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

Lupus anticoagulants and anticardiolipin antibodies are antiphospholipid antibodies (APLAb) with related antigenic specificities and are newly recognized markers for an increased risk of thrombosis. We studied 48 patients who presented with cerebral or visual dysfunction associated with APLAb to help clarify the diagnostic, clinical, laboratory, radiologic, and pathologic features in these patients. Most patients presented with transient cerebral ischemia or cerebral infarction. Recurrent and stereotypic events were frequent. Visual disturbances resulted from amaurosis fugax, retinal arterial or venous occlusion, occipital ischemia, diplopia, and migraine-like disturbances. Three patients presented with severe atypical classic migraine. Recurrent infarcts of brain and eye were significantly associated with the presence of cigarette smoking, hyperlipidemia, and a positive antinuclear antibody. During 44.4 patient-years of prospective follow-up, the combined stroke and systemic thrombotic event rate was 0.27 events per patient-year and was 0.54 events per patient-year if TIA and death were included. Forty (83%) of the patients did not have systemic lupus erythematosus (SLE). Thrombocytopenia was present in 15 (31%) and a false-positive VDRL in 11 (23%) of the patients. Cerebral angiography was normal or revealed large-vessel occlusion or stenosis without changes suggestive of vasculitis. Patients with only transient dysfunction generally had normal radiologic studies, including angiography. Organs and arterial vessels studied pathologically revealed thrombotic occlusive disease without vasculitis. APLAb are strongly associated with an immune-mediated thrombotic tendency, generally in the absence of SLE. Other stroke risk factors may add to the risk of recurrent ischemic events in patients with APLAb.
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PMID:Cerebrovascular and neurologic disease associated with antiphospholipid antibodies: 48 cases. 238 25

The lupus anticoagulant is an acquired serum immunoglobulin that prolongs several coagulation parameters, most notably the partial thromboplastin time (PTT). Most commonly, this condition is found in association with systemic lupus erythematosus (SLE) but may be seen with other collagen-vascular diseases and in otherwise healthy individuals. Despite the laboratory tests suggesting impaired coagulation, clinically the lupus anticoagulant has been associated with thrombosis. The authors present five patients with the lupus anticoagulant who came to medical attention because of branch retinal artery occlusion, ischemic optic neuropathy, transient visual loss, transient diplopia, or vertebrobasilar insufficiency. Eleven previously reported patients with the lupus anticoagulant and disturbed vision are also reviewed with additional findings of retinal venous occlusive disease and homonymous visual field loss. The relationship of these findings to retinopathy in SLE is discussed. Patients with the lupus anticoagulant (with or without SLE) may develop disturbances in vision due to thrombosis from a hypercoagulable state. We recommended obtaining a PTT, VDRL, and the more sensitive anticardiolipin antibody in patients with unexplained visual symptoms.
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PMID:Visual symptoms associated with the presence of a lupus anticoagulant. 314 Jan 58

Phenobarbital (phenobarbitone) and phenytoin are the most useful anticonvulsants in neonates because adverse effects are most readily reversed when these drugs are used. Most anticonvulsants are very rarely associated with haematological adverse effects. Platelet function is particularly vulnerable to valproic acid (sodium valproate) therapy. Barbiturates and phenytoin can precipitate metabolic bone disease. Although very infrequent, lymphadenopathy is most common with phenytoin, and lupus-like illnesses with ethosuximide. Valproic acid may precipitate underlying metabolic disorders. Nephrolithiasis can occur with topiramate. Liver disease is most likely with felbamate or valproic acid, but can occur with other anticonvulsants. Valproic acid and ethosuximide are the main precipitants of gastrointestinal symptomatology; while valproic acid and vigabatrin are frequently associated with excessive bodyweight gain. Rash is most likely to occur with barbiturates, but there is a high risk of this adverse effect if large doses of lamotrigine are given with valproic acid. Adverse cosmetic effects are most likely with phenytoin, but valproic acid may cause alopecia. All anticonvulsants may cause unwanted neurological effects: when they occur, diplopia is usually precipitated by carbamazepine; tremor by valproic acid; and other motor disturbances are probably most common with phenytoin. Most anticonvulsants can cause drowsiness. Phenobarbital leads anticonvulsants as a cause of behavioural difficulties. Effects of anticonvulsants on cognitive function are difficult to assess, but subtle changes have been reported for all anticonvulsants in use up to the 1980s. Compared with other anticonvulsant drugs, phenytoin and felbamate are more often discontinued as a result of unwanted effects.
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PMID:A comparative review of the adverse effects of anticonvulsants in children with epilepsy. 896 93

Idiopathic intracranial hypertension is a disorder of intracerebral pressure regulation and patients run the risk of permanent visual loss. Intracranial hypertension (IH) has been reported rarely in systemic lupus erythematosus (SLE). We reviewed the medical records of 127 patients with lupus nephritis (LN) who were followed up from 1987 to 1996 in our unit. There were six patients with IH which gave a disease prevalence of 4.7% in those with LN. All were females giving a disease prevalence of 5.2% for that sex, a high rate of occurrence of IH in patients with LN. Their age ranged from 22 to 34 y (27.8 +/- 3.6 y). Headache, vomiting and diplopia were the common presenting symptoms and had started 7.3 +/- 4.4 weeks prior to the diagnosis of IH. The cerebrospinal (CSF) opening pressure (413.3 +/- 77.0 mmH2O) was raised in all cases. Biochemical and cytological analyses of CSF were normal. The only abnormal radiological finding was partially empty sella in one patient on magnetic resonance imaging (MRI) (performed in three patients) or computed tomography (CT) (performed in all patients). All patients had serological evidences of active lupus disease at the time of diagnosis of IH. The renal histology was WHO type IV in four cases and III and V in one each indicating severe renal involvement. Laboratory evidences of procoagulant activity were found in the form of positive anticardiolipin antibody (aCL) in two patients, lupus anticoagulant (LA) in two and an otherwise unexplained isolated prolongation of activated partial thromboplastin time (APTT) in the other two. Clinically, one or more episodes of symptomatic venous or arterial thrombosis had occurred in all subjects. In addition to symptomatic measures, all subjects were treated with prednisolone, azathioprine, cyclophosphamide and plasmapheresis according to the protocol of our unit. One patient who did not receive plasmapheresis and cyclophosphamide had a relapse while all others recovered completely. None received anticoagulant therapy. Young females with serologically active lupus, severe forms of renal lesions, past history of venous or arterial thrombosis and laboratory evidences of procoagulant activity, appear to be at increased risk of IH. Thrombotic occlusion of the cerebral arteriolar or venous vascular bed eventually affecting the arachnoid villi and impeding CSF absorption is favoured compared to cerebral venous or sinus thrombosis as the pathogenic mechanism. Combined immunosuppression and plasmapheresis appeared to be beneficial in short and long term follow-up. We propose that patients with SLE and IH have definable risk and pathogenetic factors and are no more to be considered 'idiopathic'. The conditions calls for aggressive intervention which leads to an excellent outcome.
Lupus 1997
PMID:Treatable intracranial hypertension in patients with lupus nephritis. 930 63

We report a 40-year-old Japanese woman with antiphospholipid antibody syndrome (APS) associated with myasthenia gravis (MG). She had a history of miscarriage at the age of 27 followed by pulmonary embolism 3 weeks later. At the age of 40, she developed diplopia, bilateral ptosis and easy fatigability. Serum anti-acetylcholine receptor antibody and tensilon test were positive. She was diagnosed as having MG. The laboratory test revealed mild thrombocytopenia, prolonged activated partial thromboplastin time (aPTT) and positive findings for both beta 2-glycoprotein I-dependent anticardiolipin antibody and lupus anticoagulant. She fulfilled the diagnostic criteria of APS, but did not the criteria proposed by American Rheumatism Association for SLE. An extended total thymectomy was performed after administration of oral prednisolone and low-dose aspirin. This is a patient who had APS associated with MGs: both are known to result from autoimmune abnormality. The clinical and laboratory manifestations of APS were ameliorated after removal of the thymus, suggesting that thymectomy alleviates APS symptoms.
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PMID:[A case of antiphospholipid syndrome associated with myasthenia gravis]. 939 64

A 35-year-old woman was admitted to our hospital with complaints of a two-year history of recurrent, daily episodes of transient ischemic attacks; the symptoms consisted of scotoma of her left eye, vertical diplopia, and paresthesia of her right arm. The presence of lupus anticoagulants and anticardiolipin antibodies led to the diagnosis of antiphospholipid syndrome (APS). After thrombotest values had decreased to 30% (international normalized ratio: 1.5) with warfarin, her symptoms did not recur. This suggests that anticoagulant therapy is effective for the prevention of recurrence of ischemic events complicated by primary APS, even when they occur repeatedly.
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PMID:Primary antiphospholipid syndrome with recurrent transient ischemic attacks: report of a case and its successful treatment. 974 60

We report a 56-year-old man with external ophthalmoplegia and ataxic gait following a diarrhea, being diagnosed atypical Miller Fisher syndrome (FS). On admission, he had severe diplopia and bilateral external ophthalmoplegia were observed. The deep tendon reflexes were decreased on the right upper extremity. He could not walk straight and his tandem gait was impaired. Serum IgG anticardiolipin antibody (aCL) and APTT-lupus anticoagulant (LA) were found to be increased. The serum of the patient had low titer of anti-GQ 1 b and anti-GM 1 antibodies. After the first immunoadsorption therapy, his ophthalmoplegia was improved moderately, but peripheral facial palsy appeared. He was treated with immunoadsorption again, then all neurologic symptoms improved and a follow-up study revealed normalized aCL and LA titers. There have been no previous reports of FS associated with antiphospholipid antibody. The low titer of serum anti-GQ1b and anti-GM 1 antibodies in this patient suggests that the antiphospholipid antibodies, such as aCL and LA, may be linked to the pathogenesis of FS.
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PMID:[A case of atypical Miller Fisher syndrome associated with antiphospholipid antibodies]. 1039 71

Intracranial complications are rare in Churg-Strauss syndrome (CSS). Cerebral infarctions are the most common intracranial presentation, usually after the clinical diagnosis of CSS had been established. We present a case of vertigo and Parinaud's syndrome as presentation of CSS in an asthmatic patient. Clinical examination revealed upward gaze limitation and bilateral midriasis. A cranial computed tomography scan showed a small round hypoattenuating lesion located in the right thalamic-mesencephalic region, which was later confirmed by magnetic resonance imaging. There was eosinophilia of more than 50%, and p-ANCA were positive. After steroid treatment was started, vertigo and diplopia resolved, and eosinophilia was reduced. After 24 months follow-up, the patient remains stable, with negative p-ANCA, taking 20 mg prednisone daily. Further magnetic resonance exams have shown findings that were similar to those of previous studies. This case shows how vertigo and transient diplopia may be the first symptoms of neurological complications in patients with CSS.
Lupus 2001
PMID:Vertigo and Parinaud's syndrome as presentation of Churg-Strauss syndrome. 1167 56

This case report describes a 24-year-old female who presented with sudden onset of painless diplopia and ptosis in her left eye. Examination identified an isolated incomplete pupil-sparing left oculomotor nerve palsy. Magnetic resonance imaging demonstrated focal hyperintensity in the left midbrain with infarction suggested by diffusion-weighted imaging. A diagnosis of primary antiphospholipid syndrome was made with the demonstration of a positive lupus anticoagulant. Other autoimmune markers were present on initial assessment, but did not fulfil diagnostic criteria for systemic lupus erythematosus. Anticoagulation with warfarin was commenced, with gradual resolution of neurological deficits. This case illustrates an unusual initial manifestation of primary antiphospholipid syndrome causing midbrain stroke in a young woman.
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PMID:Isolated fascicular oculomotor nerve palsy as the initial presentation of the antiphospholipid syndrome. 1260 87

We report a case of central nervous system (CNS) lupus showing peculiar findings on cranial magnetic resonance imaging (MRI) with remarkable improvement after corticosteriod therapy. The patient was a 28-year-old woman, admitted to our hospital with severe fever, general malaise, and facial edema on June 4, 2001. After admission, she was diagnosed with systemic lupus erythematosus (SLE). On June 6, she showed diplopia at a distance, and on June 10, she suddenly became unconscious and developed general convulsions. Cranial MRI showed asymmetrical, multifocal, high signal intensity lesions on T2-weighted image (T2-WI) and low signal intensity on T1-weighted image (T1-WI). These lesions were primarily present in the subcortical white matter, with some detected in the overlying cerebral cortex. Gadolinium (Gd)-DTPA enhanced T1-WI showed marked leptomeningeal enhancement overlying the lesions on T1-WI and T2-WI. Apparent diffusion coefficient image (ADCI) showed high signal intensity in the surrounding areas of the T1-WI and T2-WI lesions, and low signal intensity in the central areas of the lesions. Diffusion weighted image (DWI) showed high signal intensity in the central areas of the low signal intensity on ADCI. Cerebrospinal fluid (CSF) examination revealed albuminocytologic dissociation (cell counts of 2/microliter and protein level of 108 mg/dl). CSF IgG index was elevated to 1.152 (normal < 0.7) and interleukin-6 (IL-6) activity to 27.2 pg/ml (normal < 4.0). On June 10, Intravenous administration of high-dose methylprednisolone (1,000 mg/day for 3 days) was started to treat CNS lesions of SLE. Her CNS manifestations, CSF findings, and the lesions on the cranial MRI improved remarkably. This is the first case report describing the lesions on both ADCI and DWI in a case of CNS lupus. The findings of ADCI and DWI suggest that the lesions of high signal intensity on ADCI indicate interstitial edema caused by inflammatory microangiopathy, and the lesions of high signal intensity on DWI and low signal intensity on ADCI indicate cytotoxic edema caused by ischemic change resembling microinfarction. We speculate that in addition to usual T1-WI and T2-WI, performing ADCI and DWI is useful for understanding the pathogenesis of CNS lupus lesions, and may play a significant role in the prognosis.
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PMID:[A central nervous system lupus showing peculiar findings on cranial magnetic resonance imaging (MRI)]. 1458 67


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