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Query: UMLS:C0008489 (chorea)
 2,102 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a 21-year-old woman in whom chorea was associated with antiphospholipid antibodies. In August 1986, she developed involuntary movement which started in the right hand but subsided spontaneously. In September 1988, she again developed right-sided involuntary movements which started in the right hand but rapidly progressed to involve the whole of the right side. In September 1990, she was admitted to our hospital for investigation of choreiform movements, because her involuntary movements had progressed to involve all four extremities. She had no family or past history of chorea, psychiatric, rheumatological or vascular disease. On admission, she had difficulty in speaking and swallowing due to choreiform movements of her mouth and tongue. Her gait was unsteady. On walking she had wild gyrations of the arms. Choreiform movements of all four extremities, neck, face, mouth and tongue were present at rest, more marked on the right side. There was no other neurological deficits. She had none of the classical features of SLE. She had none of the complications commonly associated with antiphospholipid antibody syndrome (APS) (i.e., recurrent spontaneous abortion, thrombosis and thrombocytopenia). Laboratory tests revealed that antinuclear antibody was present. Cardiolipin antibody (VDRL) was positive but specific tests for syphilis were negative. Anticardiolipin antibodies were present. All coagulation studies have failed to reveal lupus anticoagulant. Brain CT, MRI, 123IMP-SPECT and cerebral angiography were normal. Associated with her chorea, she had the serological but not the clinical features of APS. We suggest that antiphospholipid antibodies should be looked for in all unexplained cases of chorea, even when the associated clinical signs of APS are absent.
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PMID:[Chorea associated with antiphospholipid antibodies]. 130 Feb 73

Recent reports describe the association of antiphospholipid antibodies (aPL) with chorea or severe heart valve lesions in systemic lupus erythematosus, lupus-like disease, or the primary antiphospholipid antibody syndrome. We conducted a case series and a case-control investigation of patients with rheumatic fever with Sydenham chorea or other manifestations of rheumatic fever for anticardiolipin antibodies (aCL) during the acute attack and disease remission. Eighty percent of patients were positive for aCL during the rheumatic fever attack vs 40% when inactive (p = 0.035); IgG and IgM aCL increased significantly with disease activity. Individuals with or without Sydenham chorea were equally positive for aCL (76 and 83%, respectively). A significant association was found between IgM aCL and carditis: All patients with valvulitis had IgM aCL (100%) vs 37% of patients without valvular involvement (p = 0.02). aPL may play a role in the pathogenesis of some clinical manifestations of acute rheumatic fever.
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PMID:Anticardiolipin antibodies in acute rheumatic fever. 781 96

Antiphospholipid antibodies are a relatively heterogeneous mix of immunoglobulins with binding specificities for negatively charged or neutral phospholipids. Currently, the most commonly detected antiphospholipid antibodies include the anticardiolipin antibody, the lupus anticoagulant, and an antibody implicated in false-positive VDRL testing. Recently, a clinical syndrome of vaso-occlusive disorders associated with antiphospholipid antibodies has been identified and may result from immune-mediated disruption of endothelial function. This clinical syndrome encompasses arterial and venous thrombosis, recurrent fetal loss, neurologic dysfunction (eg, migraine, chorea, and encephalopathy), systemic and pulmonary arterial hypertension, and endocardial disease. Although most commonly associated with systemic lupus erythematosus, the antiphospholipid antibody syndrome also has been identified in patients with vaso-occlusive disease without systemic lupus erythematosus. Recently, identification of antiphospholipid antibodies has been facilitated by the development of a more sensitive assay for anticardiolipin antibody. In this article, case histories of three patients with arterial thrombosis and associated anticardiolipin antibodies, including the first associated case of terminal aortic thrombosis, are reviewed and the subject of the antiphospholipid antibody syndrome is discussed.
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PMID:Antiphospholipid antibodies and arterial thrombosis. Case reports and a review of the literature. 155 Apr 84

Two cases of primary antiphospholipid syndrome are described. A girl presented with myocardial infarction at the age of 6. afterward developed chorea, livedo reticularis, thrombocytopenia and circulating lupus anticoagulant (LAC). A boy, age 7, had an episode of intracranial hypertension and a deep venous thrombosis of a lower left limb, both recurrent in the following years. A high titer of IgG anticardiolipin antibodies (aCI) was detected. These observations suggest that both LAC and aCI tests should be performed in children with thromboembolic phenomena when the criteria for a definite autoimmune disease are lacking.
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PMID:Primary antiphospholipid syndrome: a report of two pediatric cases. 192 Mar 12

We report the case of a patient with the unusual combination of migraine, chorea, and retinal arterial thrombosis along with laboratory evidence of autoimmunity. In the absence of systemic lupus erythematosus, the clinical manifestations suggest the presence of the primary antiphospholipid antibody syndrome.
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PMID:Retinal migraine, chorea, and retinal artery thrombosis in a patient with primary antiphospholipid antibody syndrome. 203 Mar 76

Since the original description and definition of the antiphospholipid syndrome (APS), a number of distinct clinical manifestations related to it have appeared in the literature. These include vascular obstruction of both veins and arteries, thrombus formation on the endocardium and its consequences, as well as a group of other conditions where vascular obstructive mechanisms are either incompletely understood or unproven, eg, chorea, avascular necrosis, and pulmonary hypertension. Single vessel (large/medium) involvement or multiple vascular occlusions may cause a wide variety of presentations. Any combination of vascular occlusive events may occur in the same individual, and the time interval between them also varies considerably from weeks to months or even years. Rapid chronological occlusive events occurring over days to weeks have been termed the "catastrophic" APS. Most of these complications may be ascribed to the hypercoagulable state of which antiphospholipid antibodies appear either to be "markers" or intimately connected with the highly complex coagulation mechanisms resulting in thrombotic occlusions.
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PMID:Clinicopathologic correlations of the antiphospholipid syndrome. 774 Mar 6

A female patient with a history of migraines and chorea developed polyarthralgia at age 24 and was diagnosed with rheumatoid arthritis. In 1991 she was hospitalized because of impaired renal function and hypertension. Examination revealed thrombocytopenia and the presence of lupus anticoagulant. Antinuclear antibody was weakly positive, but anti-DNA antibody was negative, and no decrease in leukocyte count or complement level was observed. Rheumatoid arthritis with antiphospholipid syndrome was diagnosed. Renal biopsy showed renal thrombotic microangiopathy. This renal lesion was considered to be associated with antiphospholipid syndrome. Cyclophosphamide pulse therapy and anticoagulation therapy decreased proteinuria and improved renal function.
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PMID:Renal thrombotic microangiopathy in a patient with rheumatoid arthritis and antiphospholipid syndrome: successful treatment with cyclophosphamide pulse therapy and anticoagulant. 780 16

One of the clinical hallmarks of antiphospholipid syndrome is the development of neurological complications, namely cerebral ischaemia, chorea, multi-infarct dementia, amaurosis fugax, migraine and transverse myelitis. An animal model should include the development of measurable neurological deficits and evidence of cerebral infarction. Although there are a number of mouse models for fetal loss, there has been no convincing model for the neurological complications of the antiphospholipid syndrome. One explanation for the high frequency of neurological events in antiphospholipid syndrome is a vulnerability of the cerebral vasculature to the hypercoagulable state associated with the syndrome. A greater appreciation of the differences in the regulation of coagulation between the systemic and cerebral vasculatures may be key to understanding the apparent predilection for central nervous system involvement in the antiphospholipid syndrome.
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PMID:Models for central nervous system complications of antiphospholipid syndrome. 780 11

The spectrum of the primary antiphospholipid syndrome has expanded in recent years. It has been associated with a number of non-thrombotic syndromes such as pulmonary hypertension, adrenal insufficiency, chorea and avascular necrosis of bone. Yet, it has not been described in association with inflammatory pulmonary disease. We describe a young male with definite primary antiphospholipid syndrome who developed insidious diffuse pulmonary infiltrates. The histopathologic examination of the involved lung demonstrated alveolitis and fibrosis. We suggest that this pulmonary involvement may represent another manifestation of the primary antiphospholipid syndrome.
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PMID:Fibrosing alveolitis associated with primary antiphospholipid syndrome. 792 62

Antiphospholipid antibodies (APA) comprise a family of immunoglobulins characterized by their pattern of reactivity in a number of laboratory tests. Included in this family are lupus anticoagulant (LA) anticardiolipin antibodies (ACA) and antibodies causing biologic false positive serologic tests for syphilis (BFP-STS). LA and ACA occur in a variety of conditions, including other autoimmunes disorders, infectious diseases, neoplasic disorders, in association with certain drugs and in otherwise healthy individuals. Clinical interest in LA and ACA is increasing. Antiphospholipid antibody syndrome is characterized by a triad of clinical features which include fetal loss, thromboembolic disease and thrombocytopenia. Other clinical manifestations related with APA are livedo reticularis, cutaneous necrosis, hemolytic anemia, heart valve disease, chorea, migraine and obstetric problems as fetal growth retardation, pre-eclampsia, post-partum serositis or neonatal thrombosis or catastrophic antiphospholipid syndrome. Therapy is mainly directed against the widespread and diverse manifestations associated with the obstruction of small and large vessels. Long-term treatment with oral anticoagulation therapy is advised, even if the venous or arterial occlusion occurred many years previously. In patients with primary antiphospholipid syndrome there is no evidence that the prophylactic administration of steroids or immunosuppression will prevent thromboembolic events. Although the administration of more energetic immunosuppression with cyclophosphamide in pulse form is effective in reducing elevated antibody levels, there is usually a rapid rebound to pretreatment levels shortly after discontinuation of the therapy. A history of recurrent fetal loss requires mandatory treatment during pregnancy. Although the actual prospective risk of pregnancy loss in women with antiphospholipid syndrome and prior pregnancy loss is unknown, it may exceed 60%. Because of this many investigators have treated women with antiphospholipid syndrome with either antiplatelet agents, immunosuppressive agents, or anticoagulants in an attempt to improve pregnancy outcome. Unfortunately, there is no unequivocal proof that any of these therapies are fully efficacious. Despite varying treatment protocols, the live birth rate with treatment was 70%, similar to that reported in the recent randomized clinical trial. Thrombocytopenia and autoimmune hemolytic anemia in patients with APA are treated similarly as patients without APA. Treatment of asymptomatic patients isn't indicated, because only approximately 10-15% of patients with APA developed complications.
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PMID:Clinical and therapeutic aspects associated to phospholipid binding antibodies (lupus anticoagulant and anticardiolipin antibodies). 798 46


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