Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 30-year-old Caucasian male with systemic lupus erythematosus suffered acute catastrophic-onset transverse myelitis. Two years earlier aseptic meningitis, another rare CNS lupus manifestation, had been diagnosed. MRI showed involvement of the medulla oblongata and the entire spinal cord. Therapy with intravenous high-dose methylprednisolone and intravenous cyclophosphamide is discussed.
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PMID:Involvement of the entire spinal cord and medulla oblongata in acute catastrophic-onset transverse myelitis in SLE. 1079 31

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

In this paper we wanted to present cases of stroke occurring in the course of cerebral vasculitis. Cerebral vasculitis occurs most often in patients with infections, tumours and connective tissue diseases. Idiopathic vasculitis is observed very rarely and making the diagnosis meets great difficulties. Three patients (2 women and 1 man) with idiopathic cerebral arteritis aged 28-41 who underwent treatment in our department are prevented. There were no known stroke risk factors in anamnesis and diagnostic investigations. All patients had headaches before the occurrence of stroke. Electrocardiography, echocardiography were normal. Two patients had arteriography--small arterial occlusions and changes in arteries' walls were seen. Ischaemic changes in both cerebral hemispheres were shown in CT and MRI scans. One patient had high serum level of antinuclear antibodies one year later the diagnosis of lupus erythematosus was made. In patients with cerebral vasculitis extensive laboratory investigations should be carried out and combined with a detailed follow-up study. Neurological changes could be the first symptom of connective tissue disease.
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PMID:[Stroke in the course of cerebral arteritis]. 1110 76

A 22-year old woman developed erythema in her hands. She also suffered from general fatigue and arthralgia. In July of 1992, she visited the dermatology clinic of Nagoya City University Hospital. We made a diagnosis of subacute cutaneous lupus erythematosus with thrombocytopenia and gave her 30 mg of prednisolone daily. Her conditions were improved and had been stable with 5 mg of prednisolone daily. In the winter of 1993, she developed ulcer in her fingers and soles. These lesions were associated with antiphosholipid syndrome. She complained of mild headache. She was examined by CT, MRI and single photon emission computerized tomography (SPECT) using N-isopropyl-p-[123I] iodoamphetamine (IMP). Low perfusion areas in the bilateral occipital lobes and the right temporal lobe were found in SPECT, although no abnormalities were detected by CT and MRI. Low perfusion areas in antiphospholipid syndrome might be caused by microarterial thrombosis, microvenous thrombosis or spasms, although they could be reversible. Ten mg of argatroban daily for one month improved cerebral low perfusion, headache, and skin lesions. In this case, argatroban was effective for both cerebral low perfusion and skin ulcer, which were related to antiphospholipid syndrome.
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PMID:[Improvement of cerebral perfusion with argatroban in a patient with antiphospholipid syndrome]. 1121 61

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 the case of a 30-year-old caucasian woman affected by SLE who developed neurological symptoms (prosopagnosia and visual-spatial agnosia) after nine years of disease. Brain MRI showed no abnormalities while a brain SPECT scan showed diffuse uptake defects and hypoperfusion areas in the right and left frontal-parietal regions. At that time the patient was on hydroxychloroquine (400 mg/day) and oral prednisolone (0.5 mg/kg/day) as maintenance therapy. One year later the patient showed worsening of Raynaud's phenomenon with digital dystrophic lesions and was therefore treated with an intravenous infusion of Iloprost (1.5 ng/kg/min per 6h/day for 10 days consecutively), while baseline treatment remained unchanged. One month later the patient showed a dramatic improvement in her cognitive function and subsequent SPECT scans showed the gradual disappearance of perfusion abnormalities. This first report of Iloprost treatment in CNS lupus suggests the potential therapeutic usefulness of this drug in patients with SLE and functional CNS involvement.
Lupus 2002
PMID:Sustained normalization of cerebral blood-flow after iloprost therapy in a patient with neuropsychiatric systemic lupus erythematosus. 1189 21

A 61-year-old woman with a history of photosensitive dermatitis and recurrent mouth ulcers presented with progressive weakness typical of amyotrophic lateral sclerosis (ALS), and subsequently underwent extensive neurologic and rheumatologic testing. We investigated whether ALS-like motor neuron disease associated with a positive antinuclear antibody (ANA) is really ALS or rather neuropsychiatric systemic lupus erythematosus (NPSLE). On neurologic evaluation, she had prominent bulbar involvement with dysarthria and dysphagia associated with profound lingual fasciculations and a denervating pattern on electromyogram. MRI showed no evidence of cerebral ischemia. Laboratory studies revealed a positive ANA (1:2560 titer), positive antiphospholipid antibodies (GPL and MPL), circulating lupus anticoagulant, and depressed C3 and C4. Repeat MRI studies at 4 and 11 mo revealed an evolving infarct in the paramedian pons consistent with the presence of NPSLE. Therapy was initiated with corticosteroids and intravenous cyclophosphamide, and the neurologic condition did not improve, but also did not progress inexorably as would be expected with ALS. NPSLE, presumably through the mechanism of ischemic vasculopathy, may present as motor neuron disease clinically indistinguishable from ALS.
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PMID:Neuropsychiatric systemic lupus erythematosus presenting as amyotrophic lateral sclerosis. 1190 83


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