scholarly journals Internal Carotid Artery Occlusion in Systemic Lupus Erythematosus as a Potential Mimicker of Multiple Sclerosis

2017 ◽  
Vol 9 (1) ◽  
pp. 86-90 ◽  
Author(s):  
Kenichiro Sato ◽  
Mami Kanzaki ◽  
Yoshifumi Ubara ◽  
Yoshikazu Uesaka
Keyword(s):  
Multiple Sclerosis ◽  
Systemic Lupus Erythematosus ◽  
White Matter ◽  
Carotid Artery ◽  
Internal Carotid Artery ◽  
Lupus Erythematosus ◽  
Internal Carotid ◽  
White Matter Lesions ◽  
Systemic Lupus ◽  
Neuropsychiatric Sle

The diagnosis of neurological symptoms in patients with systemic lupus erythematosus (SLE) is often challenging, in part because they sometimes mimic features of multiple sclerosis (MS). Herein we report a case of a young female who presented with relapsing-remitting symptoms of unilateral visual loss and motor aphasia. Additionally, radiological findings revealed multiple white matter lesions on her brain that led to an initial diagnosis of MS based on the established diagnostic criteria. However, she was eventually diagnosed with neuropsychiatric SLE (NPSLE) presenting with extracranial internal carotid artery (ICA) occlusion. Her ICA occlusion had not been detected for 2 months until she underwent magnetic resonance angiography. Although exact underlying pathological mechanisms are unclear, both the ICA occlusion and MS-like brain white matter lesions could be attributed to SLE. This case demonstrated that both of these lesions can coexist in the same patient, suggesting that NPSLE with ICA occlusion can be a potential mimicker of MS, and vice versa. Care must be paid to avoid delay in the diagnosis.

Distribution of White Matter Lesions in Multiple Sclerosis and Systemic Lupus Erythematosus

1998 ◽  
Vol 11 (2_suppl) ◽  
pp. 17-19
Author(s):  
M.T. Peltz ◽  
L. Casciu ◽  
F.M. Manconi ◽  
G. Sanna ◽  
P. Schiffini ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Systemic Lupus Erythematosus ◽  
White Matter ◽  
Lupus Erythematosus ◽  
White Matter Lesions ◽  
Systemic Lupus

White matter lesions and brain atrophy in systemic lupus erythematosus patients: correlation to cognitive dysfunction in a cohort of systemic lupus erythematosus patients using different definition models for neuropsychiatric systemic lupus erythematosus

Lupus ◽  
2018 ◽  
Vol 27 (7) ◽  
pp. 1140-1149 ◽  
Author(s):  
B Cannerfelt ◽  
J Nystedt ◽  
A Jönsen ◽  
J Lätt ◽  
D van Westen ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
White Matter ◽  
Corpus Callosum ◽  
Cognitive Dysfunction ◽  
Lupus Erythematosus ◽  
Verbal Memory ◽  
White Matter Lesions ◽  
Cognitive Test ◽  
Systemic Lupus ◽  
Neuropsychiatric Sle

Aim The aim of this study was to evaluate the extent of white matter lesions, atrophy of the hippocampus and corpus callosum, and their correlation with cognitive dysfunction (CD), in patients diagnosed with systemic lupus erythematosus (SLE). Methods Seventy SLE patients and 25 healthy individuals (HIs) were included in the study. To evaluate the different SLE and neuropsychiatric SLE (NPSLE) definition schemes, patients were grouped both according to the American College of Rheumatology (ACR) definition, as well as the more stringent ACR-Systemic Lupus International Collaborating Clinics definition. Patients and HIs underwent a 3 Tesla brain MRI and a standardized neuropsychological test. MRI data were evaluated for number and volume of white matter lesions and atrophy of the hippocampus and corpus callosum. Differences between groups and subgroups were evaluated for significance. Number and volume of white matter lesions and atrophy of the hippocampus and corpus callosum were correlated to cognitive dysfunction. Results The total volume of white matter lesions was significantly larger in SLE patients compared to HIs ( p = 0.004). However, no significant differences were seen between the different SLE subgroups. Atrophy of the bilateral hippocampus was significantly more pronounced in patients with NPSLE compared to those with non-NPSLE (right: p = 0.010; left p = 0.023). Significant negative correlations between cognitive test scores on verbal memory and number and volume of white matter lesions were present. Conclusion SLE patients have a significantly larger volume of white matter lesions on MRI compared to HIs and the degree of white matter lesion volume correlates to cognitive dysfunction, specifically to verbal memory. No significant differences in the number or volume of white matter lesions were identified between subgroups of SLE patients regardless of the definition model used.

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

2012 ◽  
Vol 70 (10) ◽  
pp. 769-773 ◽  
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.

scholarly journals Cerebrovascular disease progression in patients with ACTA2 Arg179 Pathogenic Variants

Neurology ◽  
2020 ◽  
pp. 10.1212/WNL.0000000000011210
Author(s):  
Arne Lauer ◽  
Samantha L. Speroni ◽  
Jay B. Patel ◽  
Ellen Regalado ◽  
Myoung Choi ◽  
...  
Keyword(s):  
White Matter ◽  
Carotid Artery ◽  
Internal Carotid Artery ◽  
Disease Progression ◽  
White Matter Hyperintensities ◽  
Internal Carotid ◽  
White Matter Lesions ◽  
Critical Stenosis ◽  
Pathogenic Variants ◽  
Over Time

Objective:To establish progression of imaging biomarkers of stroke, arterial steno-occlusive disease and white matter injury in patients with smooth muscle dysfunction syndrome caused by mutations in the ACTA2 gene, we analyzed 113 cerebral MRI scans from a retrospective cohort of 27 patients with ACTA2 Arg179 pathogenic variants.Methods:Systematic quantifications of arterial ischemic strokes and white matter lesions were performed on baseline and follow-up scans using planimetric methods. Critical stenosis and arterial vessel diameters were quantified applying manual and semi-automated methods to cerebral MR angiograms. We then assessed correlations between arterial abnormalities and parenchymal injury.Results:We found characteristic patterns of acute white matter ischemic injury and progressive internal carotid artery stenosis during infancy. Longitudinal analysis of patients older than 1.2 years showed stable white matter hyperintensities but increased number of cystic-like lesions over time. Progressive narrowing of the terminal internal carotid artery occurred in 80% of patients and correlated with the number of critical stenosis in cerebral arteries and arterial ischemic infarctions. Arterial ischemic strokes occurred in same territories affected by critical stenosis.Conclusions:We found characteristic, early and progressive cerebrovascular abnormalities in patients with ACTA2 Arg179 pathogenic variants. Our longitudinal data suggests that while steno-occlusive disease progresses over time and is associated with arterial ischemic infarctions and cystic-like white matter lesions; white matter hyperintensities can remain stable over long periods. Above evaluated metrics will enable diagnosis in early infancy and be used to monitor disease progression, guide timing of stroke preventive interventions and assess response to current and future therapies.

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