Cognitive dysfunction and white matter hyperintensities in Fabry disease

2022 ◽  
Author(s):  
P. Murphy ◽  
F. Williams ◽  
I. Davagnanam ◽  
E. Chan ◽  
E. Murphy ◽  
...  
Keyword(s):  
White Matter ◽  
Fabry Disease ◽  
Cognitive Dysfunction ◽  
White Matter Hyperintensities

Abstract P366: High Burden of Tract-Specific White Matter Hyperintensities Relates to Impaired Microstructural Integrity of Distal White Matter Tracts in Community-Dwelling Elders

Stroke ◽  
2021 ◽  
Vol 52 (Suppl_1) ◽  
Author(s):  
YANPENG LIU ◽  
YIWEI XIA ◽  
XIAOXIAO WANG ◽  
YI WANG ◽  
LUMENG YANG ◽  
...  
Keyword(s):  
Executive Function ◽  
White Matter ◽  
Cognitive Dysfunction ◽  
White Matter Hyperintensities ◽  
Brain Mri ◽  
Mean Diffusivity ◽  
Community Dwelling ◽  
Superior Longitudinal Fasciculus ◽  
High Burden ◽  
Microstructural Integrity

Background and purpose: White matter hyperintensities (WMH) are common in elderly individuals and contribute to age-related cognitive dysfunction. Converging evidence indicates that WMH affect white matter (WM) microstructural integrity in WMH and their penumbra. We aimed to investigate whether this effect extends to the distal WM tracts, and to examine the association between distal WM microstructural integrity and cognitive dysfunction in community-dwelling elderly people. Methods: Brain MRI data including FLAIR and DTI sequences of 174 participants (74 ± 5 years) of the Shanghai Aging Study (SAS) were collected and analyzed. For each participant, WMH lesions were segmented automatically. Eighteen major WM tracts were reconstructed using automated quantitative tractography, and the mean diffusivity (MD) of distal WM tracts (excluding an area of 12 mm around the WMH) was calculated. Multivariable linear regression was performed. Results: A high burden of tract-specific WMH was related to a high MD of distal WM tracts in the forceps major (FMA), anterior thalamic radiations (ATR), cingulum cingulate gyrus (CCG), corticospinal tract (CST), superior longitudinal fasciculus-parietal (SLFP), superior longitudinal fasciculus-temporal (SLFT), and uncinate fasciculus (UNC). Furthermore, a high MD of distal tracts was linked to worse attention and executive function in the forceps minor (FMI), right CCG, left inferior longitudinal fasciculus (ILF), SLFP, SLFT and UNC. Conclusions: The effect of WMH on the microstructural integrity of WM tracts may propagate along tracts to distal regions farther than the penumbra and eventually might affect attention and executive function.

scholarly journals White Matter Hyperintensities and Cognitive Dysfunction in Patients With Infratentorial Stroke

2014 ◽  
Vol 38 (5) ◽  
pp. 620 ◽  
Author(s):  
Tae Won Kim ◽  
Yun-Hee Kim ◽  
Kang Hee Kim ◽  
Won Hyuk Chang
Keyword(s):  
White Matter ◽  
Cognitive Dysfunction ◽  
White Matter Hyperintensities

scholarly journals Cobalamin C Disease: Cognitive Dysfunction, Spastic Ataxic Paraparesis, and Cerebral White Matter Hyperintensities in a Genetic but Easily Treatable Cause!

2020 ◽  
Vol 0 (0) ◽  
pp. 0
Author(s):  
M.V. Padma Srivastava ◽  
Ayush Agarwal ◽  
Vibhor Upadhyay ◽  
Anu Gupta ◽  
Ajay Garg ◽  
...  
Keyword(s):  
White Matter ◽  
Cognitive Dysfunction ◽  
White Matter Hyperintensities ◽  
Cerebral White Matter

scholarly journals Associations between white matter hyperintensities and cognitive dysfunction in Alzheimer’s, vascular and mixed dementias

2020 ◽  
Vol 16 (S5) ◽  
Author(s):  
Hyunwoo Lee ◽  
Vanessa Wiggermann ◽  
Alexander Rauscher ◽  
Mirza Faisal Beg ◽  
Karteek Popuri ◽  
...  
Keyword(s):  
White Matter ◽  
Cognitive Dysfunction ◽  
White Matter Hyperintensities

White Matter Hyperintensities and Cognitive Dysfunction in Alzheimer Disease

2009 ◽  
Vol 22 (3) ◽  
pp. 207-212 ◽  
Author(s):  
Jae-Hyeok Heo ◽  
Soon-Tae Lee ◽  
Kon Chu ◽  
Hyun-Jung Park ◽  
Ji-Young Shim ◽  
...  
Keyword(s):  
White Matter ◽  
Alzheimer Disease ◽  
Cognitive Dysfunction ◽  
White Matter Hyperintensities

scholarly journals White matter integrity correlates with cognition and disease severity in Fabry disease

Brain ◽  
2020 ◽  
Vol 143 (11) ◽  
pp. 3331-3342
Author(s):  
Leonardo Ulivi ◽  
Baris Kanber ◽  
Ferran Prados ◽  
Indran Davagnanam ◽  
Aine Merwick ◽  
...  
Keyword(s):  
White Matter ◽  
Fabry Disease ◽  
Disease Severity ◽  
Fractional Anisotropy ◽  
Processing Speed ◽  
White Matter Hyperintensities ◽  
Mean Diffusivity ◽  
Healthy Controls ◽  
Healthy Control ◽  
The Mean

Abstract Cerebral white matter pathology is a common CNS manifestation of Fabry disease, visualized as white matter hyperintensities on MRI in 42–81% of patients. Diffusion tensor imaging (DTI) MRI is a sensitive technique to quantify microstructural damage within the white matter with potential value as a disease biomarker. We evaluated the pattern of DTI abnormalities in Fabry disease, and their correlations with cognitive impairment, mood, anxiety, disease severity and plasma lyso-Gb3 levels in 31 patients with genetically proven Fabry disease and 19 age-matched healthy control subjects. We obtained average values of fractional anisotropy and mean diffusivity within the white matter and performed voxelwise analysis with tract-based spatial statistics. Using a standardized neuropsychological test battery, we assessed processing speed, executive function, anxiety, depression and disease severity. The mean age (% male) was 44.1 (45%) for patients with Fabry disease and 37.4 (53%) for the healthy control group. In patients with Fabry disease, compared to healthy controls the mean average white matter fractional anisotropy was lower in [0.423 (standard deviation, SD 0.023) versus 0.446 (SD 0.016), P = 0.002] while mean average white matter mean diffusivity was higher (749 × 10−6 mm2/s (SD 32 × 10−6) versus 720 × 10−6 mm2/s (SD 21 × 10−6), P = 0.004]. Voxelwise statistics showed that the diffusion abnormalities for both fractional anisotropy and mean diffusivity were anatomically widespread. A lesion probability map showed that white matter hyperintensities also had a wide anatomical distribution with a predilection for the posterior centrum semiovale. However, diffusion abnormalities in Fabry disease were not restricted to lesional tissue; compared to healthy controls, the normal appearing white matter in patients with Fabry disease had reduced fractional anisotropy [0.422 (SD 0.022) versus 0.443 (SD 0.017) P = 0.003] and increased mean diffusivity [747 × 10−6 mm2/s (SD 26 × 10−6) versus 723 × 10−6 mm2/s (SD 22 × 10−6), P = 0.008]. Within patients, average white matter fractional anisotropy and white matter lesion volume showed statistically significant correlations with Digit Symbol Coding Test score (r = 0.558, P = 0.001; and r = −0.633, P ≤ 0.001, respectively). Average white matter fractional anisotropy correlated with the overall Mainz Severity Score Index (r = −0.661, P ≤ 0.001), while average white matter mean diffusivity showed a strong correlation with plasma lyso-Gb3 levels (r = 0.559, P = 0.001). Our findings using DTI confirm widespread areas of microstructural white matter disruption in Fabry disease, extending beyond white matter hyperintensities seen on conventional MRI. Moreover, diffusion measures show strong correlations with cognition (processing speed), clinical disease severity and a putative plasma biomarker of disease activity, making them promising quantitative biomarkers for monitoring Fabry disease severity and progression.

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 ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
White Matter ◽  
Magnetic Resonance ◽  
Enzyme Replacement Therapy ◽  
Fabry Disease ◽  
Basilar Artery ◽  
White Matter Hyperintensities ◽  
Resonance Imaging ◽  
Enzyme Replacement ◽  
Diameter Change

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.

P4-078: White matter hyperintensities and cognitive dysfunction in Alzheimer's disease

2008 ◽  
Vol 4 ◽  
pp. T691-T691
Author(s):  
Jae-Hyeok Heo ◽  
Min Ky Kim ◽  
Jin Young Ahn ◽  
Tai Hwan Park ◽  
Soon-Tae Lee ◽  
...  
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
White Matter ◽  
Cognitive Dysfunction ◽  
White Matter Hyperintensities

Cerebral White Matter Atrophy and Cognitive Dysfunction Following TBI

2007 ◽  
Author(s):  
Tracy D. Vannorsdall ◽  
Vani Rao ◽  
David J. Schretlen
Keyword(s):  
White Matter ◽  
Cognitive Dysfunction ◽  
Cerebral White Matter
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