Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of human T cell leukemia virus type I (HTLV-1) associated myelopathy (HAM)/tropical spastic paraparesis (TSP) with 14-year history of systemic lupus erythematosus (SLE) is reported. For 9 years, the numbness of the feet and sacral region progressed with occasional urinary incontinence and constipation. She was admitted to hospital due to gait disturbance and aggravation of SLE and the diagnosis of HAM/TSP was confirmed, indicating that HTLV-1 infection is associated with the development of not only HAM/TSP but also SLE.
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PMID:Human T cell leukemia virus type I-associated myelopathy in a patient with systemic lupus erythematosus. 1041 48

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

Central cord syndrome has classically been defined by disproportionately more motor impairment of the upper than the lower extremities, bladder dysfunction, and varying degrees of sensory loss below the level of the lesion. Review of the literature indicates that the majority of causes of central cord syndrome have a traumatic etiology and few are a consequence of connective tissue disorders. This is a case of a 53-year-old female with systemic lupus erythematosus who developed central cord syndrome due to an exacerbation of her disease. The patient presented with upper extremity motor and sensory deficits but only sensory deficits of the lower extremities. She had laboratory abnormalities consistent with lupus, and magnetic resonance imaging of the cervical spine revealed decreased attenuation from C-1-T-2. High-dose intravenous steroids were started in the acute care hospital with some return of proximal upper extremity strength. Upon transfer to the acute rehabilitation unit, the patient was noted to have neurogenic bladder dysfunction requiring a catheterization program. The patient was slowly tapered down on her oral prednisone with minimal improvement of strength but with return of bladder function. A review of the literature revealed few cases of central cord syndrome secondary to a connective tissue disorder or a non-traumatic etiology.
J Spinal Cord Med 2000
PMID:Systemic lupus erythematosus: a unique cause of central cord syndrome, a case report. 1075 68

We describe two patients with established antiphospholipid syndrome, who during periods of subtherapeutic anticoagulation, developed acute optic neuropathy and transverse myelopathy. Treatment with optimal anticoagulation and high dose glucocorticoids was followed by resolution of the neurologic deficits.
Lupus 2000
PMID:Acute optic neuropathy and transverse myelopathy in patients with antiphospholipid antibody syndrome: favorable outcome after treatment with anticoagulants and glucocorticoids. 1086 4

There are several non-neoplastic lesions which may mimic intramedullary spinal cord neoplasm in their radiographic and clinical presentation. These can be classified as either infectious (TB, fungal, bacterial, parasitic, syphilis, CMV, HSV) and non-infectious (sarcoid, MS, myelitis, ADEM, SLE) inflammatory lesions, idiopathic necrotizing myelopathy, unusual vascular lesions (amyloid, infarct, isolated intramedullary vascular lesions) and radiation myelopathy. Although biopsy may be indicated in many cases, the mistaken diagnosis of intramedullary neoplasm can often be eliminated pre-operatively.
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PMID:Non-neoplastic intramedullary pathology. Diagnostic dilemma: to Bx or not to Bx. 1101 44

The treatment of patients with neuropsychiatric systemic lupus erythematosus (NPSLE) can be difficult and complex owing to the variety of nervous system manifestations that can occur, which include peripheral nerve disease, headaches, seizures, cerebrovascular disease, chorea, transverse myelitis, and psychiatric and cognitive disorders. Many of these manifestations can result from metabolic abnormalities or infection or as side effects of medications. Thus, in any patient with suspected NPSLE, it is crucial to exclude secondary causes of the presenting symptoms before assuming that they are due to NPSLE. It is especially important to exclude infection because this is a common cause of both morbidity and mortality in patients with systemic lupus erythematosus (SLE). Symptoms such as anxiety and depression may or may not be related to disease activity. Treatment decisions are based on accurate diagnosis of the specific NPSLE manifestation, which is usually made using tools such as brain imaging, electroencephalography, cerebrospinal fluid analysis, nerve conduction studies, or special serologic tests (eg, determination of antiphospholipid or antiribosomal P antibody levels). It is also important to assess the degree of other SLE- mediated systemic disease activity in a patient with neurologic manifestations to determine if activation of systemic disease activity is also occurring. This is done by measuring complement levels, anti-double-stranded DNA levels, complete blood count, and urinalysis. For some NPSLE manifestations (eg, infrequent seizures, headaches, depression, anxiety, or peripheral neuropathy) that appear without activation of systemic disease, symptomatic treatment is appropriate. For others (eg, psychosis, delirium, or transverse myelopathy without other obvious cause), treatment with high-dose glucocorticoids with or without cyclophosphamide is appropriate whether there is evidence of other systemic disease activity or not. In general, the activity and severity of the leading organ manifestations dictate pharmacologic treatment.
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PMID:Neuropsychiatric Systemic Lupus Erythematosus. 1109 72

Systemic lupus erythematosus (SLE) is an autoimmunologic illness, which apart from changes in the skin, the locomotor system and the internal organs, attacks also the nervous system. The paper presents 19 neuropsychiatric symptomic syndromes which after conduction of multidisciplinary, international research were accepted by the American College of Rheumatology (ACR) as the criteria of systemic lupus erythematosus (SLE). This broadens the criteria applied since 1982, which were only considering acute symptoms and psychoses as characteristic of the neuropsychiatric form of systemic lupus erythematosus (NPSLE). In the new neurologic criteria concerning the CNS are: epileptic attacks and acute attack disorders, headaches, vascular diseases, demyelinating syndrome, aseptical meningitis, chorea, myelopathy. Psychiatric syndromes which make up the new criteria are: acute amentive state, anxiety disorders, cognitive function impairment, affective disorders, psychoses. The criteria connected to the CNS are: cranial nerve damage, mononeuropathy, damage of nerve plexus, polyneuropathy, vegetative neuropathy, myasthenia and acute inflammatory demyelinating polyneuropathy. Clinical symptoms of these syndromes were set and laboratory and visualising tests were developed, which are useful in their diagnosis. The intention of the ACR in setting new, significantly broader criteria of NPSLE, was to stress the diversity of symptoms and for a practical aspect to allow the diagnosis of NPSLE in patients having this disease, in whom the symptoms connected with the nervous system may dominate in the clinical picture, or may be before the dermatological, locomotor or internal organ symptoms.
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PMID:[New diagnostic criteria for the neuropsychiatric form of systemic lupus erythematosus]. 1130 90

The acute spinal cord infarction is a rare cause of acute-onset paraplegia. Furthermore, it is specially uncommon that the infarction occurs in patients with apparent low predisposition to vascular disease. The 20210A allele of the prothrombin gene (causing a threefold-increased risk in venous thromboembolism) was recently associated with unexplained spinal cord infarction in young women under treatment with estrogens (contraceptive pill). We report a case of anterior spinal artery syndrome resulting from an ischaemic infarction at the anterior aspect of the spinal cord in a healthy 50-year-old woman, carrying this mutation, being the first published case under treatment with transdermal estradiol. She referred the typical sudden-onset back pain associated to clinical anterior spinal artery syndrome with sphincter dysfunction and nontraumatic paraplegia. A possible multiple sclerosis was ruled out and the steroids or immunoglobulin therapy induced no clinical improvement. Cerebrospinal fluid and other investigations were all negative. Sequential MRI scans revealed development of spinal cord infarction from T10 to T11, with increased signal in T2-weighted image (T2). Because she referred a previous thrombophlebitis and suffered a deep-vein thrombosis one month after paraplegia, a complete coagulation study was performed. Antithrombin, proteins C and S, homocysteine, factor V Leiden, lupus anticoagulant and anticardiolipin antibodies were all normal or negatives. In opposite, the 20210A variation was positive (heterozygous) and the factor VIIIc level was very high (280 U/dl eight months later). We argue the relative importance of both findings. The patient had no a substantial recovery over a period of 20 months.Certainly, the prothrombin 20210A seems to be associated with unexplained ischemic myelopathy among the young women with estrogens.
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PMID:[Spinal cord infarction and recurrent venous thrombosis in association with estrogens and the 20210A allele of the prothrombin gene]. 1174 25

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

Acute transverse myelopathy (ATM) is a clinical definition of an acute neurologic condition that reflects impairment of spinal cord function. The term "myelopathy" has a different meaning from "myelitis", even if the words are often confused. Both terms indicate spinal cord involvement by some pathological event; but while myelopathy does not imply any etiological factor, myelitis refers to inflammatory diseases of the spinal cord. Acute spinal pathology can be associated with intra-axial or extra-axial lesions; extra-axial spinal pathology, however, has more often a chronic and progressive presentation. In this paper, we discuss primarily intra-axial lesions with attention on the role of neuroradiological investigations in diagnosis and differential diagnosis. Magnetic resonance imaging is the modality of choice for diagnosis; it shows signal abnormalities, usually T2 hyperintensity, focal or extensive, gadolinium enhancement and sometimes cord swelling. Despite its high sensitivity, about 40% of acute transverse myelopathies remain undemonstrated. Concerning etiology (multiple sclerosis (MS), vasculitis, infection, autoimmune disorders) no clearly different and specific patterns have been found; however small multiple enhancing lesions are more suggestive of MS (or lupus) while extensive, multilevel abnormalities reflect vasculitis as in antiphospholipid antibody syndrome.
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PMID:Diagnosis and differential diagnosis of acute transverse myelopathy. The role of neuroradiological investigations and review of the literature. 1179 82


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