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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Thrombosis, thrombocytopenia, recurrent fetal loss and a variety of non-thrombotic neurological disorders have all been associated with antiphospholipid antibodies (aPL). Cerebral ischemia associated with aPL is the most common arterial thrombotic manifestation. Depression, cognitive dysfunction, depression and psychosis have all been associated with aPL. The presumed pathophysiologic mechanism underlying these manifestations is thought to be a result of cerebral ischemia in some, but not all cases. Seizures, chorea and transverse myelitis all appear to be associated with aPL. An interaction between aPL and central nervous system cellular elements rather than aPL-associated thrombosis seems to be a more plausible mechanism for these clinical manifestations. Migraine on the other hand, does not appear to be associated with aPL in either lupus or non-lupus populations. Neuroimaging studies show an increased frequency of brain abnormalities in patients with aPL, but none appear to be specific. The best treatment strategy for preventing neurological manifestations of aPL is not fully defined. For thrombotic manifestations, both antiplatelet and anticoagulant therapies have been suggested. In some patients, immunosuppressant therapy has been used. For non-thrombotic manifestations, some combination of immunosuppressant therapy and symptomatic treatment may be warranted.
Lupus 1998
PMID:Neurological manifestations of antiphospholipid antibody syndrome. 981 77

Transverse myelitis is a rare and serious complication of systemic lupus erythematosus (SLE). Magnetic resonance imaging is the investigation of choice for diagnosis and followup. This typically shows T1 and T2 signal prolongation, cord widening, and contrast enhancement over several spinal segments. We describe a 21-year-old woman with SLE who developed very extensive SLE related transverse myelitis with longitudinal involvement of the spinal cord from C3 to T2 and from T7 to the conus medullaris. Clinically, this was manifest as leg weakness, bladder dysfunction, severe low back pain, and patchy lower limb sensory loss. She responded to treatment with pulse cyclophosphamide and high dose corticosteroids with complete recovery in 3 months. To our knowledge, this is the first case report of such an extensive "longitudinal" myelitis.
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PMID:Longitudinal involvement of the spinal cord in a patient with lupus related transverse myelitis. 997 84

Optical neuromyelitis or Devic's syndrome is a very uncommon neurological manifestation of systemic lupus erythematosus. It is also associated with antiphospholipid antibodies, limited responsiveness to glucocorticoid treatment and a poor prognosis. We report the case of a female systemic lupus erythematosus patient who developed recurrent flares of optical neuritis and transverse myelitis. These flares consistently responded to glucocorticoid therapy. Despite the absence of overt anticardiolipin antibodies in the course of the disease, long-term anticoagulant therapy has been introduced with positive results. Treatments are usually of limited efficacy in Devic's syndrome. In our patient, however, aggressive glucocorticoid treatment resulted in prolonged survival.
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PMID:Systemic lupus erythematosus with optical neuromyelitis (Devic's syndrome). A case with a 35-year follow-up. 1008 70

Magnetic resonance imaging has proven to be useful in the diagnosis of spinal cord pathology. Little has been written in the literature concerning magnetic resonance imaging of acute transverse myelitis. A case of magnetic resonance imaging of acute transverse myelitis in a young man with known systemic lupus erythematosus is presented along with a review of the radiographic approach to transverse myelitis.
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PMID:MR imaging of acute transverse myelitis. 1022 33

Neuropsychiatric forms of systemic lupus erythematosus (SLE) vary, most commonly consisting of seizures, psychiatric disturbances, or focal central nervous deficits. This is a new case of neuromyelitis optica or Devic's syndrome during the course of SLE. Few reports of this association exist in the literature. Our objective is to report this unique case of Devic's neuromyelitis optica during pregnancy in a patient with systemic lupus erythematosus. A 28-year-old woman had been diagnosed as having SLE with cutaneous and articular involvement in 1987 when she was 17 years old. She was treated with a synthetic antimalarial agent associated with corticosteroids. In 1994, during the fourth month of pregnancy, she had signs of transverse myelitis with a sensory level at T6 associated with an optic neuropathy suggesting a Devic's syndrome. The patient was managed by plasmapheresis sessions and intravenous corticosteroids. Transverse myelitis recurred postpartum and three years later at the same thoracic level. Management by bolus administration of a steroid and cyclophosphamide resulted in remission again. There have only been around a dozen reports in the literature of patients who had both Devic's neuromyelitis optica and SLE. Magnetic resonance imaging is contributive to diagnosis and therapeutic follow-up, showing spinal cord lesions with increased intensity on T2-weighted sequences. Although the clinical course of the present patient has been favourable so far, the prognosis of this neurologic disease is generally considered to be poor with elevated mortality.
Lupus 1999
PMID:Devic's neuromyelitis optica during pregnancy in a patient with systemic lupus erythematosus. 1034 19

Optic neuropathy is a well-known ocular manifestation occurring in patients with systemic lupus erythematosus (SLE), and it remains one of the major causes of blindness in these patients. We report data from six SLE patients with optic neuropathy, one of whom was considered to have antiphospholipid syndrome (APS). This patient had monolateral optic neuropathy, whereas the other five SLE patients had bilateral optic nerve disease. We believe that the monolateral occurrence of optic neuropathy in our patient can be considered as a 'focal' neurological disease due to a thrombotic event involving the ciliary vasculature. Conversely, bilateral optic nerve damage in SLE could be considered to be a 'general' neurological disease due to different immunological mechanisms, such as vasculitis. Additionally, the literature on SLE patients affected by optic neuropathy is reviewed to evaluate the major clinical features, particularly neurological features. In reviewing the literature, it appears that bilateral optic neuropathy in SLE occurs more frequently than monolateral optic neuropathy, and the main neurological manifestation seen in these patients is transverse myelitis, particularly in SLE patients with bilateral optic nerve disease. Finally, we propose a clinico-ophthalmological spectrum of APS and outline the ocular clinical manifestations that can be considered as diagnostic for the syndrome.
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PMID:Optic neuropathy in systemic lupus erythematosus and antiphospholipid syndrome (APS): clinical features, pathogenesis, review of the literature and proposed ophthalmological criteria for APS diagnosis. 1035 17

Acute transverse myelitis as complication of systemic lupus erythematosus is a known and well-characterized although uncommon clinical entity. We report here four cases of lupic myelitis collected at our hospital in the last few years and review the available literature of the last ten years, approximately the time when NMR became generally available. The clinical picture can be very variable and therefore, when facing a picture of acute myelitis, lupus should be included in the differential diagnosis; biochemistry evaluating the lupus "activity" is of poor diagnostic value, nuclear magnetic resonance is not conclusive for the etiologic diagnosis of myelitis and its prognosis has improved with therapy including pulses of steroid and immunosuppressant agents.
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PMID:[Acute transverse myelitis in systemic lupus erythematosus]. 1036 94

In a cross-sectional study of 24 Oriental children with systemic lupus erythematosus (SLE) with a mean age of 11.25 years, 75% were found to have clinical and neurophysiological evidence of cerebral lupus. Seizures were the most common manifestation affecting 11 (61%) of the cases, followed by psychosis in five (27.7%), encephalopathy in five (27.7%), headaches in five (27.7%), personality changes in four (22.2%), stroke in three (16.6%), movement disorders in three (16.6%) and myelitis in one child (5.5%). Four children had cerebral lupus as the presenting manifestation of SLE. Twenty-one children had an electroencephalogram (EEG) of which 11 were normal. Abnormalities detected in the rest included focal sharps, slowing of background and electrodecremental changes. There was a poor correlation of EEG with the clinical presentation. Sixteen children with cerebral lupus had a computed tomogram (CT) of which three were normal. The commonest abnormality was cerebral atrophy with or without infarcts. Only four of the cases had lupus anticoagulant but compliment was reduced in 13. Sixteen of the cases also had renal involvement. Treatment was generally with steroids with only two patients receiving cyclophosphamide for cerebral relapse. Eight children (44%) made a full recovery. Learning disability was the most frequent sequelae affecting one-third of children seen at a 1-year follow up. Four (22%) had epilepsy, two (11%) had motor deficits and one child had optic atrophy. One child died of cerebral haemorrhage during a hypertensive crisis.
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PMID:Childhood cerebral lupus in an Oriental population. 1039 44

Transverse myelitis is a rare but serious complication of systemic lupus erythematosus (SLE). We describe the youngest patient with transverse myelitis ever recorded in SLE. Clinical improvement was observed after prednisone and cyclophosphamide therapy. The English language literature from 1966 to the present on the subject are reviewed.
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PMID:Spinal cord involvement in pediatric systemic lupus erythematosus: case report and literature review. 1046 67

A 69-year-old woman developed paraplegia and hypesthesia on upper extremities and below T4 level. Examination of cerebrospinal fluid showed increased protein levels and pleocytosis. MRI of the cervical spinal cord revealed syrinx formation from C3 to upper thoracic cord. A diagnosis of acute transverse myelitis was made. A high dose of corticosteroid including pulse therapy did not improve her symptoms and signs of myelopathy, but the syrinx could not be found thereafter. One year later, she developed severe visual loss due to bilateral optic neuritis which was improved spontaneously. The clinical course and MRI findings were similar to those of the optic-spinal form of multiple sclerosis (MS). The presence of anticardiolipin antibodies, lupus anticoagulant and perinuclear anti-neutrophil cytoplasmic antibodies, however, strongly suggested that vasculitic and/or ischemic mechanisms induced by these autoantibodies might play a role on the development of the disease. We conclude that our case should be distinguished from MS.
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PMID:[A case of acute transverse myelopathy and bilateral optic neuritis associated with anticardiolipin antibodies, lupus anticoagulant and perinuclear antineutrophil cytoplasmic antibodies]. 1061 63


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