Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0008489 (chorea)
 2,102 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 22-year-old woman gradually developed depression, dementia, and chorea over an 8-month period. She fulfilled the criteria for the primary antiphospholipid antibody syndrome but not those for systemic lupus erythematosus. Her chorea and neurobehavioral deficits responded favorably to a regimen of prednisone, hydroxychloroquine, and aspirin. This appears to be the first report of a patient with a lupus anticoagulant and reversible dementia. The response to immunosuppressive therapy implies an antibody-mediated condition similar to Sydenham's chorea.
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PMID:Reversible dementia and chorea in a young woman with the lupus anticoagulant. 864 56

We describe a case of antiphospholipid associated thrombocytopenia in which warfarin, but not heparin, normalized the platelet count. Systemic lupus erythematosus was present in association with chorea, mitral valve stenosis, and renal thrombotic microangiopathy. No evidence of platelet consumption was detected. Low dose aspirin, pulse methylprednisolone, intravenous gammaglobulin, and cyclophosphamide also partially elevated the platelet count. Platelet activation, measured by P selectin assay, was not present during warfarin or aspirin therapy, suggesting that platelet activation may have been decreased by these treatments. We discuss possible mechanisms for correction of thrombocytopenia in antiphospholipid syndrome by warfarin.
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PMID:Thrombocytopenia corrected by warfarin in antiphospholipid syndrome. 873 Jan 44

A 12-year-old girl with chorea, thrombocytopenia and false positive VDRL had negative serological tests for SLE, but high titre of antiocardiolipine antibodies. Primary antiphospholipid antibody syndrome is rarely complicated by chorea, but should be taken into consideration in patients who do not fulfill the diagnostic criteria for SLE.
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PMID:Chorea in primary antiphospholipid syndrome. 888 98

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.
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PMID:Neurological aspects of antiphospholipid antibody syndrome. 890 59

We analyzed the clinical, radiologic, and immunologic characteristics of 50 patients with chorea and the antiphospholipid syndrome (APS) (6 from our clinics and 44 from a MEDLINE computer-assisted review of the literature from 1985 through 1995). Forty-eight (96%) patients were female and 2 (4%) were male. Twenty-nine (58%) patients had defined systemic lupus erythematosus (SLE), 6 (12%) had "lupus-like" syndrome, and 15 (30%) patients had "primary" APS. Mean age of patients in this series was 23 +/- 12 years (range, 6-77 yr); mean age at presentation of chorea was 21 +/- 12 years (range, 6-77 yr). In 11 (22%) patients, the onset of chorea was in childhood (6-14 yr), and in 2 (4%) patients it presented at 60 years or more. Six (12%) patients developed chorea soon after they started taking estrogen-containing oral contraceptives, 3 (6%) developed chorea gravidarum, and 1 (2%) patient developed chorea shortly after delivery. Most patients (66%) presented only 1 episode of chorea. Chorea was bilateral in 55% of patients. Computed tomography and magnetic resonance imaging scans reported cerebral infarcts in 35% of patients. The following antibodies were detected: lupus anticoagulant (92%), anticardiolipin antibodies (91%), antinuclear antibodies (82%), anti-DNA (59%), anti-Ro (10%), anti-RNP (8%), anti-La (2%), and anti-Sm (2%). The chorea in these patients responded to a variety of medications, for example, steroids, haloperidol, antiaggregants, anticoagulants, or a combination of therapy, usually prescribed in the presence of other manifestations of APS or SLE. However, many patients responded well to haloperidol and to the discontinuation of oral contraceptives if this was the precipitating factor.
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PMID:Chorea in the antiphospholipid syndrome. Clinical, radiologic, and immunologic characteristics of 50 patients from our clinics and the recent literature. 919 55

A 20-year-old woman with acute chorea induced by primary antiphospholipid syndrome was studied by using fluorodeoxyglucose and positron emission tomography (PET). PET sessions were conducted during an episode of severe chorea and after recovery. The symptoms predominantly affected the right side of her face and body, and PET demonstrated a corresponding increase in lentiform and caudate nucleus metabolism prevailing on the left side. After recovery, PET showed normal values in the regions previously studied. This study adds further evidence to support the theory that acute choreas are somehow the result of striatal hypermetabolism.
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PMID:Reversible chorea in primary antiphospholipid syndrome. 945 40

A workshop to be held in Sapporo will attempt to upgrade criteria for the antiphospholipid syndrome (APS). These criteria should probably be based on a scoring system using both clinical and biological items. Clinical criteria could be categorized between 'major', that is thrombosis or obstetrical criteria, and 'minor', to be selected among livedo, heart valve lesions, chorea, adrenal hemorrhage, thrombocytopenia, and others. A similar approach could be proposed for biological criteria, with persistent strong LA, high IgG aCL or antibodies to beta2GPI as major criteria if the workshop accepts antibodies directed to co-factors as APS criteria. Minor criteria could include IgM aCL, low/medium IgG aCL, and VDRL. Whether anti-prothrombin, anti-oxidised LDL, and M5 anti-mitochondrial antibodies should be added to the minor criteria, is open to discussion. In our mind, other parameters should be taken into account such as: young age--a method to avoid the questionable exclusion of arteriosclerosis in cases of arterial thrombosis--and the presence of personal and/or first-degree familial features of auto-immunity. Lastly, a differential diagnosis section is probably needed.
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PMID:Towards improved criteria for the antiphospholipid syndrome. 981 94

Three patients, aged five to 16 years, developed chorea as the only or main clinical manifestation of primary antiphospholipid syndrome. In two cases, complaints were self-limited five to eight months after onset. In one patient, the clinical course was complicated by valvulitis. Under corticosteroid treatment, chorea disappeared and cardiac involvement stabilised. Primary antiphospholipid syndrome is a probably under-recognised differential diagnosis of choreatic syndromes in childhood. Assessment of anticardiolipin antibodies and/or lupus anticoagulant should be an obligatory part of the diagnostic work-up of such patients. Early diagnosis of primary antiphospholipid syndrome may improve clinical management and prognosis.
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PMID:Chorea as the presenting clinical feature of primary antiphospholipid syndrome in childhood. 1083 88

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.
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PMID:Chorea in juvenile primary antiphospholipid syndrome. Reversible decreased circulation in the basal ganglia visualised by single photon emission computed tomography. 1056 31

Carbamazepine has been used successfully in the treatment of different movement disorders and was recently reported to be effective for nonhereditary chorea. In view of the significant side effects associated with the drugs currently used to treat chorea, we sought to further evaluate the efficacy of carbamazepine in children with rheumatic chorea. The study was prospective and included 10 children with chorea (eight females and two males; age range = 7-16 years) referred to our Pediatric Rheumatology Clinic between 1995 and 1999. Nine had rheumatic fever and one had antiphospholipid antibody syndrome that later evolved to systemic lupus erythematosus. All were treated with carbamazepine. Improvement was evident within 2-14 days of initiation of low doses of carbamazepine (4-10 mg/kg daily). The plasma drug levels were 2.8-8.2 microg/mL (therapeutic antiepileptic range = 8-12 microg/mL). The chorea disappeared within 2-12 weeks. The duration of treatment was 1-15 months. No side effects were observed. Recurrence was observed in three patients who received a second trial of carbamazepine with a good response. We suggest that carbamazepine may serve as a first-line treatment for rheumatic chorea.
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PMID:Successful treatment of rheumatic chorea with carbamazepine. 1102 Jun 40


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