Deep Medullary Vein Research Articles

Deep medullary vein abnormalities impact white matter hyperintensity volume through increases in interstitial free water

BackgroundOur intent was to explore the mediating role of interstitial free water (FW) linking deep medullary vein (DMV) score to white matter hyperintensity (WMH) volume.MethodsOur research team conducted a forward-looking analysis of initial clinical and imaging information gathered from 125 patients with cerebral small vessel disease. We identified six anatomic DMV regions on susceptibility weighted imaging (SWI) studies. Each region earned a score of 0–3, determined by the visual conditions of vessels, summing all six to generate a DMV score. We utilized fluid-attenuated inversion recovery (FLAIR) sequences to measure the volume of WMH. Additionally, we employed diffusion tensor imaging (DTI) to assess FW value.ResultsDMV score significantly positively correlated with FW value and with WMH volume (p < 0.05), and value of FW positively correlated with WMH volume (p < 0.05). The indirect effect of DMV score on WMH volume was mediated by FW (β = 0.281, 95% confidence interval [CI]: 0.178–0.388), whether adjusted for age and gender (β = 0.142, 95% CI: 0.058–0.240) or for age, gender and vascular risk factors (β = 0.141, 95% CI: 0.054–0.249).ConclusionDMV score correlate with WMH volume by virtue of FW increases in white matter.

Cerebral venous thrombosis and deep medullary vein thrombosis: Padua experience over the last two decades.

Cerebral venous thrombosis (CVT) is a cerebrovascular disorder that accounts for 20% of perinatal strokes. CVT incidence ranges from 0.67 to 1.12 per 100,000 newborns, while the incidence of "deep medullary vein thrombosis" (DMVT), a subtype of CVT, cannot be accurately estimated. This study aims to analyze the case history of CVT in the neonatal period, with a specific focus on DMVT. Newborns diagnosed with CVT, with or without DMVT, between January 2002 and April 2023, were collected using the Italian Registry of Infantile Thrombosis (RITI). Cerebral MRIs were reviewed by an expert neuroradiologist following a standardized protocol. Forty-two newborns with CVT were identified, of which 27/42 (64%) had CVT, and the remaining 15/42 (36%) had DMVT (isolated DMVT in 9/15). Symptom onset occurred in the first week of life (median 8days, IQR 4-14) with a male prevalence of 59%. The most common risk factors for CVT were complicated delivery (38%), prematurity (40%), congenital heart diseases (48%), and infections (40%). Seizures were the predominant presenting symptom in 52% of all cases. Hemorrhagic infarction was higher in cases with isolated DMVT (77%) compared to patients with CVT without DMVT (p = 0.013). Antithrombotic treatment was initiated in 36% of patients. Neurological impairment was observed in 48% of cases at discharge, while 18 out of 31 infants (58%) presented one or more neurological deficits at long term follow up. Conclusion:DMVT occurs in over a third of neonates with CVT. Multicentric studies are essential to establish standardized protocols for therapy, neuroimaging, and follow-up in these patients.

Association of ADAMTS13 activity with cerebral deep medullary vein: A community-based cross-sectional study

ObjectivesThis study aims to investigate whether circulating ADAMTS13 activity can offer insights into the mechanism of pathophysiological changes in deep medullary veins (DMVs). MethodsThis study was conducted on a community cohort of elderly individuals in Shanghai. Plasma von Willebrand factor (VWF) levels and ADAMTS13 activity were measured. A validated DMV score described the overall burden of DMV on the brain. Through ordinal regression models, we investigated the correlation between VWF levels, ADAMTS13 activity, and increasing severity of DMV score while adjusting for demographics and cardiovascular risk factors. ResultsThe study enrolled 262 subjects according to the inclusion criteria. The mean VWF level (1.35 ± 0.25) was higher in the DMV group than in the group without DMV (1.25 ± 0.30) (p = 0.025), and ADAMTS13 activity (83.76 ± 7.96) was relatively lower. After adjusting for age, sex, alcohol consumption, smoking, hypertension, and diabetes, reduced ADAMTS13 activity [β = −7.78; 95 % CI (−10.21, −5.35) p < 0.01] was associated with DMV. Moreover, correlation analysis indicated that ADAMTS13 activity was negatively correlated with the DMV score (Kendall's tau-b = −0.53, p < 0.001). DiscussionIn summary, there was an inverse correlation observed between ADAMTS13 activity and the DMV score, which may provide some clinical clues for exploring the potential pathogenesis of DMV.

Deep medullary vein damage correlates with small vessel disease in small vessel occlusion acute ischemic stroke

ObjectivesWe aim to investigate whether cerebral small vessel disease (cSVD) imaging markers correlate with deep medullary vein (DMV) damage in small vessel occlusion acute ischemic stroke (SVO-AIS) patients.MethodsThe DMV was divided into six segments according to the regional anatomy. The total DMV score (0–18) was calculated based on segmental continuity and visibility. The damage of DMV was grouped according to the quartiles of the total DMV score. Neuroimaging biomarkers of cSVD including white matter hyperintensity (WMH), cerebral microbleed (CMB), perivascular space (PVS), and lacune were identified. The cSVD score were further analyzed.ResultsWe included 229 SVO-AIS patients, the mean age was 63.7 ± 23.1 years, the median NIHSS score was 3 (IQR, 2–6). In the severe DMV burden group (the 4th quartile), the NIHSS score grade (6 (3–9)) was significantly higher than other groups (p < 0.01). The grade scores for basal ganglia PVS (BG-PVS) were positively correlated with the degree of DMV (R = 0.67, p < 0.01), rather than centrum semivole PVS (CS-PVS) (R = 0.17, p = 0.1). In multivariate analysis, high CMB burden (adjusted odds ratio [aOR], 25.38; 95% confidence interval [CI], 1.87–345.23) was associated with severe DMV scores. In addition, BG-PVS was related to severe DMV burden in a dose-dependent manner: when BG-PVS score was 3 and 4, the aORs of severe DMV burden were 18.5 and 12.19, respectively.ConclusionThe DMV impairment was associated with the severity of cSVD, which suggests that DMV burden may be used for risk stratification in SVO-AIS patients.Clinical relevance statementThe DMV damage score, based on the association between small vessel disease and the deep medullary veins impairment, is a potential new imaging biomarker for the prognosis of small vessel occlusion acute ischemic stroke, with clinical management implications.Key Points• The damage to the deep medullary vein may be one mechanism of cerebral small vessel disease.• Severe burden of the basal ganglia perivascular space and cerebral microbleed is closely associated with significant impairment to the deep medullary vein.• The deep medullary vein damage score may reflect a risk of added vascular damage in small vessel occlusion acute ischemic stroke patients.

Abstract WP227: Deep Medullary Vein Thrombosis in Neonates

Introduction: Deep medullary vein(DMV) thrombosis is a rare cause of neurological damage noted in both term and preterm infants. Although their incidence in children is not know, DMV thrombosis can occur isolated or in association with cerebral sinovenous thrombosis (CSVT). The spectrum of parenchymal radiological findings in DMV thrombosis includes edema and hemorrhage of variable extent. No clinical trials or definite treatment algorithms are available for DMV thrombosis in neonates. The choice of antithrombotic therapy is therefore based on the experience of the single centers or on indications extrapolated from available guidelines on CSVT. Aims: There are limited guidelines for the management of DMV thrombosis in literature. Recently published systematic review in neonates found a total of only 75 cases in literature. In none of these cases treatment options were described. We aim to present a single institute experience describing 3 cases of neonatal DMV thrombosis and different management methods. Methods and findings: Between July-August 2023, we prospectively studied 3 neonates with deep medullary vein thrombosis as evidenced by characteristic hemorrhage noted on MRI. For the 1st patient no anticoagulation was initially started given co-morbid conditions and critical illness. However, repeat imaging showed worsening of hemorrhage, thereby indicating progression of thrombosis resulting in venous hemorrhage. Pt was therefore stared on AC with LMWH. No worsening hemorrhage was noted thereafter. For the 2nd patient, AC was not started &amp; no progression of thrombosis was noted. For the 3rd patient AC was started from a cardiac standpoint and no worsening in hemorrhage was noted. Observation: 1. Anticoagulation in patient with progression of DMV thrombosis did not worsen existing hemorrhage and prevented further propagation of clot. 2. No anticoagulation did not result in worsening of thrombosis and it likely self resolved in the absence of other comorbid conditions. 3. Anticoagulation used for a different indication did not worsen existing intraparenchymal hemorrhage. Next steps: 1. To conduct a retrospective chart review on a larger cohort of patients. This will help future studies and management options for this rare condition.

Deep vein diameters and perivascular space scores are associated with deep medullary vein hypo-visibility in patients with white matter hyperintensity.

Deep medullary vein (DMV) hypo-visibility is correlated with white matter hyperintensity (WMH), but the underlying causes remain unclear. This study aimed to explore the relationship between deep vein diameters and perivascular space (PVS) scores, and DMV hypo-visibility in the presence of WMH. This cross-sectional study prospectively analyzed the clinical and imaging data of 190 cerebral small vessel disease patients with WMH and 40 healthy controls from the Lishui Hospital of Traditional Chinese Medicine affiliated with Zhejiang Chinese Medical University. PVS scores ranging from 0 to 4 were determined according to the PVS counts in the basal ganglia area on T2-weighted magnetic resonance images; high-grade PVS was defined as a PVS score >1. The diameters of the deep cerebral veins, including the bilateral septal veins (SVs), thalamostriate veins (TSVs), lateral ventricular veins (LVVs), and internal cerebral veins, were measured using susceptibility weighted imaging (SWI). Left and right DMV scores, ranging from 0 to 9, were calculated based on the visibility of the DMV on SWI in the ipsilateral frontal, parietal, and occipital lobes. The deep cerebral vein diameters, left and right DMV scores, and high-grade PVS differed between the healthy controls and WMH patients (P<0.05). Left DMV scores were independently associated with age {β [95% confidence interval (CI)]: 0.050 (0.018, 0.082)}, high-grade PVS [β (95% CI): 0.998 (0.262, 1.737)], and the diameters of the ipsilateral SVs [β (95% CI): -1.114 (-1.754, -0.475)], SVs [β (95% CI): -0.734 (-1.191, -0.277)], and LVVs [β (95% CI): -0.921 (-1.567, -0.275)] [all false discovery rate (FDR)-corrected P<0.05]. Right DMV scores were independently associated with age [β (95% CI): 0.071 (0.037, 0.105)], high-grade PVS [β (95% CI): 0.873 (0.111, 1.635)], and the diameters of the ipsilateral SVs [β (95% CI): -0.837 (-1.386, -0.289)], TSVs [β (95% CI): -0.875 (-1.331, -0.419)], and LVVs [β (95% CI): -1.813 (-2.484, -1.142)] (all FDR-corrected P<0.05). Decreased hypo-visibility of DMVs on SWI was associated with a higher age, the presence of high-grade PVS, and smaller diameters of the ipsilateral deep cerebral veins in individuals with WMH. Our findings provide novel insights into the probable mechanisms leading to high DMV scores.

Quantification of enlarged deep medullary vein volumes in Sturge-Weber syndrome.

Enlarged deep medullary veins (EDMVs) in patients with Sturge-Weber syndrome (SWS) may channel venous blood from the surface to the deep vein system in brain regions affected by the leptomeningeal venous malformation. Thus, the quantification of EDMV volume may provide an objective imaging marker for this vascular compensatory process. The present study proposes a novel analytical method to quantify enlarged EDMV volumes in the affected hemisphere of patients with unilateral SWS. Twenty young subjects, including 10 patients with unilateral SWS and 10 healthy siblings (age 14.5±6.7 and 16.0±7.0 years, respectively) underwent 3T brain MRI scanning using susceptibility-weighted imaging (SWI) and volumetric T1-weighted sequences. The proposed image analytic steps segmented EDMVs in white matter regions, defined on the volumetric T1-weighted images, by statistically associating the likelihood of intensity, location, and tubular shape on SWI. The volumes of the segmented EDMVs, calculated in each hemisphere, were compared between affected and unaffected hemispheres. EDMV volumes were also correlated with visually assessed EDMV scores, hemispheric white matter volumes, and cortical surface areas. Parametric tests including Pearson's correlation, unpaired and paired t-tests, were used. A P value <0.05 was considered statistically significant. It was found that EDMVs were identified well in SWS-affected hemispheres while calcified regions were excluded. Mean EDMV volumes in the SWS-affected hemispheres were 10-12-fold greater than in the unaffected or healthy control hemispheres; while white matter volumes and cortical surface areas were lower. EDMV volumes in the SWS-affected hemispheres showed a strong positive correlation with the visual EDMV scores (r=0.88, P=0.001) and an inverse correlation with cortical surface area ratios (r=-0.65, P=0.04) but no correlation with white matter volume ratios. EDMVs were detected in the SWS-affected atrophic hemispheres reliably while avoiding calcified regions. The approach can be used to quantify enlarged deep cerebral veins in the human brain, which may provide a potential marker of cerebral venous remodeling.

Deep medullary veins score is associated with atrophy in patients with cerebral small vessel disease.

To explore the relationship between the deep medullary vein (DMV) score and atrophy in patients with cerebral small vessel disease (CSVD). Imaging and clinical data from 125 patients with CSVD from January to December 2022 were reviewed. Normalized gray matter volume (GM_N) was calculated by dividing the gray matter volume by the whole brain volume. DMV scoring is conducted using susceptibility-weighted magnetic resonance imaging, wherein the DMV area is partitioned into six distinct regions: bilateral frontal, parietal, and occipital regions. Each region undergoes assessment based on the clarity and consistency of DMV visibility. Subsequently, the scores from these six regions are summed, resulting in a score ranging from 0 to 18 points. DMV score was associated with GM_N (r = -0.376, p < 0.001). Comparisons among patients according to GM_N tertiles, differences in gender, age, current smoking, DMV score, and total CSVD magnetic resonance imaging score were demonstrated (p < 0.05). Adjusting for age, gender, vascular risk factors, and total CSVD MR score, the DMV score was independently associated with GM_N [β (95% CI): -0.347 (-0.525, -0.168), p < 0.001]. DMV scores are independently associated with GM_N, and DMV dysfunction may play a role in brain atrophy.

Advancements in investigating the role of cerebral small vein loss in Alzheimer's disease-related pathological changes.

Alzheimer's disease (AD) is the prevailing type of dementia in the elderly, yet a comprehensive comprehension of its precise underlying mechanisms remains elusive. The investigation of the involvement of cerebral small veins in the advancement of AD has yet to be sufficiently explored in previous studies, primarily due to constraints associated with pathological staining techniques. However, recent research has provided valuable insights into multiple pathophysiological occurrences concerning cerebral small veins in AD, which may manifest sequentially, concurrently, or in a self-perpetuating manner. These events are presumed to be among the initial processes in the disease's progression. The impact of cerebral small vein loss on amyloid beta (Aβ) clearance through the glial lymphatic system is noteworthy. There exists a potential interdependence between collagen deposition and Aβ deposition in cerebral small veins. The compromised functionality of cerebral small veins can result in decreased cerebral perfusion pressure, potentially leading to cerebral tissue ischemia and edema. Additionally, the reduction of cerebral small veins may facilitate the infiltration of inflammatory factors into the brain parenchyma, thereby eliciting neuroinflammatory responses. Susceptibility-weighted imaging (SWI) is a valuable modality for the efficient assessment of cerebral small veins, precisely the deep medullary vein (DMV), and holds promise for the identification of precise and reliable imaging biomarkers for AD. This review presents a comprehensive overview of the current advancements and obstacles to the impairment of cerebral small veins in AD. Additionally, we emphasize future research avenues and the importance of conducting further investigations in this domain.

Impact of Decreased Visibility of Deep Medullary Veins on White Matter Integrity in Patients with Cerebral Small Vessel Disease.

Based on susceptibility-weighted imaging (SWI) visibility, deep medullary vein (DMV) scores are related to white matter damage (WMD) in patients with cerebral small vessel disease (CSVD). However, whether mechanisms are associated with DMV changes is unclear. We examined extracellular fluid (ECF) roles in white matter associations between DMV scores and white matter integrity (WMI) in patients with CSVD. We examined magnetic resonance imaging (MRI) and clinical data from 140 patients with CSVD. DMV scores (0-18) were assigned on SWI according to DMV anatomic regions and signal continuity/visibility. WMI and ECF volumes were evaluated using free water (FW) and fractional anisotropy (FA) values by diffusion tensor imaging (DTI). DMV scores were independently associated with FA after adjusting for vascular risk factors, age, white matter hyperintensity (WMH) volume, and CSVD burden [β (95% confidence interval (CI)): -0.219 (-0.375, -0.061), p = 0.006]. We also observed a significant indirect effect of DMV scores on FA in white matter (mediated by FW in white matter) after controlling for age, vascular risk factors, WMH volume, and CSVD burden. DMV scores were independently related to WMI and mediated by ECF in the white matter of patients with CSVD.

Deep Medullary Vein and MRI Markers Were Related to Cerebral Hemorrhage Subtypes.

To explore the performance of deep medullary vein (DMV) and magnetic resonance imaging (MRI) markers in different intracerebral hemorrhage (ICH) subtypes in patients with cerebral small vessel disease (CSVD). In total, 232 cases of CSVD with ICH were included in this study. The clinical and image data were retrospectively analyzed. Patients were divided into hypertensive arteriopathy (HTNA)-related ICH, cerebral amyloid angiopathy (CAA)-related ICH, and mixed ICH groups. The DMV score was determined in the cerebral hemisphere contralateral to the ICH. The DMV score was different between the HTNA-related and mixed ICH groups (p < 0.01). The MRI markers and CSVD burden score were significant among the ICH groups (p < 0.05). Compared to mixed ICH, HTNA-related ICH diagnosis was associated with higher deep white matter hyperintensity (DWMH) (OR: 0.452, 95% CI: 0.253-0.809, p < 0.05) and high-degree perivascular space (PVS) (OR: 0.633, 95% CI: 0.416-0.963, p < 0.05), and CAA-related ICH diagnosis was associated with increased age (OR: 1.074; 95% CI: 1.028-1.122, p = 0.001). The DMV score correlated with cerebral microbleed (CMB), PVS, DWMH, periventricular white matter hyperintensity (PWMH), and CSVD burden score (p < 0.05) but not with lacuna (p > 0.05). Age was an independent risk factor for the severity of DMV score (OR: 1.052; 95% CI: 0.026-0.076, p < 0.001). DMV scores, CSVD markers, and CSVD burden scores were associated with different subtypes of ICH. In addition, DMV scores were associated with the severity of CSVD and CSVD markers.

T1w/T2w ratio is associated with the brush sign and perfusion delay in watershed regions in patients with moyamoya angiopathy

BackgroundIt has been shown recently using the T1w/T2w mapping technique that white matter microstructural integrity impairments exist in watershed regions patients with moyamoya angiopathy (MMA). We hypothesized that these changes could be associated with the prominence of other neuroimaging markers of chronic brain ischemia, such as perfusion delay and the brush sign. MethodsThirteen adult patients with MMA (24 affected hemispheres) were evaluated using brain MRI and CT perfusion. The T1w/T2w signal intensity ratio, which reflects white matter integrity, was calculated in watershed regions (centrum semiovale and middle frontal gyrus). Brush sign prominence was evaluated with susceptibility-weighted MRI. Additionally, brain perfusion parameters such as cerebral blood flow (CBF), cerebral blood volume (CBF), and mean transit time (MTT) were assessed. Correlations between white matter integrity and perfusion changes in watershed regions, as well as the prominence of the brush sign, were evaluated. ResultsStatistically significant negative correlations between the prominence of the brush sign and the T1w/T2w ratio values from the centrum semiovale and middle frontal white matter were found (R = −0.62 to 0.71, adjusted p < 0.05). Furthermore, there was a positive correlation between the T1w/T2w ratio values and the MTT values from the centrum semiovale (R = 0.65, adjusted p < 0.05). ConclusionsWe revealed that T1w/T2w ratio changes are associated with the prominence of the brush sign as well as white matter hypoperfusion in watershed regions in patients with MMA. This could be explained by chronic ischemia due to venous congestion in the deep medullary vein territory.

Effect of dynamic cerebral autoregulation on the association between deep medullary vein changes and cerebral small vessel disease.

Changes in the deep medullary vein (DMV) are reported to be associated with cerebral small vessel disease (CSVD). While the mechanisms of this association are unclear, dynamic cerebral autoregulation (dCA) has been speculated to participate in this association. Thus, we aimed to verify the association between DMV changes and total CSVD burden and further investigate the effect of dCA function on this correlation. In this prospective study, 95 Asian patients aged ≥18 years were included in the final assessment. DMV scores and total CSVD burden were determined using magnetic resonance imaging sequences. Transfer function analysis was performed to analyze dCA function. Generalized linear regressions were used to assess the relationship between DMV changes and total CSVD burden as well as between DMV changes and dCA function. An interaction model was utilized to assess the effect of dCA function on the association between DMV changes and total CSVD burden. Generalized linear models showed a significant positive association between DMV changes and total CSVD burden (p = 0.039) and a significant negative association between DMV changes and dCA function (p = 0.018). The interaction model demonstrated a significant positive interaction of dCA impairment on the association between DMV changes and the total CSVD burden (p = 0.02). Thus, we came to the conclusion that changes in DMV were correlated independently with both CSVD and dCA impairment and furthermore, impaired dCA function play an interaction effect on the association between DMV changes and the total CSVD burden. Our results can help improve the understanding of the complex pathogenesis and progression of CSVD, thereby facilitating early intervention and treatment development.

Neuroimaging findings related to glymphatic system alterations in older adults with metabolic syndrome

ObjectiveThe glymphatic system is a glial-based perivascular network that promotes brain metabolic waste clearance. Reduced glymphatic flow has been observed in rat models of type 2 diabetes and hypertension, indicating the role of vascular risk factors in the glymphatic system. However, little is known about how vascular risk factors affect the human glymphatic system. The present study aims to assess the relationships between metabolic syndrome (MetS), a cluster of vascular risk factors, and the glymphatic system function using diffusion magnetic resonance imaging (MRI)-based measures of water diffusivity in the glymphatic compartments, including the brain interstitial space and perivascular spaces around the deep medullary vein. We hypothesized that vascular risk factors are associated with glymphatic dysfunction, leading to cognitive impairment in older adults. MethodsThis cross-sectional study assessed 61 older adults (age range, 65–82 years) who had participated in the Bunkyo Health Study, including 15 healthy controls (mean age, 70.87 ± 4.90 years) and 46 individuals with MetS (mean age, 71.76 ± 4.61 years). Fractional volume of extracellular-free water (FW) and an index of diffusion tensor imaging along the perivascular space (DTI-ALPS) were used as indirect indicators of water diffusivity in the interstitial extracellular and perivenous spaces of white matter, respectively. ResultsAfter adjusting for age, sex, years of education, total Fazekas scale, Pittsburgh sleep quality index (PSQI) score, and intracranial volume (ICV), a significantly (P = 0.030; Cohen's d = 1.01) higher FW was observed in individuals with MetS than in the healthy controls. Furthermore, individuals with MetS had a significantly (P = 0.031; Cohen's d = 0.86) lower ALPS index than the healthy controls, with age, sex, years of education, total Fazekas scale, PSQI score, ICV, fractional anisotropy, and mean diffusivity included as confounding factors. Higher FW was significantly associated with lower ALPS index (r = −0.37; P = 0.004). Multiple linear regression (MLR) with backward elimination analyses showed that higher diastolic blood pressure (BP; standardized β = 0.33, P = 0.005) was independently associated with higher FW, whereas higher fasting plasma glucose levels (standardized β = −0.63, P = 0.002) or higher Brinkman index of cigarette consumption cumulative amount (standardized β = −0.27, P = 0.022) were associated with lower ALPS index. The lower ALPS index (standardized β, 0.28; P = 0.040) was associated with poorer global cognitive performance, which was determined using the Japanese version of the Montreal Cognitive Assessment (MOCA-J) scores. Finally, partial correlation analyses showed a significant correlation between higher FW and lower MOCA-J scores (r = −0.35; P = 0.025) and between higher FW and higher diastolic BP (r = 0.32, P = 0.044). ConclusionThe present study shows the changes in diffusion MRI-based measures reflected by the higher FW and lower ALPS index in older adults with MetS, possibly due to the adverse effect of vascular risk factors on the glymphatic system. Our findings also indicate the associations between the diffusion MRI-based measures and elevated diastolic BP, hyperglycemia, smoking habit, and poorer cognitive performance. However, owing to the limitations of this study, the results should be cautiously interpreted.

Brain Deep Medullary Veins on 7T MRI in Dutch-Type Hereditary Cerebral Amyloid Angiopathy.

Deep medullary vein (DMV) changes occur in cerebral small vessel diseases (SVD) and in Alzheimer's disease. Cerebral amyloid angiopathy (CAA) is a common SVD that has a high co-morbidity with Alzheimer's disease. So far, DMVs have not been evaluated in CAA. To evaluate DMVs in Dutch-type hereditary CAA (D-CAA) mutation carriers and controls, in relation to MRI markers associated with D-CAA. Quantitative DMV parameters length, tortuosity, inhomogeneity, and density were quantified on 7 Tesla 3D susceptibility weighted MRI in pre-symptomatic D-CAA mutation carriers (n = 8), symptomatic D-CAA mutation carriers (n = 8), and controls (n = 25). Hemorrhagic MRI markers (cerebral microbleeds, intracerebral hemorrhages, cortical superficial siderosis, convexity subarachnoid hemorrhage), non-hemorrhagic MRI markers (white matter hyperintensities, enlarged perivascular spaces, lacunar infarcts, cortical microinfarcts), cortical grey matter perfusion, and diffusion tensor imaging parameters were assessed in D-CAA mutation carriers. Univariate general linear analysis was used to determine associations between DMV parameters and MRI markers. Quantitative DMV parameters length, tortuosity, inhomogeneity, and density did not differ between pre-symptomatic D-CAA mutation carriers, symptomatic D-CAA mutation carriers, and controls. No associations were found between DMV parameters and MRI markers associated with D-CAA. This study indicates that vascular amyloid-β deposition does not affect DMV parameters. In patients with CAA, DMVs do not seem to play a role in the pathogenesis of MRI markers associated with CAA.

Baseline Hemodynamic Impairment and Revascularization Outcome in Newly Diagnosed Adult Moyamoya Disease Determined by Pseudocontinuous Arterial Spin Labeling

The study aimed to investigate the hemodynamic features and independent predictors of neoangiogenesis after revascularization in moyamoya disease (MMD) by pseudocontinuous arterial spin labeling magnetic resonance imaging (pCASL MRI). Thirty-nine MMD patients were categorized into infarction group, hemorrhagic group, and atypical group. All patients underwent combined bypass surgery and pCASL MRI with postlabeling delays (PLD) of 1525 ms and 2525 ms. Absolute CBFMCA (cerebral blood flow in middle cerebral artery territory), relative CBFMCA (CBFMCA 2525 ms/CBFMCA 1525 ms), and spatial coefficient of variation of MCA (CoVMCA) were analyzed. Relationships between CBFMCA and the following clinical parameters were assessed: Suzuki stage, modified Rankin scale (mRS), cerebrovascular accident lesion score, and deep medullary veins score. Potential predictors for favorable neoangiogenesis and hemodynamic changes were explored as well. Preoperative CBFMCA differed among MMD patients with variable clinical presentations, Matsushima stages, modified Rankin Scale scores, CVA scores, and deep medullary vein scores. After bypass surgery, mean CBFMCA increased significantly in the infarction group (P= 0.027) and decreased in the hemorrhagic group (P= 0.043), while spatial CoVMCA was observed to decline in all groups. Higher preoperative relative CBFMCA and spatial CoVMCA were independent predictors for robust neoangiogenesis after bypass. The cutoff value of 0.330 of spatial CoVMCA at long PLD yielded the best sensitivity at 82.1% and specificity at 81.8%. Furthermore, both preoperative relative CBFMCA and spatial CoVMCA showed mild positive correlations with ΔmRS in MMD patients. pCASL-MRI with multiple PLDs could reflect preoperative hemodynamic impairment and predict the neoangiogenesis after combined bypass surgery in moyamoya patients.

Deep Medullary Vein White Matter Injury Global Severity Score Predicts Neurodevelopmental Impairment.

To examine associations between the deep medullary vein white matter injury global severity scoring system and neurodevelopmental impairment. This is a prospective observational cohort study of infants born at ≥32 weeks, diagnosed with deep medullary vein thrombosis and infarction on neuroimaging in the first month of life. Developmental testing was performed using validated measures for early, preschool, and school-age follow-up. Nineteen (37%) patients had major neurodevelopmental impairment. Global severity score was higher among patients with neurodevelopmental impairment (21.6 vs 13.4, P = .04). Overall, 78% of patients with epilepsy had neurodevelopmental impairment. A greater degree of asymmetry with right-sided injury predominance was associated with lower Bayley-III cognitive scores and presence of neurodevelopmental impairment (P < .01). Results suggest a need for targeted clinical surveillance for patients with a high global severity score and/or asymmetric, predominantly right cerebral white matter injury and for those who develop epilepsy.

New Insights in Addressing Cerebral Small Vessel Disease: Association With the Deep Medullary Veins.

ObjectiveTo assess the suitability of deep medullary vein visibility in susceptibility weighted imaging—magnetic resonance imaging studies as a method for the diagnosis and evaluation of cerebral small vessel disease progression.MethodsA total of 92 patients with CSVD were enrolled and baseline clinical and imaging data were reviewed retrospectively. Neuroimaging biomarkers of CSVD including high-grade white matter hyperintensity (HWMH), cerebral microbleed (CMB), enlarged perivascular space (PVS), and lacunar infarct (LI) were identified and CSVD burden was calculated. Cases were grouped accordingly as mild, moderate, or severe. The DMV was divided into six segments according to the regional anatomy. The total DMV score (0–18) was calculated as the sum of the six individual segmental scores, which ranged from 0 to 3, for a semi-quantitative assessment of the DMV based on segmental continuity and visibility.ResultsThe DMV score was independently associated with the presence of HWMH, PVS, and LI (P < 0.05), but not with presence and absence of CMB (P > 0.05). Correlation between the DMV score and the CSVD burden was significant (P < 0.05) [OR 95% C.I., 1.227 (1.096–1.388)].ConclusionThe DMV score was associated with the presence and severity of CSVD.

Deep medullary vein engorgement and superficial medullary vein engorgement: two patterns of perinatal venous stroke.

Perinatal venous stroke has classically been attributed to cerebral sinovenous thrombosis with resultant congestion or thrombosis of the small veins draining the cerebrum. Advances in brain MRI, in particular susceptibility-weighted imaging, have enabled the visualization of the engorged small intracerebral veins, and the spectrum of perinatal venous stroke has expanded to include isolated congestion or thrombosis of the deep medullary veins and the superficial intracerebral veins. Congestion or thrombosis of the deep medullary veins or the superficial intracerebral veins can result in vasogenic edema, cytotoxic edema or hemorrhage in the territory of disrupted venous flow. Deep medullary vein engorgement and superficial medullary vein engorgement have characteristic findings on MRI and should be differentiated from neonatal hemorrhagic stroke.

MR Imaging Scoring System for White Matter Injury after Deep Medullary Vein Thrombosis and Infarction in Neonates.

Advanced imaging techniques have allowed earlier and more accurate detection of cerebral deep medullary vein thrombosis and infarction. Our objective was to develop an MR imaging scoring system to evaluate the severity of white matter injury in neonates with deep medullary vein thrombosis and infarction. This was a retrospective study of infants born ≥32 weeks' gestation (2000-2016) diagnosed with deep medullary vein thrombosis and infarction on neuroimaging in the first 30 days of life. A 102-point deep medullary vein white matter injury global severity score was developed. MR images were scored by 2 pediatric radiologists. Subject clinical data and regional and global severity scores were recorded. Fifty-one patients (mean gestational age, 37.3 ± 2.2 weeks; mean birth weight, 3182 ± 720 g) were included with a mean age at diagnosis via MR imaging of postnatal day 10.1 ± 6.1. Global severity scores ranged from 1 to 53, with a median score of 11 (interquartile range, 5-25). Lesions were more common in the frontal and parietal regions and less common in the occipital and temporal regions. Fifty-five percent of the group had neonatal seizures. No difference in perinatal risk factors (gestational age, birthweight, 5-minute Apgar score, chorioamnionitis, delivery room resuscitation, ventilator, or inotrope requirement) was observed among severity score quartiles. An MR imaging scoring system provides a comprehensive and objective classification of WM injury after deep medullary vein thrombosis and infarction in late preterm and term neonates. The global severity score is independent of gestational age and other antenatal risk factors, consistent with presentation in previously healthy-appearing neonates.