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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Lacunar infarcts in the basal ganglia are known to cause various movement disorders, such as chorea, focal dystonia, and hemichorea-hemiballismus. We report here a case of putaminal lacunar infarction which presented with "painful tonic spasms" of the contralateral limbs. This consisted of paroxysmal brief, painful, flexor contractures of the upper, and occasionally the lower limb. These were not focal seizures but were controlled with carbamazepine, which has been used for the "painful tonic spasms" well-associated with multiple sclerosis. The putaminal infarct we describe is probably related to a lupus anticoagulant and systemic lupus erythematosus.
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PMID:Painful tonic spasms caused by putaminal infarction. 381 Jul 37

A 23-year-old woman, who had nonbullous congenital ichthyosiform erythroderma since her childhood, was diagnosed as nephrotic syndrome caused by systemic lupus erythematosus (SLE). She was pregnant but experienced fetal loss at the age of 25. Although 10 mg/day of oral prednisolone was administered, low levels of serum complement, proteinuria, thrombocytopenia (6.0 x 10(4)/mm3) and biological false positive for STS continued. When she was 27 years old, right hemichorea developed. She was admitted to our hospital at the age of 28 because of low levels of serum complement, high titers of anti ds-DNA antibody, profuse proteinuria, gingival bleeding and thrombocytopenia (1.5 x 10(4)/mm3). The nephrotic syndrome gradually improved after 1 g/day of methylprednisolone for 2 days and the oral prednisolone dosage was then increased up to 40 mg/day, and was tapered to 10 mg/day. Epileptic attack (minor seizure) occurred at the age of 29. Continuous low levels of serum complement and high titers of anti ds-DNA antibody were improved by adding 50 mg/day of cyclophosphamide. However, high levels of beta 2 GPI dependent anticardiolipin antibody and lupus anticoagulant activity were observed throughout the study. Our patient was a very rare case of congenital ichthyosis with typical antiphospholipid antibody syndrome and SLE. A few cases of acquired ichthyosis associated with SLE has been reported, and ichthyosis developed only in active stage of SLE. However, our patient's ichthyosiform lesions were not changed throughout the course.
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PMID:[Systemic lupus erythematosus and antiphospholipid antibody syndrome in a patient with congenital ichthyosiform erythroderma]. 748 67

An association exists between antiphospholipid antibodies and chorea. As these antibodies are associated with thrombosis, it has been suggested that cerebral infarction might cause chorea. However, CT and MRI typically do not demonstrate focal basal ganglionic lesions in such patients and an autoimmune mechanism for chorea has also been proposed. We report a young woman with left hemichorea and dyspnea. She was found to have lupus anticoagulant, large aortic and tricuspid vegetations, and pulmonary emboli. CT and MRI showed a small lesion in the head of the right caudate. In the presence of a definite cardiac source for emboli (valvular vegetations) with embolic activity (pulmonary emboli), it is likely that this patient's hemichorea was caused by cardioembolic caudate infarction.
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PMID:Cardioembolic caudate infarction as a cause of hemichorea in lupus anticoagulant syndrome. 833 81

A young man with systemic lupus (SLE) developed hemichorea 13 years after the onset of his illness. For the first time in the course of his illness he had a positive test for anticardiolipin antibodies (aCL). Magnetic resonance imaging (MRI) of his brain showed lesions of presumed vascular cause in the ipsilateral basal ganglia. The findings support the contention that an immune phenomenon, invisible on proton imaging by MRI, is responsible for the striatal neuronal activation. Chorea, the clinical expression of this activation, was probably blocked on the side previously affected by vascular pathology.
Lupus 1995 Aug
PMID:Hemichorea in systemic lupus erythematosus: significance of MRI findings. 852 32

Records of 108 patients with lupus erythematosus beginning in childhood (1953-1990) were reviewed; 25 had recorded neurologic findings. This is the largest group of childhood lupus erythematosus patients with neurologic disease that has been reported. The average age of children at the time of diagnosis of lupus was 154 months. There were 22 girls and 3 boys in the group. All patients met at least four of the 1982 American Rheumatism Association criteria for the classification of systemic lupus erythematosus. Average age at onset of neurologic difficulties was 168 months. In 4 patients, the neurologic symptoms preceded the diagnosis: 1 month (spastic diplegia), 1 month (bilateral weakness and spasticity), 24 months (chorea), and 26 months (chorea), respectively. Four patients had neurologic symptoms coincident with the diagnosis of lupus erythematosus. In those patients whose symptoms followed the diagnosis of lupus erythematosus, the average elapsed time until symptoms appeared was 33 months; the single lowest and highest outliers were discounted. Most frequent findings were headache (16/25) and behavioral aberrations (10/25). All behavioral manifestations were depression except in 1 patient. Other prevalent findings included hemichorea or chorea (7/25), cerebrovascular accident with hemiplegia or diplegia (7/25), seizures (5/25), visual loss (3/25), and cranial neuropathy (2/25). Vertigo and myelopathy occurred in 1 patient each. All patients were treated primarily with corticosteroids and azathioprine; in the presence of active disease, the drug dosages were increased with significant improvement in neurologic symptoms. Resolution usually occurred from days to months; most improved in a few days to a few weeks; 3-4 months was the longest period until symptoms subsided.
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PMID:Neurologic characteristics of childhood lupus erythematosus. 855 56

We report the results of a systematic study on the association of antiphospholipid antibodies (aPLs) with some neurological disease other than stroke in a childhood population. Patients affected by migraine, benign intracranial hypertension (BIH) or unilateral movement disorders, such as hemichorea and hemidystonia with acute-subacute onset, were screened for aPLs. None of them had clinical or serological evidence of Systemic Lupus Erythematosus (SLE) or other connective tissue disease. Moderate to high levels of anticardiolipin antibodies (aCL) and/or positive Lupus Anticoagulant (LA) were demonstrated in 6 out of 17 patients with migraine, in 3 out of 4 patients with BIH and in all of the 5 patients showing unilateral movement disorders. The association between aPLs and these neurological conditions, usually regarded as cryptogenic, may suggest a possible pathogenetic mechanism.
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PMID:Neurological disorders, other than stroke, associated with antiphospholipid antibodies in childhood. 883 75

Although central nervous system involvement is an important manifestation of systemic lupus erythematosus (SLE), chorea is a relatively uncommon complication. A strong association between chorea and the presence of antiphospholipid antibodies (aPLs) has been reported in patients with SLE, lupus-like disease, or primary antiphospholipid syndrome. We describe a patient with lupus nephritis and cerebral infarction, who subsequently developed recurrent hemichorea associated with increased aPLs levels. A 7-year-old boy suffered from lupus nephritis and a left middle cerebral artery infarction associated with aPLs. He subsequently experienced two episodes of right hemichorea associated with increased aPLs levels without any evidence of further neurological lesions by brain computed tomography or magnetic resonance imaging. The previous left cerebral artery infarction might have increased the susceptibility of the left basal ganglia to the effects of aPLs that contributed to the development of the right hemichorea in this patient.
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PMID:Hemichorea with antiphospholipid antibodies in a patient with lupus nephritis. 1474 Feb 86

A 68-year-old woman was admitted to our hospital because of sudden onset of involuntary movements, similar to those associated with chorea, of the right side of the body and for further evaluation of thrombocythemia. She had no family history of chorea. Neurological findings did not show any abnormality except for chorea of the right side. Laboratory studies showed increased number of white blood cells (14,000/microl) and platelets (188.3 x 10(4)/ microl). Lupus anticoagulant, anti-cardiolipin antibody, and ceruloplasmin levels were within the normal range. Her NAP score was 240, and result for bcr-abl gene expression was negative. Bone marrow puncture showed hypercellularity and increased number of megakaryocytes (550/microl), but there was no atypism. On the basis of these laboratory findings, she was diagnosed with essential thrombocythemia T1-weighted magnetic resonance imaging (MRI) showed a hyperintense lesion extending from the region around the left globus pallidum to putamen. The MRI findings of our study were similar to those related to diabetic hemichorea; however, the results of some tests did not indicate diabetes mellitus. An MRI scan showing high T1 signal intensity in the basal ganglia might not be specific for diabetic hemichorea. In this case, MRI revealed the cause of hemichorea to be micocirculatory failure or small cerebral hemorrhages.
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PMID:[Case report of essential thrombocythemia with sudden onset of hemichorea]. 2148 68