Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of an 18-year-old female, who developed an intraventricular hemorrhage and recurrent cerebral ischemic r attacks with Moyamoya like angiographical findings was reported. Lupus Anticoagulant (LA) was present in the patient's plasma. Cerebral angiography demonstrated stenosis and occlusion of the intracranial major arteries. Hemorrhagic episodes in patient's with LA is quite rare. The mechanism of intraventricular hemorrhage and the significance of LA in the differential diagnosis of Moyamoya disease are discussed.
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PMID:Intraventricular hemorrhage and cerebral ischemic attacks in the presence of lupus anticoagulant mimicking moyamoya disease. 812 82

Moyamoya disease is characterized by bilateral involvement of the internal carotid arteries. The etiology of this involvement is unknown. However, we previously reported two pediatric cases of moyamoya disease that progressed from unilateral to bilateral involvement. Some cases of unilateral occlusion in the carotid fork seem to have occurred at an early stage of definite moyamoya disease. In the present study, we examined five pediatric patients showing bilateral and/or unilateral occlusion of the internal carotid artery. In each case, the etiology was known. They included Apert syndrome, radiation therapy for pilocytic astrocytoma, systemic lupus erythematosis, von Recklinghausen disease and Down syndrome. The clinical manifestations, EEG, CT, MRI, PET, and angiographic findings in these patients were presented in comparison with those of definite moyamoya disease. This comparison led us to suggest that definite moyamoya disease might be included in these cases, and we emphasize the importance of precise examination in each case.
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PMID:[Akin moyamoya disease in children]. 842 89

The significance of antiphospholipid antibodies was examined in patients with childhood strokes. Eight patients, aged 2-13 years, who presented with acute hemiplegia were studied. On the basis of magnetic resonance imaging, magnetic resonance angiography, single photon emission computed tomography, and cerebral angiographic findings, 3 children were diagnosed as having infarctions due to moyamoya disease, and the others as having idiopathic infarctions. Serologic studies revealed elevated anticardiolipin antibody (ACA) IgG in 3 of the 5 patients with idiopathic infarction; no such elevation was revealed in patients with moyamoya disease. Values for all other tests, including ACA IgM and lupus anticoagulant, were within normal limits or negative in all patients. ACA IgG, therefore, may be a more important causative agent of childhood strokes than was previously considered.
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PMID:Antiphospholipid antibody syndrome in childhood strokes. 877 Nov 68

Sixteen patients with Moyamoya disease and four with quasi-Moyamoya disease were investigated in order to elucidate the presence of thrombophilia. The assay system for diagnosing thrombophilia consisted of assessing both the activity and antigen levels of antithrombin III, protein C, protein S, fibrinogen and plasminogen as well as detecting lupus anticoagulants. The analysis revealed that one third (four definite cases and three quasi-cases) of the examined patients demonstrated either congenital or acquired thrombotic tendency. Protein C deficiency was found in two definite cases and in two quasi-cases among whom one quasi-case was identified to have a hereditary type I Protein C deficiency. Protein S deficiency was found in one definite case and in one quasi-case. Type II plasminogen deficiency was found in one quasi-case, and lupus anticoagulant was present in one quasi-case. Based on these findings, an evaluation of thrombophilia should thus be performed when both diagnosing and treating suspected cases of Moyamoya disease.
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PMID:Thrombophilia found in patients with moyamoya disease. 940 44

We systematically screened for the aetiology of thrombophilia in 115 patients with venous, arterial and small vessel thromboses. Forty-one patients (36% of those we examined) suffering from a variety of thromboses, including deep vein thrombosis, pulmonary embolism, arterial occlusion, cerebral infarction, Moyamoya disease and ulcerative colitis, were characterized either with positive lupus anticoagulants or with decreased activities of protein S, protein C, antithrombin III and/or plasminogen. Eight mutation sites were confirmed in 11 thrombotic patients using gene analysis. Decreased protein S activity was found with a high incidence (23 out of 115) in Japanese patients who suffered from not only venous thrombosis but also arterial and small vessel thrombosis. We emphasize here the important role of protein S in the pathogenesis of thrombosis in the Japanese population.
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PMID:Screening for aetiology of thrombophilia: a high prevalence of protein S abnormality. 1045 3

Moyamoya disease is an uncommon clinical entity, characterized by bilateral occlusion of the internal carotid artery and the development of collateral arteries. An 18-year-old Saudi male with systemic lupus erythematosus (SLE) presented with mild right hemiparesis, followed by recurrent ischemic stroke. Cerebral angiography showed bilateral internal carotid artery stenosis associated with the development of collateral circulation (moyamoya vessels). There was no evidence of active SLE or other risk factors for cerebral occlusion, such as antiphospholipid antibody syndrome. Medical and surgical interventions did not influence the poor outcome of the recurrent ischemic insults.
Lupus 2000
PMID:Systemic lupus erythematosus associated with moyamoya syndrome. 1103 39

Neonatal lupus erythematosus, characterized mainly by congenital heart block and transient skin lesions, is usually self-limited. A patient with history of neonatal lupus erythematosus and congenital heart block developed central nervous system vasculopathy resembling moyamoya disease and hypertension at 17 years of age. Careful examination and follow-up of possible central nervous system complications later in life might be important in infants with neonatal lupus erythematosus.
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PMID:Central nervous system vasculopathy associated with neonatal lupus. 1181 40

Two patients with Down syndrome and the primary antiphospholipid antibody are described. One patient had a vasculopathy similar to that previously described as Moyamoya. Down syndrome is characterized by immune defects including a tendency to autoimmune phenomena. This report extends the scope of these observations and particularly draws out the potential role of antiphospholipid antibodies. Indeed antiphospholipid antibodies may well explain the well-known association of Down syndrome and stroke.
Lupus 2003
PMID:Antiphospholipid antibodies and stroke in Down syndrome. 1258 28

Aneurysms arising from a lenticulostriate artery (LSA) are uncommon. Their causes include hypertension, moyamoya disease, infection, systemic lupus erythematosis, and flow-related saccular aneurysms. Options for treating these aneurysms are limited. The authors present a case in which an LSA aneurysm was identified in a 69-year-old woman with no significant medical history, who experienced a sudden onset of right hemiparesis and aphasia due to a basal ganglia hemorrhage. The different causes and treatment options available for these rare and difficult-to-treat aneurysms are discussed.
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PMID:Surgical treatment of a lenticulostriate artery aneurysm. Case report. 1508 44

This reports describes a 12-year-old Japanese female with Noonan syndrome who had antiphospholipid syndrome and moyamoya-like vascular changes. She presented choreic movements in her face and extremities. She manifested phenotypic features of Noonan syndrome with short stature, mental retardation, and a webbed neck. Magnetic resonance angiography revealed occlusion of bilateral internal carotid arteries and moyamoya-like vascular changes around the basal ganglion region. Pimozide completely resolved the patient's choreic movements. Tests for anticardiolipin antibody and lupus anticoagulant were positive. The patient has manifested no symptoms for 2 years with pimozide, aspirin, and growth hormone treatment, without further aggravation of moyamoya-like vascular changes. This article is the first report of Noonan syndrome with antiphospholipid syndrome and moyamoya-like vascular lesions.
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PMID:Noonan syndrome, moyamoya-like vascular changes, and antiphospholipid syndrome. 1551 21


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