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Background and Objectives: NOTCH3 pathologic variants cause cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), which presents with stroke and dementia and is characterized by white matter hyperintensities (WMHs) on brain MRI. The R544C variant is a common pathologic variant in Taiwan, but not all carriers exhibit significant symptoms. We investigated whether WMHs occur before clinical symptoms in carriers with pathogenic variants, examined factors associated with WMHs, and explored their relationship with cognitive functions., Methods: We enrolled 63 R544C carriers without overt clinical disease (WOCD) and 37 age-matched and sex-matched noncarriers as controls from the Taiwan Precision Medicine Initiative data set. All participants underwent clinical interviews, comprehensive neuropsychological assessments, and brain MRI. We calculated total and regional WMH volumes, determined the age at which WMHs began increasing in carriers, and examined the relationship between WMHs and neuropsychological performance. Factors associated with WMH volumes were analyzed using multivariable linear regression models., Results: Compared with controls, R544C carriers WOCD had increased WMH volume, except in the occipital and midbrain areas, and showed a rapid increase in WMHs starting at age 48. They scored lower on the Mini-Mental State Examination (median = 28.4 vs 29.0, p = 0.048), Montreal Cognitive Assessment (MoCA) (median = 28.3 vs 29.0, p = 0.013), and memory and executive function tests than controls. After adjusting for age, sex, and education, MoCA scores were associated with whole-brain (r = -0.387, p adj = 0.008) and regional WMHs (all p adj < 0.05) except in the midbrain area. Age (β = 0.034, 95% CI 0.021-0.046, p < 0.001), hypercholesterolemia (β = 0.375, 95% CI 0.097-0.653, p = 0.009), and the vascular risk factor (VRF) index (β = 0.132, 95% CI 0.032-0.242, p = 0.019) were associated with the WMH severity in carriers., Discussion: Our study revealed that WMHs are extensively distributed in R544C carriers WOCD. They exhibited a rapid increase in WMHs beginning at age 48, approximately 7 years earlier than the reported age at symptomatic onset. Age was the strongest predictive factor of WMHs, and VRF, particularly hypercholesterolemia, might be modifying factors of WMHs.

To evaluate the necessity of performing whole spine magnetic resonance imaging (MRI) as opposed to solely lumbosacral MRI for detecting high-level spinal lesions (in the brain, cervical, or thoracic spine) in patients with anorectal malformations (ARM). This retrospective cohort study included 69 patients treated for ARM at a medical center between January 2011 and January 2022. Before March 2021, lumbosacral MRI was used for patients with ARM. Since March 2021, anomalies in the posterior cranial fossa and whole spine were assessed using routine MRI. Both male (n = 30, 43.4%) and female patients were included in the study. Out of the 69 patients, 19 (27.5%) underwent whole spine MRI, and 50 (72.5%) underwent lumbosacral MRI. In the whole spine group, 4 patients had anomalies beyond the lumbosacral area. In the lumbosacral group, 4 patients subsequently underwent whole spine MRI due to symptoms, with 3 revealing higher-level anomalies.High-level spinal and brain anomalies, although infrequent, are present in patients with ARM. The findings suggest considering routine whole spine MRI in screening ARM patients to avoid missing high-level anomalies., (© 2024. The Author(s).)

This review article provides a comprehensive overview of fetal MR imaging in supratentorial cerebral malformations. It emphasizes the importance of fetal MR imaging as an adjunct diagnostic tool used alongside ultrasound, improving the detection and characterization of prenatal brain abnormalities. This article reviews a spectrum of cerebral malformations, their MR imaging features, and the clinical implications of these findings. Additionally, it outlines the growing importance of fetal MR imaging in the context of perinatal care., Competing Interests: Disclosure The authors have nothing to disclose., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Idiopathic normal pressure hydrocephalus (iNPH) is a potentially reversible cause of dementia-like symptoms among the elderly. Current diagnostic guidelines for iNPH rely on clinical manifestations and ventricular morphology, which often lack accuracy. While magnetic resonance imaging (MRI) CSF flowmetry of the cerebral aqueduct provides a noninvasive aid to differential diagnosis, previous studies suffered from small sample sizes. This study compares the accuracy of different CSF flow parameters for iNPH diagnosis in a general patient population. From 2016 to 2018, a total of 216 subjects over 60 years of age were retrospectively enrolled, including 38 patients with iNPH and 178 patients with non-iNPH neurological conditions. All participants received phase-contrast MRI (PC-MRI) CSF flowmetry, with measurements performed independently by two radiologists. Flow parameters of iNPH and non-iNPH groups were compared along with their diagnostic accuracy. Absolute stroke volume (ABSV), forward flow, backward flow, mean flux and peak velocity were significantly higher in iNPH patients (P < 0.001, P < 0.001, P < 0.001, P = 0.008, P = 0.038, respectively). Backward flow had the highest diagnostic accuracy, followed by ABSV and forward flow. Net caudocranial aqueductal flow was observed in both groups, but with greater volume in the iNPH group. PC-MRI provides a non-invasive method of CSF flowmetry across the cerebral aqueduct and may aid in iNPH diagnosis. ABSV and its component flow values may provide better accuracy in identifying iNPH than other parameters., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2022. Published by Elsevier Ltd.)

Objective: Most patients with spinal cerebrospinal fluid (CSF) leakage require an epidural blood patch (EBP); however, the response to treatment is varied. This study aimed to compare the MRI findings at follow-up between EBP effective and non-effective groups and to identify imaging findings that predict EBP treatment failure., Methods: We retrospectively reviewed 48 patients who received EBP treatment for spinal CSF leakage. These patients were stratified into two groups: EBP effective ( n = 27) and EBP non-effective ( n = 21) using the results of the 3 month MRI as the end point., Results: Compared to the EBP non-effective group, the patients in the EBP effective group had a lower spinal CSF leakage number (2.67 vs 12.48; p = 0.001), lower spinal epidural fluid accumulation levels (3.00 vs 7.48; p = 0.004), brain descend (11.11% vs 38.10%; p = 0.027), pituitary hyperemia (18.52% vs 57.14%; p = 0.007), and decreased likelihood of ≥three numbers of spinal CSF leakage (25.93% vs 90.48%; p = 0.001) in the post-EBP MRI. Clinical non-responsiveness (OR: 57.84; 95% CI: 3.47-972.54; p = 0.005) and ≥three numbers of spinal CSF leakage (OR: 15.13; 95% CI: 1.45-159.06; p = 0.023) were associated with EBP failure. Between these variables, ≥three numbers of spinal CSF leakage identified using the post-EBP MRI demonstrated greater sensitivity in predicting EBP failure compared to clinical non-responsiveness (90.48% vs 61.9%)., Conclusion: The number of spinal CSF leakage identified using the post-EBP MRI with a cut-off value of three is an effective predictor of EBP failure., Advances in Knowledge: Compared to clinical responsiveness, the post-EBP MRI provided a more objective approach to predict the effectiveness of EBP treatment in patients with spinal CSF leakage.

Accurate quantification of brain tissue is a fundamental and challenging task in neuroimaging. Over the past two decades, statistical parametric mapping (SPM) and FMRIB's Automated Segmentation Tool (FAST) have been widely used to estimate gray matter (GM) and white matter (WM) volumes. However, they cannot reliably estimate cerebrospinal fluid (CSF) volumes. To address this problem, we developed the TRIO algorithm (TRIOA), a new magnetic resonance (MR) multispectral classification method. SPM8, SPM12, FAST, and the TRIOA were evaluated using the BrainWeb database and real magnetic resonance imaging (MRI) data. In this paper, the MR brain images of 140 healthy volunteers (51.5 ± 15.8 y/o) were obtained using a whole-body 1.5 T MRI system (Aera, Siemens, Erlangen, Germany). Before classification, several preprocessing steps were performed, including skull stripping and motion and inhomogeneity correction. After extensive experimentation, the TRIOA was shown to be more effective than SPM and FAST. For real data, all test methods revealed that the participants aged 20-83 years exhibited an age-associated decline in GM and WM volume fractions. However, for CSF volume estimation, SPM8-s and SPM12-m both produced different results, which were also different compared with those obtained by FAST and the TRIOA. Furthermore, the TRIOA performed consistently better than both SPM and FAST for GM, WM, and CSF volume estimation. Compared with SPM and FAST, the proposed TRIOA showed more advantages by providing more accurate MR brain tissue classification and volume measurements, specifically in CSF volume estimation., Competing Interests: The authors declare that there is no conflict of interest regarding the publication of this paper., (Copyright © 2021 Hsian-Min Chen et al.)

Background: According to the Standards for Reporting Vascular Changes on Neuroimaging, White Matter Hyperintensities (WMHs) are cerebral white matter lesions that are characterized by abnormal tissues of variable sizes and appear hyperintense in T2-weighted Magnetic Resonance (MR) measurements without cavitation (i.e., their tissue signals differ from those of Cerebrospinal Fluid or CSF). Such abnormal tissue regions are typically observed in the MR images of brains of healthy older adults and are associated with a number of geriatric neurodegenerative diseases. Explanations of the exact causes and mechanisms of these diseases remain inconclusive. Moreover, WMHs are typically identified by visual assessment and manual examination, both of which require considerable time. This brings up a need of developing a method for detecting WMHs more objectively and enabling patients to be treated early. As a consequence, damages on nerve cells can be limited and the severity of patients' conditions can be contained., Aims: This paper presents a computer-aided technique for automatically detecting and segmenting anomalies in MR images., Methods: The method has two steps: (1) a Band Expansion Process (BEP) to expand the dimensions of brain MR images nonlinearly and (2) anomaly detection algorithms to detect WMHs. Synthesized MR images provided by BrainWeb were used as benchmarks against which the detection performance of the algorithms was determined., Results: The most notable findings are as follows: Firstly, compared with the other anomaly detection algorithms and the Lesion Segmentation Tool (LST), BEP-anomaly detection is shown to be the most effective in detecting WMHs. Secondly, across all levels of background noise and inhomogeneity, the mean Similarity Index (SI) produced by our proposed algorithm is higher than that produced by LST, indicating that the algorithm is more effective than LST in segmenting WMHs from brain MR images., Conclusion: Experimental results demonstrated a significantly high accuracy of the BEP-K/R-RX method in detection of synthetic brain MS lesion data. In the meantime, it also effectively enhances the detection of brain lesions., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

Background: Diagnosis of spontaneous intracranial hypotension (SIH) relies on the ability of medical staff to recognize cerebrospinal fluid (CSF) leakage at the spine. However, difficulties with interobserver discrepancy sometimes occurred while reading magnetic resonance myelography (MRM) because clear image definition was lacking. In this study, we tried to determine which pattern of CSF distribution is more reliable for diagnosis of CSF leakage by using MRM., Methods: From January 2012 to August 2014, 19 SIH patients and 27 healthy controls (HC) were recruited into our study; 10 of the 19 patients were recovered (SIH-R) after treatment. Whole spine MRM was performed using the 3D-SPACE (three-dimensional sampling perfection with application-optimized contrasts using different flip-angle evolutions) sequence, and interpreted by two experienced neuroradiologists. Two 4-point classification systems of CSF distribution were used to evaluate the three-dimensional maximum intensity projection (3D MIP) and the thin-slice axial multiplanar reconstruction (MPR) images, respectively., Results: The interobserver agreement between the two readers interpreting the 3D MIP and thin-slice axial MPR MRM were moderate to good (κ=0.60-0.78). Grade 3 of 3D MIP and Type D of axial MPR MRM were only noticed in the SIH. Overall, Grade 3 of MIP and Type D of MPR showed significant difference (p<0.008) between the SIH and the HC in the whole spine. Type C at the T-spine was more frequently noted in the SIH than in the HC (p<0.038). By using "Grade 3", "Type D", "Type D and Type C at T-spine" as the diagnostic criteria of CSF leakage, the sensitivity, specificity, positive predict value (PPV), and negative predict value (NPV) were all > 70%., Conclusion: Grade 3 on 3D MIP and Type D on axial MPR MRM were definite criteria of MRM for localizing CSF leakage, and Type C in the T-spine was a probable leakage sign with high sensitivity and NPV., (Copyright © 2016 The Authors. Published by Elsevier Taiwan LLC.. All rights reserved.)

A new TRIO algorithm method integrating three different algorithms is proposed to perform brain MRI segmentation in the native coordinate space, with no need of transformation to a standard coordinate space or the probability maps for segmentation. The method is a simple voxel-based algorithm, derived from multispectral remote sensing techniques, and only requires minimal operator input to depict GM, WM, and CSF tissue clusters to complete classification of a 3D high-resolution multislice-multispectral MRI data. Results showed very high accuracy and reproducibility in classification of GM, WM, and CSF in multislice-multispectral synthetic MRI data. The similarity indexes, expressing overlap between classification results and the ground truth, were 0.951, 0.962, and 0.956 for GM, WM, and CSF classifications in the image data with 3% noise level and 0% non-uniformity intensity. The method particularly allows for classification of CSF with 0.994, 0.961 and 0.996 of accuracy, sensitivity and specificity in images data with 3% noise level and 0% non-uniformity intensity, which had seldom performed well in previous studies. As for clinical MRI data, the quantitative data of brain tissue volumes aligned closely with the brain morphometrics in three different study groups of young adults, elderly volunteers, and dementia patients. The results also showed very low rates of the intra- and extra-operator variability in measurements of the absolute volumes and volume fractions of cerebral GM, WM, and CSF in three different study groups. The mean coefficients of variation of GM, WM, and CSF volume measurements were in the range of 0.03% to 0.30% of intra-operator measurements and 0.06% to 0.45% of inter-operator measurements. In conclusion, the TRIO algorithm exhibits a remarkable ability in robust classification of multislice-multispectral brain MR images, which would be potentially applicable for clinical brain volumetric analysis and explicitly promising in cross-sectional and longitudinal studies of different subject groups.

Background: Most diagnostic tools for spontaneous intracranial hypotension (SIH) are either invasive or occasionally inconsistent with the clinical condition. In this study, we examined the cerebrospinal fluid (CSF) dynamics in SIH using phase-contrast magnetic resonance (PC-MR) imaging., Materials and Method: Seventeen SIH patients and 32 healthy individuals, matched by sex and age, were recruited. Each person underwent brain and PC-MR imaging using 3-Tesla MRI. We evaluated the differences in image parameters among patients during the initial and recovery stages against the status of the control group., Results: SIH patients had lower CSF flow-volume, flux, peak velocity, and higher systolic-to-diastolic time ratio, as well as systolic-to-diastolic volume ratio compared to the control group and the conditions when they recovered. The flow time and volume of the diastolic phase markedly increased after treatment. The discriminating power of PC-MR for SIH was good. Diffuse pachymeningeal enhancement and venous engorgement were present when their PC-MR values were lower than the cut-off values for SIH diagnosis. The headache scores correlated with the peak velocity and pituitary volume., Conclusion: Noninvasive PC-MR could provide valid parameters for diagnosis and treatment follow-up in SIH patients. It may be more sensitive than conventional brain MRI., (© International Headache Society 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.)

Objective: This study's objective was to explore whether certain parameters measurable by dual-energy computed tomography (DECT) performed 24 h after endovascular thrombectomy (EVT) can predict subsequent hemorrhagic transformation. Material and Methods: We retrospectively reviewed patients with acute ischemic stroke (AIS) managed with EVT who had follow-up DECT within 24 h post-EVT between January 2019 and December 2023. Clinical and image parameters were recorded for predictive factor analysis. The primary outcome was hemorrhagic transformation, which was determined by using follow-up computed tomography (CT) or magnetic resonance imaging (MRI). The secondary outcomes were in-hospital mortality and 3-month post-EVT favorable functional outcome, as defined by a modified ranking scale (mRS) score of ≤2. Results: A total of 152 patients were included in this study. Multivariable analysis showed that the VNC-ASPECT score (p = 0.002) and superior sagittal sinus density (p = 0.01) were significantly associated with hemorrhagic transformation. For in-hospital survival rate analysis, post-EVT NIHSS measured 24 h post-EVT was an effective predictor, with a cutoff value of 23 (≤23: 88% vs. >23: 52.1%; p < 0.001). For functional outcome analysis, age (p < 0.001), tPA prior to EVT (p = 0.017), NIHSS 24 h post-EVT (p = 0.001), and VNC-ASPECT score (p < 0.003) were associated with a favorable functional outcome 3 months after EVT. Conclusions: The VNC-ASPECT score was associated with both hemorrhagic transformation and a 3-month post-EVT favorable functional outcome, and could therefore be an useful predictor for the development of hemorrhagic transformation. [ABSTRACT FROM AUTHOR]

Acute ischemic stroke in young adults typically carries significant implications for morbidity, mortality, and long-term disability. In this study, we describe the case of a 34-year-old male with no prior medical history who presented with symptoms of right-sided weakness and slurred speech, suggesting an acute ischemic stroke. Initial CT angiography revealed an occlusion in the left M2 segment middle cerebral artery (MCA). The occlusion was successfully recanalized through emergent endovascular thrombectomy, which also identified a dissection as the cause of the stroke. Follow-up assessments at 3 days and three months, which included advanced vessel wall MRI, highlighted the critical role of intracranial artery dissection in strokes among young adults and provided essential images for ongoing evaluation. [ABSTRACT FROM AUTHOR]

Acute hemorrhagic encephalomyelitis (AHEM) is the most severe form of acute disseminated encephalomyelitis (ADEM). Patients with AHEM usually have unfavorable outcomes with high mortality rate. We reported a middle-aged male, who was diagnosed with AHEM and died 35 days after admission even under intensive immune therapy. Clinical courses were recorded and serial MR images were demonstrated to illustrate the rapidly changes in brain parenchyma. By highlighting these aspects, we hope to provide valuable insights for future studies and potential advancements in the management of AHEM. [ABSTRACT FROM AUTHOR]

Objectives: Neuropsychiatric systemic lupus erythematosus (NPSLE) is associated with adverse outcomes; however, imaging abnormalities are only detectable by conventional brain magnetic resonance imaging (MRI) in up to 50% of patients. This study investigated the variability in cortical thickness and diffusion tensor imaging (DTI) parameters among patients with NPSLE whose brain morphology appeared normal on conventional MRI. Methods: This retrospective study enrolled 27 female patients with NPSLE (median age: 41.0 years, range: 22–63 years) and 34 female healthy controls (median age: 37.0 years, range: 24–55 years). None exhibited evident abnormalities on conventional MRI. Regional volumes, cortical thickness, and DTI parameters, including fractional anisotropy (FA) and mean diffusivity (MD), were compared. Age-adjusted multivariable logistic regression analysis was conducted to detect significant NPSLE-associated differences. Results: No significant differences in grey or white matter volume fractions were observed between the groups. However, the NPSLE group demonstrated significant cortical thinning in the right pars opercularis (2.45 vs 2.52 mm, p = 0.007), reduced FA values in the fornix (0.35 vs 0.40, p = 0.001) and left anterior limb of internal capsule (0.50 vs 0.52, p = 0.012), and increased MD in the fornix (1.71 vs 1.48, p = 0.009) and left posterior corona radiata (0.80 vs 0.76, p = 0.005) compared with those of healthy controls. Conclusions: Cortical thickness measurements and DTI analyses can be used to detect differential variations in patients with NPSLE who exhibit an otherwise normal brain structure on conventional MRI, indicating the existence of subtle changes despite the absence of obvious macrostructural central nervous system involvement of lupus. [ABSTRACT FROM AUTHOR]

Differentiation cerebellar multiple systemic atrophy (MSA-C) from spinocerebellar ataxia (SCA) is important. The "hot cross bun" sign (HCBS) at pons and magnetic resonance spectroscopy (MRS) are helpful. However, the prevalence of HCBS and the alteration of cerebellar MRS parameters are evolving with disease progression. We hypothesized that since the HCBS and MRS are evolving with time, different parameters for differentiation of MSA-C and SCA are required at different disease stages. The aim of this study was to evaluate the HCBS and MRS changes in patients with MSA-C and SCA at different disease stages. A total of 398 patients with molecularly confirmed SCA (SCA1, 2, 3, 6, 17) and 286 patients diagnosed with probable MSA-C (without mutations in SCA1, 2, 3, 6, 17 genes), who had received brain magnetic resonance imaging (MRI) and MRS from January 2000 to January 2020, were recruited. Twenty-five patients were molecularly identified as having SCA1, 68 as SCA2, 253 as SCA3, 34 as SCA6, and 18 as SCA17. We compared their clinical parameters and neuroimaging features at different disease stages. The presence of HCBS was assessed using an axial T2 fast spin-echo or FLAIR sequence. Proton MRS was recorded with voxel of interest focusing on cerebellar hemispheres and cerebellar vermis and avoiding cerebrospinal fluid spaces space using a single-voxel stimulated echo acquisition mode sequence. We found that patients with MSA-C tend to have a higher prevalence of pontine HCBS, worse Scale for the Assessment and Rating of Ataxia scores, lower cerebellar N-acetyl aspartate (NAA)/creatinine (Cr), and choline (Cho)/Cr, compared to patients with SCA at corresponding disease stages. In MSA-C patients with a disease duration < 1 year and without pontine HCBS, a cerebellar NAA/Cr ≤ 0.79 is a good indicator of the possibility of MSA-C. By using the pontine HCBS and cerebellar MRS, discerning MSA-C from SCA became possible. This study provides cutoff values of MRS to serve as clues in differentiating MSA-C from SCAs. [ABSTRACT FROM AUTHOR]

Objectives: Epidural blood patching (EBP) is the mainstay therapy for spontaneous intracranial hypotension (SIH). MRI is used for evaluating spinal CSF leakage. Post-EBP MRI has been. shown to be effective in predicting the efficacy of EBP. However, there are few reports on how post-EBP MRI findings may change with time. The aim of this study was to evaluate the relationship between post-EBP MRI findings at different time points and the corresponding effectiveness of EBP. Methods: We retrospectively reviewed 63 SIH patients who had received target EBP. All patients received an MRI follow-up within 10 days (post-EBP MRI) and at 3 months after EBP (3-month MRI). A sub-group analysis was performed at different post-EBP MRI time points (0–2, 3–6, and 7–10 days). The relationships between the post-EBP MRI findings and the EBP effectiveness were evaluated. Results: Thirty-five (55.56%) patients were assigned to the EBP-effective group, and 28 (44.44%) were assigned to the EBP non-effective group according to the 3-month MRI. Compared to the EBP non-effective group, the EBP-effective group had significantly lower numbers of spinal CSF leakage in the post-EBP MRI (4.49 vs. 11.71; p = 0.000) and greater numbers of leakage improvement (7.66 vs. 2.96; p = 0.003). For patients who received post-EBP MRI during periods of 0–10, 0–2, 3–6, and 7–10 days, the cutoff values of numbers of spinal CSF leakage for predicting EBP failure were 4, 6, 4, and 5, respectively, with an AUC above 0.77. Conclusion: By using post-EBP MRI, which only takes approximately 20 min, predicting EBP efficacy became possible in SIH patients. This study provides cutoff values of numbers of spinal CSF leakage at different follow-up times to serve as clues of if further EBP is needed, which provides the novelty of the current study. [ABSTRACT FROM AUTHOR]

Background: Spinal cerebrospinal fluid (CSF) leakage is frequently encountered clinically after lumbar puncture or spontaneous events. Although some patients recover without treatment or after intensive hydration, some require an epidural blood patch (EBP). The risks of nonresponsive hydration remain unknown. Therefore, we identified the risk factors for patients with spinal CSF leakage nonresponsive to hydration. Methods: We retrospectively reviewed patients diagnosed with spinal CSF leakage between January 2010 and March 2021. Clinical data, including patient age, sex, etiology, and radiological indications in magnetic resonance imaging, were compared between patients who were responsive and non-responsive to hydration. Results: Of the 74 patients with spinal CSF leakage, 25 were responsive to hydration and 49 required EBP. Patients who were nonresponsive to hydration were older (39.27 vs. 34.32 years, P = 0.01), had a higher percentage of spontaneous intracranial hypotension (93.88% vs. 68.00%, P = 0.005), had more spinal CSF leakage (12.04 vs. 8.04, P = 0.01), and had a higher percentage of dural sinus engorgement (81.63% vs. 60.00%, P = 0.044). Spontaneous intracranial hypotension (odds ratio [OR]: 4.63; 95% confidence interval [CI]: 1.00-21.38) and having ≥9 spinal CSF leakages (OR: 3.29; 95% CI: 1.08-10.01), as indicated by magnetic resonance myelography, are considered risk factors for noneffective hydration. Conclusions: Patients with spinal CSF leakage who have spontaneous intracranial hypotension and those with ≥9 spinal CSF leakages are considered at risk for noneffective hydration. EBP should be considered early in these patients. [ABSTRACT FROM AUTHOR]

Background: Isolated sulfite oxidase deficiency (ISOD) is a very rare autosomal recessive inherited neurometabolic disease. The most striking postnatal neuroimaging finding is multicystic encephalomalacia, which occurs rapidly within days to weeks after birth and mimics severe hypoxic-ischemic encephalopathy. The aim of this study was to describe the prenatal neuroimaging features in a neonate and a fetus diagnosed with ISOD.Results: We report an 11-day-old female neonate who presented with feeding difficulties, decreased activity, neonatal seizures, and movement disorders within a few days after birth. Brain MRI at 9 days of age showed cystic lesions over the left frontal and temporal areas, diffuse and evident T2 high signal intensity of bilateral cerebral cortex, and increased T2 signal intensity of the globus pallidi. A pronounced low level of plasma cysteine and normal level of plasma uric acid were noted. Mutation analysis of SUOX revealed homozygous c.1200C > G mutations, resulting in an amino acid substitution of tyrosine to a stop codon (Y400X). The diagnosis of ISOD was made. The brain MRI of a prenatally diagnosed ISOD fetus of the second pregnancy of the mother of the index case showed poor gyration and differentiation of cortical layers without formation of cystic lesions at gestational age 21 weeks.Conclusion: Cystic brain destruction might occur prenatally and neurodevelopment of gyration and differentiation of the cortical layers in the developing brain could be affected by sulfite accumulation early during the second trimester in ISOD patients. This is the first description of the prenatal neurodevelopment of brain disruption in ISOD. [ABSTRACT FROM AUTHOR]

Time-resolved magnetic resonance angiography (TR MRA) is a promising less invasive technique for the diagnosis of intracranial vascular lesions and hypervascular tumors. Similar to 4-dimensional computed tomographic angiography obtaining high frame rate images, TR MRA utilizes acceleration techniques to acquire sequential arterial and venous phase images for identifying, localizing, and classifying vascular lesions. Because of the good agreement with digital subtraction angiography for grading brain arteriovenous malformations with the Spetzler–Martin classification and the good sensitivity for visualizing arteriovenous fistulas, studies have suggested that TR MRA could serve as a screening or routine follow-up tool for diagnosing intracranial vascular disorders. In this pictorial essay, we report on the use of TR MRA at 3.0 T to diagnose intracranial vascular lesions and hypervascular tumors, employing DSA as the reference technique. [ABSTRACT FROM AUTHOR]

Rupture of a right hepatic artery pseudoaneurysm into the gallbladder is very rare. We demonstrated a 20-mm dumbbell-shaped pseudoaneurysm in the gallbladder lumen by using contrast-enhanced magnetic resonance angiography in a 73-year-old man with acute right upper abdominal pain. Inflammation of the gallbladder caused by calculous cholecystitis, which leads to biliary leakage and erodes the right hepatic artery, could have been the cause. [Copyright &y& Elsevier]