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

Transverse myelopathy is an uncommon complication of systemic lupus erythematosus (SLE). Three patients with SLE are reported who developed transverse myelopathy, including the neuropathological findings in one patient on whom necropsy was performed. Paraparesis was present in all three cases, but definite sensory changes were present in only one patient. In two patients, the CSF findings were remarkable for elevated protein and depressed glucose concentrations. Microscopic examination of the brain demonstrated small, scattered foci of recent necrosis consistent with microinfarctions. Striking abnormalities were found in the spinal cord at all levels, including multiple foci of vacuolar spongy degeneration in the peripheral white matter, as well as ballooning of myelin sheaths, swollen axons, myelin pallor, and loss of glial nuclei. The pathological findings in previously reported cases of SLE-related transverse myelopathy are reviewed, and the possible pathogenesis of the findings in our case are discussed.
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PMID:Lupus-related myelopathy: report of three cases and review of the literature. 140 76

A 52-year-old woman who developed acute transverse myelopathy following systemic lupus erythematosus (SLE) was reported. At the age of 46, she was diagnosed as having atypical psychosis. Neurological examination revealed mild depressive state, paraparesis, diffuse hyperreflexia, hypesthesia below the breasts, and urinary disturbance. Gait was slightly ataxic and Romberg sign was positive. Laboratory study disclosed lymphocytopenia, positive antinuclear antigen, false positive Venereal Disease Research Laboratories flocculation test and prolonged activated partial thromboplastin time. IgG anticardiolipin antibody (aCLA) was positive, whereas IgM aCLA was negative. Cerebrospinal fluid was normal except the elevation of %IgG. Nerve conduction studies were normal and no abnormality was detected in the brain and spinal cord by MRI and CT. We treated her by two series of steroid pulse therapy, which resulted in marked improvement of symptoms and disappearance of aCLA. Before and after the pulse therapy, symptoms were fluctuated in parallel with the levels of aCLA. These findings suggest the relation of aCLA to the transverse myelopathy in SLE. This is the first case report of a good prognosis of myelopathy in a SLE patient who was treated by steroid pulse therapy with the aim of disappearance of aCLA.
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PMID:[Transverse myelopathy in a patient with systemic lupus erythematosus associated with positive anticardiolipin antibody--a case report]. 142 46

Postmortem examination of 21 patients showed a vacuolar myelopathy resembling that associated with the acquired immunodeficiency syndrome. Underlying diseases included six cases of leukemia or lymphoma, five of carcinoma, three of systemic lupus erythematosus, two of chronic lung disease, and one each of cadaveric renal transplant, cirrhosis, diabetes, hemophagocytic syndrome, and viral encephalitis. Fourteen patients were on long-term steroid therapy and 10 of these also had immunosuppressive chemotherapy. No patient had the acquired immunodeficiency syndrome, although one received blood transfusions in 1978. Signs and symptoms consistent with myelopathy included paraparesis in seven patients, ataxia in one, and bilateral extensor plantar reflexes in one. Microscopic examination showed vacuolation in spinal cord white matter primarily located in posterior and lateral columns. Lipid-laden macrophages and axonal changes were proportional to the severity of the vacuolation, which was severe in five patients, moderate in 10, and mild in six. Eight patients had coexistent viral diseases elsewhere in the central nervous system, but viral-associated antigens or genomic material was not found in regions of vacuolated spinal cord white matter. Although the etiology of these myelopathies is unknown, their association with immune suppression and coexistent viral infection of the central nervous system suggests that an opportunistic viral infection may be important.
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PMID:Idiopathic myelopathies with white matter vacuolation in non-acquired immunodeficiency syndrome patients. 186 65

Systemic lupus erythematosus (SLE) affects the nervous system in 75% of cases (1). A female with several neurological manifestations in the case history presented with severe headache, psychiatric disturbances, and increasing paraparesis. She was found to have bilateral subdural hematomas, and after evacuation her neuropsychiatric symptoms, including headache, disappeared. It is speculated that the reported low incidence of subdural hematomas in SLE may be more apparent than real. On the basis of our case, we recommend repeated neuroradiological investigations to uncover this important, treatable and otherwise potentially fatal cause of headache.
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PMID:Subdural hematoma presenting as headache in systemic lupus erythematosus. 231 50

Myelopathy is a well recognised but infrequent neurological manifestation of systemic lupus erythematosus (SLE). The case of a 27 year old woman with SLE of seven years' duration who developed a spastic paraparesis during her second pregnancy is reported. Magnetic resonance imaging did not show any intrinsic abnormality of the spinal cord. Anticardiolipin antibody was weakly positive and C4 was low. The patient responded dramatically to steroids.
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PMID:Transverse myelitis occurring during pregnancy in a patient with systemic lupus erythematosus. 293 Feb 67

Paired serum and cerebrospinal fluid specimens from 19 patients with SLE and central nervous system dysfunction were studied with respect to cerebrospinal fluid IgG index (a measure of intrathecal IgG synthesis), isoelectric focusing using immunoperoxidase staining techniques to detect oligoclonal IgG, and determination of the cerebrospinal fluid/serum albumin quotient (Q albumin) as a measure of blood-brain barrier integrity. Twenty-five patients without neurologic disease and 70 patients with a variety of non-SLE neurologic disorders were also studied for comparison. Of most interest was the observation that 42 percent of the patients with SLE had cerebrospinal fluid oligoclonal IgG, usually in association with elevation of the cerebrospinal fluid IgG index. In addition, two of the cerebrospinal fluid specimens that exhibited oligoclonal IgG also had increased titers of alpha-interferon. Q albumin was normal (under 9.0) in 12 of 13 patients with SLE, who had seizure, psychosis, or cranial neuropathy as principal central nervous system manifestations (mean +/- SD = 5.3 +/- 2.4), but was significantly elevated (mean +/- SD = 27.4 +/- 18.8, p less than 0.001) in five of six patients with diffuse, major central nervous system injury, for example, encephalopathy with coma, transverse myelopathy, paraparesis. Blood-brain barrier impairment was not correlated either with presence of circulating immune complexes or with other clinical or serologic evidence for extra-central nervous system disease activity. Taken together, the data suggest that, within the limitations of the techniques used, impairment of the blood-brain barrier in SLE may be secondary to the central nervous system lesion, rather than a result of systemic immune complex injury. In addition, substantial evidence is provided for an ongoing humoral immune response within the central nervous system in this disorder, which, in certain patients, may be associated with the production of intrathecal alpha-interferon.
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PMID:Intrathecal IgG synthesis and blood-brain barrier impairment in patients with systemic lupus erythematosus and central nervous system dysfunction. 683 7

Transverse myelitis has been cited as a rare and unusual complication of systemic lupus erythematosus (SLE). A review of the literature reveals only 10 cases of transverse myelitis as the initial presentation of SLE, and only one with reported benefits from antimalarial therapy. The case of a 30-year-old woman is reviewed. She presented to the emergency room with complaints of hypogastric and low back pain. The ensuing course was one of frank urinary retention and rapidly progressing quadriparesis. Magnetic resonance imaging of the spine revealed marked edema of the cervical and thoracic spine. A diagnosis of SLE was based on positive antinuclear antibodies and leukopenia. The patient was treated with high dose methylprednisolone, plasmapheresis and pulse cyclophosphamide for 3 months. Subsequently, treatment was begun with hydroxychloroquine, and significant improvement in her neurologic and functional status was achieved after 1 month of therapy. Ten months after her onset of symptoms, the patient suffered an acute exacerbation of paraparesis and urinary retention. Again, she improved clinically after high dose methylprednisolone and pulse cyclophosphamide for 1 month. Hydroxychloroquine was continued throughout the duration of therapy.
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PMID:Transverse myelitis complicating systemic lupus erythematosus: treatment including hydroxychloroquine. Case report. 851 79

The objective of the study was to examine the clinical features of lupus patients who present with transverse myelopathy (TM) and ascertain functional outcomes when treated early with high dose corticosteroids and/or cyclophosphamide. Case records of nine patients who developed a total of 14 episodes of TM were retrospectively studied. All the patients were female and their ages ranged from 21 to 59 years. Nine episodes of paraparesis, three of tetraparesis, one of numbness and one of neurogenic bladder were reported early in the diagnosis of SLE (median of two years). Neurogenic bowel and bladder and presence of ANA and ds-DNA were invariable. Urodynamics assessment in six patients showed abnormal detrusor behavior in all. CT scans and myelograms were uninformative and CSF studies were normal. ESR and complement levels were insensitive as markers of disease activity. The treatment regimens included pulses of methylprednisolone and/or cyclophosphamide followed by prednisolone and high dose prednisolone from onset. The functional outcomes were uniformly good-with independent ambulation in all except three (who needed assistive devices) and improvement of motor scores. Acute hospital stays were short (range of three to 45 days) whilst only two were referred for inpatient rehabilitation. Bladder abnormalities persisted despite motor recovery and would require long-term review.
Lupus 1996 Aug
PMID:Transverse myelopathy in SLE: clinical features and functional outcomes. 886 1

A 22 year old woman with recently diagnosed systemic lupus erythematosus presented with subacute progressive areflexic paraparesis, electrophysiologically identified as a pure axonal polyradiculopathy. Sural nerve biopsy disclosed necrotising vasculitis. A striking radiological feature was marked enhancement of the cauda equina with gadolinium.
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PMID:Vasculitic polyradiculopathy in systemic lupus erythematosus. 1020 82

Myelopathy is a rare central nervous system (CNS) complication associated with systemic lupus erythematosus (SLE). Acute transverse myelitis (ATM) is the most frequent form of SLE-related myelopathy. Magnetic resonance imaging (MRI) typically shows increased signal intensity in T2-weighted images and cord swelling. In the present paper, we describe six cases of SLE-related myelopathy with multiple increased signals in the T2-weighted images involving continuous levels of the cervical and thoracic spinal cord, a distinctive feature recently named 'longitudinal myelitis'. The clinical and laboratory findings are similar to those presented by ATM patients, including paraparesis, sensory level and sphincter disturbances. Four patients had positive antiphospholipid antibodies (aPL) suggesting that this could be a characteristic of longitudinal myelitis. Treatment in all cases included high doses of corticosteroids and immunosuppressive agents (intravenous (i.v.) cyclophosphamide). Anticoagulation therapy was given to one patient and two others received low doses of aspirin. The outcome was mainly unfavorable with slow improvement in only one case, no improvement in two and relapse of the myelopathy in the remaining three. In conclusion, longitudinal myelitis is an unusual form of SLE-related myelopathy, it might be associated with aPL and it has a poor prognosis.
Lupus 2001
PMID:Longitudinal myelitis associated with systemic lupus erythematosus: clinical features and magnetic resonance imaging of six cases. 1178 74


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