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

Young patients with migraine are at increased risk for stroke, particularly patients with an aura of focal neurologic deficits. Other causes of ischemia are often identified in patients with migraine, including patent foramen ovale, lupus anticoagulant, cervical carotid dissection, arteriovenous malformation, and hyperactivity of the clotting system. Migrainous stroke is only diagnosed when all other possible causes of stroke have been eliminated and the patient has irreversibility of the usual aura, associated with an ischemic infarct in the appropriate brain territory. Prophylactic therapy of migraine with aura may be beneficial in preventing migrainous stroke.
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PMID:Stroke and migraine. 1858 14

Neuropsychiatric manifestations are commonly observed in systemic lupus erythematosus (SLE) patients; however, cerebellar involvement has rarely been reported. In the presence of acute cerebellar ataxia, etiologies related (focal edema and ischemia) and not related (infections, malignancy and paraneoplastic syndromes) to lupus have to be considered and they imply different treatment strategies. We report the clinical and radiological features of 3 SLE patients who presented with acute cerebellar ataxia. A review of the literature was performed by documenting cases of cerebellar ataxia in SLE and the importance of neuroimaging in the evaluation of these patients.
Lupus 2008 Dec
PMID:Cerebellar ataxia in systemic lupus erythematosus. 1902 81

Lupus mesenteric vasculitis (LMV) is a unique clinical entity found in patients who present with gastrointestinal manifestations of systemic lupus erythematosus, and is the main cause of acute abdominal pain in these patients. LMV usually presents as acute abdominal pain with sudden onset, severe intensity and diffuse localization. Other causes of abdominal pain, such as acute gastroenteritis, peptic ulcers, acute pancreatitis, peritonitis, and other reasons for abdominal surgery should be ruled out. Prompt and accurate diagnosis of LMV is critical to ensure implementation of appropriate immunosuppressive therapy and avoidance of unnecessary surgical intervention. The pathology of LMV comprises immune-complex deposition and complement activation, with subsequent submucosal edema, leukocytoclastic vasculitis and thrombus formation; most of these changes are confined to small mesenteric vessels. Abdominal CT is the most useful tool for diagnosing LMV, which is characterized by the presence of target signs, comb signs, and other associated findings. The presence of autoantibodies against phospholipids and endothelial cells might provide information about the likelihood of recurrence of LMV. Immediate, high-dose, intravenous steroid therapy can lead to a favorable outcome and prevent serious complications such as bowel ischemia, necrosis and perforation.
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PMID:Lupus mesenteric vasculitis can cause acute abdominal pain in patients with SLE. 1941 94

Central nervous system symptoms occur in a substantial portion of patients with systemic lupus erythematosus. However, coma is a rare presentation and is usually secondary to complications such as subarachnoid hemorrhage, seizure, or ischemia. Here, we present a 49-year-old woman with lupus erythematosus and a history of recurrent aseptic meningitis and mild subarachnoid hemorrhage who presented with altered mental status and lethargy that progressed rapidly over hours to the herniation syndrome of coma, extensor posturing, and unilateral pupillary dilation. Spinal fluid showed massive protein elevation (>1600), and head computed tomography revealed global cerebral edema. The clinical and radiologic findings rapidly reversed with intravenous corticosteroids and mannitol within 24 hours, and her mental status improved to baseline. Her course was complicated by 2 episodes of recurrent encephalopathy when corticosteroids were tapered; these resolved after resuming high dosing. Because of ongoing pancytopenia, chemotherapy immunosuppression was delayed, and instead she received intravenous immunoglobulin with improvement in the pancytopenia. She remained cognitively intact during subsequent corticosteroid tapering. Rapid development of coma in lupus patients may be due to a primary process of the disease impacting blood brain barrier integrity. Although rare, this potentially fatal complication may be reversible with acute corticosteroid administration.
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PMID:Transient life-threatening cerebral edema in a patient with systemic lupus erythematosus. 1945 59

Diplopia is frequently encountered in neurological practice and may occur as part of the clinical spectrum of 3rd cranial nerve palsy. Correct localization of the underlying problem is the first step in making an accurate diagnosis. Pathologies affecting the supranuclear structures, cranial nerves and nuclei, extraocular muscles and the neuromuscular junction may cause or simulate 3rd nerve palsy. We report a case of a patient with longstanding quiescent SLE who presented with sudden onset of diplopia and discuss possible aetiologies including ischemia, demyelination, neuromuscular conditions and medication-induced.
Lupus 2009 Aug
PMID:Third cranial nerve palsy or pseudo 3rd nerve palsy of myasthenia gravis? A challenging diagnosis in systemic lupus erythematosus. 1957 9

Systemic sclerosis (SSc) is an autoimmune systemic disease characterized by small vessel involvement that leads to tissue ischemia and fibroblast stimulation resulting in accumulation of collagen (fibrosis) in the skin and internal organs. Lipomembranous panniculitis is a peculiar type of fat necrosis and has been reported with clinical conditions, commonly with peripheral vascular diseases. We describe a case of a 43-year-old woman with SSc manifestations, who presented with black scaly skin plaques, associated with thickening of the subcutaneous fat tissue, on the lateral surface of her thighs, her calves, gluteal area and lower abdomen. Biopsy revealed lipomembranous panniculitis. Lipomembranous changes have been seen in connective tissue disorders such as lupus profundus, morphea, systemic sclerosis and panniculitis associated with dermatomyositis, but rarely in thighs, calves, gluteal area and lower abdomen.
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PMID:Panniculitis-an unusual cutaneous manifestation of systemic sclerosis. 1961 30

We report a patient with Down syndrome, under treatment with carbamazepine, levopromazine and clonazepam. After urinary infection he developed glans necrosis requiring excision of prepuce. Six hours post surgery he presented right-hand ischemia followed by arterial and venous thrombosis of the right thoracic extremity. Later, he progressed to a compartment syndrome and presented ischemia of toes. All the clinical manifestations developed over a week. Anticardiolipin (aCL) antibodies, lupus anticoagulant and perinuclear antineutrophil antibodies were positive. Anticoagulant and immunosuppressive treatment were initiated. Owing to the failure of both treatments, the patient underwent amputation of right hand and a toe. Histopathology revealed recent and old thrombosis of medium- and small-sized vessels without vasculitis. Diagnosis of catastrophic antiphospholipid syndrome (CAPS) was made. At present, the patient continues on oral anticoagulants, IgG aCL remains positive, and no further episodes of thromboses have been observed after 4 years of follow-up. To our knowledge, this is the first case of CAPS in a patient with Down syndrome.
Lupus 2009 Oct
PMID:Catastrophic antiphospholipid syndrome in a patient with Down syndrome. 1976 87

A 48-year-old woman presented with bilateral lower extremity critical limb ischemia. In addition to this, her work-up revealed multiple other thromboembolic insults including cerebral and visceral emboli. Initial laboratory findings were significant for an indeterminate platelet count, secondary to platelet clumping. After appropriate emergent surgical treatment including bilateral lower extremity embolectomy, the patient was empirically anticoagulated with a direct thrombin inhibitor. Further embolic work-up discovered bilateral renal and splenic infarctions as well as a large mobile mitral vegetation. Finally, an upper extremity duplex revealed left axillary, left subclavian, and right internal jugular acute deep vein thromboses. Mitral valve replacement was performed to remove the septic source. A series of hypercoagulability studies was done, and results were positive for lupus anticoagulants. Months after her recovery, the patient was tested and found to be positive for sticky platelet syndrome.
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PMID:Sticky platelet syndrome: an unusual presentation of arterial ischemia. 2036 2

The antiphospholipid syndrome (APS) is an acquired thrombophilic disorder characterized by the presence of autoantibodies to a variety of phospholipids and phospholipid-binding proteins. Clinical manifestations range from being asymptomatic to having imminently life-threatening events. Catastrophic antiphospholipid syndrome (CAPS) occurs in <1% of patients with APS and is defined by multiple small-vessel occlusions that lead to multiple-organ failure and is associated with high morbidity and mortality rates. Here we report the case of a 3-month-old boy with probable CAPS who presented to us with digital necrosis and pulmonary hemorrhage. In addition, a skin biopsy demonstrated multiple small-vessel thromboses without signs of vasculitis. Results of testing for autoantibodies were positive for anti-beta(2) glycoprotein I (anti-beta(2)-GPI) only. His treatment consisted of high-dose steroids, immunoglobulin therapy, exchange transfusion, cyclophosphamide, and rituximab as well as iloprost and bosentan as vasodilators for his ischemia; he showed an excellent clinical response. To the best of our knowledge, this is the youngest patient with probable CAPS, the first reported patient to test positive for anti-beta(2)-GPI antibodies and negative for anticardiolipin antibodies and lupus anticoagulant, and the second patient reported to be successfully treated with an immunomodulatory regimen including rituximab.
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PMID:Infant with probable catastrophic antiphospholipid syndrome successfully managed with rituximab. 2047 43

Maintenance of the foot at 90 degrees to the lower leg following posterior calf lengthening or to prevent an equinus contracture in situations in which splint, cast, or external fixation is deemed inappropriate is a challenge. The author presents an observational case series involving 9 extra-articular ankle stabilizations performed in 9 consecutive patients. Each patient underwent his or her index surgery followed by percutaneous placement of 2 smooth 2.8-mm or larger diameter Steinmann pins extra-articular to the ankle joint. There were 6 men and 3 women with a mean age of 56.1 years (range, 31-73 years). Five patients had diabetes with peripheral neuropathy, 2 had critical limb ischemia, 1 had alcohol-induced neuropathy, 1 had lupus, and 1 was an active smoker. Eight patients had posterior calf lengthening, and 1 had open metatarsal fractures with severe soft-tissue disruption with an inability to use splint immobilization. Three patients had a transmetatarsal amputation, 2 patients had Chopart amputations, 2 patients had forefoot plastic surgery reconstructive procedures, 1 had a complex Charcot reconstruction, and 1 had a splittibialis anterior tendon transfer. Extra-articular ankle stabilization fixation was retained for a mean of 5.5 weeks (range, 2-10 weeks). Mean follow-up was 12 months (range, 1-17 months). All extra-articular stabilization procedures were deemed successful. When properly performed, extra-articular stabilization to maintain the foot at 90 degrees to the lower leg represents a safe, simple, reliable, and minimally invasive technique useful in situations in which traditional splint or cast immobilization is not possible and when external fixation is deemed inappropriate.
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PMID:Extra-articular ankle stabilization: a case series. 2050 12


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