MR Diffusion Tractography to Identify and Characterize Microstructural White Matter Tract Changes in Systemic Lupus Erythematosus Patients

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.

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White Matter Microstructure and Cognition in Non-neuropsychiatric Systemic Lupus Erythematosus

Cognitive and Behavioral Neurology ◽

10.1097/wnn.0b013e318190d174 ◽

2009 ◽

Vol 22 (1) ◽

pp. 38-44 ◽

Cited By ~ 37

Author(s):

Christopher M. Filley ◽

Elizabeth Kozora ◽

Mark S. Brown ◽

David E. Miller ◽

Sterling G. West ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

White Matter ◽

Lupus Erythematosus ◽

Systemic Lupus ◽

Neuropsychiatric Systemic Lupus Erythematosus ◽

White Matter Microstructure

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An uncommon central nervous system manifestation in systemic lupus erythematosus: the diffuse and symmetrical lesions of white matter

European Journal of Neurology ◽

10.1046/j.1468-1331.2000.t01-1-00118.x ◽

2000 ◽

Vol 7 (5) ◽

pp. 585-585 ◽

Cited By ~ 3

Author(s):

N. Aydin ◽

U. Utku ◽

T. E. Huseyin Ozer

Keyword(s):

Systemic Lupus Erythematosus ◽

Central Nervous System ◽

Nervous System ◽

White Matter ◽

Lupus Erythematosus ◽

Central Nervous System Manifestation ◽

Systemic Lupus ◽

System Manifestation

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Functional impairments in white matter syndrome of neuropsychiatric systemic lupus erythematosus are similar to those observed in patients with multiple sclerosis

Arquivos de Neuro-Psiquiatria ◽

10.1590/s0004-282x2012001000004 ◽

2012 ◽

Vol 70 (10) ◽

pp. 769-773 ◽

Cited By ~ 1

Author(s):

Denise Sisterolli-Diniz ◽

Aline de Oliveira ◽

Daiany Silva de Paula ◽

Rosangela Vieira Rodrigues ◽

Nilzio Antônio da Silva

Keyword(s):

Multiple Sclerosis ◽

Systemic Lupus Erythematosus ◽

White Matter ◽

Lupus Erythematosus ◽

Signs And Symptoms ◽

Functional System ◽

Functional Impairments ◽

Systemic Lupus ◽

Symptoms Of Depression ◽

Neuropsychiatric Systemic Lupus Erythematosus

OBJECTIVE: In order to compare white matter syndrome of neuropsychiatric systemic lupus erythematosus (NPSLE) and multiple sclerosis (MS), an assessment on demographic, medical history, and clinical data was proposed. METHODS: Sixty-four patients with NPSLE and 178 with MS answered a questionnaire and were evaluated regarding functional system, expanded disability status scale (EDSS), Beck depression inventory (BDI), and Beck anxiety inventory (BAI). RESULTS: The prevalence of autoimmune diseases and altered consciousness was similar in both groups, however it was higher than in the general population. Systemic signs and symptoms occurred from 2.9 to 61.9% of the MS cases, while neurological signs and symptoms occurred in 9.4 to 76.4% of the NPSLE ones. The motor, visual, and mental systems were the most affected in both diseases. The BDI in NPSLE had higher scores and the BAI in MS. CONCLUSIONS: The functional impairments in NPSLE were similar to those of MS, although greater impairment of the functional systems of cerebellar, sensitivity, and sphincters occurred in MS cases, and greater symptoms of depression, anxiety, and headache also occurred in it.

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Are serum autoantibodies associated with brain changes in systemic lupus erythematosus? MRI data from the Leiden NP-SLE cohort

Lupus ◽

10.1177/0961203318816819 ◽

2018 ◽

Vol 28 (1) ◽

pp. 94-103 ◽

Cited By ~ 9

Author(s):

C. Magro-Checa ◽

S. Kumar ◽

S. Ramiro ◽

L.J. Beaart-van de Voorde ◽

J. Eikenboom ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Sensitivity Analysis ◽

White Matter ◽

Basal Ganglia ◽

Lupus Erythematosus ◽

Brain Abnormalities ◽

Systemic Lupus ◽

Lacunar Infarcts ◽

Mri Brain ◽

Brain Changes

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.

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Brain network reorganisation and spatial lesion distribution in systemic lupus erythematosus

Lupus ◽

10.1177/0961203320979045 ◽

2020 ◽

pp. 096120332097904

Author(s):

Maria del C Valdés Hernández ◽

Keith Smith ◽

Mark E Bastin ◽

E. Nicole Amft ◽

Stuart H Ralston ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

White Matter ◽

Lupus Erythematosus ◽

Structural Connectivity ◽

Brain Network ◽

White Matter Hyperintensity ◽

Healthy Controls ◽

Systemic Lupus ◽

Disease Indicators ◽

Network Organisation

Objective This work investigates network organisation of brain structural connectivity in systemic lupus erythematosus (SLE) relative to healthy controls and its putative association with lesion distribution and disease indicators. Methods White matter hyperintensity (WMH) segmentation and connectomics were performed in 47 patients with SLE and 47 healthy age-matched controls from structural and diffusion MRI data. Network nodes were divided into hierarchical tiers based on numbers of connections. Results were compared between patients and controls to assess for differences in brain network organisation. Voxel-based analyses of the spatial distribution of WMH in relation to network measures and SLE disease indicators were conducted. Results Despite inter-individual differences in brain network organization observed across the study sample, the connectome networks of SLE patients had larger proportion of connections in the peripheral nodes. SLE patients had statistically larger numbers of links in their networks with generally larger fractional anisotropy weights (i.e. a measure of white matter integrity) and less tendency to aggregate than those of healthy controls. The voxels exhibiting connectomic differences were coincident with WMH clusters, particularly the left hemisphere’s intersection between the anterior limb of the internal and external capsules. Moreover, these voxels also associated more strongly with disease indicators. Conclusion Our results indicate network differences reflective of compensatory reorganization of the neural circuits, reflecting adaptive or extended neuroplasticity in SLE.

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