Quantitative susceptibility mapping in patients with systemic lupus erythematosus: detection of abnormalities in normal-appearing basal ganglia

2015 ◽  
Vol 26 (4) ◽  
pp. 1056-1063 ◽  
Author(s):  
Atsushi Ogasawara ◽  
Shingo Kakeda ◽  
Keita Watanabe ◽  
Satoru Ide ◽  
Issei Ueda ◽  
...  
2017 ◽  
Vol 8 (1) ◽  
pp. 31-34 ◽  
Author(s):  
Meghan Romba ◽  
Yujie Wang ◽  
Shu-Ching Hu ◽  
Sandeep Khot

Dystonia as a manifestation of neuropsychiatric lupus erythematosus (NPSLE) is uncommon. We report a 25-year-old woman who experienced progressive confusion, reduced speech, and difficulty opening her mouth approximately 2 weeks after development of a facial rash. Brain imaging showed bilateral, symmetric signal abnormalities within the basal ganglia and subcortical white matter. Despite treatment with high-dose steroids, she continued to have difficulty speaking with evidence of jaw dystonia on examination. Jaw dystonia rapidly improved with the initiation of levodopa. Repeat evaluation 3 months later exhibited the absence of jaw dystonia and near resolution of the imaging abnormalities. Our patient demonstrated a unique presentation with jaw dystonia refractory to traditional treatment for NPSLE. Such a presentation likely represents a severe variant of NPSLE requiring both immunosuppressive and symptomatic therapies.


Lupus ◽  
2018 ◽  
Vol 28 (1) ◽  
pp. 94-103 ◽  
Author(s):  
C. Magro-Checa ◽  
S. Kumar ◽  
S. Ramiro ◽  
L.J. Beaart-van de Voorde ◽  
J. Eikenboom ◽  
...  

Objective The effect of serum autoantibodies on the brain of systemic lupus erythematosus (SLE) patients remains unclear. We investigated whether serum autoantibodies, individually and assessed in groups, are associated with specific brain-MRI abnormalities or whether these structural changes are associated with other SLE-related or traditional cardiovascular disease risk factors. Methods All patients underwent brain 3Tesla-MRI. White matter hyperintensities (WMHs), ischemic lesions, inflammatory-like lesions and cerebral atrophy were scored. Serum autoantibodies analyzed included lupus anticoagulant (LAC), anticardiolipine (aCL) IgG and IgM (first 3 also grouped into antiphospholipid autoantibodies (aPL)), anti-dsDNA, anti-SSA, anti-SSB, anti-RNP, and anti-Sm (the latter 5 grouped into SLE-related autoantibodies). Associations were assessed using logistic regression analysis adjusted for potential confounders. Furthermore, a sensitivity analysis including anti-Beta2 glycoprotein-1 antibodies (anti-β2GP1) in the aPL group was performed and the potential modification role of the neuropsychiatric clinical status in the model was assessed. Results 325 patients (mean age 42 years (SD 14), 89% female) were included. The following MRI-brain abnormalities were found: WMHs (71%), lacunar infarcts (21%), gliosis (11%), micro-hemorrhages (5%), large hemorrhages (2%), inflammatory-like lesions (6%) and atrophy (14%). No associations were found between individual or total SLE-related autoantibodies and inflammatory-like lesions. A higher number of positive aPL was associated with lacunar infarcts (OR 1.37 (95%CI 1.02–1.99) and gliosis (OR 2.15 (1.37–3.37)). LAC was associated with lacunar infarcts in white matter (OR 3.38 (1.32–8.68)) and atrophy (OR 2.49 (1.01–6.15)), and aCL IgG with gliosis (OR 2.71 (1.05–7.02)). Among other variables, SLE patients with hypertension presented a higher chance for WMHs (OR 5.61 (2.52–12.48)) and lacunar infarcts in WM (OR 2.52 (1.10–5.74)) and basal ganglia (OR 8.34 (2.19–31.70)), while cumulative SLE-damage was correlated with lacunar infarcts in WM (OR 1.43 (1.07–1.90)), basal ganglia (OR 1.72 (1.18–2.51)) and cerebellum (OR 1.79 (1.33–2.41)). These associations were confirmed in the sensitivity analysis. Conclusions Brain abnormalities in SLE represent different underlying pathogenic mechanisms. aPL are associated with ischemic brain changes in SLE, while the presence of SLE-related serum autoantibodies is not related to inflammatory-like lesions. Hypertension and cumulative SLE-damage associate with ischemic MRI-brain changes in SLE, suggesting the importance of accelerated atherosclerosis in this process.


1986 ◽  
Vol 75 (1) ◽  
pp. 83-88 ◽  
Author(s):  
Mitsuo MAEDA ◽  
Yoshiaki KITAMURA ◽  
Mitsuko TAKATA ◽  
Yasushi NAKAMURA ◽  
Shogo MASUDA ◽  
...  

2018 ◽  
Vol 39 (8) ◽  
pp. 1648-1658 ◽  
Author(s):  
Mari Miyata ◽  
Shingo Kakeda ◽  
Kohsuke Kudo ◽  
Shigeru Iwata ◽  
Yoshiya Tanaka ◽  
...  

The purposes of this study are to assess the oxygen extraction fraction (OEF) changes on MRI-based quantitative susceptibility mapping (QSM) in systemic lupus erythematosus (SLE) patients and to determine whether QSM-OEF is associated with disease activity in SLE. We enrolled 42 SLE patients and 20 healthy subjects (HS) who had no pathologies on conventional brain MRI. Disease activity was assessed using SLE Disease Activity Index (SLEDAI). For the measurement of QSM-OEF, QSM data were analysed using the Perfusion Mismatch Analyzer software program. Spearman's or Pearson's correlation coefficients were calculated, and independent predictors were identified through a multiple linear regression analysis. QSM-OEF was significantly higher in SLE than that in HS (51.3 ± 10.1 vs. 40.5 ± 3.7, p < 0.001). QSM-OEF was positively correlated with SLEDAI and the presence of neuropsychiatric symptom (NPS) scores (ρ = 0.663, p < 0.001 and ρ = 0.340, p = 0.028). At multiple linear regression analysis, SLEDAI and NPS were independently associated with QSM-OEF (standardized β = 0.426, p = 0.016 and standardized β = 6.148, p = 0.029). In the SLE patients, QSM-OEF is associated with disease activity, which might predict an increased risk of stroke in SLE.


Author(s):  
Francis R. Comerford ◽  
Alan S. Cohen

Mice of the inbred NZB strain develop a spontaneous disease characterized by autoimmune hemolytic anemia, positive lupus erythematosus cell tests and antinuclear antibodies and nephritis. This disease is analogous to human systemic lupus erythematosus. In ultrastructural studies of the glomerular lesion in NZB mice, intraglomerular dense deposits in mesangial, subepithelial and subendothelial locations were described. In common with the findings in many examples of human and experimental nephritis, including many cases of human lupus nephritis, these deposits were amorphous or slightly granular in appearance with no definable substructure.We have recently observed structured deposits in the glomeruli of NZB mice. They were uncommon and were found in older animals with severe glomerular lesions by morphologic criteria. They were seen most commonly as extracellular elements in subendothelial and mesangial regions. The deposits ranged up to 3 microns in greatest dimension and were often adjacent to deposits of lipid-like round particles of 30 to 250 millimicrons in diameter and with amorphous dense deposits.


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