Functional MRI, DTI and neurophysiology in horizontal gaze palsy with progressive scoliosis

Introduction: Horizontal gaze palsy with progressive scoliosis (HGPPS) is an autosomal recessive disease due to a mutation in the ROBO3 gene. This rare disease is of particular interest because the absence, or at least reduction, of crossing of the ascending lemniscal and descending corticospinal tracts in the medulla predicts abnormal ipsilateral sensory and motor systems. Methods: We evaluated the use of functional magnetic resonance imaging (fMRI) for the first time in this disease and compared it to diffusion tensor imaging (DTI) tractography and neurophysiological findings in the same patient with genetically confirmed ROBO3 mutation. Results: As expected, motor fMRI, somatosensory evoked potentials (SSEP) and motor evoked potentials (MEP) were dominantly ipsilateral to the stimulation side. DTI tractography revealed ipsilateral ascending and descending connectivity in the brainstem yet normal interhemispheric connections in the corpus callosum. Auditory fMRI revealed bilateral auditory activation to monaural left-sided auditory stimulation. No significant cortical activation was observed after monaural right-sided stimulation, a hearing defect having been excluded. Prosaccades fMRI showed no activations in the eye-movement network. Conclusion: Motor fMRI confirmed the established findings of DTI and neurophysiology in the same patient. In suspected HGPPS, any technique appears appropriate for further investigation. Auditory fMRI suggests that a monaural auditory system with bilateral auditory activations might be a physiological advantage as compared to a binaural yet only unilateral auditory system, in analogy to anisometropic amblyopia. Moving-head fMRI studies in the future might show whether the compensatory head movements instead of normal eye movements activate the eye-movement networ

Investigation of the critical heat flux in a rod bundle configuration under low pressure conditions

Diverse boiling phenomena occur during the operation of light-water reactors. Their understanding is necessary to guarantee a safe service and to avoid unstable operating modes. For example, the comportment of the coolant could either be subcooled boiling during normal operation or even critical boiling during the occurrence of a disturbance. Besides, boiling effects also appear on the secondary loop of the steam generator. The boiling process allows significantly higher heat transfer rates compared to the single-phase convection. But this heat transport can be suddenly decreased when the limit of the critical heat flux (CHF) is reached. The occurrence of the boiling crisis leads generally to severe damage of the facility components and has to be avoided during reactor operation. Until today, there is no reliable method predicting this phenomenon based on universally valid correlations. A substantial benefit for the reactor safety research would be a prediction method which is based on the solution of the transport equations for the two-phase flow of water and steam. There exist many correlations based on observations in experiments or theoretical reflections which try to explain the occurrence and the development of the critical heat flux. Unfortunately, they cannot be combined to one complete model as they are counter-predicting effects or are set up on different physical effects. For example, the ‘Near Wall Bubble Crowding Model’ [Kandlikar, S. G., 2011] postulates the decrease of the liquid flow to the wall due to turbulence with increasing heat flux as bubbles will concentrate near the wall. Whereas the ‘Interfacial Lift-Off Model’ [Galloway, J., Mudawar, I., 1993] predicts pseudo-periodic ‘wetting-fronts’ which cause the agglomeration of steam leading to the CHF as these zones lift off from the wall. Using the COSMOS-L test facility, IKET at KIT tries to contribute to analyzing the different existing theories and to examine specific phenomena like flow pattern or void distribution for flow boiling

In vivo visualization and analysis of 3-D hemodynamics in cerebral aneurysms with flow-sensitized 4-D MR imaging at 3T

Introduction: Blood-flow patterns and wall shear stress (WSS) are considered to play a major role in the development and rupture of cerebral aneurysms. These hemodynamic aspects have been extensively studied in vitro using geometric realistic aneurysm models. The purpose of this study was to evaluate the feasibility of in vivo flow-sensitized 4-D MR imaging for analysis of intraaneurysmal hemodynamics. Methods: Five cerebral aneurysms were examined using ECG-gated, flow-sensitized 4-D MR imaging at 3T in three patients. Postprocessing included quantification of flow velocities, visualization of time-resolved 2-D vector graphs and 3-D particle traces, vortical flow analysis, and estimation of WSS. Flow patterns were analyzed in relation to aneurysm geometry and aspect ratio. Results: Magnitude, spatial and temporal evolution of vortical flow differed markedly among the aneurysms. Particularly unstable vortical flow was demonstrated in a wide-necked parophthalmic ICA aneurysm (high aspect ratio). Relatively stable vortical flow was observed in aneurysms with a lower aspect ratio. Except for a wide-necked cavernous ICA aneurysm (low aspect ratio), WSS was reduced in all aneurysms and showed a high spatial variation. Conclusion: In vivo flow-sensitized 4-D MR imaging can be applied to analyze complex patterns of intraaneurysmal flow. Flow patterns, distribution of flow velocities, and WSS seem to be determined by the vascular geometry of the aneurysm. Temporal and spatial averaging effects are drawbacks of the MR-based analysis of flow patterns as well as the estimation of WSS, particularly in small aneurysms. Further studies are needed to establish a direct link between definitive flow patterns and different aneurysm geometrie

Time-resolved 3D contrast-enhanced MRA with GRAPPA on a 1.5-T system for imaging of craniocervical vascular disease: initial experience

Introduction: For three-dimensional (3D) imaging with magnetic resonance angiography (MRA) of the cerebral and cervical circulation, both a high temporal and a high spatial resolution with isovolumetric datasets are of interest. In an initial evaluation, we analyzed the potential of contrast-enhanced (CE) time-resolved 3D-MRA as an adjunct for neurovascular MR imaging. Methods: In ten patients with various cerebrovascular disorders and vascularized tumors in the cervical circulation, high-speed MR acquisition using parallel imaging with the GeneRalized Autocalibrating Partially Parallel Acquisitions (GRAPPA) algorithm on a 1.5-T system with a temporal resolution of 1.5s per dataset and a nearly isovolumetric spatial resolution was applied. The results were assessed and compared with those from conventional MRA and digital subtraction angiography (DSA). Results: CE time-resolved 3D-MRA enabled the visualization and characterization of high-flow arteriovenous shunts in cases of vascular malformations or hypervascularized tumors. In steno-occlusive disease, the method provided valuable additional information about altered vessel perfusion compared to standard MRA techniques such as time-of-flight (TOF) MRA. The use of a nearly isovolumetric voxel size allowed a free-form interrogation of 3D datasets. Its comparatively low spatial resolution was found to be the major limitation. Conclusion: In this preliminary analysis, CE time-resolved 3D-MRA was revealed to be a promising complementary MRA sequence that enabled the visualization of contrast flow dynamics in various types of neurovascular disorders and vascularized cervical tumor

Muscular involvement assessed by MRI correlates to motor function measurement values in oculopharyngeal muscular dystrophy

Oculopharyngeal muscular dystrophy (OPMD) is a progressive skeletal muscle dystrophy characterized by ptosis, dysphagia, and upper and lower extremity weakness. We examined eight genetically confirmed OPMD patients to detect a MRI pattern and correlate muscle involvement, with validated clinical evaluation methods. Physical assessment was performed using the Motor Function Measurement (MFM) scale. We imaged the lower extremities on a 1.5T scanner. Fatty replacement was graded on a 4-point visual scale. We found prominent affection of the adductor and hamstring muscles in the thigh, and soleus and gastrocnemius muscles in the lower leg. The MFM assessment showed relative mild clinical impairment, mostly affecting standing and transfers, while distal motor capacity was hardly affected. We observed a high (negative) correlation between the validated clinical scores and our visual imaging scores suggesting that quantitative and more objective muscle MRI might serve as outcome measure for clinical trials in muscular dystrophie

MTR variations in normal adult brain structures using balanced steady-state free precession

Introduction: Magnetization transfer (MT) is sensitive to the macromolecular environment of water protons and thereby provides information not obtainable from conventional magnetic resonance imaging (MRI). Compared to standard methods, MT-sensitized balanced steady-state free precession (bSSFP) offers high-resolution images with significantly reduced acquisition times. In this study, high-resolution magnetization transfer ratio (MTR) images from normal appearing brain structures were acquired with bSSFP. Methods: Twelve subjects were studied on a 1.5T scanner. MTR values were calculated from MT images acquired in 3D with 1.3mm isotropic resolution. The complete MT data set was acquired within less than 3.5 min. Forty-one brain structures of the white matter (WM) and gray matter (GM) were identified for each subject. Results: MTR values were higher for WM than GM. In general, MTR values of the WM and GM structures were in good accordance with the literature. However, MTR values showed more homogenous values within WM and GM structures than previous studies. Conclusions: MT-sensitized bSSFP provides isotropic high-resolution MTR images and hereby allows assessment of reliable MTR data in also very small brain structures in clinically feasible acquisition times and is thus a promising sequence for being widely used in the clinical routine. The present normative data can serve as a reference for the future characterization of brain pathologie

Rheological Changes After Stenting of a Cerebral Aneurysm: A Finite Element Modeling Approach

Hemodynamic changes in intracranial aneurysms after stent placement include the appearance of areas with stagnant flow and low shear rates. We investigated the influence of stent placement on blood flow velocity and wall shear stress of an intracranial aneurysm using a finite element modeling approach. To assess viscosity changes induced by stent placement, the rheology of blood as non-Newtonian fluid was taken into account in this model. A two-dimensional model with a parent artery, a smaller branching artery, and an aneurysm located at the bifurcation, before and after stent placement, was used for simulation. Flow velocity plots and wall shear stress before and after stent placement was calculated over the entire cardiac circle. Values for dynamic viscosity were calculated with a constitutive equation that was based on experimental studies and yielded a viscosity, which decreases as the shear rate increases. Stent placement lowered peak velocities in the main vortex of the aneurysm by a factor of at least 4 compared to peak velocities in the main artery, and it considerably decreased the wall shear stress of the aneurysm. Dynamic viscosity increases after stent placement persisted over a major part of the cardiac cycle, with a factor of up to 10, most pronounced near the dome of the aneurysm. Finite element modeling can offer insight into rheological changes induced by stent treatment of aneurysms and allows visualizing dynamic viscosity changes induced by stent placemen

Lobar Dementia due to Extreme Widening of Virchow-Robin Spaces in One Hemisphere

Widened perivascular spaces known as Virchow-Robin spaces (VRS) are often seen on MRI and are usually incidental findings. It is unclear if enlarged VRS can be associated with neurological deficits. In this report, we describe a case of lobar dementia associated with unusual VRS widening in one cerebral hemisphere. A 77-year-old woman, seen at a memory clinic, presented with progressive cognitive decline, left hemianopsia, and mild pyramidal signs on the left side. On MRI, unusually wide VRS were visible, predominantly in the right centrum semiovale and the right temporo-occipital white matter. The clinical syndrome was consistent with the extent and location of the abnormally dilated VRS. The high MR signal in white matter bridges between the VRS suggested parenchymal damage, possibly representing gliotic white matter. No evidence for another etiology was found on cerebral MRI and rCBF SPECT. As a conclusion, enlarged VRS in one cerebral hemisphere may be associated with cognitive change and neurological deficits

Patients profiling for Botox® (onabotulinum toxin A) treatment for migraine: a look at white matter lesions in the MRI as a potential marker

BACKGROUND: To evaluate if white matter lesions (WML) on MRI can be a potential marker for onabotulinum toxin A (Botox®) treatment success in migraine, given the limited response rate and high costs per treatment. METHODS: Retrospective data base and MRI analysis of 529 migraineurs who received Botox® between 2002 and 2009. Responders were defined as patients who underwent three or more treatments, whereas non-responders had only one or two treatments. MRIs were analysed on axial T2 and coronar FLAIR (fluid attenuated inversion recovery) sequences for the presence of WML. Statistical analysis was done with the Chi-Square-Test and the Mann-Whitney-U-Test. RESULTS: Of 529 Botox® treated migraineurs, 111 patients had a MRI. Of these 111 patients, 47 were responders, 64 non-responders to Botox®. Response rate to Botox® in migraineurs with WML was 55.3%, in migraineurs without WML 44.7%. In the investigated items "age", "age at onset", "gender", "attack duration", "frequency", "aura", "WML", "size of WML", we found no statistical significant difference between the two groups. 55% of the responders and 50% of the non-responders showed WML. All WML were located supratentorially, anteriorly, mostly of small size (3-5 mm). CONCLUSION: WML on MRIs cannot serve as a marker to predict a positive response to Botox®

Flow diversion treatment: intra-aneurismal blood flow velocity and WSS reduction are parameters to predict aneurysm thrombosis

Background: To evaluate the haemodynamic changes induced by flow diversion treatment in cerebral aneurysms, resulting in thrombosis or persisting aneurysm patency over time. Method: Eight patients with aneurysms at the para-ophthalmic segment of the internal carotid artery were treated by flow diversion only. The clinical follow-up ranged between 6days and 12months. Computational fluid dynamics (CFD) analysis of pre- and post-treatment conditions was performed in all cases. True geometric models of the flow diverter were created and placed over the neck of the aneurysms by using a virtual stent-deployment technique, and the device was simulated as a true physical barrier. Pre- and post-treatment haemodynamics were compared, including mean and maximal velocities, wall-shear stress (WSS) and intra-aneurysmal flow patterns. The CFD study results were then correlated to angiographic follow-up studies. Results: Mean intra-aneurysmal flow velocities and WSS were significantly reduced in all aneurysms. Changes in flow patterns were recorded in only one case. Seven of eight aneurysms showed complete occlusion during the follow-up. One aneurysm remaining patent after 1year showed no change in flow patterns. One aneurysm rupturing 5days after treatment showed also no change in flow pattern, and no change in the maximal inflow velocity. Conclusions: Relative flow velocity and WSS reduction in and of itself may result in aneurysm thrombosis in the majority of cases. Flow reductions under aneurysm-specific thresholds may, however, be the reason why some aneurysms remain completely or partially patent after flow diversio