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)

We present a case of a 20-year-old woman admitted with new onset severe systemic lupus erythematosus (SLE) with multiple manifestations, including lupus nephritis with renal failure and significant central nervous system (CNS) lupus. She presented with a forbidding condition and deteriorated despite a regimen of high dose corticosteroids and cyclophosphamide administration. She had progressive CNS disease initially, with multiple generalized tonic-clonic seizures and later with dense left sided hemiparesis. After treatment with high dose intravenous immunoglobulin (IVIG), there was remarkable improvement in overall lupus disease activity and resolution of neurologic deficits. A head magnetic resonance imaging (MRI) scan early in her hospital course revealed multiple areas of vasogenic and cortical edema consistent with ischemic changes, possibly from lupus vasculitis. A repeat MRI after IVIG treatment revealed dramatic interval improvement and resolution of most of the multifocal areas of signal abnormality.The use of IVIG has been reported in a variety of autoimmune disorders, and there have also been several case reports of the successful use of IVIG in the treatment of SLE. IVIG seems to be a relatively safe treatment alternative and may have a promising role in the future of lupus treatment. We have found only five case reports in the literature specifically involving the use of IVIG in CNS lupus, but they show substantial benefits. Our case is unique because the demonstration of dramatic response to IVIG by a head MRI has not previously been reported. Based on the available information and the observations in this case, IVIG remains an experimental but promising treatment option for patients with SLE.
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PMID:Treatment of severe CNS lupus with intravenous immunoglobulin. 1907 92

Systemic lupus erythematosus (SLE) is a multisystem, autoimmune connective tissue disorder. Neuropsychiatric SLE (NPSLE) has varied clinical and radiological manifestations. Clinical manifestations range from subtle abnormalities of neurocognitive functions and mood changes to overt psychiatric or neurological manifestations such as seizures, stroke, and psychosis. Magnetic resonance imaging (MRI) may show various types of abnormalities. Cerebral white matter lesions are most common (60-86%). Here, we are discussing a young female who presented with alteration of sensorium and right-sided hemiparesis, and MRI of the brain showed extensive involvement of brainstem in the form of possible demyelination.
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PMID:A case of systemic lupus erythematosus with extensive brain stem involvement. 1934 71

We report the case of a 25-year-old woman with systemic lupus erythematosus. She complained of headache and left hemiparesis and was admitted to our hospital. She was in a coma after admission. Brain MRI showed no abnormalities, but brain single photon emission computed tomography showed diffuse hyperperfusion in the right frontal and temporal lobes. The calculated measured regional cerebral blood flow in the right hemisphere was increased to 40 to 55 mL/100 g/min, and that in the left was within the normal range of 25 to 35 mL/100 g/min. Two months later, the hyperperfusion was disappeared. The hyperperfusion could be explained by inflammatory changes.
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PMID:Transient brain hyperperfusion detected with I-123 IMP SPECT in a patient with neuropsychiatric systemic lupus erythematosus. 1969 23

Patients with advanced HIV disease with low CD4 count are more prone to thrombo-embolism and various predisposing factors have been identified. These include the presence of anticardiolipin antibodies and the lupus anticoagulant, deficiencies of proteins C and S, heparin co-factor II and antithrombin. Increased levels of Von Willebrand factor and d-dimers have also been linked with thrombo-embolism, as has the presence of concurrent infections and malignancies. We report a case of an AIDS patient who presented with acute hemiparesis. He was severely immunosuppressed. Computed tomography of the head confirmed cerebral infarction with haemorrhagic transformation. He had no known risk factors apart from being severely immunocompromised and had high anticardiolipin antibodies and low free protein S.
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PMID:Haemorrhagic transformation of cerebral infarction in an AIDS patient--thrombophilia screen essential! 1983 95

Primary antiphospholipid syndrome (APS) is a well-defined entity characterized by spontaneous and recurrent abortion, thrombocytopenia and recurrent vascular thromboses (arterial and venous). Left ventricular thrombus mimicking primary cardiac tumor with recurrent systemic embolism has not been previously reported. In this report we describe a 39 year-old man admitted to hospital presenting with left hemiparesis and a peripheral embolism. He had no history of thrombotic events. Transthoracic echocardiography showed a large, polypoid and mobile mass (4.0 x 1.2 cm) attached to the apex of the left ventricle, highly suggestive of primary cardiac tumor. The patient subsequently underwent open heart surgery. The histological examination showed an older thrombus and a fresh thrombus. Post-operative laboratory tests showed lupus anticoagulant activity, confirming the primary APS diagnosis. The patient initiated treatment with oral anticoagulation (INR levels between 2 and 3) and was discharged 29 days after surgery. At ten month follow-up, he was symptom-free with long-term anticoagulation therapy. No evidence of intracardiac mass recurrence on two-dimensional echocardiography was seen. Intracardiac thrombus has been rarely reported as a complication of primary APS. Left ventricular mass mimicking primary cardiac tumor with recurrent systemic embolism has not been previously reported. Pre-operative investigations could not distinguish such a thrombus from a cardiac tumor and the diagnosis was made post-operatively.
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PMID:Left ventricular thrombus mimicking primary cardiac tumor in a patient with primary antiphospholipid syndrome and recurrent systemic embolism. 1995 93

Primitive neuroectodermal tumor is a rare brain tumor composed of undifferentiated or poorly differentiated neuroepithelial cells with a high malignant potential that usually occurs in children, and which is only occasionally encountered in adults. A 19-year-old female with systemic lupus erythematosus presented with right hemiparesis and a headache of 10 days duration. Brain magnetic resonance imaging showed a large solid mass with necrotic portions in the left frontoparietal lobe. Primitive neuroectodermal tumor was confirmed by a neuronavigator-guided brain biopsy. This is the first case report of primitive neuroectodermal tumor associated with systemic lupus erythematosus and moyamoya disease. This case demonstrates that brain tumors, such as primitive neuroectodermal tumor, should be included in the differential diagnosis of neurological manifestations in children and adolescent patients with systemic lupus erythematosus.
Lupus 2010 Jul
PMID:De novo appearance of primitive neuroectodermal tumor in a patient with systemic lupus erythematosus and moyamoya disease. 2041 Jan 55

We report a 50-year-old woman with systemic lupus erythematosus (SLE) who developed stroke-like symptoms, including disturbance of consciousness and severe left hemiparesis. Despite the stroke-like symptoms, MR imaging showed quite atypical findings for stroke; broad high-intensity areas in the right fronto-parietal lobes on diffusion-weighted images were shown mainly as iso-intensity areas on the ADC map, without any findings of stenosis of the cerebral large arteries. The cerebral blood flow in these areas was significantly decreased, as evaluated by single photon emission computed tomography. The present case suggests that small vessel vasculopathies localized in the unilateral hemisphere could cause "stroke-like" symptoms in SLE.
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PMID:A case of SLE presenting stroke-like symptoms. 2132 72

This study aimed to investigate the maternal, pre- and perinatal, and prothrombotic factors with congenital hemiparesis due to presumed perinatal stroke (PPS). Prothrombotic risk factors including protein C and S, antithrombin III, lipoprotein (a), homocystein, factor VIII levels; anticardiolipin antibodies and lupus anticoagulant; methylenetetrahydrofolate reductase mutations, factor V Leiden, prothrombin G20210A mutations were investigated. Arterial ischemic stroke was detected in 60% and periventricular venous infarction in 40%. At least one prothrombotic risk factor was present in 69%, two in 17%, and three or more in 8.5% of cases. The most common combination was methylenetetrahydrofolate reductase C677T and factor V Leiden heterozygosity. The etiology and pathogenesis of PPS is still unclear. According to this study, most of the patients with PPS might have one or more prothrombotic risk factors and certain prenatal risk factors including intrauterine growth retardation, twin gestation and preeclampsia might be related to PPS.
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PMID:Etiological analysis of presumed perinatal stroke. 2156 29

Systemic lupus erythematosus (SLE) is a chronic inflammatory multi-system disease characterised by varied clinical manifestation and immunological abnormalities. The clinical presentation of the disorder has wide spectra, from an asymptomatic presentation to a severe life-threatening disease affecting several organs. The sole manifestation of lupus erythematosus could be neurological syndrome, where diagnosis of SLE is difficult to establish. The authors intended to report a young female, who initially developed left-sided hemiparesis due to tumefactive demyelination, later on diagnosed as case of SLE. The association of tumefactive demyelination and SLE has not been previously reported in the literature.
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PMID:Tumefactive demyelination associated with systemic lupus erythematosus. 2260 71

A 42-year-old woman, a diagnosed case of systemic lupus erythematosus (SLE), developed severe headache followed by left hemiparesis and cortical blindness. Magnetic resonance imaging (MRI) of brain demonstrated right parieto-occipital infarct and the patient was initiated on high-dose steroids and antiplatelet agents with which the patient had clinical and radiological deterioration. Magnetic Resonance angiography showed severe narrowing of bilateral anterior, middle, and posterior cerebral arteries (PCA) suggestive of reversible cerebral vasoconstriction syndrome (RCVS). Patient was treated with IV nimodipine and she recovered over a period of 2 weeks. Repeat MR-angiography done on day-30 was normal. The diagnosis of RCVS in patients with SLE is of practical importance, because treatment and prognosis for SLE-associated cerebral vasculitis differ strongly from the treatment of RCVS. Empirical high-dose glucocorticoid therapy should be avoided in patients with typical features of RCVS.
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PMID:Reversible cerebral vasoconstriction syndrome in a patient with systemic lupus erythematosus. 2328 28


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