scholarly journals Effect of Enzyme Replacement Therapy on Basilar Artery Diameter in Male Patients With Fabry Disease

Stroke ◽  
2019 ◽  
Vol 50 (4) ◽  
pp. 1010-1012 ◽  
Author(s):  
Kaori Miwa ◽  
Yoshiki Yagita ◽  
Manabu Sakaguchi ◽  
Kazuo Kitagawa ◽  
Norio Sakai ◽  
...  

Background and Purpose— The effect of enzyme replacement therapy (ERT) on cerebrovascular complications remains largely unexplored. We aimed to investigate the relationship between basilar artery (BA) diameter and long-term ERT in patients with Fabry disease. Methods— We obtained baseline magnetic resonance imaging data from 30 patients (40.5±16.3 years, male: 14) in the single institution; among them, 21 patients prospectively had follow-up magnetic resonance imaging assessments. The short axis of BA diameter was measured at the midpons level on axial T2-weighted images. Brain magnetic resonance imaging measurements included markers of cerebral small vessel disease (lacunas, white matter hyperintensities, and cerebral microbleeds). We assessed variables associated with baseline BA diameter and annual BA diameter change using linear regression analyses. Results— Hypertension, left ventricular hypertrophy, estimated glomerular filtration rate, and white matter hyperintensities correlated with the initial BA diameter. After a mean interval of 7.2±4.6 years, the annual BA diameter change correlated positively with severe white matter hyperintensities and inversely with the duration of ERT (β=−0.48, P =0.033). After stratifying patients by sex, a significant correlation between the duration of ERT and BA diameter was found only in men (β=−0.72, P =0.019). Conclusions— Our results show a possible relationship between ERT and changes in BA diameter. Future studies to elucidate the clinical impact of BA changes as a potential surrogate marker are needed.

2015 ◽  
Vol 13 (3) ◽  
pp. 261-276 ◽  
Author(s):  
Maria Eugenia Caligiuri ◽  
Paolo Perrotta ◽  
Antonio Augimeri ◽  
Federico Rocca ◽  
Aldo Quattrone ◽  
...  

2009 ◽  
Vol 172 (2) ◽  
pp. 117-120 ◽  
Author(s):  
Adam M. Brickman ◽  
Amir Zahra ◽  
Jordan Muraskin ◽  
Jason Steffener ◽  
Christopher M. Holland ◽  
...  

2021 ◽  
Vol 15 ◽  
Author(s):  
Wha Jin Lee ◽  
Cindy W. Yoon ◽  
Sung-Woo Kim ◽  
Hye Jin Jeong ◽  
Seongho Seo ◽  
...  

Early- and late-onset Alzheimer’s disease (AD) patients often exhibit distinct features. We sought to compare overall white matter connectivity and evaluate the pathological factors (amyloid, tau, and vascular pathologies) that affect the disruption of connectivity in these two groups. A total of 50 early- and 38 late-onset AD patients, as well as age-matched cognitively normal participants, were enrolled and underwent diffusion-weighted magnetic resonance imaging to construct fractional anisotropy-weighted white matter connectivity maps. [18F]-THK5351 PET, [18F]-Flutemetamol PET, and magnetic resonance imaging were used for the evaluation of tau and related astrogliosis, amyloid, and small vessel disease markers (lacunes and white matter hyperintensities). Cluster-based statistics was performed for connectivity comparisons and correlation analysis between connectivity disruption and the pathological markers. Both patient groups exhibited significantly disrupted connectivity compared to their control counterparts with distinct patterns. Only THK retention was related to connectivity disruption in early-onset AD patients, and this disruption showed correlations with most cognitive scores, while late-onset AD patients had disrupted connectivity correlated with amyloid deposition, white matter hyperintensities, and lacunes in which only a few cognitive scores showed associations. These findings suggest that the pathogenesis of connectivity disruption and its effects on cognition are distinct between EOAD and LOAD.


2015 ◽  
Vol 5 (3) ◽  
pp. 387-394 ◽  
Author(s):  
William Reginold ◽  
Angela C. Luedke ◽  
Angela Tam ◽  
Justine Itorralba ◽  
Juan Fernandez-Ruiz ◽  
...  

Background/Aims: This study used 3-Tesla magnetic resonance imaging (MRI) tractography to determine if there was an association between tracts crossing white matter hyperintensities (WMH) and cognitive function in elderly persons. Methods: Brain T2-weighted fluid-attenuated inversion recovery (FLAIR) and diffusion tensor MRI scans were acquired in participants above the age of 60 years. Twenty-six persons had WMH identified on T2 FLAIR scans. They completed a battery of neuropsychological tests and were classified as normal controls (n = 15) or with Alzheimer's dementia (n = 11). Tractography was generated by the Fiber Assignment by Continuous Tracking method. All tracts that crossed WMH were segmented. The average fractional anisotropy and average mean diffusivity of these tracts were quantified. We studied the association between cognitive test scores with the average mean diffusivity and average fractional anisotropy of tracts while controlling for age, total WMH volume and diagnosis. Results: An increased mean diffusivity of tracts crossing WMH was associated with worse performance on the Wechsler Memory Scale-III Longest Span Forward (p = 0.02). There was no association between the fractional anisotropy of tracts and performance on cognitive testing. Conclusion: The mean diffusivity of tracts crossing WMH measured by tractography is a novel correlate of performance on the Wechsler Memory Scale-III Longest Span Forward in elderly persons.


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