Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Magnetic resonance imaging (MRI) of the brain is a sensitive method to detect parenchymal tissue lesions. Its value in the diagnosis of central nervous system (CNS) lupus is disputed. To address this question, we have conducted an open and prospective study in a population of 44 SLE patients. We investigated 24 patients (mean age 33 +/- 13 yr) with past or active CNS lupus (group A) that included organic brain syndrome (12), migraine (8), focal neurological signs (7), seizures (2), myelopathy (1) and narcolepsy-cataplexy (1), and 20 patients (mean age 32 +/- 12 yr) without CNS lupus (group B). Health controls comprising nine females and one male aged 31 +/- 9 yr were also studied for comparison (group C). MRI was performed using sagittal T1-weighted images, axial and coronal spin density, and T2-weighted images. All scans were read blindly. Thirteen patients in group A and 10 in group B had well-identified lesions on sequences with long repetition time. Lesions were mostly multiple, small, punctate areas of increased signal at periventricular or subcortical white matter of both cerebral hemispheres. The number and location of lesions were not significantly different in both groups. None of the group C patients had MRI lesions. The presence of lesions was significantly associated with age at study and disease duration, but not with the presence of CNS lupus. In summary, MRI abnormalities are detected in neurologically asymptomatic SLE patients. Whether this represents subclinical brain involvement remains unknown.
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PMID:Magnetic resonance imaging of the brain in systemic lupus erythematosus. 854 7

Central nervous system involvement is a common but rarely reviewed feature of pediatric systemic lupus erythematosus (SLE). We retrospectively reviewed the charts of 91 patients with pediatric SLE and using a standardized data abstraction form documented 40 patients with central nervous system (CNS-SLE) involvement. The mean age of onset of SLE was 13.3 years. In 19 patients the CNS manifestation was a presenting symptom, in 12 patients CNS involvement was present within the first year of diagnosis, and in 9 patients it took up to 7 years for CNS disease to become evident. Nineteen children (48%) manifested neuropsychiatric SLE, which included depression, concentration or memory problems, and frank psychosis. Seizures were present in 8 patients (20%), 6 had cerebral ischemic events (15%), 1 had chorea (3%), 2 had papilledema (5%), and 2 patients had a peripheral neuropathy (5%). Nine patients (22%) had severe headache consistent with lupus headache. Seven children had more than one CNS manifestation. In the investigation of CNS-SLE, computed tomography and/or magnetic resonance imaging scans were helpful in patients with focal ischemic lesions and venous sinus thrombosis. Electroencephalography was abnormal only in 33% of patients with seizure disorders and rarely helpful in patients with diffuse neuropsychiatric symptoms. Single-photon emission computed tomography scans were abnormal in most patients with neuropsychiatric SLE, especially in those with frank psychosis. The lupus anticoagulant was present in the patient with chorea and was frequently present in patients with cerebral vascular events. Long-term outcome was good: only 1 child died of cerebral hemorrhagic infarction and 3 others had significant persistent CNS deficits. The majority of patients (90%) had excellent recovery from CNS-SLE.
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PMID:Neurologic manifestations of pediatric systemic lupus erythematosus. 855 55

Records of 108 patients with lupus erythematosus beginning in childhood (1953-1990) were reviewed; 25 had recorded neurologic findings. This is the largest group of childhood lupus erythematosus patients with neurologic disease that has been reported. The average age of children at the time of diagnosis of lupus was 154 months. There were 22 girls and 3 boys in the group. All patients met at least four of the 1982 American Rheumatism Association criteria for the classification of systemic lupus erythematosus. Average age at onset of neurologic difficulties was 168 months. In 4 patients, the neurologic symptoms preceded the diagnosis: 1 month (spastic diplegia), 1 month (bilateral weakness and spasticity), 24 months (chorea), and 26 months (chorea), respectively. Four patients had neurologic symptoms coincident with the diagnosis of lupus erythematosus. In those patients whose symptoms followed the diagnosis of lupus erythematosus, the average elapsed time until symptoms appeared was 33 months; the single lowest and highest outliers were discounted. Most frequent findings were headache (16/25) and behavioral aberrations (10/25). All behavioral manifestations were depression except in 1 patient. Other prevalent findings included hemichorea or chorea (7/25), cerebrovascular accident with hemiplegia or diplegia (7/25), seizures (5/25), visual loss (3/25), and cranial neuropathy (2/25). Vertigo and myelopathy occurred in 1 patient each. All patients were treated primarily with corticosteroids and azathioprine; in the presence of active disease, the drug dosages were increased with significant improvement in neurologic symptoms. Resolution usually occurred from days to months; most improved in a few days to a few weeks; 3-4 months was the longest period until symptoms subsided.
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PMID:Neurologic characteristics of childhood lupus erythematosus. 855 56

A 50-year-old Japanese female with a long history of Raynaud's phenomenon presented with progressive dyspnea due to pulmonary hypertension. The diagnosis of systemic lupus erythematosus was confirmed by proteinuria, lymphocytopenia, bilateral pleurisy, and a seizure of convulsion which was consistent with neurological manifestations of systemic lupus erythematosus, whereas the antinuclear antibody showed a low titer. Despite improvement in the activity of systemic lupus erythematosus, steroid treatment did not alter the progression of pulmonary hypertension, which increased in severity, eventually resulting in her death. We believe pulmonary hypertension to be an unusual but critical complication of systemic lupus erythematosus.
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PMID:Systemic lupus erythematosus with pulmonary hypertension. 865 23

The contraceptive implant depot medroxyprogesterone acetate (DMPA) may offer advantages to women with medical problems which contraindicate the use of estrogen. In such women, the risks of pregnancy must be weighted against the risk posed by a contraceptive method. While young women with well-controlled hypertension can use DMPA or the combined oral contraceptive, patients with uncontrolled hypertension or other risk factors may be better managed with DMPA. DMPA is also an appropriate choice for many women with cardiac disorders which can be associated with an extremely high risk of adverse pregnancy outcomes. Because it is not associated with increased thrombotic risk, DMPA is also safe in women over 35 years old who smoke. The contraceptive is likewise indicated in women with a history of thromboembolic disease (despite package labeling which was based on trials of high doses of DMPA as a cancer treatment). DMPA also is safe in women with sickle cell disease and actually has been shown to reduce the incidence of sickle cell crisis. Evidence also suggests that DMPA injections in anticoagulated women do not increase the incidence of hematoma formation. DMPA will not protect women with underlying predisposing causes of thrombosis from experiencing a thrombotic event. Whereas the contraceptive efficacy of hormonal contraception may be reduced in epileptic women using hepatic enzyme induction agents, DMPA has been reported to reduce seizure frequency with few contraceptive failures in such women. In diabetic women with peripheral vascular disease and in women with systemic lupus erythematosus, DMPA, unlike contraceptives with estrogen, avoids the enhanced risk of thrombosis. Because the best pregnancy outcome in women with medical problems occurs when the pregnancy is planned, such women should use the most effective contraceptive methods available to them.
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PMID:Depot medroxyprogesterone acetate contraception in women with medical problems. 872 4

An 18-year-old female with 7 years' history of epilepsy was admitted for developing malar rash. She had been treated with hydantoin for 7 years. Laboratory examinations revealed leukopenia and high titer of anti-dsDNA antibodies. Renal biopsy also showed diffuse segmental mesangial proliferative glomerulonephritis. A diagnosis of systemic lupus erythematosus (SLE) was made, and she received 40 mg of prednisolone daily. At follow up 4 months later since her first visit, she developed choreiform movements involving the right upper and lower limbs, despite no signs of increase in her disease activity. Neither biological false positive testing for syphilis nor the lupus anticoagulant (LAC) was detected. MRI demonstrated no signal abnormalities in the brain. Administration of haloperidol was started and the choreiform movements were decreased. Anticonvulsants are associated with drug-induced lupus. On the other hand, seizure is known to be one of the first manifestations of SLE. In drug-induced lupus, positive testing for anti-dsDNA, anti-Sm antibodies, hypocomplementemia and renal involvement are not a frequent as in SLE. In this case, laboratory findings showed high titer of anti-dsDNA antibodies, positive testing for antihistone, anti-SSA, anti-Ki antibodies, and hypocomplementemia. And mesangial proliferative glomerulonephritis was detected. So we diagnosed her as SLE and suggested that epileptic seizure developed 7 years ago had been the first manifestations of SLE. Neurologic complications of SLE are common, but chorea has been rarely reported. Since it is known that LAC is associated with thrombosis, it has been suggested that small infarctions in the basal ganglia may play a part in the pathogenesis of chorea in SLE. In this case, the LAC was negative and MRI showed no detectable abnormalities. As a result another mechanism may be attributed to chorea in this case.
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PMID:[A case of systemic lupus erythematosus diagnosed 7 years after epileptic seizure and developed chorea during prednisolone treatment]. 877 91

To examine the association between anticardiolipin (aCL) antibodies and epilepsy, we investigated the serum titers of aCL antibodies in a total 252 systemic lupus erythematosus (SLE) patients recruited in a prospective study. Twenty-one cases with epilepsy which were not attributable to any causes other than SLE were identified after being followed-up for five years. The clinical manifestations were recorded and blood samples were tested for the presence of aCL antibodies (IgG, IgM and IgA isotypes). Among 21 patients with epilepsy, 12 (57.1%), 2 (9.5%) and 2 (9.5%), respectively, had elevated baseline serum levels of IgG, IgM and IgA aCL antibodies. There was a dose-response relationship between risk of seizure and the baseline serum level of aCL antibodies (P < 0.01). The odds ratio of developing seizure were 3.7 for those who had a high level of aCL antibodies compared with those without a detectable level of aCL antibodies as the referent. Our results indicate that epilepsy as a primary neuropsychiatric event among lupus patients is associated with a high titer of aCL antibodies.
Lupus 1996 Aug
PMID:Elevated levels of anticardiolipin antibodies and epilepsy in lupus patients. 922 72

Antiphospholipid antibodies (aPL) have been associated with a variety of neurological disorders, mostly linked to focal neuroparenchymal ischemia or infarction. Cerebral ischemia associated with the antiphospholipid syndrome (APS) occurs at a younger age than typical atherothrombotic cerebrovascular disease, is often recurrent, and high positive GPL values are usually linked to the presence of a lupus anticoagulant. When other features of the syndrome are not present and cerebral ischemia occurs only associated with anticardiolipin immunoreactivity, there appears to be no discerning features of these patients unless GPL > 40 for which recurrent thrombo-occlusive events appear to occur more frequently. Other neurological manifestations associated with aPL include cerebral venous sinus thrombosis, ocular ischemia, dementia, including ischemic encephalopathy, and chorea. The role of aPL in migrainous events is controversial and may not play a role in recent, large case-controlled studies. Most seizures in patients harboring aPL are associated with focal brain infarction.
Lupus 1996 Oct
PMID:Neurological aspects of antiphospholipid antibody syndrome. 890 59

Antiphospholipid' (aPL) antibodies are of important clinical significance because of their association with thrombosis both arterial and venous, recurrent foetal loss, specific neurological sequelae like seizures and chorea, cardiac valvular abnormalities and thrombocytopenia. Traditionally these autoantibodies have been assayed using phospholipid (PL) dependent tests and are classified as lupus anticoagulants (LA) and anticardiolipin (aCL) antibodies based on the method of detection. The antibodies thus, had been thought to bind PLs but it has now become clear that the true antigens are PL-binding proteins. The major protein consistently found as the target antigen for these autoantibodies is beta 2-glycoprotein I (beta 2-GPI). Other candidate PL-binding proteins have also been investigated including prothrombin, protein C and protein S but thus far appear to play less important roles in the binding of these antibodies.
Lupus 1996 Oct
PMID:beta 2-Glycoprotein I: target antigen for autoantibodies in the 'antiphospholipid syndrome'. 890 65

Neuropsychiatric symptoms are recognized to occur in a significant percentage of systemic lupus erythematosus patients and to be a leading cause of morbidity and morality in lupus. Recent findings regarding the clinical presentation, diagnosis, pathogenesis, and treatment of neuropsychiatric lupus erythematosus are reviewed. The study of neurocognitive deficits and psychosocial functioning in systemic lupus erythematosus patients continues to be an area of great research interest worldwide. Severe neuropsychiatric manifestations can be divided into diffuse, focal, and seizure presentations, which can each have a different etiopathogenesis. New techniques for magnetic resonance imaging and single-photon emission CT of the brain may improve the utility and sensitivity of these neuroradiographic tests. Certain combinations of serologic, cerebrospinal fluid, and neuroimaging tests appear to be most useful diagnostically when ordered based on the patient's neurologic presentation. The role of complement, cytokines, and endothelial cell activation in causing the vascular pathology observed in the brains of neuropsychiatric lupus erythematosus patients is an area of promising research. Treatment remains empiric, but intravenous pulse cyclophosphamide and intrathecal administration of immunosuppressive medications are new approaches that have been used successfully to treat patients with severe and refractory symptoms.
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PMID:Lupus and the central nervous system. 894 43


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