2006
DOI: 10.1191/0961203306lu2265cr
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Neuromyelitis optica (Devic’s syndrome) as first manifestation of systemic lupus erythematosus

Abstract: Neurologic symptoms rarely occur as presenting feature of systemic lupus erythematosus (SLE). We describe a 37-year old woman who presented with several episodes of transverse myelitis and optic neuritis. Clinical, radiologic and laboratory findings were compatible with neuromyelitis optica (NMO). Seven years after disease onset clinical and laboratory findings were diagnostic for SLE. This case illustrates that NMO may represent a first manifestation of SLE for many years.

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Cited by 50 publications
(31 citation statements)
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“…LM is a characteristic finding in NMO/NMOSD and its presence may be used to differentiate from myelitis in classic MS, which rarely extends beyond a single vertebral segment (33). Longitudinal cord involvement has been reported in SLE and SS as well (7,8,14,17,22,23,30,34,35). These reports have identified a high prevalence of the NMO-IgG antibody and additional features consistent with concurrent NMO/NMOSD in these patients.…”
Section: Discussionmentioning
confidence: 80%
See 1 more Smart Citation
“…LM is a characteristic finding in NMO/NMOSD and its presence may be used to differentiate from myelitis in classic MS, which rarely extends beyond a single vertebral segment (33). Longitudinal cord involvement has been reported in SLE and SS as well (7,8,14,17,22,23,30,34,35). These reports have identified a high prevalence of the NMO-IgG antibody and additional features consistent with concurrent NMO/NMOSD in these patients.…”
Section: Discussionmentioning
confidence: 80%
“…Lumbar puncture (LP) may serve as an additional tool to help differentiate NMO from MS: 70% of cases of NMO are negative for oligoclonal bands (OCBs), in contrast to MS, where Ͼ95% are typically positive (17,36). In our cohort, 12 patients had LP data, 10 of which had testing for OCBs.…”
Section: Discussionmentioning
confidence: 99%
“…(2) Analysis of the CSF in these patients with normal spinal MRI, demonstrated the presence of OCB or elevated protein levels, but no vigorous inflammatory reaction, along with the lack of demyelination, suggest that parenchymatous neurons are the sites of the pathologic involvement, and are are analogous to the acute lesions of axonal polyneuropathy. Such cases could be variants of DEM although similar cases were described in patient with SLE and was regarded as part of NMO spectrum (45). It was found that about 40% of acute transverse myelopathies cannot be documented by MRI (17)…”
Section: Inflammatory Myelopathiesmentioning
confidence: 98%
“…For example, Provenzale et al reported eight episodes of transverse myelitis in four patients and consistently observed prolonged signals on T1-or T2-weighted MRIs as well as cord enlargement in 75% of the episodes [36]. Moreover, Deodhar et al reported the first case of SLE with longitudinal myelitis in 1999 [37] and other groups have reported MRI findings of NPSLE with longitudinal spinal involvement [38,39] (Figure 1C), many of which showed T2-hyperintense lesions in central regions of the spinal cord and cord swelling, which are the typical MRI findings in NMO ( Figure 1D …”
Section: Mris Of Npsle With Optic Neuritis or Myelitismentioning
confidence: 99%