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Simple Summary: The identification of gliomas and the differentiation between different types is essential to evaluate patients' prognosis and guide optimal clinical management. The ideal multiparametric magnetic resonance imaging (MRI) protocol for the assessment of gliomas is a current topic of research. This study aimed to explore the performance of dynamic contrast-enhanced (DCE) MRI and diffusion kurtosis imaging (DKI) in differentiating molecular subtypes of adult-type diffuse gliomas. The results showed that a combined evaluation of DCE-MRI and DKI parameters reveals the best prediction of high-grade vs. low-grade gliomas, IDH1/2 wildtype vs. mutated gliomas, and astrocytomas/glioblastomas vs. oligodendrogliomas. The aim was to explore the performance of dynamic contrast-enhanced (DCE) MRI and diffusion kurtosis imaging (DKI) in differentiating the molecular subtypes of adult-type gliomas. A multicenter MRI study with standardized imaging protocols, including DCE-MRI and DKI data of 81 patients with WHO grade 2–4 gliomas, was performed at six centers. The DCE-MRI and DKI parameter values were quantitatively evaluated in ROIs in tumor tissue and contralateral normal-appearing white matter. Binary logistic regression analyses were performed to differentiate between high-grade (HGG) vs. low-grade gliomas (LGG), IDH1/2 wildtype vs. mutated gliomas, and high-grade astrocytic tumors vs. high-grade oligodendrogliomas. Receiver operating characteristic (ROC) curves were generated for each parameter and for the regression models to determine the area under the curve (AUC), sensitivity, and specificity. Significant differences between tumor groups were found in the DCE-MRI and DKI parameters. A combination of DCE-MRI and DKI parameters revealed the best prediction of HGG vs. LGG (AUC = 0.954 (0.900–1.000)), IDH1/2 wildtype vs. mutated gliomas (AUC = 0.802 (0.702–0.903)), and astrocytomas/glioblastomas vs. oligodendrogliomas (AUC = 0.806 (0.700–0.912)) with the lowest Akaike information criterion. The combination of DCE-MRI and DKI seems helpful in predicting glioma types according to the 2021 World Health Organization's (WHO) classification. [ABSTRACT FROM AUTHOR]

Simple Summary: Interest in applying artificial intelligence to medical imaging to enhance image quality has grown in both clinical practice and research. Nonetheless, these artificial intelligence models require further validation for use in diagnosing and monitoring disease progression to effectively compare them with traditional techniques. This study aimed to assess the effectiveness of a deep learning-optimized glioma protocol for MRI scans in patients with glioblastoma. The study protocol focuses on improving image quality and shortening scan time, which might be particularly beneficial for critically ill or uncooperative patients. The results showed that deep learning-reconstructed sequences can save scan time and simultaneously increase subjective image quality but with less subjective image noise. In addition, tumor volume measurements and neuro-oncologic response assessments were not affected negatively by deep learning. This study demonstrated the feasibility of the deep learning protocol for MRI of glioblastomas. A fully diagnostic MRI glioma protocol is key to monitoring therapy assessment but is time-consuming and especially challenging in critically ill and uncooperative patients. Artificial intelligence demonstrated promise in reducing scan time and improving image quality simultaneously. The purpose of this study was to investigate the diagnostic performance, the impact on acquisition acceleration, and the image quality of a deep learning optimized glioma protocol of the brain. Thirty-three patients with histologically confirmed glioblastoma underwent standardized brain tumor imaging according to the glioma consensus recommendations on a 3-Tesla MRI scanner. Conventional and deep learning-reconstructed (DLR) fluid-attenuated inversion recovery, and T2- and T1-weighted contrast-enhanced Turbo spin echo images with an improved in-plane resolution, i.e., super-resolution, were acquired. Two experienced neuroradiologists independently evaluated the image datasets for subjective image quality, diagnostic confidence, tumor conspicuity, noise levels, artifacts, and sharpness. In addition, the tumor volume was measured in the image datasets according to Response Assessment in Neuro-Oncology (RANO) 2.0, as well as compared between both imaging techniques, and various clinical–pathological parameters were determined. The average time saving of DLR sequences was 30% per MRI sequence. Simultaneously, DLR sequences showed superior overall image quality (all p < 0.001), improved tumor conspicuity and image sharpness (all p < 0.001, respectively), and less image noise (all p < 0.001), while maintaining diagnostic confidence (all p > 0.05), compared to conventional images. Regarding RANO 2.0, the volume of non-enhancing non-target lesions (p = 0.963), enhancing target lesions (p = 0.993), and enhancing non-target lesions (p = 0.951) did not differ between reconstruction types. The feasibility of the deep learning-optimized glioma protocol was demonstrated with a 30% reduction in acquisition time on average and an increased in-plane resolution. The evaluated DLR sequences improved subjective image quality and maintained diagnostic accuracy in tumor detection and tumor classification according to RANO 2.0. [ABSTRACT FROM AUTHOR]

Purpose: Patients with Moyamoya Angiopathy (MMA) require hemodynamic assessment to evaluate the risk of stroke. Hemodynamic evaluation by use of breath-hold-triggered fMRI (bh-fMRI) was proposed as a readily available alternative to the diagnostic standard [15O]water PET. Recent studies suggest voxel-wise hemodynamic delay correction in hypercapnia-triggered fMRI. The aim of this study was to evaluate the effect of delay correction of bh-fMRI in patients with MMA and to compare the results with [15O]water PET. Methods: bh-fMRI data sets of 22 patients with MMA were evaluated without and with voxel-wise delay correction within different shift ranges and compared to the corresponding [15O]water PET data sets. The effects were evaluated combined and in subgroups of data sets with most severely impaired CVR (apparent steal phenomenon), data sets with territorial time delay, and data sets with neither steal phenomenon nor delay between vascular territories. Results: The study revealed a high mean cross-correlation (r = 0.79, p < 0.001) between bh-fMRI and [15O]water PET. The correlation was strongly dependent on the choice of the shift range. Overall, no shift range revealed a significantly improved correlation between bh-fMRI and [15O]water PET compared to the correlation without delay correction. Delay correction within shift ranges with positive high high cutoff revealed a lower agreement between bh-fMRI and PET overall and in all subgroups. Conclusion: Voxel-wise delay correction, in particular with shift ranges with high cutoff, should be used critically as it can lead to false-negative results in regions with impaired CVR and a lower correlation to the diagnostic standard [15O]water PET. [ABSTRACT FROM AUTHOR]

This study aims to describe the posterolateral epidural supra-C2-root approach (PESCA), which might be a good alternative to the transoral, anterolateral, and other posterolateral approaches for biopsy of lesions of the odontoid process (OP). The preoperative planning of PESCA included computerized tomography (CT), CT-angiography, and three-dimensional reconstruction (if possible, even with three-dimensional print) to analyze the angle of the trajectory and the anatomy of the vertebral artery (VA). For PESCA, the patient is positioned under general anesthesia in prone position. In case of an osteolytic lesion with fracture of the OP, an X-ray is performed after positioning to verify anatomic alignment. In the first step, in case of instability and compression of the spinal cord, a craniocervical fusion and decompression is performed (laminectomy of the middle part of the C1 arc and removal of the lower part of the lateral C1 arc). The trajectory is immediately above the C2 root (and under the upper rest of the lateral part of C1 arc). Even if the trajectory is narrowed, it is possible to perform PESCA without relevant traction of the spinal cord. The vertical segment of V3 of the VA at the level of C2 is protected by the vertebral foramen, and the horizontal part of V3 is protected by the remnant upper lateral part of the C1 arc (in case of normal variants). PESCA might be a good choice for biopsy of selected lesions of the OP in same sitting procedure after craniocervical stabilization and decompression. [ABSTRACT FROM AUTHOR]

In diffusion MRI, the advent of high angular resolution diffusion imaging (HARDI) and HARDI with compressed sensing (HARDI+CS) has led to clinically practical signal acquisition techniques which allow for the assessment of white matter architecture in routine patient studies. However, the reconstruction and visualization of fiber pathways by tractography has not yet been established as a standard methodology which can easily be applied. This is due to various algorithmic problems, such as a lack of robustness, error propagation and the necessity of fine-tuning parameters depending on the clinical question. In the framework of a clinical study of glioma patients, we compare two different whole-brain tracking methods to a local connectivity mapping approach which has recently shown promising results in an adaptation to diffusion MRI. The ability of the three methods to correctly depict fiber affection is analyzed by comparing visualization results to representations of local diffusion profiles provided by orientation distribution functions (ODFs). Our results suggest that methods beyond fiber tractography, which visualize local connectedness rather than global connectivity, should be evaluated further for pre-surgical assessment of fiber affection. [ABSTRACT FROM AUTHOR]

Flow diverters (FDs) are designed for the endovascular treatment of complex intracranial aneurysm configurations. From February 2009 to March 2013 28 patients (22 females, 6 males) were treated with FD; mean age was 57 years. Data, including aneurysm features, clinical presentation, history of previous bleeding, treatment, and follow-up results, are presented. Early postinterventional neurological deficits (transient: n=3/enduring: n=1) appeared in 4/28 patients (14%), and early improvement of neurological symptoms was observed in 7 patients with previous restriction of cranial nerve function. The overall occlusion rate was 20/26 (77%; 59% after 3 months). 77% achieved best results according to O’Kelly-Marotta score grade D with no contrast material filling (70% of those after 3 months). In 4/6 patients who did not achieve grade D, proximal and/or distal stent overlapping ≥5 mm was not guaranteed sufficiently. During follow-up we did not detect any aneurysm recurrence or haemorrhage. In-stent stenosis emerged as the most frequent complication (4/27; 15%) followed by 2 cases of vascular obliteration (AICA/VA). In conclusion endovascular reconstruction using a FD represents a modern and effective treatment in those aneurysms that are not suitable for conventional interventional or surgical treatment. The appearance of severe complications was rare. [ABSTRACT FROM AUTHOR]

Very preterm (PT) birth (≤32 weeks of gestation) carries a high risk for an adverse neurodevelopmental outcome. In recent years, the importance of neurocognitive deficits in the language domain has been increasingly recognized, which can be well-characterized using neuropsychological testing and noninvasive imaging approaches. We compared former early PT born children and adolescents (PT, n = 29, 20M) and typically developing children (TD, n = 19, 7M), using conventional fMRI group analyses as well as functional connectivity analyses. We found only small regions with significantly different group activation (PT > TD) but significantly stronger connectivity between superior temporal lobe (STL) language regions in TD participants. There were also significant differences in local and global network efficiency (TD > PT). Surprisingly, there was a stronger connectivity of STL regions with non-STL regions both intrahemispherically and interhemispherically in PT participants, suggesting the coexistence of reduced and increased connectivity in the language network of former PTs. Very similar results were obtained when using task-based versus resting state functional connectivity approaches. Finally, lateralization of functional connectivity correlated with verbal comprehension abilities, suggesting that a more bilateral language comprehension representation is associated with better performance. Our results underline the importance of interhemispheric crosstalk for language comprehension. Hum Brain Mapp 35:3372-3384, 2014. © 2013 Wiley Periodicals, Inc. [ABSTRACT FROM AUTHOR]

To assess the clinical spectrum of ataxia and cerebellar oculomotor deficits in the most common spinocerebellar ataxias (SCAs), we analysed the baseline data of the EUROSCA natural history study, a multicentric cohort study of 526 patients with either spinocerebellar ataxia type 1, 2, 3 or 6. To quantify ataxia symptoms, we used the Scale for the Assessment and Rating of Ataxia (SARA). The presence of cerebellar oculomotor signs was assessed using the Inventory of Non-Ataxia Symptoms (INAS). In a subgroup of patients, in which magnetic resonance images (MRIs) were available, we correlated MRI morphometric measures with clinical signs on an exploratory basis. The SARA subscores posture and gait (items 1-3), speech (item 4) and the limb kinetic subscore (items 5-8) did not differ between the genotypes. The scores of SARA item 3 (sitting), 5 (finger chase) and 6 (nose-finger test) differed between the subtypes whereas the scores of the remaining items were not different. In SCA1, ataxia symptoms were correlated with brainstem atrophy and in SCA3 with both brainstem and cerebellar atrophy. Cerebellar oculomotor deficits were most frequent in SCA6 followed by SCA3, whereas these abnormalities were less frequent in SCA1 and SCA2. Our data suggest that vestibulocerebellar, spinocerebellar and pontocerebellar circuits in SCA1, SCA2, SCA3 and SCA6 are functionally impaired to almost the same degree, but at different anatomical levels. The seemingly low prevalence of cerebellar oculomotor deficits in SCA1 and SCA2 is most probably related to the defective saccadic system in these disorders. [ABSTRACT FROM AUTHOR]

Abstract: Sensory representations in the postcentral gyrus are supposed to be strictly lateralised and to provide spatially unbiased representations of limb positions. However, electrophysiological and behavioural measurements in humans and non-human primates tentatively suggested some degree of bilateral processing even in early somatosensory areas. We report a patient who suffered a small and confined lesion of the hand area in the postcentral gyrus that resulted in a proprioceptive deficit without any concomitant primary motor impairment. We performed a finger position-matching task with target locations being defined proprioceptively. Without visual feedback of either hand, the patient demonstrated a significant leftward shift of perceived locations when reaching with the ipsilesional right hand to her contralesional left hand and an opposite rightward shift when reaching with the left hand to the position of the right hand. Although these directional errors improved when vision of the active hand was allowed, errors were still significantly larger than those of age-matched healthy controls with unconstrained view of the active contralesional hand. Reaching to visual targets without visual online feedback the patient revealed comparable errors with both hands. Reaching to visual targets with full visual feedback, she was as accurate as controls with either hand. In summary, our data demonstrate an effect of the right postcentral lesion on proprioceptive information processing for both hands. The results suggest an integration of contralateral and ipsilateral proprioceptive information already at this early processing stage possibly mediated by callosal connections. [Copyright &y& Elsevier]

Magnetic resonance (MR) imaging is widely used to visualize atrophic processes that occur during the pathogenesis of spinocerebellar ataxias (SCAs). T1-weighted images are utilized to rate the atrophy of cerebellar vermis, cerebellar hemispheres, pons and midbrain. Signal changes in the basal ganglia and ponto-cerebellar fibers are evaluated by T2-weighted and proton density-weighted images. However, two-dimensional (2D) images do not allow a reliable quantification of the degree of atrophy. The latter is now possible through the application of three-dimensional (3D) true volumetric methods, which should be used for research purposes. Ideally, these methods should allow automated segmentation of contrast-defined boundaries by using region growing algorithms, which can be applied successfully in structures of the posterior fossa and basal ganglia. Thin slice thickness helps to minimize partial volume effects. Whereas volumetric approaches rely on predetermined anatomical boundaries, voxel-based morphometry has been developed to determine group differences between different types of SCA (cross-sectional studies) or within one SCA entity (longitudinal studies). We will review recent results and how these methods are currently used to (i) separate sporadic and dominantly inherited forms of cerebellar ataxias; (ii) identify specific SCA genotypes; (iii) correlate patho-anatomical changes with SCA disease symptoms or severity; and (iv) visualize and estimate the rate of progression in SCA. [ABSTRACT FROM AUTHOR]

To visualize and quantify disease progression in multiple system atrophy (MSA) from cerebellar type (MSA-C), we combined two magnetic resonance imaging (MRI) techniques, voxel-based morphometry (VBM) and 3D-based volumetry. Patients suffering from MSA-C (n = 14) were imaged twice with an interval of 2.0 ± 0.2 years. We first applied VBM to map brain morphology changes between MSA patients and controls and to identify brain areas that showed a significant amount of atrophy. Using 3D-based volumetry, we confirmed that in MSA-C patients, the brainstem including medulla and pons, vermis and cerebellar hemispheres, caudate nucleus and putamen showed significant atrophy compared with controls. Next, we used 3D-based volumetry to analyze the atrophy rates. Atrophy rates in patients with MSA were significantly different from controls for putamen (−11.4% ± 2.6%/year), vermis (−12.3% ± 2.9%/year), and cerebellar hemispheres (−6.6% ± 1.1%/year). The results show that 3D-based MRI volumetry is a tool that allows the disease progression of MSA to be followed over a time period of 2 years and suggest that it may serve as a surrogate marker in clinical trials to measure disease progression. © 2006 Movement Disorder Society [ABSTRACT FROM AUTHOR]

Because there are no biological markers for the clinical diagnosis of progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD), we established a mathematical model based on three-dimensional magnetic resonance (MR) imaging to differentiate between these parkinsonian disorders. Using MR imaging-based volumetry we studied the pattern of atrophic changes in patients with probable, possible or definite PSP (n = 33) or CBD (n = 18). Patients were compared with 22 controls with similar age. To establish a mathematical model that would allow for differentiation of PSP, CBD and controls we performed a discriminant analysis. We found a significant reduction in average brain, brainstem, midbrain and frontal gray matter volumes in patients with PSP, whereas patients with CBD showed atrophy of parietal cortex and corpus callosum. With the exception of reduced midbrain volumes in PSP, the measured volumes of anatomical structures showed an extensive overlap with the normal range on an individual basis. Using only post mortem confirmed cases of PSP (n = 8) and CBD (n = 7) as well as all controls, the volumes of midbrain, parietal white matter, temporal gray matter, brainstem, frontal white matter and pons were identified to separate best between groups and were used to construct a model with two canonical variables. This model allowed to correctly predict the diagnosis in 95% of controls as well as in 76% of all PSP and 83% of all CBD patients. Similar results were obtained only when patients with a possible and probable diagnosis of PSP and CBD, who were not involved in the development of the discriminant analysis, were classified. 3D-MR imaging-based volumetry may help to differentiate PSP from CBD ante mortem. [Copyright &y& Elsevier]

Endothelial dysfunction represents a potential pathomechanism of neurological post-COVID-19 syndrome (PCS). A recent study demonstrated reduced cerebrovascular reactivity (CVR) in patients with PCS. The aim of this pilot study was to prospectively assess CVR in patients with PCS using breath-hold functional MRI (bh-fMRI). Fourteen patients with neurological PCS and leading symptoms of fatigue/memory issues/concentration disorder (PCSfmc), 11 patients with PCS and leading symptoms of myopathy/neuropathy (PCSmn), and 17 healthy controls underwent bh-fMRI. Signal change and time to peak (TTP) were assessed globally and in seven regions of interest and compared between the subgroups using one-way ANCOVA adjusting for age, time since infection, Fazekas score, and sex. No significant differences were observed. In PCS patients, the global CVR exhibited a slight, non-significant tendency to be lower compared to healthy controls (PCSfmc: 0.78 ± 0.11%, PCSmn: 0.84 ± 0.10% and 0.87 ± 0.07%). There was a non-significant trend towards lower global TTP values in the PCS subgroups than in the control group (PCSfmc: 26.41 ± 1.39 s, PCSmn: 26.32 ± 1.36 s versus 29.52 ± 0.93 s). Endothelial dysfunction does not seem to be the sole pathomechanism of neurological symptoms in PCS. Further studies in larger cohorts are required. [ABSTRACT FROM AUTHOR]

Current standard care for acute cerebral venous sinus thrombosis (CVST) includes either intravenous heparin or subcutaneous low-molecular-weight heparin, but patients with refractory CVST, despite adequate anticoagulation therapy, may benefit from mechanical thrombectomy (MT). A retrospective study of patients with CVST, who underwent MT between 2011 and 2019, was performed looking at procedure success rate and clinical outcomes. Two raters evaluated the cerebral venous system of every patient before and after the intervention using the following scoring system: (0) No obvious thrombosis; (1) thrombosis without impaired blood flow; (2) thrombosis with impaired blood flow; (3) and thrombosis with complete vascular occlusion. The success of MT was measured using a score quotient (Q = A/B), dividing the sum of the patient's scores after the intervention (A) by the sum of scores before the intervention (B). Overall, 21 MTs were performed on 20 patients with refractory or severe CVST. Clinical improvement was seen in 61.9% during hospital stay and in 80% at 6-month follow-up, with complete recovery in 70% of patients. Patients with favorable outcomes had significantly lower recanalization quotients (p = 0.008). Our study provides evidence supporting that MT may be a safe and effective treatment with favorable clinical outcomes for selected patients with CVST. [ABSTRACT FROM AUTHOR]

Before revascularization, moyamoya patients require hemodynamic evaluation. In this study, we evaluated the scoring system Prior Infarcts, Reactivity and Angiography in Moyamoya Disease (PIRAMID). We also devised a new scoring system, MRI-Based Assessment of Risk for Stroke in Moyamoya Angiopathy (MARS-MMA), and compared the scoring systems with respect to the capability to predict impaired [15O]water PET cerebral perfusion reserve capacity (CPR). We evaluated 69 MRI, 69 DSA and 38 [15O]water PET data sets. The PIRAMID system was validated by ROC curve analysis with neurological symptomatology as a dependent variable. The components of the MARS-MMA system and their weightings were determined by binary logistic regression analysis. The comparison of PIRAMID and MARS-MMA was performed by ROC curve analysis. The PIRAMID score correlated well with the symptomatology (AUC = 0.784). The MARS-MMA system, including impaired breath-hold-fMRI, the presence of the Ivy sign and arterial wall contrast enhancement, correlated slightly better with CPR impairment than the PIRAMID system (AUC = 0.859 vs. 0.827, Akaike information criterion 140 vs. 146). For simplified clinical use, we determined three MARS-MMA grades without loss of diagnostic performance (AUC = 0.855). The entirely MRI-based MARS-MMA scoring system might be a promising tool to predict the risk of stroke. [ABSTRACT FROM AUTHOR]

Purpose: Vessel-encoded arterial spin labeling (VE-ASL) is able to provide noninvasive information about the contribution of individual arteries to the cerebral perfusion. The aim of this study was to compare VE-ASL to the diagnostic standard digital subtraction angiography (DSA) with respect to its ability to visualize vascular territories. Methods: In total, 20 VE-ASL and DSA data sets of 17 patients with Moyamoya angiopathy with and without revascularization surgery were retrospectively analyzed. Two neuroradiologists independently assessed the agreement between VE-ASL and DSA using a 4-point Likert scale (no- very high agreement). Additionally, grading of the vascular supply of subterritories (A1-A2, M1-M6) on the VE-ASL images and angiograms was performed. The intermodal agreement was calculated for all subterritories in total and for the subdivision into without and after revascularization (direct or indirect bypass). Results: There was a very high agreement between the VE-ASL and the DSA data sets (median = 1, modus = 1) with a substantial inter-rater agreement (kw = 0.762 (95% CI 0.561–0.963)). The inter-modality agreement between VE-ASL and DSA in vascular subterritories was almost perfect for all subterritories (k = 0.899 (0.865–0.945)), in the subgroup of direct revascularized subterritories (k = 0.827 (0.738–0.915)), in the subgroup of indirect revascularized subterritories (k = 0.843 (0.683–1.003)), and in the subgroup of never revascularized subterritories (k = 0.958 (0.899–1.017)). Conclusion: Vessel-encoded ASL seems to be a promising non-invasive method to depict the contributions of individual arteries to the cerebral perfusion before and after revascularization surgery. [ABSTRACT FROM AUTHOR]

Purpose: Patients with Moyamoya Angiopathy (MMA) require hemodynamic evaluation to assess the risk of stroke. Assessment of cerebral blood flow with [15O]water PET and acetazolamide challenge is the diagnostic standard for the evaluation of the cerebral perfusion reserve (CPR). Estimation of the cerebrovascular reactivity (CVR) by use of breath-hold-triggered fMRI (bh-fMRI) as an index of CPR has been proposed as a reliable and more readily available approach. Recent findings suggest the use of resting-state fMRI (rs-fMRI) which requires minimum patient compliance. The aim of this study was to compare rs-fMRI to bh-fMRI and [15O]water PET in patients with MMA. Methods: Patients with MMA underwent rs-fMRI and bh-fMRI in the same MRI session. Maps of the CVR gained by both modalities were compared retrospectively by calculating the correlation between the mean CVR of 12 volumes of interest. Additionally, the rs-maps of a subgroup of patients were compared to CPR-maps gained by [15O]water PET. Results: The comparison of the rs-maps and the bh-maps of 24 patients revealed a good correlation (Pearson's r = 0.71 ± 0.13; preoperative patients: Pearson's r = 0.71 ± 0.17; postoperative patients: Pearson's r = 0.71 ± 0.11). The comparison of 7 rs-fMRI data sets to the corresponding [15O]water PET data sets also revealed a high level of agreement (Pearson's r = 0.80 ± 0.19). Conclusion: The present analysis indicates that rs-fMRI might be a promising non-invasive method with almost no patient cooperation needed to evaluate the CVR. Further prospective studies are required. [ABSTRACT FROM AUTHOR]

Background and Purpose: This study explores the use of deep learning (DL) techniques in MRI of the orbit to enhance imaging. Standard protocols, although detailed, have lengthy acquisition times. We investigate DL‐based methods for T2‐weighted and T1‐weighted, fat‐saturated, contrast‐enhanced turbo spin echo (TSE) sequences, aiming to improve image quality, reduce acquisition time, minimize artifacts, and enhance diagnostic confidence in orbital imaging. Methods: In a 3‐Tesla MRI study of 50 patients evaluating orbital diseases from March to July 2023, conventional (TSES) and DL TSE sequences (TSEDL) were used. Two neuroradiologists independently assessed the image datasets for image quality, diagnostic confidence, noise levels, artifacts, and image sharpness using a randomized and blinded 4‐point Likert scale. Results: TSEDL significantly reduced image noise and artifacts, enhanced image sharpness, and decreased scan time, outperforming TSES (p <.05). TSEDL showed superior overall image quality and diagnostic confidence, with relevant findings effectively detected in both DL‐based and conventional images. In 94% of cases, readers preferred accelerated imaging. Conclusion: The study proved that using DL for MRI image reconstruction in orbital scans significantly cut acquisition time by 69%. This approach also enhanced image quality, reduced image noise, sharpened images, and boosted diagnostic confidence. [ABSTRACT FROM AUTHOR]

Aim: To assess how atypical language organization after early left‐hemispheric brain lesions affects grey matter in the contralesional hemisphere. Method: This was a cross‐sectional study with between‐group comparisons of 14 patients (six female, 8–26 years) with perinatal left‐hemispheric brain lesions (two arterial ischemic strokes, 11 periventricular haemorrhagic infarctions, one without classification) and 14 typically developing age‐matched controls (TDC) with functional magnetic resonance imaging (fMRI) documented left‐hemispheric language organization (six female, 8–28 years). MRI data were analysed with SPM12, CAT12, and custom scripts. Language lateralization indices were determined by fMRI within a prefrontal mask and right‐hemispheric grey matter group differences by voxel‐based morphometry (VBM). Results: FMRI revealed left‐dominance in seven patients with typical language organization (TYP) and right‐dominance in seven patients with atypical language organization (ATYP) of 14 patients. VBM analysis of all patients versus controls showed grey matter reductions in the middle temporal gyrus of patients. A comparison between the two patient subgroups revealed an increase of grey matter in the middle frontal gyrus in the ATYP group. Voxel‐based regression analysis confirmed that grey matter increases in the middle frontal gyrus were correlated with atypical language organization. Interpretation: Compatible with a non‐specific lesion effect, we found areas of grey matter reduction in patients as compared to TDC. The grey matter increase in the middle frontal gyrus seems to reflect a specific compensatory effect in patients with atypical language organization. What this paper adds: Perinatal stroke leads to decreased grey matter in the contralesional hemisphere.Atypical language organization is associated with grey matter increases in contralesional language areas. [ABSTRACT FROM AUTHOR]

A 61-year-old patient was diagnosed with a left-sided falx meningioma. Histopathological analysis following extirpation showed a meningothelial meningioma ZNS WHO grade 1 with sparse mitoses. Over the course of 12 years, the patient received irradiation (54.0 Gy), peptide radio-receptor therapy (177Lu-DOMITATE) and targeted therapy (mTOR inhibitor). Follow-up imaging revealed an increased size of the residual tumor. Due to increased liver function parameters, imaging of the liver was performed, showing widespread space-occupying lesions with atypical appearance. Biopsy revealed metastasis of the meningioma, now with 2.7 mitoses/mm2, necrosis and homozygous CDKN2A/B deletion, corresponding to an anaplastic CNS meningioma WHO grade 3. A second small meningioma on the left petroclival side has been consistent in size over 12 years. Metastatic meningiomas pose a pertinent clinical challenge due to poor prognosis. The lung, bone, liver and cervical lymph nodes are the most common sites of extracranial metastasis. According to the World Health Organization criteria, the most important predictive factor for recurrence and metastasis is the tumor grade. [ABSTRACT FROM AUTHOR]

Background: Nerve injuries are a frequent problem in routine clinical practice and require intensive interdisciplinary care. Objective: The current status of imaging to confirm the diagnosis of nerve injuries is described. The role of high-resolution ultrasound and magnetic resonance imaging (MRI) in the diagnostics and follow-up of peripheral nerve injuries is elaborated. Material and methods: Review of the current state of imaging to confirm the diagnosis of nerve injuries. Results: Depending on the suspected site of damage, the primary domain of magnetic resonance (MR) imaging (MR neurography) is injuries in the region of the spine, nerve roots, brachial plexus and lumbar plexus, pelvis and proximal thigh. In contrast, in other peripheral nerve lesions of the extremities the advantages of high-resolution nerve ultrasound in a dynamic setting predominate. The MR neurography is indicated here, especially in the frequent bottleneck syndromes and only in very isolated and selected cases. Conclusion: In addition to a correct anatomical assignment, the timely decision for a possible intervention and the appropriate concomitant treatment are an important basis for a favorable prognosis of nerve injuries. Imaging techniques should therefore be used early in the diagnostics and follow-up controls of peripheral nerve injuries. [ABSTRACT FROM AUTHOR]

Spinal arachnoid web (SAW) is a rare disease entity characterized as band-like arachnoid tissue that can cause spinal cord compression and syringomyelia. This study aimed to analyze the surgical management of the spinal arachnoid web in patients with syringomyelia, focusing on surgical strategies and outcomes. A total of 135 patients with syringomyelia underwent surgery at our department between November 2003 and December 2022. All patients underwent magnetic resonance imaging (MRI), with a special syringomyelia protocol (including TrueFISP and CINE), and electrophysiology. Among these patients, we searched for patients with SAW with syringomyelia following careful analysis of neuroradiological data and surgical reports. The criteria for SAW were as follows: displacement of the spinal cord, disturbed but preserved CSF flow, and intraoperative arachnoid web. Patients were evaluated for initial symptoms, surgical strategies, and complications by reviewing surgical reports, patient documents, neuroradiological data, and follow-up data. Of the 135 patients, 3 (2.22%) fulfilled the SAW criteria. The mean patient age was 51.67 ± 8.33 years. Two patients were male, and one was female. The affected levels were T2/3, T6, and T8. Excision of the arachnoid web was performed in all cases. No significant change in intraoperative monitoring was noted. Postoperatively, none of the patients presented new neurological symptoms. The MRI 3 months after surgery revealed that the syringomyelia improved in all cases, and caliber variation of the spinal cord could not be detected anymore. All clinical symptoms improved. In summary, SAW can be safely treated by surgery. Even though syringomyelia usually improves on MRI and symptoms also improve, residual symptoms might be observed. We advocate for clear criteria for the diagnosis of SAW and a standardized diagnostic (MRI including TrueFISP and CINE). [ABSTRACT FROM AUTHOR]

We have previously established an fMRI task battery suitable for mapping the language processing network in children. Among the tasks used, the synonyms and the vowel identification task induced robust task-related activations in children with average language abilities; however, the fixed presentation time seems to be a drawback in participants with above- or below-average language abilities. This feasibility study in healthy adults ( n = 20) was aimed at adapting these tasks to the individual level of each patient by implementing a self-paced stimulus presentation. The impact of using a block- versus an event-related statistical approach was also evaluated. The self-paced modification allowed our participants with above-average language abilities to process stimuli much faster than originally implemented, likely increasing task adherence. A higher specificity of the event-related analysis was confirmed by stronger left inferior frontal and crossed cerebellar activations. We suggest that self-paced paradigms and event-related analyses may both increase specificity and applicability. [ABSTRACT FROM AUTHOR]

Background: Management of intracerebral hemorrhage (ICH) is challenged by limited therapeutic options and a complex relationship between blood pressure (BP) dynamics, especially BP variability (BPV) and ICH outcome.In an exploratory analysis of prospectively collected data on consecutive patients with nontraumatic ICH between 2015 and 2020, continuous BP accessed via an arterial line extracted from the Intellispace Critical Care and Anesthesia information system (Philips Healthcare) was analyzed over the first 72 h post admission. Arterial lines were used as part of standard clinical practice in the intensive care, ensuring high fidelity and real-time data essential for acute care settings. BPV was assessed through successive variation (SV), standard deviation (SD), and coefficient of variation using all available BP measurements. Multivariate regression models were applied to evaluate the association between BPV indices and functional outcome at 3 months.Among 261 patients (mean age 69.6 ± 15.2 years, 47.9% female, median National Institutes of Health Stroke Scale [NIHSS] score 6 [interquartile range 2–12]) analyzed, lower systolic BP upon admission (< 140 mm Hg) and lower systolic BPV were significantly associated with favorable outcome, whereas higher diastolic BPV correlated with improved outcomes. In the multivariate analysis, diastolic BPV (SD, SV) within the first 72 h post admission emerged as an independent predictor of good functional outcome (modified Rankin Scale score < 3; odds ratio 1.123, 95% confidence interval CI 1.008–1.184, p = 0.035), whereas systolic BPV (SD) showed a negative association. Patients with better outcomes also exhibited distinct clinical characteristics, including younger age, lower median NIHSS scores, and less prevalence of anticoagulation therapy upon admission.This study shows the prognostic value of BPV in the acute phase of ICH. Lower systolic BPV (SD) and higher diastolic BPV (SD, SV) were associated with better functional outcomes, challenging traditional BP management strategies. These findings might help to tailor a personalized BP management in ICH.Methods: Management of intracerebral hemorrhage (ICH) is challenged by limited therapeutic options and a complex relationship between blood pressure (BP) dynamics, especially BP variability (BPV) and ICH outcome.In an exploratory analysis of prospectively collected data on consecutive patients with nontraumatic ICH between 2015 and 2020, continuous BP accessed via an arterial line extracted from the Intellispace Critical Care and Anesthesia information system (Philips Healthcare) was analyzed over the first 72 h post admission. Arterial lines were used as part of standard clinical practice in the intensive care, ensuring high fidelity and real-time data essential for acute care settings. BPV was assessed through successive variation (SV), standard deviation (SD), and coefficient of variation using all available BP measurements. Multivariate regression models were applied to evaluate the association between BPV indices and functional outcome at 3 months.Among 261 patients (mean age 69.6 ± 15.2 years, 47.9% female, median National Institutes of Health Stroke Scale [NIHSS] score 6 [interquartile range 2–12]) analyzed, lower systolic BP upon admission (< 140 mm Hg) and lower systolic BPV were significantly associated with favorable outcome, whereas higher diastolic BPV correlated with improved outcomes. In the multivariate analysis, diastolic BPV (SD, SV) within the first 72 h post admission emerged as an independent predictor of good functional outcome (modified Rankin Scale score < 3; odds ratio 1.123, 95% confidence interval CI 1.008–1.184, p = 0.035), whereas systolic BPV (SD) showed a negative association. Patients with better outcomes also exhibited distinct clinical characteristics, including younger age, lower median NIHSS scores, and less prevalence of anticoagulation therapy upon admission.This study shows the prognostic value of BPV in the acute phase of ICH. Lower systolic BPV (SD) and higher diastolic BPV (SD, SV) were associated with better functional outcomes, challenging traditional BP management strategies. These findings might help to tailor a personalized BP management in ICH.Results: Management of intracerebral hemorrhage (ICH) is challenged by limited therapeutic options and a complex relationship between blood pressure (BP) dynamics, especially BP variability (BPV) and ICH outcome.In an exploratory analysis of prospectively collected data on consecutive patients with nontraumatic ICH between 2015 and 2020, continuous BP accessed via an arterial line extracted from the Intellispace Critical Care and Anesthesia information system (Philips Healthcare) was analyzed over the first 72 h post admission. Arterial lines were used as part of standard clinical practice in the intensive care, ensuring high fidelity and real-time data essential for acute care settings. BPV was assessed through successive variation (SV), standard deviation (SD), and coefficient of variation using all available BP measurements. Multivariate regression models were applied to evaluate the association between BPV indices and functional outcome at 3 months.Among 261 patients (mean age 69.6 ± 15.2 years, 47.9% female, median National Institutes of Health Stroke Scale [NIHSS] score 6 [interquartile range 2–12]) analyzed, lower systolic BP upon admission (< 140 mm Hg) and lower systolic BPV were significantly associated with favorable outcome, whereas higher diastolic BPV correlated with improved outcomes. In the multivariate analysis, diastolic BPV (SD, SV) within the first 72 h post admission emerged as an independent predictor of good functional outcome (modified Rankin Scale score < 3; odds ratio 1.123, 95% confidence interval CI 1.008–1.184, p = 0.035), whereas systolic BPV (SD) showed a negative association. Patients with better outcomes also exhibited distinct clinical characteristics, including younger age, lower median NIHSS scores, and less prevalence of anticoagulation therapy upon admission.This study shows the prognostic value of BPV in the acute phase of ICH. Lower systolic BPV (SD) and higher diastolic BPV (SD, SV) were associated with better functional outcomes, challenging traditional BP management strategies. These findings might help to tailor a personalized BP management in ICH.Conclusions: Management of intracerebral hemorrhage (ICH) is challenged by limited therapeutic options and a complex relationship between blood pressure (BP) dynamics, especially BP variability (BPV) and ICH outcome.In an exploratory analysis of prospectively collected data on consecutive patients with nontraumatic ICH between 2015 and 2020, continuous BP accessed via an arterial line extracted from the Intellispace Critical Care and Anesthesia information system (Philips Healthcare) was analyzed over the first 72 h post admission. Arterial lines were used as part of standard clinical practice in the intensive care, ensuring high fidelity and real-time data essential for acute care settings. BPV was assessed through successive variation (SV), standard deviation (SD), and coefficient of variation using all available BP measurements. Multivariate regression models were applied to evaluate the association between BPV indices and functional outcome at 3 months.Among 261 patients (mean age 69.6 ± 15.2 years, 47.9% female, median National Institutes of Health Stroke Scale [NIHSS] score 6 [interquartile range 2–12]) analyzed, lower systolic BP upon admission (< 140 mm Hg) and lower systolic BPV were significantly associated with favorable outcome, whereas higher diastolic BPV correlated with improved outcomes. In the multivariate analysis, diastolic BPV (SD, SV) within the first 72 h post admission emerged as an independent predictor of good functional outcome (modified Rankin Scale score < 3; odds ratio 1.123, 95% confidence interval CI 1.008–1.184, p = 0.035), whereas systolic BPV (SD) showed a negative association. Patients with better outcomes also exhibited distinct clinical characteristics, including younger age, lower median NIHSS scores, and less prevalence of anticoagulation therapy upon admission.This study shows the prognostic value of BPV in the acute phase of ICH. Lower systolic BPV (SD) and higher diastolic BPV (SD, SV) were associated with better functional outcomes, challenging traditional BP management strategies. These findings might help to tailor a personalized BP management in ICH. [ABSTRACT FROM AUTHOR]

Hereditary spastic paraplegias (HSP) are a group of clinically and genetically heterogeneous disorders with the hallmark of progressive spastic gait disturbance. We used advanced neuroimaging to identify brain regions involved in SPG4, the most common HSP genotype. Additionally, we analyzed correlations between imaging and clinical findings. We performed 3T MRI scans including isotropic high-resolution 3D T1, T2-FLAIR, and DTI sequences in 15 adult patients with genetically confirmed SPG4 and 15 age- and sex-matched healthy controls. Brain volume loss of gray and white matter was evaluated through voxel-based morphometry (VBM) for supra- and infratentorial regions separately. DTI maps of axial diffusivity (AD), radial diffusivity (RD), mean diffusivity (MD), fractional anisotropy (FA), and measured anisotropy (MA1) were analyzed through tract-based special statistics (TBSS). VBM and TBSS revealed a widespread affection of gray and white matter in SPG4 including the corpus callosum, medio-dorsal thalamus, parieto-occipital regions, upper brainstem, cerebellum, and corticospinal tract. Significant correlations with correlation coefficients r > 0.6 between clinical data and DTI findings could be demonstrated for disease duration and disease severity as assessed by the spastic paraplegia rating scale for the pontine crossing tract (AD) and the corpus callosum (RD and FA). Imaging also provided evidence that SPG4 underlies a primarily axonal rather than demyelinating damage in accordance with post-mortem data. DTI is an attractive tool to assess subclinical affection in SPG4. The correlation of imaging findings with disease duration and severity suggests AD, RD, and FA as potential progression markers in interventional studies. [ABSTRACT FROM AUTHOR]

Background: Progressive multifocal leukoencephalopathy is a severe demyelinating disease caused by the polyoma JC virus in patients with reduced immunocompetence. A few cases of progressive multifocal leukoencephalopathy have been reported in patients treated with fumaric acid esters. Case presentation: A 53-year-old Caucasian woman reported to our clinic with a first focal epileptic seizure and mild cognitive impairment. Since 1.5 years, she was treated with fumaderm for her psoriasis. During that time, her lymphocyte counts ranged between 450 and 700/µl. Cerebral magnet resonance imaging showed multifocal subcortical T2 hyperintense lesions with partial gadolinium enhancement. She did not have antibodies against human immunodeficiency virus 1 and 2 and cerebrospinal fluid-polymerase chain reaction for viral infections including a sensitive JC-virus polymerase chain reaction were negative. The diagnosis of progressive multifocal leukoencephalopathy was established by histological analysis and detection of JC-virus desoxyribonucleic acid in brain biopsy specimens. Dimethyl fumarate was stopped and Mirtazapin and Mefloquin were initiated. Neurological examination and imaging remained stable. Conclusions: Progressive multifocal leukoencephalopathy can occur in patients with lymphocyte counts between 450 and 700/µl, produce only faint symptoms and is not excluded by negative JC-virus-polymerase chain reaction in cerebrospinal fluid. The incidence of progressive multifocal leukoencephalopathy may thus be underestimated and a more careful surveillance of patients would be necessary. [ABSTRACT FROM AUTHOR]

• Common age-related cervical neuroforaminal stenoses on MRI/CT are not per se associated with nerve root enlargement on ultrasound. • Degeneration of the cervical spine is unlikely to confound the ultrasound assessment of cervical nerve roots in polyneuropathy. • Older age is associated with neuroforaminal stenosis, but is not a significant covariate of nerve root diameter in this cohort. Ultrasound can detect enlargement of cervical nerve roots, which has been described in polyneuropathies (PNP), mainly of demyelinating pathology. This study investigates whether neuroforaminal stenosis, as a common but often asymptomatic degenerative change, is associated with nerve root enlargement on ultrasound and whether neuroforaminal stenosis thus confounds cervical nerve root ultrasound findings in the diagnostic assessment of PNP. We retrospectively studied 182 patients (62 demyelinating, 71 axonal PNP; 49 without evidence of PNP) who had undergone ultrasound of the cervical nerve roots C5 and C6 and magnetic resonance or computed tomography of the cervical spine that was reviewed with respect to neuroforaminal stenoses. Patients with demyelinating PNP had larger nerve roots than those with axonal/without PNP. No significant differences in cervical nerve root diameters were found between groups with vs. without neuroforaminal stenosis. The diagnostic performance of the discrimination of PNP subtypes based on ultrasound nerve root measurements did not differ significantly when including or excluding subjects with neuroforaminal stenoses. Neuroforaminal stenosis per se does not entail relevant cervical nerve root enlargement detectable by nerve ultrasound. Ultrasound assessment of cervical nerve root size in the diagnostic evaluation of PNP is unlikely to be confounded by common degenerative changes of the cervical spine. [ABSTRACT FROM AUTHOR]

Moyamoya angiopathy (MMA) related cerebral perfusion deficits or infarctions might influence quality of life (QoL). This study examines preoperative QoL in adult patients with MMA and correlates these with findings obtained via diagnostic imaging. Sixty-seven adult Moyamoya patients underwent preoperative neuropsychological testing including questionnaires to determine QoL, as well as psychiatric and depressive symptoms. The results were checked for correlation with territorial hypoperfusions seen in H215O PET with acetazolamide (ACZ) challenge (cerebrovascular reserve) and infarction patterns observed in MRI. Each vascular territory was analyzed separately and correlated with QoL. Physical role function was restricted in 41.0% of cases and emotional role function in 34.4% of cases (SF-36). Obsessive–compulsive disorder (39.3%) (SCL-90-R), psychoticism (34.4%) (SCL-90-R), and depression (32.7%) (BDI-II) were also very common. Psychoticism was significantly more frequent in cases where perfusion deficits in PET CT were observed in both MCA territories (left p = 0.0124, right p = 0.0145) and infarctions in MRI were present in the right MCA territory (p = 0.0232). Depression was significantly associated with infarctions in the right MCA territory (SCL-90-R p = 0.0174, BDI-II p = 0.0246). Women were affected more frequently by depression (BDI-II, p = 0.0234). Physical role function impairment was significantly associated with perfusion deficits in the left MCA territory (p = 0.0178) and infarctions in the right MCA territory (p = 0.0428). MMA leads to impairments in different areas of QoL. Approximately one-third of all adult MMA patients suffered from depression, with women being most affected. In addition to depression, presence of executive dysfunctions and mental disorders such as psychoticism, obsessive–compulsive disorder, and impaired physical and emotional role function affected QoL. These patients showed significantly more often infarctions and perfusion deficits in the right MCA territory. Long-term studies with follow-up results are necessary to clarify a possible beneficial impact of early surgical revascularization on QoL and depression in adult MMA patients. [ABSTRACT FROM AUTHOR]

Abstract: In this study, we analyzed the structural connectivity of cortico-cortical and cortico-subcortical language networks in healthy children, using probabilistic tractography based on high angular resolution diffusion imaging. In addition to anatomically defining seed and target regions for tractography, we used fMRI to target inferior frontal and superior temporal cortical language areas on an individual basis. Further, connectivity between these cortical and subcortical (thalamus, caudate nucleus) language regions was assessed. Overall, data from 15 children (8f) aged 8–17years (mean age 12.1±3years) could be included. A slight but non-significant trend towards leftward lateralization was found in the arcuate fasciculus/superior longitudinal fasciculus (AF/SLF) using anatomically defined masks (p >.05, Wilcoxon rank test), while the functionally-guided tractography showed a significant lateralization to the left (p <.01). Connectivity of the thalamus with language regions was strong but not lateralized. Connectivity of the caudate nucleus with inferior-frontal language regions was also symmetrical, while connectivity with superior-temporal language regions was strongly lateralized to the left (p <.01). To conclude, we could show that tracking the arcuate fasciculus/superior longitudinal fasciculus is possible using both anatomically and functionally-defined seed and target regions. With the latter approach, we could confirm the presence of structurally-lateralized cortico-cortical language networks already in children, and finally, we could demonstrate a strongly asymmetrical connectivity of the caudate nucleus with superior temporal language regions. Further research is necessary in order to assess the usability of such an approach to assess language dominance in children unable to participate in an active fMRI study. [Copyright &y& Elsevier]

Purpose: The aim of the present work was to test the feasibility of the time-resolved MR-angiography (TWIST-MRA) of cervical arteries using double bolus injection. Material and Methods: TWIST-MRA with a temporal resolution of 8.4 seconds for each frame and a spatial resolution with a voxel size of 0.61 × 0.58 × 0.8 mm³ was performed in 24 patients. A biphasic bolus injection protocol was used with the second injection being started 21 seconds after the first contrast dye bolus. Diagnostic image quality was rated according to a 4-point scale. Results: In 12 patients (50%) no clear separation between the cervical venous and arterial vessels was evident after the first bolus injection. Using TWIST-MRA data acquired after the second bolus a sufficient diagnostic image quality (rating ≥3, mean 3.5) could be obtained in 22 of 24 patients (92%). Discussion. The double bolus injection protocol using TWIST-MRA allows for very good separation of the cervical arteries. [ABSTRACT FROM AUTHOR]

Abstract: In order to increase the rate of successful functional MR studies in children it is helpful to shorten the time spent in the scanner. To this effect, assessing two cognitive functions with one task seems to be a promising approach. The hypothesis of this study was that the control condition of an established language task (vowel identification task, VIT) requires visuospatial processing and that the control condition (VITCC) therefore may also be applicable to localize visuospatial functions. As a reference task, a visual search task (VST, previously established for use in children) was employed. To test this hypothesis, 43 children (19 f, 24 m; 12.0±2.6, range 7.9 to 17.8years) were recruited and scanned using both tasks. Second-level random effects group analyses showed activation of left inferior-frontal cortex in the active condition of the VIT, as in previous studies. Additionally, analysis of the VITCC demonstrated activation in right-dominant superior parietal and high-frontal brain regions, classically associated with visuospatial functions; activation seen in the VST was similar with a substantial overlap. However, lateralization in the parietal lobe was significantly more bilateral in the VST than in the VITCC. This suggests that the VIT can not only be applied to assess language functions (using the active>control contrast), but also that the control>active condition is useful for assessing visuospatial functions. Future task design may benefit from such a “dual use” approach to performing fMRI not only, but also particularly in children. [Copyright &y& Elsevier]

Homozygous mutations in the PINK1 gene have been shown to cause early-onset parkinsonism. Here, we describe a novel homozygous mutation (Q126P), identified in two affected German sisters with a clinical phenotype typical for PINK1-associated parkinsonism.We analysed lactate, pyruvate, carnitine and acylcarnitine blood levels, lactate levels under exercise and in the cerebrospinal fluid, activity of respiratory chain complexes I–IV in muscle biopsies and proteasomal activity in immortalized lymphoblasts, but found no evidence for mitochondrial or proteasomal dysfunction. MR spectroscopy revealed raised myoinositol levels in the basal ganglia of both patients, reflecting possible astroglial proliferation. [ABSTRACT FROM AUTHOR]

Objective: To determine the area most at risk of delayed cerebral ischemia (DCI) in relation to the location of the ruptured aneurysm in patients with aneurysmal subarachnoid hemorrhage (aSAH) and, therefore, help to choose the site for focal multimodal neuromonitoring. Methods: We retrospectively analyzed angiographic findings, CCT scans, and patient charts of patients who were admitted with aSAH to our neurosurgical intensive care unit between 2009 and 2017. DCI was defined as infarction on CCT 2–6 weeks after aSAH. Results: DCI occurred in 17.9% out of 357 included patients. A DCI occurring in the vascular territory of the artery carrying the ruptured aneurysm was found in 81.0% of patients with anterior circulation aneurysms but only in 16.7% with posterior circulation aneurysms (Fisher's exact, p=0.003). The vascular territory most frequently showing a DCI was the ipsilateral MCA territory (86.7%) in ICA aneurysms, the contra- (71.4%) and the ipsilateral (64.3%) ACA territory in ACA aneurysms, the right (93.8%) and the left (81.3%) ACA territory in AcomA aneurysms, and the ipsilateral MCA territory in MCA aneurysms (69.2%) as well as in VA/PICA/SCA aneurysms (100.0%). DCI after the rupture of a BA aneurysm occurred with 33.3% in 6 out of 8 vascular territories, respectively. DCI of multiple vascular territories occurred in 100.0% of BA aneurysms, 87.5% of AcomA aneurysms, 71.4% of ACA aneurysms, 40.0% of ICA aneurysms, 38.5% of MCA aneurysms, and 33.3% of VA/PICA/SCA aneurysms. Discussion: Few studies exist that could determine the area most at risk of a DCI after an aSAH. Our data could identify the territory most at risk for DCI with a probability of > 60% except for BA aneurysms, which showed DCI in various areas and patients suffering from multiple DCIs. Either the ipsilateral ACA or MCA were affected by the DCI in about 80% of ACA and more than 90% of AcomA, ICA, MCA, and VA/PICA/SCA aneurysms. Therefore, local intraparenchymal neuromonitoring in the ACA/MCA watershed area might detect the vast majority of DCIs for all aneurysm locations, except for BA aneurysms. In ACA and AcomA aneurysms, bilateral DCI of the ACA territory was common, and bilateral probe positioning might be considered for monitoring high-risk patients. Non-focal monitoring methods might be preferably used after BA aneurysm rupture. [ABSTRACT FROM AUTHOR]

Background and Purpose: Hemodynamic evaluation of moyamoya patients is crucial to decide the treatment strategy. Recently, CO2-triggered BOLD MRI has been shown to be a promising tool for the hemodynamic evaluation of moyamoya patients. However, the longitudinal reliability of this technique in follow-up examinations is unknown. This study aims to analyze longitudinal follow-up data of CO2-triggered BOLD MRI to prove the reliability of this technique for long-term control examinations in moyamoya patients. Methods: Longitudinal CO2 BOLD MRI follow-up examinations of moyamoya patients with and without surgical revascularization have been analyzed for all 6 vascular territories retrospectively. If revascularization was performed, any directly (by the disease or the bypass) or indirectly (due to change of collateral flow after revascularization) affected territory was excluded based on angiography findings (group 1). In patients without surgical revascularization between the MRI examinations, all territories were analyzed (group 2). Results: Eighteen moyamoya patients with 39 CO2 BOLD MRI examinations fulfilled the inclusion criteria. The median follow-up between the 2 examinations was 12 months (range 4–29 months). For 106 vascular territories analyzed in group 1, the intraclass correlation coefficient was 0.784, p < 0.001, and for group 2 (84 territories), it was 0.899, p < 0.001. Within the total follow-up duration of 140 patient months, none of the patients experienced a new stroke. Conclusions: CO2 BOLD MRI is a promising tool for mid- and long-term follow-up examinations of cerebral hemodynamics in moyamoya patients. Systematic prospective evaluation is required prior to making it a routine examination. [ABSTRACT FROM AUTHOR]

To evaluate the feasibility of a multimodal approach involving dynamic contrast-enhanced (DCE) perfusion imaging and diffusion kurtosis imaging (DKI) in the preoperative imaging of brain tumors in a multicenter setting, and to evaluate the effect on diagnostic confidence and accuracy for tumor grade and type prediction. One hundred and thirty-three patients with brain tumors were imaged in six hospitals with a standardized multimodal protocol. Standard imaging and six parameter maps derived from DCE and DKI sequences were reviewed off-site by two independent readers. Image quality and diagnostic confidence were evaluated in qualitative analyses. Quantitative analyses were performed to assess diagnostic accuracy and the performance of DKI and DCE parameters for tumor grade differentiation and molecular tumor type determination. Standardized acquisition of DCE and DKI maps was feasible with excellent image quality. Diagnostic confidence was significantly improved from 85 % to 96 % (p = 0.0005) by additional review of the DCE and DKI maps. The combination of mean kurtosis and CBV was particularly advantageous for differentiating low-grade and high-grade glioma, oligodendroglial vs. astrocytic, and IDH1/2 wild type vs. mutated tumors. A multimodal imaging approach with DCE and DKI improves diagnostic confidence and yields higher diagnostic accuracy for predicting tumor grade and type in adult-type glioma. [ABSTRACT FROM AUTHOR]

Background: Diffusion-weighted imaging (DWI) has become an important tool for lesion characterization. Advanced techniques of DWI may be used to improve image quality. Purpose: To evaluate multi-shot segmented DWI (rs-EPI, RESOLVE) and reduced field-of view DWI (rFOV-EPI, "zoomed" EPI) in patients with ocular melanoma and to compare image quality and diagnostic performance in differentiation of melanoma from retinal detachment. Material and Methods: In this prospective and IRB-approved trial, we examined 26 patients using methods including conventional single-shot echo-planar DWI (ss-EPI), rs-EPI, and rFOV-EPI. Subjective image quality was compared using a four-point score and the maximum tumor length was measured in all sequences. Apparent diffusion coefficient (ADC) measurements were performed using a region-of interest analysis. Tumor delineation and differences in ADC values between melanomas and retinal detachments were compared. Results: Diffusion restriction was markedly reduced in melanomas in all applied image techniques. Subjective image quality was significantly higher for rFOV-EPI (score = 3.5 ± 0.5) compared with rs-EPI (score = 3.3 ± 0.6) and ss-EPI (score = 2.5 ± 0.9). Regarding tumor diameter measurements, rFOV-EPI showed the best agreement compared with high-resolution conventional sequences. ADC measurements of the tumor and retinal detachment differed significantly (P < 0.001) with the rFOV-EPI performing best (sensitivities and specificities compared with T1-weighted ss-EPI 61%/82%; rFOV-EPI 86%/92%; rs-EPI 79%/92%, respectively). Conclusion: rFOV-EPI showed improved image quality compared with ss-EPI and rs-EPI, the most accurate tumor delineation and the best differentiation from retinal detachments in our patients. [ABSTRACT FROM AUTHOR]

Whole-brain voxel-based morphometry (VBM) studies revealed patterns of patchy atrophy within the cerebellum of Friedreich's ataxia patients, missing clear clinico-anatomic correlations. Studies so far are lacking an appropriate registration to the infratentorial space. To circumvent these limitations, we applied a high-resolution atlas template of the human cerebellum and brainstem (SUIT template) to characterize regional cerebellar atrophy in Friedreich's ataxia (FRDA) on 3-T MRI data. We used a spatially unbiased voxel-based morphometry approach together with T2-based manual segmentation, T2 histogram analysis, and atlas generation of the dentate nuclei in a representative cohort of 18 FRDA patients and matched healthy controls. We demonstrate that the cerebellar volume in FRDA is generally not significantly different from healthy controls but mild lobular atrophy develops beyond normal aging. The medial parts of lobule VI, housing the somatotopic representation of tongue and lips, are the major site of this lobular atrophy, which possibly reflects speech impairment. Extended white matter affection correlates with disease severity across and beyond the cerebellar inflow and outflow tracts. The dentate nucleus, as a major site of cerebellar degeneration, shows a mean volume loss of about 30%. Remarkably, not the atrophy but the T2 signal decrease of the dentate nuclei highly correlates with disease duration and severity. [ABSTRACT FROM AUTHOR]

Purpose: This paper aims to evaluate a new iterative metal artifact reduction algorithm for post-interventional evaluation of brain tissue and intracranial arteries. Methods: The data of 20 patients that underwent follow-up cranial CT and cranial CT angiography after clipping or coiling of an intracranial aneurysm was retrospectively analyzed. After the images were processed using a novel iterative metal artifact reduction algorithm, images with and without metal artifact reduction were qualitatively evaluated by two readers, using a five-point Likert scale. Moreover, artifact strength was quantitatively assessed in terms of CT attenuation and standard deviation alterations. Results: The qualitative analysis yielded a significant increase in image quality ( p = 0.0057) in iteratively processed images with substantial inter-observer agreement ( ĸ = 0.72), while the CTA image quality did not differ ( p = 0.864) and even showed vessel contrast reduction in six cases (30%). The mean relative attenuation difference was 27% without metal artifact reduction vs. 11% for iterative metal artifact reduction images ( p = 0.0003). Conclusions: The new iterative metal artifact reduction algorithm enhances non-enhanced CT image quality after clipping or coiling, but in CT-angiography images, the contrast of adjacent vessels can be compromised. [ABSTRACT FROM AUTHOR]

Objective: We investigate how known differences in myelin architecture between regions along the cortico-spinal tract and frontal white matter (WM) in 19 healthy adolescents are reflected in several quantitative MRI parameters that have been proposed to non-invasively probe WM microstructure. In a clinically feasible scan time, both conventional imaging sequences as well as microstructural MRI parameters were assessed in order to quantitatively characterise WM regions that are known to differ in the thickness of their myelin sheaths, and in the presence of crossing or parallel fibre organisation. Results: We found that diffusion imaging, MR spectroscopy (MRS), myelin water fraction (MWF), Magnetization Transfer Imaging, and Quantitative Susceptibility Mapping were myelin-sensitive in different ways, giving complementary information for characterising WM microstructure with different underlying fibre architecture. From the diffusion parameters, neurite density (NODDI) was found to be more sensitive than fractional anisotropy (FA), underlining the limitation of FA in WM crossing fibre regions. In terms of sensitivity to different myelin content, we found that MWF, the mean diffusivity and chemical-shift imaging based MRS yielded the best discrimination between areas. Conclusion: Multimodal assessment of WM microstructure was possible within clinically feasible scan times using a broad combination of quantitative microstructural MRI sequences. By assessing new microstructural WM parameters we were able to provide normative data and discuss their interpretation in regions with different myelin architecture, as well as their possible application as biomarker for WM disorders. [ABSTRACT FROM AUTHOR]

Background: Progressive multifocal leukoencephalopathy is a severe demyelinating disease caused by the polyoma JC virus in patients with reduced immunocompetence. A few cases of progressive multifocal leukoencephalopathy have been reported in patients treated with fumaric acid esters.Case Presentation: A 53-year-old Caucasian woman reported to our clinic with a first focal epileptic seizure and mild cognitive impairment. Since 1.5 years, she was treated with fumaderm for her psoriasis. During that time, her lymphocyte counts ranged between 450 and 700/μl. Cerebral magnet resonance imaging showed multifocal subcortical T2 hyperintense lesions with partial gadolinium enhancement. She did not have antibodies against human immunodeficiency virus 1 and 2 and cerebrospinal fluid-polymerase chain reaction for viral infections including a sensitive JC-virus polymerase chain reaction were negative. The diagnosis of progressive multifocal leukoencephalopathy was established by histological analysis and detection of JC-virus desoxyribonucleic acid in brain biopsy specimens. Dimethyl fumarate was stopped and Mirtazapin and Mefloquin were initiated. Neurological examination and imaging remained stable.Conclusions: Progressive multifocal leukoencephalopathy can occur in patients with lymphocyte counts between 450 and 700/μl, produce only faint symptoms and is not excluded by negative JC-virus-polymerase chain reaction in cerebrospinal fluid. The incidence of progressive multifocal leukoencephalopathy may thus be underestimated and a more careful surveillance of patients would be necessary. [ABSTRACT FROM AUTHOR]

See Klockgether (doi:10.1093/awv253) for a scientific commentary on this article.The spinocerebellar ataxias types 2 (SCA2) and 3 (SCA3) are autosomal dominantly inherited cerebellar ataxias which are caused by CAG trinucleotide repeat expansions in the coding regions of the disease-specific genes. Although previous post-mortem studies repeatedly revealed a consistent neurodegeneration of the dopaminergic substantia nigra in patients with SCA2 and with SCA3, parkinsonian motor features evolve only rarely. As the pathophysiological mechanism how SCA2 and SCA3 patients do not exhibit parkinsonism is still enigmatic, we performed a positron emission tomography and a post-mortem study of two independent cohorts of SCA2 and SCA3 patients with and without parkinsonian features. Positron emission tomography revealed a significant reduction of dopamine transporter levels in the striatum as well as largely unaffected postsynaptic striatal D2 receptors. In spite of this remarkable pathology in the motor mesostriatal pathway, only 4 of 19 SCA2 and SCA3 patients suffered from parkinsonism. The post-mortem investigation revealed, in addition to an extensive neuronal loss in the dopaminergic substantia nigra of all patients with spinocerebellar ataxia, a consistent affection of the thalamic ventral anterior and ventral lateral nuclei, the pallidum and the cholinergic pedunculopontine nucleus. With the exception of a single patient with SCA3 who suffered from parkinsonian motor features during his lifetime, the subthalamic nucleus underwent severe neuronal loss, which was clearly more severe in its motor territory than in its limbic or associative territories. Our observation that lesions of the motor territory of the subthalamic nucleus were consistently associated with the prevention of parkinsonism in our SCA2 and SCA3 patients matches the clinical experience that selective targeting of the motor territory of the subthalamic nucleus by focal lesions or deep brain stimulation can ameliorate parkinsonian motor features and is likely to counteract the manifestation of parkinsonism in SCA2 and SCA3 despite a severe neurodegeneration of the dopaminergic substantia nigra. [ABSTRACT FROM AUTHOR]

Purpose To prospectively compare the image quality and diagnostic performance of orbital MR images obtained by using a dual-source parallel transmission (pTX) 3D sequence (Sampling Perfection with Application optimized Contrasts using different flip angle Evolution, SPACE) with the image quality of conventional high-resolution standard protocol for clinical use in patients at 3T. Materials and methods After obtaining institutional review board approval and patient consent, 32 patients with clinical indication for orbital MRI were examined using a high-resolution conventional sequences and 3D pTX SPACE sequences. Quantitative measurements, image quality of the healthy orbit, incidence of artifacts, and the subjective diagnostic performance to establish diagnosis was rated. Statistical significance was calculated by using a Student's t -test and nonparametric Wilcoxon signed rank test. Results Length measurements were comparable in the two techniques, 3D pTX SPACE resulted in significant faster image acquisition with higher spatial resolution and less motion artifacts as well as better delineation of the optic nerve sheath. However, estimated contrast-to-noise and signal-to-noise and overall image quality as well as subjective scores of the conventional TSE imaging were rated significantly higher. The conventional MR sequences were the preferred techniques by the readers. Conclusion This study demonstrates the feasibility of 3D pTX SPACE of the orbit resulting in a rapid acquisition of isotropic high-resolution images. Although no pathology was missed in 3D pTX SPACE, conventional MRI techniques showed the higher diagnostic confidence in our study, presumably due to the higher signal-to-noise and contrast-to-noise ratios. We observed high-resolution TSE imaging to be the preferred technique, 3D pTX SPACE cannot replace conventional MRI so far. [ABSTRACT FROM AUTHOR]

Early postnatal infection with human cytomegalovirus (hCMV) may contribute to an adverse cognitive outcome in early preterm-born children (PT). We here set out to explore whether long-term neurobiological consequences of such an infection are detectable using fMRI in children and adolescents who were born very preterm and who either did (PThCMV+) or did not (PThCMV−) suffer from an early postnatal hCMV-infection, when compared with typically developing healthy control (HC) subjects. Overall, data from 71 children and adolescents could be included, 34 PT (of which 15 were PT hCMV+ and 19 were PT hCMV−) and 37 HC. Using a recently established 'dual use' fMRI task, we investigated language and visuospatial functions. There were significant activation differences in the left hippocampus (PT > HC and PThCMV+ > HC), and in the right anterior cingulate cortex (PThCMV− > PThCMV+) when performing the language task. Surprisingly, only a small region in the occipital cortex showed a significant activation difference (HC > PT HCMV−) when performing the visuospatial task. Targeted analyses revealed differences in gray matter volume, but not density, in several brain regions. Our results suggest that long-term neurobiological consequences of an early postnatal hCMV infection are detectable even in older children and adolescents formerly born very preterm, compatible with a higher effort when performing a cognitive task. This suggests that measures to prevent such an infection are warranted. Furthermore, an interrelation of brain structure and function was detected that may constitute a severe confound when using fMRI to compare structurally differing groups. Hum Brain Mapp 35:2594-2606, 2014. © 2013 Wiley Periodicals, Inc. [ABSTRACT FROM AUTHOR]

Complex visuospatial processing relies on distributed neural networks involving occipital, parietal and frontal brain regions. Effects of physiological maturation (during normal brain development) and proficiency on tasks requiring complex visuospatial processing have not yet been studied extensively, as they are almost invariably interrelated. We therefore aimed at dissociating the effects of age and performance on functional MRI (fMRI) activation in a complex visual search task. In our cross-sectional study, healthy children and adolescents (n = 43, 19 females, 7-17 years) performed a complex visual search task during fMRI. Resulting activation was analysed with regard to the differential effects of age and performance. Our results are compatible with an increase in the neural network's efficacy with age: within occipital and parietal cortex, the core regions of the visual exploration network, activation increased with age, and more so in the right than in the left hemisphere. Further, activation outside the visual search network decreased with age, mainly in left inferior frontal, middle temporal, and inferior parietal cortex. High-performers had stronger activation in right superior parietal cortex, suggesting a more mature visual search network. We could not see effects of age or performance in frontal cortex. Our results show that effects of physiological maturation and effects of performance, while usually intertwined, can be successfully disentangled and investigated using fMRI in children and adolescents. [ABSTRACT FROM AUTHOR]

Spinocerebellar ataxias are dominantly inherited disorders that are associated with progressive brain degeneration, mainly affecting the cerebellum and brainstem. As part of the multicentre European integrated project on spinocerebellar ataxias study, 37 patients with spinocerebellar ataxia-1, 19 with spinocerebellar ataxia-3 and seven with spinocerebellar ataxia-6 were clinically examined and underwent magnetic resonance imaging at baseline and after a 2-year follow-up. All patients were compared with age-matched and gender-matched healthy control subjects. Magnetic resonance imaging analysis included three-dimensional volumetry and observer-independent longitudinal voxel-based morphometry. Volumetry revealed loss of brainstem, cerebellar and basal ganglia volume in all genotypes. Most sensitive to change was the pontine volume in spinocerebellar ataxia-1, striatal volume in spinocerebellar ataxia-3 and caudate volume in spinocerebellar ataxia-6. Sensitivity to change, as measured by standard response mean, of the respective MRI measures was greater than that of the most sensitive clinical measure, the Scale for the Assessment and Rating of Ataxia. Longitudinal voxel-based morphometry revealed greatest grey matter loss in the cerebellum and brainstem in spinocerebellar ataxia-1, in the putamen and pallidum in spinocerebellar ataxia-3 and in the cerebellum, thalamus, putamen and pallidum in spinocerebellar ataxia-6. There was a mild correlation between CAG repeat length and volume loss of the bilateral cerebellum and the pons in spinocerebellar ataxia-1. Quantitative volumetry and voxel-based morphometry imaging demonstrated genotype-specific patterns of atrophy progression in spinocerebellar ataxias-1, 3 and 6, and they showed a high sensitivity to detect change that was superior to clinical scales. These structural magnetic resonance imaging findings have the potential to serve as surrogate markers, which might help to delineate quantifiable endpoints and non-invasive methods for rapid and reliable data acquisition, encouraging their use in clinical trials. [ABSTRACT FROM AUTHOR]

Abstract: A mutation in the coding region of the Tor1A gene, resulting in a deletion of a glutamic acid residue in the torsinA protein (∆ETorA), is the major cause of the inherited autosomal-dominant early onset torsion dystonia (DYT1). The pathophysiological consequences of this amino acid loss are still not understood. Currently available animal models for DYT1 dystonia provided important insights into the disease; however, they differ with respect to key features of torsinA associated pathology. We developed transgenic rat models harboring the full length human mutant and wildtype Tor1A gene. A complex phenotyping approach including classical behavioral tests, electrophysiology and neuropathology revealed a progressive neurological phenotype in ∆ETorA expressing rats. Furthermore, we were able to replicate key pathological features of torsinA associated pathology in a second species, such as nuclear envelope pathology, behavioral abnormalities and plasticity changes. We therefore suggest that this rat model represents an appropriate new model suitable to further investigate the pathophysiology of ∆ETorA and to test for therapeutic approaches. [Copyright &y& Elsevier]

Abstract: Background and objective: Biomarkers to monitor neurological dysfunction in autosomal dominant inherited spinocerebellar ataxias (SCA) are lacking. We therefore aimed to visualize, quantify and correlate localized brain atrophy with clinical symptoms in SCA1, SCA3, and SCA6. Methods: We compared patients suffering from SCA1 (n =48), SCA3 (n =24), and SCA6 (n =10) with 32 controls using magnetic resonance imaging (MRI) on four different scanners in eight centers followed by voxel-based morphometry (VBM) and quantitative three-dimensional (3D) volumetry. Results: SCA1 and SCA3 patients presented with severe atrophy in total brainstem (consisting of midbrain, pons, and medulla), pons, medulla, total cerebellum, cerebellar hemispheres and cerebellar vermis, putamen and caudate nucleus. Atrophy in the cerebellar hemispheres was less severe in SCA3 than in SCA1 and SCA6. Atrophy in SCA6 was restricted to the grey matter of the cerebellum (VBM and volumetry), total brainstem and pons (volumetry only). Overall, we did not observe substantial atrophy in the cerebral cortex. A discriminant analysis taking into account data from pons, cerebellar hemispheres, medulla, midbrain and putamen achieved a reclassification probability of 81.7% for SCA1, SCA3, and SCA6. The repeat length of the expanded allele showed a weak negative correlation with the volume of the brainstem, pons, caudate nucleus and putamen in SCA3, and a weak correlation with the pons in SCA1, whereas no such correlation was found in SCA6. Clinical dysfunction as measured by the Scale for the Assessment and Rating of Ataxia (SARA) and the Unified Huntington''s Disease Rating Scale functional assessment correlated best with the atrophy of pons in SCA1, with total brainstem atrophy in SCA3 and atrophy of total cerebellum in SCA6. Conclusions: Our data provide strong evidence that MRI is an attractive surrogate marker for clinical studies of SCA. In each SCA genotype clinical dysfunction may be caused by different patho-anatomical processes. [Copyright &y& Elsevier]