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Query: UMLS:C0409974 (lupus)
 22,386 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

We present a 47-year-old white woman who had a definitive diagnosis of systemic lupus erythematosus (SLE), and developed cerebellar ataxia of sudden onset. MR imaging showed changes characteristics of cerebellar infarct. Due to the absence of vascular risk factors and to the previous diagnosis of SLE, we postulate that the cerebellar infarct is related to SLE. We are not aware of previous descriptions of the MRI findings in lupus ataxia.
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PMID:Magnetic resonance imaging findings in lupus ataxia. 145 90

The most frequent neurological lesions found on Cranial Computerized Tomography (CT scan) in patients with Systemic Lupus Erythematosus (SLE) are cortical atrophy (psychosis, although in general atrophy is not associated with a particular clinical presentation), infarcts and haemorraghes (strokes). We describe a patient diagnosed of SLE who developed a psychotic clinical picture followed by generalized epileptic seizures within a context of diffuse lupus encephalopathy. On the neurological examination, she presented cortical blindness, generalized piramidalism and extrapiramidal rigidity. Various electroencephalographic recordings showed signs of diffuse cerebral involvement predominantly in posterior regions. Cerebrospinal fluid analysis showed a slight increase of IgG without oligoclonal bands and a positive anti-DNA antibody with an homogeneous pattern. Sequential CT images were carried out demonstrating hypodense areas initially in the occipital regions that extended progressively to the temporal, parietal and finally to the frontal lobes, with a moderate mass effect on the lateral ventricles and with no contrast enhancement. The images of the lesions on the CT disappeared completely with the adequate treatment as the symptomatology resolved. A MRI and cerebral angiography were performed when the patient recovered. Both MRI and angiography showed no alterations. We consider that this neuroradiological finding is exceptional, because of its total resolution. Nevertheless, more observations will be necessary to determine the exact meaning of these abnormalities.
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PMID:[Atypical neuroradiologic manifestation of systemic lupus erythematosus]. 149 82

We report a case of a 47-year-old man with anti-phospholipid antibody syndrome associated with subdural hematoma. The patient had several episodes of arthritis during his thirties. He developed venous thrombosis in his right lower leg at the age of 35, when laboratory studies demonstrated prolongation of activated partial thromboplastin time (APTT) and a biological false-positive result occurred on a syphilis test. When bulbar palsy and Horner's syndrome in the right eye suddenly appeared at the age of 42, he was diagnosed as having brain stem infarction. At the age of 47, he developed constructional apraxia, dyscalculia, skilled movement disturbances and generalized convulsions. Subdural hematoma and multiple lacunes in the cerebral white matter were demonstrated with brain MRI. Furthermore, the patient was positive for Rumpel-Leede phenomenon. Laboratory studies revealed mild thrombocytopenia, prolonged bleeding time and APTT, positive antinuclear antibody and positive test results for both lupus anticoagulant and an anti-cardiolipin antibody, namely anti-phospholipid antibodies. Based on these findings, we consider that the tendency of this patient to bleed may have been due to antiphospholipid antibodies, attacking the platelet membranes and that the bridging veins in the subdural space may be the site at which the bleeding tendency easily appears. Anti-phospholipid antibody syndrome accompanied by hemorrhagic complications had rarely been reported. We suggest that special attention should be given to hemorrhagic complications in patients with anti-phospholipid antibody syndrome associated with fragility of the vessels and/or platelet dysfunction.
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PMID:[A case of anti-phospholipid antibody syndrome associated with subdural hematoma]. 180 71

An 11-year-old boy developed florid choreic movements in his right extremities after having had an episode of febrile illness. He was evaluated at our hospital where MRI disclosed a honeycomb-like low signal intensity area rimmed by a thin Gd-enhanced layer in the left putamen. Arteriography revealed the lenticulostriate arteries being segmentally narrowed and a "ground glass" staining was observed in the left putamen in late venous phase. Sydenham's chorea, that had been the initial impression, was not substantiated because of negative pharyngeal culture for streptococci, negative ASLO/ASK titers and because of lack of clinical stigmata of rheumatic fever. However, prothrombin time was prolonged, and activated partial thromboplastin time (APTT), that had been also prolonged, was not normalized by adding healthy serum, indicating the presence of lupus anticoagulant. VDRL was false positive and anticardiolipin antibodies, both IgM and IgG classes, were also detected. However, systemic lupus erythematosus was unlikely in view of negative antinuclear antibody and LE phenomenon. He deteriorated rapidly due to development of severe bilateral chorea, thereby he was unable to walk or feed himself. He received a 3-day course of mega-dose intravenous methylprednisolone, that temporarily lessened the chorea, but soon it became worse. A second course of mega-dose methylprednisolone was given, followed by daily maintenance dose of prednisolone. His chorea gradually improved in severity and after 2 months only a trace of choreic movements was detected in his hands. He has been followed at our outpatient clinic where he no longer shows chorea and the APTT has improved to nearly normal time.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of chorea as a sole presentation of primary anti-phospholipid antibody syndrome]. 181 92

Neurologic manifestations, afflicting up to 70% of SLE patients, include psychosis, seizures, chorea, neuropathies, and stroke. MRI is useful in evaluation of lupus patients and several reports have documented cerebral atrophy or focal hyperintensities. We report an unusual MRI appearance in a 56-year-old woman with SLE, diagnosed on the basis of pleuritis, lymphopenia, anti-DNA antibodies, and neurologic involvement. She reported recent onset of Raynaud's phenomenon and generalized macular rash. She presented after two months of gradual deterioration with memory loss, flattened affect, dysphagia, dysarthria, anomia, and somnolence, without focal neurologic signs. Investigations included elevated ESR, reduced complement, normal CSF without oligoclonal bands, negative viral serology, normal hormone and vitamin levels, normal renal and hepatic function. Neuropsychologic testing showed widespread impairment (WAIS-R: FSIQ-63; WMS-69; DRS-98; RCPM-14; WAB AQ-78.8). CT was normal but MRI showed strikingly symmetric, confluent hyperintensities extensively involving cerebral and cerebellar white matter on T1 and T2 weighted scans. Basal ganglia and subependymal and subcortical white matter were spared. Treated with prednisone, the patient made a gradual, but incomplete, recovery. These MRI findings may reflect widespread vasculopathy or direct immunologic brain insult with or without immunologic blood-brain barrier disruption.
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PMID:Dementia with leukoencephalopathy in systemic lupus erythematosus. 191 71

A 28-year-old female patient with SLE was found to have complications from intrauterine fetal death, eclampsia, and brainstem vascular damage during pregnancy. These symptoms were associated with lupus anticoagulant (LAC). An LAC positive SLE case with accompanying cerebrovascular disease and fetal loss at the same time has rarely been reported. The first brain CT scan revealed the presence of multiple middle pons infarction. Ten days after onset, a brain MRI showed the change into hemorrhagic infarction in the same region, suggesting disseminated intravascular coagulation (DIC) caused by severe obstetric complications. Although the severity of the brain damage implied poor prognosis, her symptoms were alleviated by intensive care with a large amount of steroid therapy and active rehabilitation. LAC-positive SLE patients are apt to encounter obstetric complications in pregnancy. Therefore they must be treated by medical specialists in cooperation with gynecologists. When they also have accompanying with cerebrovascular diseases, active therapy for SLE is necessary for a good prognosis.
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PMID:[A case of pregnant woman with lupus anticoagulant positive systemic lupus erythematosus complicated with intrauterine fetal death, eclampsia, and brainstem vascular damage]. 192 97

A 21-year-old male with SLE developed seizure, loss of consciousness and focal signs referable to involvement of the front-temporal brain regions. MRI (magnetic response imaging) image revealed high signal areas in the temporal lobes. By these findings, herpes simplex encephalitis (HSE) was suspected at first. But neither isolation of herpes simplex virus nor HSV specific IgM by ELISA was detected. Acyclovir administration by intravenous infusion was'nt effective but corticosteroid pulse therapy was effective. The level of anticardiolipin antibody was very high. Finally, the diagnosis of CNS-lupus with HSE-like characteristics was made in this case.
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PMID:[A case of systemic lupus erythematosus with the central nervous system manifestations (CNS-lupus) mimicking herpes simplex encephalitis (HSE)]. 194 88

Twenty patients with "idiopathic" bone infarction were studied. There were 18 men and 2 women, age 30 to 69 years, at the time of the diagnosis (mean age = 49 years). Sixty-five lesions were recorded with a marked predominance to the lower extremities (77 p. cent are located around the knees) and to the metaphysis (only three pure diaphyseal lesions). Considered asymptomatic, these lesions were painful in 6 patients. They are multiple, and in this case symmetrical, in 12 patients (60 p. cent). X-Rays disclose the classic heterogeneous ball-like, smoke twirled or encapsulated calcifications. A periosteal thickening opposite the lesions was observed in 6 patients; this may be the only radiological sign. Finally, 18 of the 65 lesions were not visible on standard X-Rays, and obvious on MRI. The MRI aspect is characteristic and may be superposed on the basic lesions already described in the course of aseptic osteonecroses of the femoral head. This is, besides, the most sensitive test, snowing a larger number of more extended necroses than the other examinations. CT scanning as well as scintigraphy present a limited advantage. More than half of the patients also present epiphyseal aseptic osteonecroses, often multiple (55 sites for 13 patients), and often unrecognized. The etiological factors are in fact common to both of these diseases: steroid therapy, alcoholism, dyslipidemia, idiopathic forms. Among the possible causes, the literature mentions lupus erythematosus, renal transplant, cytosteatonecrosis, arteriopathies while there is no post-traumatic form. All of the characteristics of these bone infarctions determine a topographic form of the osteonecrotic disease.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Bone infarction, or idiopathic metaphyseal and diaphyseal aseptic osteonecrosis of the long bones. Update and contribution of new imaging technics]. 219 73

This report describes the case of a young woman with biopsy-proven neurofibromatosis and a history of recurrent fetal wastage who had sudden onset of slurred speech and left-sided weakness. CT scan and MRI revealed multiple cerebral infarcts. Laboratory evaluation revealed lupus anticoagulant and high levels of anticardiolipin antibody. This case illustrates the thrombotic tendencies seen in patients with lupus anticoagulant and anticardiolipin antibody, and is the first reported case of LA in association with neurofibromatosis.
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PMID:Lupus anticoagulant and cerebrovascular accident in a patient with neurofibromatosis. 250 76


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