scholarly journals Deep Gray Matter Iron Deposition and Its Relationship to Clinical Features in Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy Patients

Stroke ◽  
2020 ◽  
Vol 51 (6) ◽  
pp. 1750-1757 ◽  
Author(s):  
Chengyue Sun ◽  
Yue Wu ◽  
Chen Ling ◽  
Zhiying Xie ◽  
Qingle Kong ◽  
...  

Background and Purpose— Distribution patterns of iron deposition in deep gray matter and their association with clinical characteristics in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) remain unclear. We aimed to evaluate iron deposition in deep gray matter in patients with CADASIL using 7.0-T susceptibility-weighted imaging and mapping and to explore its correlations with clinical characteristics. Methods— Thirty-nine patients with CADASIL, confirmed via genetic analysis or skin biopsy, were enrolled. We examined patients using the Mini-Mental State Examination, modified Rankin Scale, and brain 7.0-T magnetic resonance imaging and obtained magnetic resonance imaging lesion loads, small vessel disease scores, and susceptibility mapping. The following regions of interest were selected: caudate nucleus, putamen, globus pallidus, thalamus, substantia nigra, and red nucleus. The quantitative differences in the susceptibility of deep gray matter between the CADASIL and control groups and the correlations between deep gray matter susceptibility and clinical characteristics were identified. Results— Compared with the control group, the CADASIL group showed significantly increased susceptibility of caudate nucleus, putamen, thalamus, substantia nigra, and red nucleus. The susceptibility of deep gray matter in basal ganglia region, including caudate nucleus, putamen, and thalamus, significantly increased with age or disease duration and positively correlated with small vessel disease scores in patients with CADASIL. Moreover, the susceptibility of thalamus positively correlated with modified Rankin Scale scores after adjusting for age and disease duration and that of putamen negatively correlated with Mini-Mental State Examination scores in patients with CADASIL after adjusting for age. Conclusions— Our findings indicate an association between abnormal iron deposition in deep gray matter of patients with CADASIL and their clinical characteristics. Therefore, excess iron deposition in deep gray matter, as indicated by 7.0-T susceptibility-weighted imaging and mapping, might not only be a novel magnetic resonance imaging feature but also a potential biomarker for CADASIL severity.

Author(s):  
Zen Kobayashi ◽  
Sawako Sakai ◽  
Sakiko Itaya ◽  
Yoshiyuki Numasawa ◽  
Kiyobumi Ota ◽  
...  

2013 ◽  
Vol 20 (4) ◽  
pp. 418-423 ◽  
Author(s):  
Lei Zhang ◽  
Aimin Wu ◽  
Bingjun Zhang ◽  
Suqin Chen ◽  
Xuejiao Men ◽  
...  

Background: Deep gray matter lesions have been reported in patients with acute disseminated encephalomyelitis (ADEM), multiple sclerosis (MS), and neuromyelitis optica (NMO). Objectives: The purpose of this study was to compare the features of deep gray matter lesions on magnetic resonance imaging (MRI) among adult patients with ADEM, MS, and NMO. Methods: Ninety-five adult patients with ADEM ( n=12), MS ( n=60), and NMO ( n=23) who had deep gray matter lesions on MRI were enrolled. Morphological features of deep gray matter lesions among these patients were assessed. Results: Putamen involvement was more common in patients with ADEM than in patients with MS and NMO. Differing from children, thalamus involvement might not be helpful in differentiating ADEM from MS in adults. Hypothalamus involvement was more common in patients with NMO than in patients with ADEM and MS. More importantly, bilateral hypothalamus involvement was more helpful in differentiating NMO from MS. The diameter of the thalamus lesions in patients with ADEM was larger than that in patients with NMO. Conclusions: Morphological features of deep gray matter lesions vary among adult patients with ADEM, MS, and NMO, and may be helpful in distinguishing these diseases.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1832.1-1832
Author(s):  
P. Falsetti ◽  
E. Conticini ◽  
C. Baldi ◽  
M. Bardelli ◽  
S. Gentileschi ◽  
...  

Background:SIJ involvement is a characteristic feature of Spondylarthritis (SpA). Magnetic Resonance imaging (MRI) has been included in the new Assessment of SpA International Society (ASAS) criteria for the classification of Axial SpA. Gray scale US, Color Doppler ultrasound (CDUS), contrast-enhanced CDUS, and spectral Doppler (SD) US has been used in few works to evaluate the inflammatory activity of the SIJ with not conclusive results. Power Doppler ultrasound (PDUS) was not yet applied to the study of SIJ with active SI.Objectives:The aim of this work was to study with PDUS and SD US the SIJ of patients with suspected active SI, to describe inflammatory flows with spectral wave analysis (SWA) in duplex Doppler US, and to correlate US data with clinical characteristics and the presence of bone marrow edema (BME) in MRI.Methods:22 patients (18 females and 4 males, mean age 35 years) with new onset of inflammatory back pain (IBP), were included. Every patient underwent an US examination in prone position. The sonographers were blinded to the clinical data of the patient. A Esaote Twice US machine, equipped with a convex multifrequency 1-8 MHz probe, was used, with standardized parameters: 1-5 MHz for gray scale, 1.9-2.3 MHz frequency for Doppler with Pulse Repetition Frequency (PRF) of 1.0 KHz and a color gain just under the artifact limit. SIJ was located as the hypoechoic triangle delimited between the sacrum and iliac bone, and the posterior SI ligament as the upper margin. The first sacral foramen was always localized to avoid measurement of the normal pre-sacral arteries. The PDUS was applied, and if any signals were detected in the SIJ, they were scored with a 3-points scale: 0= absence of signals, 1= isolate vessels, 2= more than one vessel. The signals were also classified as intra-articular or peri-articular. The same vessels were also evaluated using quantitative SD calculating the Resistive Index (RI=peak of systolic flow- end diastolic flow/peak systolic flow), ranging between 0 and 1. Every patient underwent MRI of SIJ within the same week, before treatment. A statistical analysis was performed, estimating the sensitivity and specificity against the gold standard (presence of BME in the same SIJ according to ASAS criteria). The Spearman rank not-parametric test was applied to correlate the presence and grading of BME with PDUS grading and RI. A regression analysis was applied between PDUS results and clinical characteristics.Results:In 14/22 SIJ MRI revealed BME. In 13 of them, PDUS confirmed abnormal hypervascularisation in the intrarticular portion of SI, and in 3 in the periarticular site too. Two SIJ showed hypervascularisation at PD with no BME in MRI. A significant correlation was demonstrated between positivity and grading of PD and presence of BME in MRI (p=0.0005). SD analysis demonstrated low Resistance Index (RI) values in 14 SIJ (mean 0.57). An inverse correlation was demonstrated between RI and grading of BME in MRI (r= -0,6229, p= 0,044). The diagnostic accuracy of SD for detection of active SI varied on the basis of RI cut-off value. The best values of sensitivity (62,5%) and specificity (61,5%) were obtained with a RI cut-off values of 0.60. A multiple regression model demonstrated a significant relationship between PDUS signals and ASDAS (p=0.0382), but not with inflammatory reactants.Conclusion:PDUS and SD US of SIJ can be useful as first imaging assessment in suspected active SI, demonstrating a good diagnostic accuracy compared with MRI. Intra-articular low RI values (<0.60) on SD indicate active SI with good accuracy. Moreover, PDUS signals into the SIJ correlate with clinical symptoms but not with inflammation reactants.Figure 1.Doppler US in SI.Right SIJ with a Doppler signal along the posterior SIJ ligament, and another Doppler signal into the joint, where SD analysis gave a RI of 0,62.Disclosure of Interests:None declared


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 957.1-957
Author(s):  
P. Ruscitti ◽  
A. Barile ◽  
O. Berardicurti ◽  
S. Iafrate ◽  
P. DI Benedetto ◽  
...  

Background:Adult onset Still’s disease (AOSD) is a rare systemic autoinflammatory disease and joint involvement is one of its clinical manifestations [1]. Arthritis, either oligoarthritis or bilateral symmetrical rheumatoid arthritis-like polyarthritis, is another common clinical feature of AOSD, with a migrating pattern at the beginning and becoming stable over the time [1].Objectives:The aims of the study were to assess joint involvement in AOSD by using magnetic resonance imaging (MRI), to describe main patterns of involvement, and associated clinical characteristics, and to evaluate the global transcriptomic profile of synovial tissues in AOSD to elucidate possible pathogenic pathways involved with.Methods:AOSD patients, who underwent to magnetic resonance imaging (MRI) exam on joints, were assessed to describe patterns of joint involvement and associated clinical characteristics. Some synovial tissues were collected for RNA-sequencing purposes.Results:In this study, 31 patients with AOSD (mean age 42.3 ± 15.2 years, 54.8% male gender), who underwent to at least one MRI exam on joints, were assessed. The most common MRI finding was the presence of synovitis on 60.5%, mainly in peripheral affected joints. MRI revealed a mild to moderate proliferative synovitis, as thickening of the synovial membrane, with low to intermediate signal intensity on T1-weighted images and intermediate to high signal intensity on T2-fat saturated weighted and STIR images, suggesting the presence of a hyperplastic than of a hypertrophied synovial tissue. Bone oedema and bone erosions were reported on 34.9% and 25.6% MRI exams, respectively. In all patients but one, bone erosions were synchronous with bone oedema, overlapping completely the locations. Assessing clinical characteristics in patients with MRI-erosions, a higher prevalence of splenomegaly, a more frequent chronic disease course, lower levels of erythrocyte sedimentation rate and ferritin was observed.Assessing the synovial tissues of some AOSD patients, a moderate perivascular mononuclear infiltrate in the sub-lining stroma of hip synovial tissues was observed, whereas the lining cells were relatively unremarkable. In addition, interleukin (IL)-1β, IL-6, TNF, and heavy ferritin subunit (FeH) were found on AOSD synovial tissues.An RNA-sequencing analysis assessed the global transcriptomic profile of synovial tissues on AOSD patients and matched-controls. Assessing IL-1 pathway, we found an increased expression of il1a, il1b, il1rap, il1r1, il18r1, and Il18bp on AOSD tissues when compared with controls. In IL-6 pathway, we found an increased expression of il6 and il6st/gp130 on AOSD synovial tissues whereas an increased expression of il6r was shown on the controls. Among genes involved in TNF pathway, tnf, traf1, traf2, tnfaip3 and tnfrsf1a resulted to be more expressed in AOSD synovial tissues than in controls. Finally, fth1 and ftl were more expressed in AOSD patients than controls, when we explored the iron uptake and transport pathway.Conclusion:A peculiar MRI pattern of joint involvement in AOSD was reported; the most common finding was the presence of synovitis, characterised by intermediate to high signal intensity on T2-fat-saturated weighted and STIR images. Bone erosions and bone oedema were also observed. This MRI pattern was associated with a hyper-activation of IL-1, IL-6, and TNF pathways together with a hyper-expression of ferritin genes on AOSD synovial tissues.References:[1]Giacomelli R, Ruscitti P, Shoenfeld Y. A comprehensive review on adult onset Still’s disease. J Autoimmun. 2018;93:24-36.Disclosure of Interests:None declared


Nosotchu ◽  
1996 ◽  
Vol 18 (1) ◽  
pp. 10-18
Author(s):  
Tatsuo Kohriyama ◽  
Shinya Yamaguchi ◽  
Eiji Tanaka ◽  
Yasuhiro Yamamura ◽  
Shigenobu Nakamura

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