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

Sensory neuroophthalmic abnormalities due to cerebral lupus erythematosus, with involvement of visual pathways posterior to the optic chiasm, occurred in 12 patients with systemic lupus erhthematosus. Five underwent detailed evaluation because of an hallucination, 4 for visual loss, and 3 for both. Hallucinations were either unformed (for example, bright lights, straight lines) or highly formed (for example, faces), in which case they were invariably recognized by the patient as inappropriate. In no instance did they occur in association with delirium, confusion, or use of hallucinogenic drugs. Patients with loss of vision had scotomas, homonymous field defects, and cortical blindness. These features indicate disease in the posterior cerebral artery circulation, a localization often supported by ancillary neurologic findings, for example, vocal cord paralysis, diminished gag reflex. Thus, various visual dysfunctions may occur in systemic lupus erythematosus due to cerebral vasculitis. At times they may be the most prominent and disabling feature of the disorder.
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PMID:Cerebral disorders of vision in systemic lupus erythematosus. 114 49

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

The optic neuritis of systemic lupus erythematosus (S.L.E.) more frequently results in the persistence of a central scotoma or complete blindness after a single attack than demyelinating optic neuritis, although the initial clinical presentations may be identical. A significant number of patients, however, recover normal vision. Optic neuritis may be the presenting symptom of S.L.E. and as myelopathy may also occur in the course of the disease, confusion with multiple sclerosis may result, especially if there are no arthritic, cutaneous nor visceral manifestations. We report a case of lupus optic neuritis associated with anticardiolipin antibodies and a circulating lupus anticoagulant and suggest these may be a marker for vasculitic optic neuritis and play a role in its aetiology.
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PMID:Optic neuritis and myelopathy in systemic lupus erythematosus. 308 11

We describe a patient with systemic lupus erythematosis and renal involvement and who developed visual impairment in the absence of hypertension and anti-cardiolipin antibodies. This was attributed to occlusive vascular disease and choroidal infarction in the context of active lupus. Complete and irreversible blindness developed rapidly despite treatment and the patient died of cerebral haemorrhage 2 months after discharge.
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PMID:Irreversible blindness in systemic lupus erythematosis. 840 6

This paper summarizes results from genetic studies of Nordic carnivore populations bred in captivity. The conservation genetic implications of those results for the management of wild populations of the same species are discussed. Inbreeding depression has been documented in the brown bear (Ursus arctos), wolf (Canis lupus), and lynx (Lynx lynx) populations held in Nordic zoos. The characters negatively affected by inbreeding include litter size (brown bear and wolf), longevity (lynx and wolf), female reproduction, and weight (wolf). In addition, hereditary defects caused by single autosomal alleles occur in the wolf and brown bear populations. These deleterious alleles cause blindness (wolf) and albinism (brown bear) in the homozygous state. The amount of inbreeding depression observed in Nordic carnivores are similar to that documented in other species. The captive populations have the same genetic background as the current wild ones and inbreeding depression is therefore a potential threat to wild carnivore populations in Sweden. This threat is presently not being adequately recognized in the management of these species. Frequently occurring misunderstandings regarding the kind of conclusions that can be drawn from the presented genetic observations are also discussed.
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PMID:Conservation genetics of Nordic carnivores: lessons from zoos. 1050 37

Autoimmune bullous diseases (AIBD) are characterized by autoantibodies targeted against adhesion molecules, impairing their formation. According to localization criteria, pemphigus (intraepidermal blister and desmosomal involvement) and pemphigoid (subepidermal blister and dermoepidermal junction involvement) can be distinguished. In two-thirds of the cases, pemphigus vulgaris begins with oral lesions (mainly the buccal mucosa and palate, rarely the gingiva). Skin lesions are usual. Excepting paraneoplastic pemphigus (a recently individualized entity), oral lesions are uncommon in other types of pemphigus. Cicatricial pemphigoid mainly involves oral mucosa, frequently other mucous membranes, and rarely the skin. Gingival involvement is frequent. In case of desquamative gingivitis, the clip sign gives the diagnosis of cicatricial pemphigoid. Ocular involvement is frequent and causes blindness. Epidermolysis bullosa acquisita and IgA linear dermatosis are rare. Bullous pemphigoid and bullous lupus rarely involve the oral mucosa. Diagnosis of AIBD requires a biopsy within the mucosal membrane lesion for pathology examination and another biopsy in a lesion-free area for direct immunofluorescence detection of antibody fixation. Immunoelectron microscopy or immunoblast transfer may be needed for positive diagnosis. Corticosteroids are used to treat pemphigus and dapsone is used for cicatricial pemphigoid. Immunosuppressive therapy is rarely needed.
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PMID:[Bullous autoimmune diseases of the oral mucosa]. 1060 15

An 87-year-old woman with antiphospholipid syndrome accompanied by cortical blindness and thalamic syndrome resulting from infarction of the posterior cerebral artery is reported. She was hospitalized because of laceration of the head. Two months later, she complained of loss of visual acuity, sharp pain and numbness involving the left half of the body except her face. New right posterior lobe infarction and the existence of old left infarctions were confirmed by serial CT scans. Helical CT scan revealed embolization of the posterior cerebral artery with atherosclerotic stenosis. Serological examination showed biologically false-positive and positive findings for lupus anticoagulant. She was treated with warfarin potassium and clonazepam.
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PMID:Antiphospholipid syndrome with cortical blindness resulting from infarction around the posterior cerebral artery in an elderly woman. 1088 6

Diabetic retinopathy (DR) is a major cause of blindness in the working population of the Western world and there is no doubt that its prevalence is strongly related to the duration of diabetes and the glycemic control. However, although intensive diabetes management, with the goal of achieving near-normal glycemia, has been shown to prevent and/or delay the onset of DR, there is now ample of evidence that the development of this microangiopathy is a multifactorial process in which genetic, metabolic, haemostatic and growth factors play an important role. Moreover, given the suggestions that immunological mechanisms might have a role in the pathogenesis of diabetic microangiopathy via immune complex deposition, it has been hypothesized that antiphospholipid antibodies (A-Ab) directed against endothelial antigens could be responsible for initiating vascular injury. In particular, not only A-Ab production was found to be increased in patients with overt nephropathy or macroangiopathy but also Lupus Anticoagulant positivity, representing an intersection point between immune and haemostatic alterations, has been highlighted as a potential and additional risk factor in the pathogenesis of microangiopathy in type 1 diabetics. Moreover, given the high levels of activated protein C, endothelin-1 and thrombo-modulin that have been observed in normo-albuminuric and uncomplicated patients, it has been concluded that the vascular endothelium shifts pathologically from an antithrombotic to a prothrombotic state even in the early phases of the disease. This condition was found to be more pronounced in subjects with anticardiolipin positivity and/or high circulating immune complexes concentrations, since they possess the ability not only to induce platelet activation and aggregation but also to activate the complement system via the classical pathway. Therefore, a potential synergism between generation of autoantibodies, haemostatic alterations and endothelial stress has been suggested, a stimulating hypothesis that needs further studies to be clarified in its complexity.
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PMID:Are phospholipid-binding antibodies implicated in the pathogenesis of diabetic microangiopathy? 1523 81

Although the eye itself is regarded an 'immune-privileged' organ, systemic lupus erythematosus (SLE) can affect every ocular structure, leading, if left untreated, to significant visual loss or even blindness. Since ocular inflammation in SLE can antedate the diagnosis of the systemic disease and cause significant morbidity, prompt diagnosis and treatment of the underlying systemic autoimmune disease is imperative.
Lupus 2006
PMID:Ocular manifestations of systemic lupus erythematosus: a clinical review. 2095 63

Posterior reversible encephalopathy syndrome (PRES) associates various neurological manifestations (headaches, seizures, altered mental status, cortical blindness, focal neurological deficits, vomiting) and transient changes on neuroimaging consistent with cerebral edema. Posterior reversible encephalopathy syndrome mainly occurs in the setting of hypertension, eclampsia, renal failure and/or use of immunosuppressive drugs. We report four cases of PRES complicating systemic lupus erythematosus (SLE). In all our cases, renal involvement and hypertension were present. Neurological symptoms were typical. Magnetic resonance imaging showed posterior cerebral edema and in one case hemorrhagic complication. With symptomatic treatment and immunosuppressor withdrawal when they were previously used, symptoms fully resolved within 15 days in all cases, but one who had only partial regression related to cerebral hemorrhage. Including our cases, we reviewed a total of 46 patients with SLE and PRES. Their clinical and radiological presentation was not specific. The peculiar role of SLE itself in the occurrence of PRES was not clear, since hypertension (95%), renal involvement (91%), recent onset of immunosuppressive drugs (54%) and/or recent treatment with high intravenous dose of steroids (43%) were often present. The hypertension and other worsening factors should be treated. Finally, the evolution of this clinical and radiological spectacular syndrome is generally rapidly favorable.
Lupus 2008 Feb
PMID:Posterior reversible encephalopathy syndrome during systemic lupus erythematosus: four new cases and review of the literature. 1825 Jan 39


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