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 14-year-old man was admitted with sudden onset of paralysis in his lower extremities paralysis and of sensory loss below Th10 level. On admission, linear high intensity signals was seen in the spinal cord from Th 8 to Th 12 level on thoracic MRI T2 weighted image. Laboratory data on admission indicated existence of lupus anticoagrant in the patient's serum. Systemic lupus erythematodes was negative in his past history. The diagnosis of transverse myelitis caused by primary antiphospholipid syndrome was made accordingly. Plasmapheresis was performed to remove the lupus anticoagrant. After plasma pheresis coagulopathy was normalized with disappearance of the lupus anticoagrant. The lesion of the spinal cord is too extensive to be caused by single obstruction of any one blood vessel branch of the spinal cord. The transverse myelitis may be aggravated by the direct invasion of the lupus anticoagrant into nervous tissue from the vasculature whose blood brain barrier had been compromised by intravascular coagulation of this substance.
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PMID:[A case of transverse myelitis caused by primary antiphospholipid antibody syndrome]. 1061 66

This case report describes a young woman with systemic lupus erythematosus starting at 16 years of age and giving rise to severe neurological complications including bilateral opticus neuritis and transverse myelitis. Despite heavy immunosuppression her condition steadily aggravated. At this point it was decided to perform autologous stem cell transplantation. Haematopoietic stem cells were mobilised with cyclophosphamide and granulocyte colony stimulating factor. Enrichment of CD34(+)cells was followed by depletion of peripheral T and B cells. The post-transplantation course was uneventful, and all the neurological deficits improved promptly during the 15 months of follow up. This is the first description of successful autologous stem cell transplantation in a case of life threatening central nervous system lupus.
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PMID:Autologous stem cell transplantation in a case of treatment resistant central nervous system lupus. 1070 Apr 36

A 30-year-old Caucasian male with systemic lupus erythematosus suffered acute catastrophic-onset transverse myelitis. Two years earlier aseptic meningitis, another rare CNS lupus manifestation, had been diagnosed. MRI showed involvement of the medulla oblongata and the entire spinal cord. Therapy with intravenous high-dose methylprednisolone and intravenous cyclophosphamide is discussed.
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PMID:Involvement of the entire spinal cord and medulla oblongata in acute catastrophic-onset transverse myelitis in SLE. 1079 31

Acute transverse myelitis (ATM) with moderate symptomatology and smaller multiple magnetic resonance imaging lesions is often caused by multiple sclerosis. Severe ATM with extensive magnetic resonance imaging lesions with or without associated meningitis often has a viral cause, particularly in the younger age groups, whereas vascular disorders may prevail among older patients. Previously, one had to rely on indirect evidence such as viral serology or viral identification in throat washings to confirm a diagnosis of myelitis. Thus, mycoplasma myelitis may occur coincident with a mycoplasma pneumonia. Viral myelitis is now often diagnosed by specific polymerase chain reaction of the cerebrospinal fluid, for echovirus, Coxsackie virus, mumps virus, herpes simplex virus or varicella-zoster virus, but an autoimmune component may still be important. An anterior horn syndrome may be produced by the tick-borne encephalomyelitis virus. Severe ATM may also be a postinfectious or postvaccinal disorder [i.e. a partial acute disseminated encephalomyelitis (ADEM)]. Neuromyelitis optica, a combination of severe myelitis and optic neuritis, is often a manifestation of ADEM or systemic lupus erythematosus. Many of these disorders are potentially treatable with specific antiviral agents or immunosuppression. 'Idiopathic' ATM is probably a consequence of inadequate examination and follow up. The differential diagnoses-viral myelitis, multiple sclerosis, ADEM, neuromyelitis optica, spinal arteriovenous malformation and arteritis-should be considered and are usually identified by a rapid diagnostic work-up, leaving few ATM cases undiagnosed.
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PMID:Myelitis. 1087 Dec 57

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

A 26-year-old woman suffered from acute myelitis at Th 6 level associated with systemic lupus erythematosus. Methyl-prednisolone pulse therapy, intravenous high-dose immunoglobulin administration and plasmapheresis were not effective. Her neurological signs had persisted in spite of subsequent administration of oral prednisolone and azathiopurine. Magnetic resonance imaging (MRI) of spinal cord at the onset showed a marked swelling with intramedullary high intensity signals on T2WI along the whole thoracic cord. Three years later, MRI demonstrated a severe longitudinal and segmental atrophy of the mid to low thoracic cord which resulted in transverse spinal signs.
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PMID:Development of severe longitudinal atrophy of thoracic spinal cord following lupus-related myelitis. 1133 99

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

Lupus-related transverse myelitis is a rare but serious complication. A 25-year-old Japanese woman with systemic lupus erythematosus (SLE) was admitted because of numbness of the face and left upper extremity, headache, and intermittent fever. Six days later, she developed tetraplegia. MRI of the spinal cord showed longitudinal high intensity signals from medulla oblongata to C5, and from Th12 to conus medullaris on T2-weighted image. These MRI findings were consistent with acute catastrophic neurological abnormalities. Despite administration of the combination of methylprednisolone and cyclophosphamide pulse therapies, as well as plasmapheresis, her condition did not improve. Any vasculopathy in addition to the autoimmune pathogenesis, and narrow therapeutic window may relate to the present refractory case.
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PMID:Systemic lupus erythematosus related transverse myelitis presenting longitudinal involvement of the spinal cord. 1186 6


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