scholarly journals Increased resting cerebral blood flow in adult Fabry disease

Neurology ◽  
2018 ◽  
Vol 90 (16) ◽  
pp. e1379-e1385 ◽  
Author(s):  
Po Phyu ◽  
Aine Merwick ◽  
Indran Davagnanam ◽  
Fay Bolsover ◽  
Fatima Jichi ◽  
...  
Keyword(s):  
Blood Flow ◽  
Cerebral Blood Flow ◽  
Fabry Disease ◽  
Arterial Spin Labeling ◽  
Cerebral White Matter ◽  
Healthy Controls ◽  
Cross Sectional ◽  
Whole Brain ◽  
The Mean ◽  
Control Study

ObjectiveTo assess resting cerebral blood flow (CBF) in the whole-brain and cerebral white matter (WM) and gray matter (GM) of adults with Fabry disease (FD), using arterial spin labeling (ASL) MRI, and to investigate CBF correlations with WM hyperintensity (WMH) volume and the circulating biomarker lyso-Gb3.MethodsThis cross-sectional, case-control study included 25 patients with genetically confirmed FD and 18 age-matched healthy controls. We quantified resting CBF using Quantitative Signal Targeting With Alternating Radiofrequency Labeling of Arterial Regions (QUASAR) ASL MRI. We measured WMH volume using semiautomated software. We measured CBF in regions of interest in whole-brain, WM, and deep GM, and assessed correlations with WMH volume and plasma lyso-Gb3.ResultsThe mean age (% male) for FD and healthy controls was 42.2 years (44%) and 37.1 years (50%). Mean whole-brain CBF was 27.56 mL/100 mL/min (95% confidence interval [CI] 23.78–31.34) for FD vs 22.39 mL/100 mL/min (95% CI 20.08–24.70) for healthy controls, p = 0.03. In WM, CBF was higher in FD (22.42 mL/100 mL/min [95% CI 17.72–27.12] vs 16.25 mL/100 mL/min [95% CI 14.03–18.48], p = 0.05). In deep GM, CBF was similar between groups (40.41 mL/100 mL/min [95% CI 36.85–43.97] for FD vs 37.46 mL/100 mL/min [95% CI 32.57–42.35], p = 0.38). In patients with FD with WMH (n = 20), whole-brain CBF correlated with WMH volume (r = 0.59, p = 0.006), not with plasma lyso-Gb3.ConclusionIn FD, resting CBF is increased in WM but not deep GM. In FD, CBF correlates with WMH, suggesting that cerebral perfusion changes might contribute to, or result from, WM injury.

Evaluation of the reasons for the microvascular changes in patients with Fabry disease using optic coherence tomography angiography

2020 ◽  
pp. 112067212097428
Author(s):  
Cezmi Dogan ◽  
Busenur Gonen ◽  
Mevlut Tamer Dincer ◽  
Burak Mergen ◽  
Ertugrul Kiykim ◽  
...  
Keyword(s):  
Blood Flow ◽  
Fabry Disease ◽  
Renal Involvement ◽  
Vascular Density ◽  
Optic Coherence Tomography ◽  
Healthy Controls ◽  
Duration Of Treatment ◽  
Flow Area ◽  
Cross Sectional ◽  
Significant Difference

Purpose: To investigate the blood flow changes in the choriocapillaris and the superficial and deep capillary plexus of the retina using optic coherence tomography angiography (OCTA) in patients with Fabry disease (FD) and reveal any possible association of these changes with the systemic findings. Methods: This cross-sectional study included 38 patients with FD and age- and gender-matched 40 healthy controls. OCTA images were obtained from all patients. Superficial (sCVD) and deep capillary vascular density (dCVD) in the foveal, parafoveal, and perifoveal zones and the whole image were recorded for each patient. Flow area in the choriocapillaris and central macular thickness (CMT) were also recorded. Results: Patients with FD showed a lower whole image (54.45 ± 5.99% vs 57.32 ± 6.71%, p = 0.004), foveal (34.94 ± 7.60% vs 39.65 ± 7.03%, p = 0.003), parafoveal (57.41 ± 4.85% vs 59.19 ± 4.67%, p = 0.043), and perifoveal (55.87 ± 6.43% vs 58.87 ± 7.02%, p = 0.003) dCVD compared to the healthy controls without a significant difference in the sCVD and choriocapillaris blood flow ( p > 0.05). A significantly lower whole image and foveal dCVD in the FD patients with renal involvement was observed compared to the healthy controls ( p = 0.027 and p = 0.024, respectively) without any significant difference between the FD patients without renal involvement and healthy controls ( p = 0.17 and p = 0.13, respectively). CMT was significantly higher in FD patients with renal involvement compared to the ones without renal involvement (252.1 ± 18.5 µm vs 235.5 ± 17.6 µm, p = 0.016). Conclusion: Patients with FD showed a lower dCVD without any change in sCVD and choriocapillaris compared to the healthy controls. This decrease was associated mostly with the renal involvement and duration of treatment.

scholarly journals Longitudinal Cerebral Blood Flow Changes in Normal Aging and the Alzheimer’s Disease Continuum Identified by Arterial Spin Labeling MRI

2021 ◽  
pp. 1-9
Author(s):  
Aldo Camargo ◽  
Ze Wang ◽  
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Blood Flow ◽  
Cerebral Blood Flow ◽  
Prefrontal Cortex ◽  
Arterial Spin Labeling ◽  
Spin Labeling ◽  
Normal Aging ◽  
Perfusion Magnetic Resonance Imaging ◽  
Cross Sectional

Background: Cross-sectional studies have shown lower cerebral blood flow (CBF) in Alzheimer’s disease (AD), but longitudinal CBF changes in AD are still unknown. Objective: To reveal the longitudinal CBF changes in normal control (NC) and the AD continuum using arterial spin labeling perfusion magnetic resonance imaging (ASL MRI). Methods: CBF was calculated from two longitudinal ASL scans acquired 2.22±1.43 years apart from 140 subjects from the Alzheimer’s Disease Neuroimaging Initiative (ADNI). At the baseline scan, the cohort contained 41 NC, 74 mild cognitive impairment patients (MCI), and 25 AD patients. 21 NC converted into MCI and 17 MCI converted into AD at the follow-up. Longitudinal CBF changes were assessed using paired-t test for non-converters and converters separately at each voxel and in the meta-ROI. Age and sex were used as covariates. Results: CBF reductions were observed in all subjects. Stable NC (n = 20) showed CBF reduction in the hippocampus and precuneus. Stable MCI patients (n = 57) showed spatially more extended CBF reduction patterns in hippocampus, middle temporal lobe, ventral striatum, prefrontal cortex, and cerebellum. NC-MCI converters showed CBF reduction in hippocampus and cerebellum and CBF increase in caudate. MCI-AD converters showed CBF reduction in hippocampus and prefrontal cortex. CBF changes were not related with longitudinal neurocognitive changes. Conclusion: Normal aging and AD continuum showed common longitudinal CBF reductions in hippocampus independent of disease and its conversion. Disease conversion independent longitudinal CBF reductions escalated in MCI subjects.

scholarly journals Simultaneous Measurement of Cerebral Blood Flow and Arterial Transit Time for Sickle Cell Disease

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 1298-1298 ◽  
Author(s):  
Qin Qin ◽  
Wenbo Li ◽  
Dexiang Liu ◽  
John J Strouse
Keyword(s):  
Blood Flow ◽  
Cerebral Blood Flow ◽  
Transit Time ◽  
Gray Matter ◽  
Whole Brain ◽  
Non Invasive ◽  
3D Acquisition ◽  
The Mean ◽  
Arterial Transit Time ◽  
Large Vessels

Abstract Introduction Studies of patients with ischemic stroke and silent cerebral infarcts from sickle cell anemia (SCA) have revealed abnormalities of both large and small vessels. Cerebral blood flow (CBF), a measure of the microvascular perfusion of the brain, has been recognized as a potentially sensitive and specific indicator of early cerebrovascular impairment in both children and adults with SCA. Arterial spin labeling (ASL) MRI is a non-invasive technique to acquire perfusion-weighted signal. This is typically at a single post-labeling delay (PLD) and provides only CBF measurement. The addition of multiple PLDs also permits the measurement of arterial transit time (ATT), the transit time from the labeled plane to the imaging voxels. ATT is prolonged with stenosis and/or occlusion of large vessels. In order to allow detection of both small and large vessel disease simultaneously for SCA, we employed a 4D whole-brain ASL protocol to measure CBF and ATT concurrently. Methods Experiments were performed on a 3T Philips Achieva scanner using a 32-channel head coil for reception. Seven healthy volunteers (age 36 ± 8 yrs; 3M / 4F) and 5 SCA patients with no history of stroke, recent transfusion, or renal impairment (21 ± 3 yrs; 3M / 2F) were enrolled after informed consent. The clinical laboratory performed complete blood counts on venous blood obtained the same day. The Pseudo continuous ASL (PCASL) sequence was implemented with 1000ms labeling duration and 12 PLDs (from 500 ms to 2700 ms in 200 ms intervals). A 3D acquisition scheme was employed with a field of view of 240 x 240 x 140 mm3 and acquisition resolution = 6.7 x 7.4 x 7 mm3. Total scan time was about 5 min. Fitting was performed per voxel using nonlinear-least-squares algorithm (Matlab) and maps of CBF and ATT were extracted concurrently. For each subject, five ROIs in the gray matter (frontal lobe, temporal lobe, parietal lobe, occipital lobe, and cerebellum) were manually drawn bilaterally from the corresponding anatomical image. Results Figure 1 display representative CBF and ATT maps of a participant with SCA estimated from the multi-delay PCASL scans. CBF maps (Figure 1a) were found to be uniform within the gray matter. White matter has lower CBF than gray matter and shows a longer transit time as expected. ATT maps (Figure 1b) reflected the heterogeneity between different brain regions. ATT values were about 200-400 ms shorter in the temporal lobe and medial frontal lobe, compared to the parietal/occipital lobes and cerebellum. Averaged CBF values from the five ROIs of all the subjects were calculated. For the control group (Hb = 14.1 ± 1.5 g/dL) and SCA group (Hb = 9.1 ± 2.1 g/dL), the mean CBF values were 49 ± 15 mL/100g/min vs 102 ± 23 mL/100g/min, and the mean ATT values were 1662 ± 317 ms vs 1245 ± 171 ms, respectively. Linear regression identified significant correlations between mean CBF and hemoglobin: CBF = - 9.8 Hb + 189 (r = -0.94; p < 0.001) and ATT = 81.2 Hb + 511 (r = 0.76; p = 0.004) (Figure 2). Discussion We have successfully implemented a fast and non-invasive MRI technique to measure two perfusion metrics (CBF and ATT) with a whole-brain coverage on SCA patients. It was well established only using other perfusion imaging modalities that CBF, among subjects with normal hemodynamic regulation and without neurovascular impairment, is inversely correlated with hemoglobin concentration. Conversely, a linear correlation between ATT and Hb is expected as a result of adaptive vasodilatation and lower blood viscosity. Our study's cross-subject validation of this relationship using the multi-delay PCASL method with 3D acquisition shows the potential of this technique to accurately define blood flow from both small and large vessels. This may be useful to identify people with SCA at increased risk of brain injury from silent cerebral infarct and stroke. Figure 1 Representative CBF (a) and ATT (b) maps acquired with 3D whole-brain coverage in axial, coronal and sagittal planes. Figure 1. Representative CBF (a) and ATT (b) maps acquired with 3D whole-brain coverage in axial, coronal and sagittal planes. Figure 2 Linear relationship between Hb and (a) CBF; (b) ATT. Figure 2. Linear relationship between Hb and (a) CBF; (b) ATT. Disclosures No relevant conflicts of interest to declare.

Abstract 13327: Worse Cerebral Blood Flow in Single Right verses Left Ventricle After Fontan Completion

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Nancy A PIKE ◽  
Bhaswati Roy ◽  
Nancy J Halnon ◽  
Alan B Lewis ◽  
Mary A Woo ◽  
...  
Keyword(s):  
Blood Flow ◽  
Cerebral Blood Flow ◽  
Left Ventricle ◽  
Brain Mri ◽  
Region Of Interest ◽  
Healthy Controls ◽  
Cross Sectional ◽  
Common Space ◽  
Variable Function ◽  
Brain Changes

Introduction: Single ventricle heart disease (SVHD) adolescents with a single right ventricle (RV) have worse cognition and mood function compared to single left ventricle (LV) which may result from variability in ventricular function or structure related sequela after Fontan completion. However, it is unclear whether RV SVHD has worse cerebral blood flow (CBF) in cognitive and mood regulatory areas over LV SVHD compared to healthy controls. Methods: Cross-sectional, comparative design, 14 adolescents with RV SVHD (age 16.1±1.5 years; 7 male), 6 LV SVHD (age, 16.3±1.0 years; 4 male), and 25 healthy controls (age, 15.9±1.4 years; 13 male) were studied. SVHD participants were recruited who have undergone surgical palliation with Fontan completion from local pediatric cardiology clinics. Self-reported healthy controls were recruited from the community. Brain MRI studies were performed using a 3.0-Tesla MRI scanner and 3D pseudo-continuous arterial spin labelling data were collected. We calculated whole-brain CBF maps, normalized to a common space, and assessed brain changes between RV and LV SVHD and controls [ANCOVA; covariates, age and sex; p<0.005]. Region of interest analyses were performed in cognitive and mood control brain sites. Results: Regional brain CBF was reduced in single RV over LV SVHD. Multiple brain sites showed more widespread reduced CBF values in RV over LV SVHD compared to controls (Figure 1, p<0.005), including the prefrontal cortices, caudate, insula (a, f), anterior (b, g), mid (c, h), and posterior (d, i) cingulate, and hippocampus (e, j). Conclusion: Single RV adolescents show more widespread reduced CBF than single LV in cognitive and mood regulatory sites, which may result from variable function or structure related sequela between ventricle types. The findings indicate that the therapeutic approach should recognize the differences in CBF based on ventricle type and investigate interventions to optimize CBF in single RV SVHD.

scholarly journals Altered cerebral blood flow in patients with unilateral venous pulsatile tinnitus: an arterial spin labeling study

2021 ◽  
pp. 20200990
Author(s):  
Xiaoshuai Li ◽  
Pengfei Zhao ◽  
Xiaoyu Qiu ◽  
Han Lv ◽  
Heyu Ding ◽  
...  
Keyword(s):  
Blood Flow ◽  
Cerebral Blood Flow ◽  
Neuronal Activity ◽  
Arterial Spin Labeling ◽  
Brain Perfusion ◽  
Spin Labeling ◽  
Pulsatile Tinnitus ◽  
Lingual Gyrus ◽  
Healthy Control ◽  
The Mean

Objectives: Abnormal neuronal activity and functional connectivity have been reported in patients with venous pulsatile tinnitus (PT). As neuronal activity is closely coupled to regional brain perfusion, the purpose of this study was to investigate the cerebral blood flow (CBF) alterations in patients with unilateral venous PT using arterial spin labeling (ASL). Methods: This study included patients with right-sided PT between January 2018 and July 2019. A healthy control (HC) group matched 1:1 for gender and age was also recruited. All subjects underwent ASL scanning using 3.0T MRI. The correlation between altered CBF and Tinnitus Handicap Inventory (THI) score as well as PT duration was analyzed. Results Twenty-one patients with right-sided PT and 21 HCs were included. The mean PT duration of the patients was 35.9 ± 32.2 months, and the mean THI score was 64.1 ± 20.3. Compared with the HCs, the PT patients exhibited increased CBF in the left inferior parietal gyrus and decreased CBF in the bilateral lingual gyrus (family-wise error corrected, p < 0.05). The increased CBF in the left inferior parietal gyrus showed a positive correlation with the THI score in PT patients (r = 0.501, p = 0.021). Conclusions PT patients exhibit regional CBF alterations. The increased CBF in the left inferior parietal gyrus may reflect the severity of PT. Advances in knowledge: This study not only presents evidence for the potential neuropathology of PT from the perspective of CBF alterations but also offers a new method for investigating the neuropathological mechanism of PT.

Author response: Increased resting cerebral blood flow in adult Fabry disease: MRI arterial spin labeling study

Neurology ◽  
2018 ◽  
Vol 91 (23) ◽  
pp. 1072-1072
Author(s):  
David John Werring ◽  
Aine Merwick ◽  
Indran Davagnanam ◽  
Po Phyu ◽  
Fay Bolsover ◽  
...  
Keyword(s):  
Blood Flow ◽  
Cerebral Blood Flow ◽  
Fabry Disease ◽  
Arterial Spin Labeling ◽  
Spin Labeling ◽  
Author Response

Heterogeneity of Cerebral Blood Flow: a Fractal Approach

2000 ◽  
Vol 39 (02) ◽  
pp. 37-42 ◽  
Author(s):  
P. Hartikainen ◽  
J. T. Kuikka
Keyword(s):  
Blood Flow ◽  
Cerebral Blood Flow ◽  
Fractal Dimension ◽  
Frontal Lobe ◽  
Fractal Analysis ◽  
Relative Dispersion ◽  
Emission Computed Tomography ◽  
Brain Blood Flow ◽  
Healthy Controls ◽  
Fractal Approach

Summary Aim: We demonstrate the heterogeneity of regional cerebral blood flow using a fractal approach and singlephoton emission computed tomography (SPECT). Method: Tc-99m-labelled ethylcysteine dimer was injected intravenously in 10 healthy controls and in 10 patients with dementia of frontal lobe type. The head was imaged with a gamma camera and transaxial, sagittal and coronal slices were reconstructed. Two hundred fifty-six symmetrical regions of interest (ROIs) were drawn onto each hemisphere of functioning brain matter. Fractal analysis was used to examine the spatial heterogeneity of blood flow as a function of the number of ROIs. Results: Relative dispersion (= coefficient of variation of the regional flows) was fractal-like in healthy subjects and could be characterized by a fractal dimension of 1.17 ± 0.05 (mean ± SD) for the left hemisphere and 1.15 ± 0.04 for the right hemisphere, respectively. The fractal dimension of 1.0 reflects completely homogeneous blood flow and 1.5 indicates a random blood flow distribution. Patients with dementia of frontal lobe type had a significantly lower fractal dimension of 1.04 ± 0.03 than in healthy controls. Conclusion: Within the limits of spatial resolution of SPECT, the heterogeneity of brain blood flow is well characterized by a fractal dimension. Fractal analysis may help brain scientists to assess age-, sex- and laterality-related anatomic and physiological changes of brain blood flow and possibly to improve precision of diagnostic information available for patient care.

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