Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Central nervous involvement in Sjogren's syndrome (CNS-SS) is not uncommon and has a variety of manifestations. We describe a 47-year-old woman with Sjogren's syndrome who presented with distal renal tubular acidosis with severe hypokalemia and hypokalemic myopathy. She developed progressive obtundation after years of stable disease. ANA, anti-Ro antibodies were positive. Brain MRI showed a cleft in the mid pons which was hypointense on T1 and hyperintense on T2 which was considered to be classical of central pontine myelinolysis. Serial MRI showed initial enlargement of the lesion which persisted despite successful immunosuppressive therapy with pulse methylprednisolone, pulse cyclophosphamide, plasmapheresis and IVIG.
Lupus 2000
PMID:Central pontine myelinolysis--a rare manifestation of CNS Sjogren's syndrome. 1098 55

Parkinsonism complicating systemic lupus erythematosus (SLE) is extremely rare. We report two girls with SLE who developed extrapyramidal parkinsonian features after an initial stormy course. One patient presented with generalized tonic clonic seizure and was then noted to have akinetic mutism and masked face. MRI brain revealed abnormal signals in bilateral basal ganglia and single photon emission computed tomography (SPECT) showed hypoperfusion in the same area. EEG background was slow and disorganized. Symptoms persisted despite high dose intravenous methylprednisolone and cyclophosphamide. Intravenous immunoglobulin (IVIG) was prescribed empirically and was followed by complete recovery. Both EEG and MRI brain were normal on follow-up. The second patient was found unconscious and then developed bradykinesia, mutism and shuffling gait. MRI and SPECT both detected abnormalities in basal ganglia. EEG was slow. Intravenous immunoglobulin was given after methylprednisolone and cyclophosphamide. This was followed by clinical improvement. The pathogenesis of basal ganglia injury in SLE, along with the management of cerebral lupus and the mechanisms of action of IVIG, are discussed.
Lupus 2000
PMID:Parkinsonism as unusual neurological complication in childhood systemic lupus erythematosus. 1098 56

This study shows the contribution of MRI to diagnosis and follow up of the cerebral lesions in neuro-lupus. Ten cases of neuro-lupus have been reported. The affected patients are 7 women and 3 men having an average age of 34 years. For 8 patients, MRI revealed lesions that were not detected using computed tomography. These lesions were high signal zones on T2, scattered with predilection to deep and sub-cortical cerebral white matter. MRI has also shown cortical and sub-cortical cerebral atrophy for 3 cases, cortical and deep infarcts for 2 cases and both atrophy and infarcts in one case. The MRI diagnosis was normal for the two remaining patients. MRI contributes to the diagnosis confirmation and localization of the cerebral lesions and to the follow-up. In spite of MRI sensitivity, the detection of these lesions remains non specific to neuro-lupus. New methods of exploration as the spectroscopy by magnetic resonance and the cerebral scintigraphy with Technetium 99m could be very helpful in diagnosis of cases for which MRI has shown limitations.
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PMID:[Value of magnetic resonance imaging in neuro-lupus: report of 10 cases]. 1102 22

We report a case of a 14 year old girl with SLE who developed neurological involvement in the form of posterior internuclear ophthalmoplegia (pINO). An MRI showed lesion involving pons which corroborated with the pINO.
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PMID:Unilateral inter-nuclear ophthalmoplegia in systemic lupus erythematosus. 1128 Feb 33

A rare manifestation of systemic lupus erythematosus (SLE) is cerebral venous sinus thrombosis (CVST), in which early diagnosis and aggressive therapy are of prime importance for favorable outcome. The pathogenesis of CVST is largely unknown, but it is thought to be caused by cerebral vasculitis, antiphospholipid antibodies or other conditions associated with enhanced coagulability. We describe two cases of SLE with CVST which were not associated with antiphospholipid antibodies. Both cases were treated with immunosuppressants (intravenous methylprednisolone and cyclophosphamide pulse therapy) and anticoagulant drugs (heparin and subsequent maintenance therapy with warfarin). There was a marked improvement of neurologic symptoms with the disappearance of thrombus in a follow-up MRI. The possibility of CVST should be considered in any patients with SLE who show neuropsychiatric manifestations.
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PMID:Systemic lupus erythematosus complicated with cerebral venous sinus thrombosis : a report of two cases. 1141 Jun 99

We report the case of a 36-year-old woman affected by systemic lupus erythematosus who developed rapidly progressive multifocal leukoencephalopathy. Cidofovir therapy induced disappearance of JC virus genome from the cerebrospinal fluid and stabilization of the MRI picture. Despite the fatal outcome after a few months of disease, cidofovir treatment deserves further testing as single antiviral therapy in HIV-negative PML patients.
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PMID:Reversal of CSF positivity for JC virus genome by cidofovir in a patient with systemic lupus erythematosus and progressive multifocal leukoencephalopathy. 1148 86

Cognitive dysfunction is found in a considerable proportion of patients with systemic lupus erythematosus (SLE). SPECT provides an estimate of regional cerebral blood flow (rCBF) which has been claimed to be sensitive to detect brain involvement in SLE. It is, however, uncertain if these perfusion defects are related to cognitive dysfunction. In the present study we investigated whether cerebral dysfunction assessed by neuropsychological measures was associated with changes in rCBE Fifty-two SLE patients were examined with a battery of neuropsychological tests and MRI of the brain. For each patient 99mTC-HMPAO-SPECT was performed with the visual cortex as reference, and a reduction in rCBF of > 15% was considered abnormal. Regional CBF was performed with an automated computer program quantitatively estimating blood perfusion in 16 symmetrical sectors of the brain. Several sectors of the brain showed varying areas of reduced rCBF with the temporal lobes most frequently involved. There were generally no associations between cognitive level of functioning and reduced rCBF. MRI demonstrated cerebral infarcts in 9 (17%) patients. In general rCBF was reduced in all sectors of the brain in patients with infarcts, although statistical significant difference in rCBF between patients with and without infarcts was only seen in the parietal lobe. Several neuropsychological functions were influenced by the presence of cerebral infarcts. There was no significant association between immunological measures and SPECT findings or neuropsychological measures. Neuropsychological dysfunction in SLE was associated with the presence of cerebral infarcts detected by MRI, but not by changes in rCBF. SPECT seems to add little if any information to that obtained by clinical examination, neuropsychological testing, and MRI. Since anticoagulation may prevent cerebral infarcts, such prophylactic intervention may be of importance in preventing cognitive deterioration.
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PMID:Neuropsychological dysfunction in systemic lupus erythematosus is not associated with changes in cerebral blood flow. 1151 2

The acute spinal cord infarction is a rare cause of acute-onset paraplegia. Furthermore, it is specially uncommon that the infarction occurs in patients with apparent low predisposition to vascular disease. The 20210A allele of the prothrombin gene (causing a threefold-increased risk in venous thromboembolism) was recently associated with unexplained spinal cord infarction in young women under treatment with estrogens (contraceptive pill). We report a case of anterior spinal artery syndrome resulting from an ischaemic infarction at the anterior aspect of the spinal cord in a healthy 50-year-old woman, carrying this mutation, being the first published case under treatment with transdermal estradiol. She referred the typical sudden-onset back pain associated to clinical anterior spinal artery syndrome with sphincter dysfunction and nontraumatic paraplegia. A possible multiple sclerosis was ruled out and the steroids or immunoglobulin therapy induced no clinical improvement. Cerebrospinal fluid and other investigations were all negative. Sequential MRI scans revealed development of spinal cord infarction from T10 to T11, with increased signal in T2-weighted image (T2). Because she referred a previous thrombophlebitis and suffered a deep-vein thrombosis one month after paraplegia, a complete coagulation study was performed. Antithrombin, proteins C and S, homocysteine, factor V Leiden, lupus anticoagulant and anticardiolipin antibodies were all normal or negatives. In opposite, the 20210A variation was positive (heterozygous) and the factor VIIIc level was very high (280 U/dl eight months later). We argue the relative importance of both findings. The patient had no a substantial recovery over a period of 20 months.Certainly, the prothrombin 20210A seems to be associated with unexplained ischemic myelopathy among the young women with estrogens.
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PMID:[Spinal cord infarction and recurrent venous thrombosis in association with estrogens and the 20210A allele of the prothrombin gene]. 1174 25

Lupus-related transverse myelitis is a rare but serious complication. A 25-year-old Japanese woman with systemic lupus erythematosus (SLE) was admitted because of numbness of the face and left upper extremity, headache, and intermittent fever. Six days later, she developed tetraplegia. MRI of the spinal cord showed longitudinal high intensity signals from medulla oblongata to C5, and from Th12 to conus medullaris on T2-weighted image. These MRI findings were consistent with acute catastrophic neurological abnormalities. Despite administration of the combination of methylprednisolone and cyclophosphamide pulse therapies, as well as plasmapheresis, her condition did not improve. Any vasculopathy in addition to the autoimmune pathogenesis, and narrow therapeutic window may relate to the present refractory case.
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PMID:Systemic lupus erythematosus related transverse myelitis presenting longitudinal involvement of the spinal cord. 1186 6

We report a case of Tolosa-Hunt syndrome (THS) in a patient with systemic lupus erythematosus studied with MRI. Magnetic resonance showed enlargement of the cavernous sinus and compression of the carotid syphon by enhancing tissue. In particular, fat-suppressed T1-weighted images before and after contrast agent injection and MR angiography showed extension of the abnormal tissue to the apex of the orbit and narrowing of the internal carotid artery. A presumptive diagnosis of THS was made and steroid treatment was started with rapid relief of symptoms. Follow-up MR study after steroid therapy demonstrated sub-total resolution of the neuroradiological findings. Neuroradiological findings in THS are quite typical but they may be subtle; furthermore, the presence of a systemic disease may suggest secondary involvement of the cavernous sinus. Utilization of the appropriate MR techniques and follow-up exams may contribute to the diagnosis of THS even in the presence of other systemic diseases.
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PMID:Tolosa-Hunt syndrome in a patient with systemic lupus erythematosus. 1187 Apr 31


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