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)

Manifestations of dementia occurred in two young patients with disseminated lupus erythematosus who had no signs of focalized neurological deficit. In case 1 followed for 8 years, a probably autonomous and familial psychiatric syndrome was complicated during a lupus flare-up by a prolonged dementia syndrome which regressed to a large extent. In the second patient with moderate dementia and familial hearing loss, severe mental deterioration suddenly occurred with long-term degradation of the cognitive capacity. In both of these cases with neurolupus, the frontal clinical signs, the neuropsychological deficits evidenced at testing, and the lesions demonstrated at CT scan and magnetic resonance imaging favored a frontal and/or frontobasal type dementia. Treatment with cyclophosphamide was effective in case 1 after failure of corticosteroid therapy.
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PMID:[Neurolupus with dementia manifestations. 2 cases]. 968 6

In 27 of 47 patients with Sneddon's syndrome (33 females, 14 males age 40 years) enzyme immunoassay has detected IgG-antibodies to prothrombin (aPT)--one of cofactor proteins responsible for binding of antiphospholipid antibodies (aPL) to phospholipids. Other aPL were also found: antibodies to cardiolipin (aCL), lupus anticoagulant (LA) in 14 and 27 patients, respectively. 37 (79%) patients had at least one of the studied aPL suggesting that such patients belong to patients with primary antiphospholipid syndrome. A correlation exists between aPT and LA: LA is detectable in 67% of aPT-positive patients compared to 45% of aPT-negative patients (p < 0.05). This is in agreement with the fact that prothrombin is a cofactor for most aPL registered as LA. Comparison of two subgroups of aPL patients different by dominant antigenic specificity (18 patients with aPT but free of ACL and 6 patients with aCP but free of aPT) demonstrated that the latter developed disorders of cerebral circulation, head ache, dementia and renal syndrome less frequently. aPT in Sneddon's syndrome seems to be a marker of comparatively low risk of thrombosis and less severe course of the disease.
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PMID:[Sneddon syndrome new clinical and immunological data]. 969 98

Neuropsychiatric symptoms in systemic lupus erythematosus (SLE) are varied. The exact etiology and appropriate treatment are difficult to determine. We describe a 43-year-old woman who was diagnosed 18 years earlier with SLE. She developed dementia, gait disturbance, urinary incontinence and deterioration of consciousness. Her cerebrospinal fluid pressure was not elevated. Brain computerized tomography scan revealed enlarged ventricle and cortical sulci. Normal pressure hydrocephalus (NPH) was diagnosed. She was treated by establishing a ventriculo-peritoneal shunt, resulting in the return of normal consciousness. But she still had slurred speech, slow mentation and poor calculation ability. Her urinary incontinence persisted. Along with a literature review on NPH, we discuss its etiology, diagnosis and treatment.
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PMID:Normal pressure hydrocephalus in a patient with systemic lupus erythematosus: a case report. 979 5

A 37-year-old man sought medical advice because of an 8-year history of a slowly progressive dementing illness with no clinically apparent discrete strokelike episodes. Cognitive functioning was markedly, globally impaired without lateralizing or localizing features. Widespread livedo reticularis led to a diagnosis of Sneddon's syndrome. Antiphospholipid antibodies and lupus anticoagulant were negative. Magnetic resonance imaging showed widespread cerebral atrophy, cortical and subcortical cerebral infarcts, and extensive periventricular white matter abnormalities. Cerebral angiography revealed diffuse medium- and small-vessel occlusive disease, with numerous collaterals in the mid and distal circulation but no evidence of atherosclerosis or vasculitis. No other cause of a dementing illness was found. We postulate that our patient's dementia was due to the cumulative effects of multiple cerebral infarcts.
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PMID:Gradually progressive dementia without discrete cerebrovascular events in a patient with Sneddon's syndrome. 1059 61

Among various rheumatic diseases, systemic lupus erythematosus and Behcet's disease are frequently complicated with serious neurological involvement, called CNS lupus and neuro-Behcet's syndrome (NB), respectively. CNS lupus includes lupus psychosis, such as organic brain syndrome and non-organic psychosis, as well as non-psychotic CNS lupus, such as epilepsy and focal lesions. Anti-ribosomal P antibody is closely associated with lupus psychosis, whereas anti-phospholipid antibodies appear to be involved in some manifestations of non-psychotic CNS lupus. NB includes acute type and chronic progressive type. Acute NB is characterized by acute meningoencephalitis with focal lesions, which respond to steroid therapy. By contrast, chronic progressive NB is characterized by intractable slowly progressive dementia and/or psychosis with persistent elevation of cerebrospinal fluid IL-6 activity, which is resistant to conventional steroid therapy. However, recent studies suggest the efficacy of low dose methotrexate in chronic progressive NB.
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PMID:[Central nervous system involvement in rheumatic diseases]. 1007 15

Antiphospholipid antibodies (aPL) are associated with neurological diseases such as stroke, migraine, epilepsy and dementia and are thus associated with both vascular and non-vascular neurological disease. We have therefore examined the possibility that these antibodies interact directly with neuronal tissue by studying the electrophysiological effects of aPL on a brain synaptosoneurosome preparation. IgG from patients with high levels of aPL and neurological involvement was purified by protein-G affinity chromatography as was control IgG pooled from ten sera with low levels of aPL. Synaptoneurosomes were purified from perfused rat brain stem. IgG from the patient with the highest level of aPL at a concentration equivalent to 1:5 serum dilution caused significant depolarization of the synaptoneurosomes as determined by accumulation of the lipophylic cation [3H]-tetraphenylphosphonium. IgG from this patient as well as IgG from two elderly patients with high levels of aPL were subsequently shown to permeabilize the synaptosomes to labeled nicotinamide adenine dinucleotide (NAD) and pertussis toxin-ADP-ribose transferase (PTX-A protein) as assayed by labeled ADP-ribosylation of G-proteins in the membranes. No such effects were seen with the control IgG. aPL may thus have the potential to disrupt neuronal function by direct action on nerve terminals. These results may explain some of the non-thromboembolic CNS manifestations of the antiphospholipid syndrome.
Lupus 1999
PMID:Antiphospholipid antibodies permeabilize and depolarize brain synaptoneurosomes. 1019 7

The prevalence of Borna disease virus (BDV)-specific antibodies among patients with psychiatric disorders and healthy individuals has varied in several reports using several different serological assay methods. A reliable and specific method for anti-BDV antibodies needs to be developed to clarify the pathological significance of BDV infections in humans. We developed a new electrochemiluminescence immunoassay (ECLIA) for the antibody to BDV that uses two recombinant proteins of BDV, p40 and p24 (full length). Using this ECLIA, we examined 3,476 serum samples from humans with various diseases and 917 sera from blood donors in Japan for the presence of anti-BDV antibodies. By ECLIA, 26 (3.08%) of 845 schizophrenia patients and 9 (3.59%) of 251 patients with mood disorders were seropositive for BDV. Among 323 patients with other psychiatric diseases, 114 with neurological diseases, 75 with chronic fatigue syndrome, 85 human immunodeficiency virus-infected patients, 50 with autoimmune diseases including rheumatoid arthritis and systemic lupus erythematosis and 17 with leprosy, there was no positive case except one case each with alcohol addiction, AIDS, and dementia. Although 19 (1.36%) of 1,393 patients with various ocular diseases, 10 (1.09%) of 917 blood donors, and 3 (4.55%) of 66 multitransfused patients were seropositive for BDV-specific antigen, high levels of seroprevalence in schizophrenia patients and young patients (16 to 59 years old) with mood disorders were statistically significant. The immunoreactivity of seropositive sera could be verified for specificity by blocking with soluble p40 and/or p24 recombinant protein. Anti-p24 antibody was more frequent than p40 antibody in most cases, and in some psychotic patients antibody profiles showed only p40 antibody. Although serum positive for both p40 and p24 antibodies was not found in this study, the p40 ECLIA count in schizophrenia patients was higher than that of blood donors. Furthermore, we examined 90 sera from Japanese feral horses. Antibody profiles of control human samples are similar to that of naturally BDV-infected feral horses. We concluded that BDV infection was associated in some way with psychiatric disorders.
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PMID:Detection of borna disease virus-reactive antibodies from patients with psychiatric disorders and from horses by electrochemiluminescence immunoassay. 1047 20

The primary antiphospholipid antibody syndrome (PAPS) has been described in patients with a history of fetal loss, thrombocytopenia and arterial or venous thrombosis. In PAPS, a prothrombotic state is mediated by antiphospholipid antibodies (aPLs) leading to disseminated thromboembolic vascular occlusion. Today, the presence of aPLs in the serum is considered as a distinct risk factor for recurrent stroke in young adults. Some PAPS patients develop a multi-infarct-syndrome with a stepwise decline of higher cortical functions. We report on a 55-year-old man suffering from progressive dementia and PAPS, in whom cerebral glucose metabolism and blood flow were examined by positron emission tomography (PET). Cerebral atrophy and moderate signs of leukaraiosis were detected in magnetic resonance imaging (MRI), whereas the PET scans showed a considerable diffuse impairment of cortical glucose metabolism combined with a reduced cerebral perfusion in the arterial border zones. These findings indicate that PAPS-associated vascular dementia is accompanied by a cortical neuronal loss, presumably caused by a small-vessel disease with immune-mediated intravascular thrombosis. This case shows that pathological findings in PAPS are congruent to cerebral changes of metabolism and blood flow in systemic lupus erythematosus (SLE).
Lupus 2000
PMID:Cerebral blood flow and glucose metabolism in multi-infarct-dementia related to primary antiphospholipid antibody syndrome. 1134 Nov 10

Home parenteral nutrition (HPN) is usually conducted after hospital training and home trials, but in more than a few cases self-care is virtually impossible or the ability of carers is insufficient. We investigated the problem points in the cases of two HPN patients from our hospital. Patient 1 was a 76-year-old man who had undergone surgery for esophageal cancer. He was rehospitalized with passage disorders due to eating difficulties such as dysmasesis and dysphagia and an insufficient ability to comprehend meals. Self-care was virtually impossible owing to his lack of understanding of the disease and his dementia. Even if subcutaneous leakage of the subcutaneously implanted port occurred or the connecting portion became dislocated, the patient would not be able to alert others to this by himself. His wife, the key person in his care, could not undergo hospital training because of her advanced age. She received instruction on the techniques for the completion of IVH for one month from the visiting carer, but handling the syringe and needle and the clamp maneuver were difficult for her, and she later developed an infection and was hospitalized. Upon consultation with the primary physician, a change was made to a Groshong catheter, which reduced the maneuver burden, but the prepared checklist was not used and there were problems in the handling of the catheter and management during the period when the maneuvers were being carried out. In addition, discord arose in the family relations, so a grandchild who was a university student rather than the daughter-in-law received instruction according to the manual in order to care for the patient on the nurse's days off. Currently, HPN is being carried out 3/week with meal instructions adjusted to the patient's dysphagia and contact with the family on the nurse's days off. An issue remaining for the future is the use of informal resources in terms of both micro-intervention, including selection of a catheter with consideration of care ability and meal instruction matched to his eating function, and macro-intervention with consideration of the family environment and interpersonal relationships. Patient 2 was a 41-year-old woman with SLE. She was a former nurse, but self-care was not possible due to steroid myopathy. Her main carer was her mother, but due to Alzheimer's-type dementia her mother had difficulty with sterile maneuvers, and sometime allowed the syringe, needle, and set to get dirty or refused to administer the medication or change the batteries on the pump. A visiting nurse and helper visit twice/week each and another volunteer provides daily support, but to continue home care in the future it will be essential to further train the helper and deepen the cooperation between all related.
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PMID:[Two cases of home parenteral nutrition in which home care was difficult]. 1119 Mar 16

Dehydro-3-epiandrosterone is a steroid hormone synthesized in large quantities by the adrenal gland whose physiologic role remains unclear. The effects of DHEA could be estrogenic or androgenic, depending on the hormonal milieu. Low levels of DHEA are associated with aging, cardiovascular disease in men, and an increased risk of pre-menopausal breast and ovarian cancer. High levels of DHEA might increase the risk of postmenopausal breast cancer. Therapeutically DHEA might be useful for improving psychological well-being in the elderly, reducing disease activity in people with mild to moderate systemic lupus erythematosus and myotonic dystrophy, improving mood in those clinically depressed, and improving various parameters in women with adrenal insufficiency. Although many other claims have been made for DHEA in diverse conditions, such as aging, dementia, and AIDS, no well-designed clinical trials have clearly substantiated the utility and safety of long-term DHEA supplementation.
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PMID:Uses of DHEA in aging and other disease states. 1203 47


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