrombosis in the right femoral vein and in the veins of the right leg. autoantibodies against cardiolipin were strongly positive, while antinuclear antibodies and antibodies against double-stranded deoxyribonucleic acid were absent. There was no evidence of antiphospholipid syndrome associated with drugs, connective tissue disorders, or malignancies, strongly suggesting the diagnosis of primary antiphospholipid syndrome. The development of adrenal insufficiency has been reported in primary antiphospholipid syndrome due to adrenal hemorrhage following vascular occlusion of adrenal vessels or secondary to anticoagulant therapy. It was interesting to note that in our patient adrenal gland insufficiency preceded other clinical evidence of the syndrome by 3 months. The primary antiphospholipid syndrome should be considered a possible cause of Addison's disease when the etiology is not obvious.- - - - - - - - - - ranking = 5.8826348402063keywords = gland (Clic here for more details about this article) |
7/19. Antiphospholipid antibodies and down syndrome: a case series.To describe the clinical profiles of five patients with down syndrome and elevated levels of antiphospholipid antibodies. medical records of all 149 patients screened for anticardiolipin antibodies (aCL) in the pediatric hematology or pediatric rheumatology clinics at new england Medical Center between 1996 and 1998 were retrospectively reviewed, and patients with down syndrome identified. Thirty-four patients (23%) had elevated IgG titers of aCL antibodies. Of these, five had down syndrome (15%). Two presented with discoloration of the distal digits, and one each with thrombocytopenia, autoimmune hepatitis, and undifferentiated autoimmune disease. The mothers of two of the four individuals with available family history had experienced frequent miscarriages. An association may exist between down syndrome and antiphospholipid antibodies, in particular an increased frequency of aCL antibodies. Screening patients with down syndrome and certain clinical findings may prove useful.- - - - - - - - - - ranking = 1.4706587100516keywords = gland (Clic here for more details about this article) |
8/19. Primary antiphospholipid syndrome associated with acute adrenal failure.We describe a 48-year-old woman with primary antiphospholipid syndrome who developed acute adrenal failure after an operation for a uterine myoma. After surgery, she developed a preshock state with hypotension, hypoglycemia, and hyponatremia. A diagnosis of primary antiphospholipid syndrome was made based not only on her past history of skin ulceration and recurrent spontaneous abortions but also on the presence of anticardiolipin antibodies. An abdominal computed tomography showed a bilateral enlargement of the adrenal glands but no high-density region in either gland. The patient recovered from the shock-like syndromes after the administration of glucocorticoids. Because it is possible that patients with antiphospholipid syndrome have acute or chronic adrenal failure caused by repeated hemorrhage or thrombosis, it may be important to monitor adrenal function in patients when the presence of this antibody is detected.- - - - - - - - - - ranking = 2.9413174201032keywords = gland (Clic here for more details about this article) |
9/19. antiphospholipid syndrome and multiple ischemic strokes in a patient with myasthenia gravis.Little is known about ischemic stroke occurrence in patients with myasthenia gravis (MG), although antiphospholipid antibodies are detectable in many MG patients. A 47-year-old woman with a 20-year history of generalized MG had an acute onset of right hemiparesis. She had undergone thymectomy 10 years previously and was treated for phlebothrombosis of the lower extremity 3 years previously. Computed tomography (CT) demonstrated an old infarct in the left frontal lobe and a new lesion in the right parietal lobe. Multiple small cortical and subcortical infarcts were demonstrated on fluid attenuated inversion recovery (FLAIR) images. thrombocytopenia (5.9 x 10(4)/microL), a prolonged activated partial thromboplastin time (aPTT; 50.2 sec), and an elevation of beta 2-IgG-glycoprotein I anticardiolipin antibodies (beta 2-GPIaCL; 55.7 U/mL) were observed. Neurological defects improved significantly over 2 weeks. She then was treated with oral prednisolone (30 mg/day) for 18 months, with resolution of laboratory abnormalities and no new cerebrovascular events or findings on imaging. We believe that our patient's multiple infarcts are caused by antiphospholipid antibodies and recommend glucocorticoid therapy to prevent recurrent of ischemic stroke in similar case.- - - - - - - - - - ranking = 2keywords = lobe (Clic here for more details about this article) |
10/19. mitral valve vegetation and cerebral emboli in a primary antiphospholipid syndrome patient who had hepatitis c virus infection: report of a case and review of the literature.We report the case of 36-year-old woman who came to us with a history of recurrent miscarriages and who was later diagnosed as having primary antiphospholipid syndrome (PAPS) and chronic hepatitis c virus (HCV) infection. The patient was referred to us with generalised seizures; cranial MRI revealed multiple embolic infarcts in both frontal lobes and a focal cortical infarct in the left frontoparietal lobe. Her echocardiography showed mitral valve vegetation and insufficiency. The patient was put on oral anticoagulant therapy and during her 8-month follow-up period no thrombotic events occurred. We report this case because it was the first in which PAPS, valvular disease, a cerebral embolic event and HCV infection were coexistent in the same patient. We also review other cases in which there was valvular vegetation and a cerebral ischaemic event associated with PAPS.- - - - - - - - - - ranking = 2keywords = lobe (Clic here for more details about this article) |
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