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

Magnetic resonance (MR) imaging and computed tomography (CT) are useful for the evaluation of central nervous system (CNS) lupus. This report describes the use of cranial MR and CT in 21 patients with systemic lupus erythematosus (SLE) with acute neuropsychiatric symptoms manifested by headache, seizures, focal neurological deficits, psychosis, or organic brain syndrome. Computed tomography was found to be insensitive and detected only diffuse atrophy (two cases), cerebral infarct (one case), and intracerebral haemorrhage (one case) in the 21 patients. Cranial MR images obtained with a General Electric 1.5 tesla Signa unit detected labile and fixed areas of increased proton intensity interpreted as focal oedema (eight cases), infarct (10 cases), haemorrhage (one), atrophy (seven), and acute sinusitis (two). Focal oedema was characterised by labile, high intensity lesions in the gray or white matter of the cerebellum, cerebrum, or brain stem, which completely resolved after aggressive corticosteroid treatment. Most high intensity reversible or fixed lesions evident on MR were not apparent on cranial CT images. In several patients sequential MR images were valuable in monitoring the efforts of treatment. Although histological confirmation of the high intensity brain lesions apparent on MR is desirable, prior necropsy studies suggest that pathological confirmation may be difficult owing to the paucity of recognisable brain lesions in patients with CNS lupus. It is concluded that for the evaluation of acute neuropsychiatric SLE MR is useful and provides more information than cranial CT.
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PMID:Magnetic resonance and computed tomographic imaging in the evaluation of acute neuropsychiatric disease in systemic lupus erythematosus. 261 53

A 50-year-old man with systemic lupus erythematosus developed organic brain syndrome. He responded to corticosteroid therapy and recovered completely from acalculia, apraxia and memory disturbance. Throughout his course, the cerebrospinal fluid (CSF) IgM, IgA and IgG indices were decreased in relation to the progression of normal alpha activity in the electroencephalogram. CSF Ig indices may be useful for monitoring central nervous system lupus disease activity.
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PMID:A serial study of changes in intrathecal immunoglobulin synthesis in a patient with central nervous system systemic lupus erythematosus. 343 May 11

The validity of the hypothesis that some of the neuropsychiatric manifestations of systemic lupus erythematosus (SLE) are mediated by the direct effects of antibody binding to neuronal cell membranes is dependent on the demonstration of antineuronal activity within the central nervous system of patients with active central nervous system disease. Using a radiolabelled staphylococcal protein A assay, we tested cerebrospinal fluid from 27 patients with SLE and central nervous system manifestations, and cerebrospinal fluid from 18 additional patients with SLE but free of central nervous system disease for antibody reactive with the cultured human neuronal cell line SK-N-SH. Cerebrospinal fluid from 20 of 27 patients with active lupus central nervous system disease had increased immunoglobulin G (IgG) antineuronal activity compared with cerebrospinal fluid from two of 18 patients with SLE without central nervous system disease. Ninety percent of the patients with psychosis, organic brain syndrome or generalized seizures had increased IgG antineuronal activity as compared with only 25 percent of the patients who presented with hemiparesis or with chorea/hemiballismus. Antineuronal activity per microgram of IgG was concentrated eightfold in the cerebrospinal fluid of patients with active central nervous system disease as compared with the serum activity. Patients with or without active central nervous system disease did not differ significantly in the amount of serum antineuronal binding activity. The results are consistent with the hypothesis that the more diffuse central nervous system manifestations of SLE are a direct result of the interaction of antibody with neuronal cell membranes.
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PMID:Cerebrospinal fluid antibodies to neuronal cells: association with neuropsychiatric manifestations of systemic lupus erythematosus. 746 11

We describe a patient with systemic lupus erythematosus (SLE) with severe organic brain syndrome, who showed diffuse low density areas in the cerebral white matter on computed axial tomography (CAT) scans. A 43-year-old woman with SLE showed disturbances in various intellectual functions, followed by the development of consciousness disturbances and urinary incontinence. CAT scans, single photon emission computed tomography (SPECT) and cerebrospinal fluid (CSF) interleukin 6 (IL-6) were serially examined during the clinical course before and after treatment with high doses of prednisolone. CSF IL-6 activity paralleled the central nervous system (CNS) disease activity most consistently. By contrast, the diffuse low density areas in cerebral white matter on CAT scans as well as the decreased blood flow in the cerebral cortices on SPECT persisted long after the improvement of the CNS manifestations. Magnetic resonance imaging (MRI) scans also revealed the diffuse areas of increased signal intensity in the subcortical white matter on T2 weighted images. The diffuse lesions in the cerebral white matter are considered to reflect the edema as well as other irreversible changes that have resulted from the disturbed cerebral circulation presumably due to the diffuse microangiopathies in the cerebral cortices and not to be necessarily correlated with the CNS disease activities. Moreover, it is suggested that the inflammatory process evidenced by the elevation of CSF cytokines in addition to the disturbed circulation in the cerebral cortices might play an important role in the pathogenesis of CNS lupus in our patient.
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PMID:Systemic lupus erythematosus presenting with diffuse low density lesions in the cerebral white matter on computed axial tomography scans: its implication in the pathogenesis of diffuse central nervous system lupus. 779 63

Magnetic resonance imaging (MRI) of the brain is a sensitive method to detect parenchymal tissue lesions. Its value in the diagnosis of central nervous system (CNS) lupus is disputed. To address this question, we have conducted an open and prospective study in a population of 44 SLE patients. We investigated 24 patients (mean age 33 +/- 13 yr) with past or active CNS lupus (group A) that included organic brain syndrome (12), migraine (8), focal neurological signs (7), seizures (2), myelopathy (1) and narcolepsy-cataplexy (1), and 20 patients (mean age 32 +/- 12 yr) without CNS lupus (group B). Health controls comprising nine females and one male aged 31 +/- 9 yr were also studied for comparison (group C). MRI was performed using sagittal T1-weighted images, axial and coronal spin density, and T2-weighted images. All scans were read blindly. Thirteen patients in group A and 10 in group B had well-identified lesions on sequences with long repetition time. Lesions were mostly multiple, small, punctate areas of increased signal at periventricular or subcortical white matter of both cerebral hemispheres. The number and location of lesions were not significantly different in both groups. None of the group C patients had MRI lesions. The presence of lesions was significantly associated with age at study and disease duration, but not with the presence of CNS lupus. In summary, MRI abnormalities are detected in neurologically asymptomatic SLE patients. Whether this represents subclinical brain involvement remains unknown.
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PMID:Magnetic resonance imaging of the brain in systemic lupus erythematosus. 854 7

We report here a case of neuropsychiatric lupus erythematosus with organic brain syndrome and transverse myelitis which was successfully managed by plasmapheresis. A 27-year-old female with facial rash, arthralgia and fever was diagnosed as having SLE and treated with oral prednisolone (PSL) in June 1996. After 6 weeks she demonstrated muscle pain and a spiking temperature. The dose of PSL was increased but clinical symptoms did not improve. In August, pulse methyl-PSL was performed and she subsequently-developed delirium, impairment of orientation, memory and perception, which were followed by paraplegia of the lower extremities and loss of sphincter control. Intravenous bolus cyclophosphamide was not effective, but liver dysfunction, bone marrow suppression and respiratory failure due to an infection of pneumocystis carinii were observed. We then performed plasmapheresis or immunoabsorption several times. After this treatment steady improvement was observed. High values of antiribosomal P protein antibodies in the serum and interleukin-6 in the cerebrospinal fluid decreased. Small foci of increased signal intensity detected on cranial magnetic resonance imaging and hypoperfused areas on single-photon emission CT diminished. The patient was maintained on low-dose PSL and no recurrence has been observed 15 months from the onset.
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PMID:[A case of severe neuropsychiatric lupus erythematosus treated by plasmapheresis: diagnostic values of serum antiribosomal P protein antibodies and interleukin-6 in cerebrospinal fluid]. 979 79

Among various rheumatic diseases, systemic lupus erythematosus and Behcet's disease are frequently complicated with serious neurological involvement, called CNS lupus and neuro-Behcet's syndrome (NB), respectively. CNS lupus includes lupus psychosis, such as organic brain syndrome and non-organic psychosis, as well as non-psychotic CNS lupus, such as epilepsy and focal lesions. Anti-ribosomal P antibody is closely associated with lupus psychosis, whereas anti-phospholipid antibodies appear to be involved in some manifestations of non-psychotic CNS lupus. NB includes acute type and chronic progressive type. Acute NB is characterized by acute meningoencephalitis with focal lesions, which respond to steroid therapy. By contrast, chronic progressive NB is characterized by intractable slowly progressive dementia and/or psychosis with persistent elevation of cerebrospinal fluid IL-6 activity, which is resistant to conventional steroid therapy. However, recent studies suggest the efficacy of low dose methotrexate in chronic progressive NB.
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PMID:[Central nervous system involvement in rheumatic diseases]. 1007 15

Neuropsychiatric systemic lupus erythematosus (NPSLE) has become a popular term designing all neurological and psychiatric complications in patients with systemic lupus erythematosus (SLE). It occurs in up to two thirds of all SLE patients and it covers a vast array of disorders ranging from peripheral neuropathy to stroke, psychosis, and dementia. Mechanisms associated with the pathogenesis of NPSLE include anti-neuronal antibodies, antiphospholipid antibody associated thrombosis, emboli from cardiac source and, rarely, vasculitis by immune complex depositions. Although the most common manifestations is cognitive dysfunction (50%), NPSLE may also present itself as peripheral neuropathy (15%), psychosis (10%), or other central nervous system abnormalities (stroke, organic brain syndrome, seizures). In lupus patients, one should always look for secondary causes of the neuropsychiatric manifestation, including infection, toxic metabolic abnormalities, and hypertension. We present two cases of SLE, which developed neuropsychiatric manifestations.
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PMID:[Neuropsychiatric manifestations in systemic lupus erythematosus]. 1780 40

Patients with SLE show in a variety of neuropsychiatric symptoms, although we could not use standardized methods for evaluating and making diagnosis of the syndromes. ACR felt to develop objective and valuable tools for the diagnosis and classification of neuropsychiatric lupus, therefore, they proposed a new tentative set of nomenclatures describing neuropsychiatric lupus syndromes in accordance with 4th edition of Diagnostic and Statistical Manual of Mental Disorders provided by American Psychiatric Association. For this purpose, The Ad Hoc Committee collected and evaluated 108 case presentations of neuropsychiatric lupus from USA, Canada, and UK. Re-evaluation of the tentative nomenclatures selected 19 neuropsychiatric syndrome to facilitate and enhance clinical research. They grouped into peripheral and central nervous system lupus, and central nervous system lupus was divided into neurologic syndromes and diffuse psychiatric/ neuropsychological syndromes. Instead of organic brain syndrome, a term of acute confusional state was introduced.
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PMID:[Neuropsychiatric lupus (CNS lupus and PNS lupus)]. 1928 Sep 28