Development of Gestational Age-Based Fetal Brain and Intracranial Volume Reference Norms Using Deep Learning.

BACKGROUND AND PURPOSE: Fetal brain MR imaging interpretations are subjective and require subspecialty expertise. We aimed to develop a deep learning algorithm for automatically measuring intracranial and brain volumes of fetal brain MRIs across gestational ages. MATERIALS AND METHODS: This retrospective study included 246 patients with singleton pregnancies at 19-38 weeks gestation. A 3D U-Net was trained to segment the intracranial contents of 2D fetal brain MRIs in the axial, coronal, and sagittal planes. An additional 3D U-Net was trained to segment the brain from the output of the first model. Models were tested on MRIs of 10 patients (28 planes) via Dice coefficients and volume comparison with manual reference segmentations. Trained U-Nets were applied to 200 additional MRIs to develop normative reference intracranial and brain volumes across gestational ages and then to 9 pathologic fetal brains. RESULTS: Fetal intracranial and brain compartments were automatically segmented in a mean of 6.8 (SD, 1.2) seconds with median Dices score of 0.95 and 0.90, respectively (interquartile ranges, 0.91-0.96/0.89-0.91) on the test set. Correlation with manual volume measurements was high (Pearson r = 0.996, P < .001). Normative samples of intracranial and brain volumes across gestational ages were developed. Eight of 9 pathologic fetal intracranial volumes were automatically predicted to be >2 SDs from this age-specific reference mean. There were no effects of fetal sex, maternal diabetes, or maternal age on intracranial or brain volumes across gestational ages. CONCLUSIONS: Deep learning techniques can quickly and accurately quantify intracranial and brain volumes on clinical fetal brain MRIs and identify abnormal volumes on the basis of a normative reference standard.

Survey of the American Society of Neuroradiology Membership on the Use and Value of Extracranial Carotid Vessel Wall MRI.

BACKGROUND AND PURPOSE: Extracranial vessel wall MRI (EC-VWI) contributes to vasculopathy characterization. This survey study investigated EC-VWI adoption by American Society of Neuroradiology (ASNR) members and indications and barriers to implementation. MATERIALS AND METHODS: The ASNR Vessel Wall Imaging Study Group survey on EC-VWI use, frequency, applications, MR imaging systems and field strength used, protocol development approaches, vendor engagement, reasons for not using EC-VWI, ordering provider interest, and impact on clinical care was distributed to the ASNR membership between April 2, 2019, to August 30, 2019. RESULTS: There were 532 responses; 79 were excluded due to minimal, incomplete response and 42 due to redundant institutional responses, leaving 411 responses. Twenty-six percent indicated that their institution performed EC-VWI, with 66.3% performing it ≤1-2 times per month, most frequently on 3T MR imaging, with most using combined 3D and 2D protocols. Protocols most commonly included pre- and postcontrast T1-weighted imaging, TOF-MRA, and contrast-enhanced MRA. Inflammatory vasculopathy (63.3%), plaque vulnerability assessments (61.1%), intraplaque hemorrhage (61.1%), and dissection-detection/characterization (51.1%) were the most frequent applications. For those not performing EC-VWI, the reasons were a lack of ordering provider interest (63.9%), lack of radiologist time/interest (47.5%) or technical support (41.4%) for protocol development, and limited interpretation experience (44.9%) and knowledge of clinical applications (43.7%). Reasons given by 46.9% were that no providers approached radiology with interest in EC-VWI. If barriers were overcome, 51.1% of those not performing EC-VWI indicated they would perform it, and 40.6% were unsure; 48.6% did not think that EC-VWI had impacted patient management at their institution. CONCLUSIONS: Only 26% of neuroradiology groups performed EC-VWI, most commonly due to limited clinician interest. Improved provider and radiologist education, protocols, processing techniques, technical support, and validation trials could increase adoption.

Arterial Spin-Labeling Perfusion for PHACE Syndrome.

BACKGROUND AND PURPOSE: Arterial stroke is a rare-but-reported complication in patients with posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities (PHACE) syndrome. Currently, stroke risk is inferred by the severity of arterial anomalies identified on MRA, though no evidenced-based data exist. The purpose of our study was to determine whether arterial spin-labeling MR imaging perfusion can detect alterations in CBF in patients with PHACE syndrome. MATERIALS AND METHODS: Records were reviewed from 3 institutions for all patients with PHACE syndrome who underwent arterial spin-labeling from 2000 to 2019. CBF was qualitatively investigated with arterial spin-labeling to determine whether there was decreased or normal perfusion. Arterial anomalies were characterized on MRA imaging, and parenchymal brain findings were evaluated on conventional MR imaging sequences. RESULTS: Forty-one patients with PHACE syndrome had arterial spin-labeling imaging. There were 30 females and 11 males (age range, 7 days to 15 years). Of the 41 patients, 10 (24%) had decreased CBF signal corresponding to a major arterial territory. Ten of 10 patients had decreased CBF signal in the anterior circulation, 2/10 had decreased anterior and posterior circulation CBF signal, 2/10 had decreased bilateral anterior circulation CBF signal, and 1/10 had globally decreased CBF signal. Forty of 41 (97.5%) patients had at least 1 arteriopathy, and in those with decreased CBF signal, the arteriopathy corresponded to the CBF signal alteration in 10/10 patients. CONCLUSIONS: Arterial spin-labeling can potentially characterize hemodynamic changes in patients with PHACE syndrome.

Artificial Intelligence in Neuroradiology: Current Status and Future Directions.

Fueled by new techniques, computational tools, and broader availability of imaging data, artificial intelligence has the potential to transform the practice of neuroradiology. The recent exponential increase in publications related to artificial intelligence and the central focus on artificial intelligence at recent professional and scientific radiology meetings underscores the importance. There is growing momentum behind leveraging artificial intelligence techniques to improve workflow and diagnosis and treatment and to enhance the value of quantitative imaging techniques. This article explores the reasons why neuroradiologists should care about the investments in new artificial intelligence applications, highlights current activities and the roles neuroradiologists are playing, and renders a few predictions regarding the near future of artificial intelligence in neuroradiology.

Predictive Value of Noncontrast Head CT with Negative Findings in the Emergency Department Setting.

BACKGROUND AND PURPOSE: Noncontrast head CTs are routinely acquired for patients with neurologic symptoms in the emergency department setting. Anecdotally, noncontrast head CTs performed in patients with prior negative findings with the same clinical indication are of low diagnostic yield. We hypothesized that the rate of acute findings in noncontrast head CTs performed in patients with a preceding study with negative findings would be lower compared with patients being imaged for the first time. MATERIALS AND METHODS: We retrospectively evaluated patients in the emergency department setting who underwent noncontrast head CTs at our institution during a 4-year period, recording whether the patient had undergone a prior noncontrast head CT, the clinical indication for the examination, and the examination outcome. Positive findings on examinations were defined as those that showed any intracranial abnormality that would necessitate a change in acute management, such as acute hemorrhage, hydrocephalus, herniation, or interval worsening of a prior finding. RESULTS: During the study period, 8160 patients in the emergency department setting underwent a total of 9593 noncontrast head CTs; 88.2% (7198/8160) had a single examination, and 11.8% (962/8160) had at least 1 repeat examination. The baseline positive rate of the "nonrepeat" group was 4.3% (308/7198). The 911 patients in the "repeat" group with negative findings on a baseline/first CT had a total of 1359 repeat noncontrast head CTs during the study period. The rate of positive findings for these repeat examinations was 1.8% (25/1359), significantly lower than the 4.3% baseline rate (P < .001). Of the repeat examinations that had positive findings, 80% (20/25) had a study indication that was discordant with that of the prior examination, compared with only 44% (593/1334) of the repeat examinations that had negative findings (P < .001). CONCLUSIONS: In a retrospective observational study based on approximately 10,000 examinations, we found that serial noncontrast head CT examinations in patients with prior negative findings with the same study indication are less likely to have positive findings compared with first-time examinations or examinations with a new indication. This finding suggests a negative predictive value of a prior noncontrast head CT examination with negative findings with the same clinical indication.

Revisiting classic MRI findings of venous malformations: Changes in protocols may lead to potential misdiagnosis.

Introduction Magnetic resonance imaging (MRI) is most sensitive and specific for characterizing venous malformations (VMs). VMs typically demonstrate central enhancement on delayed-contrast imaging. Fluid-fluid levels (FFLs) are uncommon in VMs and common in lymphatic malformations (LMs). Technology has advanced since the initial description of these findings. Rates of detection of these MRI findings in VMs may have changed as MRI technology and techniques have evolved. Methods and methods A prospectively maintained database from a multidisciplinary vascular anomalies clinic was reviewed to identify patients with final diagnosis of VM or LM. Patients with reviewable contrast-enhanced MRIs were selected, reviewing the oldest MRI studies in the database against the newest MRI studies to identify equal numbers of patients from the temporal extremes. Imaging was reviewed to assess for presence of FFLs. Enhancement was quantified by measuring signal in the same location of the lesion both on pre- and postcontrast sequences Results Forty patients were identified for analysis. Twenty studies with sufficient archived imaging for review were performed between 1995 and 2006; 20 such studies were performed between 2011 and 2012. The new imaging cohort had higher rates of FFL visualization ( p = 0.001). Correlation was found between time to imaging following contrast and degree of enhancement ( p < 0.001). Inverse correlation was found between scan date and time to contrast ( p = 0.001) and scan date and enhancement ( p = 0.021). Conclusion FFLs should no longer be considered exclusionary for the diagnosis of VMs. Timing following contrast administration should be maximized to increase degree of enhancement to confirm the diagnosis of VMs.

Long-Term Effectiveness of Direct CT-Guided Aspiration and Fenestration of Symptomatic Lumbar Facet Synovial Cysts.

BACKGROUND AND PURPOSE: Lumbar facet synovial cysts are commonly seen in facet degenerative arthropathy and may be symptomatic when narrowing the spinal canal or compressing nerve roots. The purpose of this study was to analyze the safety, effectiveness, and long-term outcomes of direct CT-guided lumbar facet synovial cyst aspiration and fenestration for symptom relief and for obviating an operation. MATERIALS AND METHODS: We retrospectively reviewed the medical records and imaging studies of 64 consecutive patients between 2006 and 2016 who underwent 85 CT-guided lumbar facet synovial cyst fenestration procedures in our department. We recorded patient demographics, lumbar facet synovial cyst imaging characteristics, presenting symptoms, change in symptoms after the procedure, and whether they underwent a subsequent operation. We also assessed long-term outcomes from the medical records and via follow-up telephone surveys with patients. RESULTS: Direct CT-guided lumbar facet synovial cyst puncture was technically successful in 98% of procedures. At first postprocedural follow-up, 86% of patients had a complete or partial symptomatic response. During a mean follow-up of 49 months, 56% of patients had partial or complete long-term relief without the need for an operation; 44% of patients underwent an operation. Patients with calcified, thick-rimmed, or low T2 signal intensity cysts were less likely to respond to the procedure and more likely to need an operation. CONCLUSIONS: CT-guided direct lumbar facet synovial cyst aspiration and fenestration procedures are safe, effective, and minimally invasive for symptomatic treatment of lumbar synovial facet cysts. This procedure obviates an operation in a substantial number of patients, even at long-term follow-up, and should be considered before surgical intervention.

Entorhinal Cortex: Antemortem Cortical Thickness and Postmortem Neurofibrillary Tangles and Amyloid Pathology.

BACKGROUND AND PURPOSE: The entorhinal cortex, a critical gateway between the neocortex and hippocampus, is one of the earliest regions affected by Alzheimer disease-associated neurofibrillary tangle pathology. Although our prior work has automatically delineated an MR imaging-based measure of the entorhinal cortex, whether antemortem entorhinal cortex thickness is associated with postmortem tangle burden within the entorhinal cortex is still unknown. Our objective was to evaluate the relationship between antemortem MRI measures of entorhinal cortex thickness and postmortem neuropathological measures. MATERIALS AND METHODS: We evaluated 50 participants from the Rush Memory and Aging Project with antemortem structural T1-weighted MR imaging and postmortem neuropathologic assessments. Here, we focused on thickness within the entorhinal cortex as anatomically defined by our previously developed MR imaging parcellation system (Desikan-Killiany Atlas in FreeSurfer). Using linear regression, we evaluated the association between entorhinal cortex thickness and tangles and amyloid-ß load within the entorhinal cortex and medial temporal and neocortical regions. RESULTS: We found a significant relationship between antemortem entorhinal cortex thickness and entorhinal cortex (P = .006) and medial temporal lobe tangles (P = .002); we found no relationship between entorhinal cortex thickness and entorhinal cortex (P = .09) and medial temporal lobe amyloid-ß (P = .09). We also found a significant association between entorhinal cortex thickness and cortical tangles (P = .003) and amyloid-ß (P = .01). We found no relationship between parahippocampal gyrus thickness and entorhinal cortex (P = .31) and medial temporal lobe tangles (P = .051). CONCLUSIONS: Our findings indicate that entorhinal cortex-associated in vivo cortical thinning may represent a marker of postmortem medial temporal and neocortical Alzheimer disease pathology.

Microstructure of the Default Mode Network in Preterm Infants.

BACKGROUND AND PURPOSE: Diffusion and fMRI has been providing insights to brain development in addition to anatomic imaging. This study aimed to evaluate the microstructure of white matter tracts underlying the default mode network in premature infants by using resting-state functional MR imaging in conjunction with diffusion tensor imaging-based tractography. MATERIALS AND METHODS: A cohort of 44 preterm infants underwent structural T1-weighted imaging, resting-state fMRI, and DTI at 3T, including 21 infants with brain injuries and 23 infants with normal-appearing structural imaging as controls. Neurodevelopment was evaluated with the Bayley Scales of Infant Development at 12 months' adjusted age. Probabilistic independent component analysis was applied to resting-state fMRI data to explore resting-state networks. The localized clusters of the default mode network were used as seeding for probabilistic tractography. The DTI metrics (fractional anisotropy, mean diffusivity, axial diffusivity, and radial diffusivity) of the reconstructed primary tracts within the default mode network-cingula were measured. RESULTS: Results revealed decreased fractional anisotropy (0.20 ± 0.03) and elevated radial diffusivity values (1.24 ± 0.16) of the cingula in the preterm infants with brain injuries compared with controls (fractional anisotropy, 0.25 ± 0.03; P < .001; radial diffusivity, 1.06 ± 0.16; P = .001). The Bayley Scales of Infant Development cognitive scores were significantly associated with cingulate fractional anisotropy (P = .004) and radial diffusivity (P = .021); this association suggests that the microstructural properties of interconnecting axonal pathways within the default mode network are of critical importance in the early neurocognitive development of infants. CONCLUSIONS: This study of combined resting-state fMRI and DTI at rest suggests that such studies may allow the investigation of key functional brain circuits in premature infants, which could function not only as diagnostic tools but also as biomarkers for long-term neurodevelopmental outcomes.

Intracranial Vessel Wall MRI: Principles and Expert Consensus Recommendations of the American Society of Neuroradiology.

Intracranial vessel wall MR imaging is an adjunct to conventional angiographic imaging with CTA, MRA, or DSA. The technique has multiple potential uses in the context of ischemic stroke and intracranial hemorrhage. There remain gaps in our understanding of intracranial vessel wall MR imaging findings and research is ongoing, but the technique is already used on a clinical basis at many centers. This article, on behalf of the Vessel Wall Imaging Study Group of the American Society of Neuroradiology, provides expert consensus recommendations for current clinical practice.

More than memory impairment in voltage-gated potassium channel complex encephalopathy.

BACKGROUND AND PURPOSE: Autoimmune encephalopathies (AEs) are a heterogeneous group of neurological disorders that affect cognition. Although memory difficulties are commonly endorsed, few reports of AEs inclusively assess all cognitive domains in detail. Our aim was to perform an unbiased cognitive evaluation of AE patients with voltage-gated potassium channel complex antibodies (VGKCC -Abs) in order to delineate cognitive strengths and weaknesses. METHODS: Serial VGKCC -Ab AE subjects (n = 12) were assessed with a comprehensive evaluation of memory, executive functions, visuospatial skills and language. Clinical magnetic resonance imaging (MRI) (n = 10/12) was evaluated. Five subjects had serial cognitive testing available, permitting descriptive analysis of change. RESULTS: Subjects demonstrated mild to moderate impairment in memory (mean Z = -1.9) and executive functions (mean Z = -1.5), with variable impairments in language and sparing of visuospatial skills. MRI findings showed T2 hyperintensities in medial temporal lobe (10/10) and basal ganglia (2/10). Serial cognitive examination revealed heterogeneity in cognitive function; whereas most patients improved in one or more domains, residual impairments were observed in some patients. CONCLUSIONS: This study augments previous neuropsychological analyses in VGKCC -Ab AE by identifying not only memory and executive function deficits but also language impairments, with preservation of visuospatial functioning. The study further highlights the importance of domain-specific testing to parse out the complex cognitive phenotypes of VGKCC -Ab AE.

Structural MRI connectome in development: challenges of the changing brain.

MRI connectomics is an emerging approach to study the brain as a network of interconnected brain regions. Understanding and mapping the development of the MRI connectome may offer new insights into the development of brain connectivity and plasticity, ultimately leading to improved understanding of normal development and to more effective diagnosis and treatment of developmental disorders. In this review, we describe the attempts made to date to map the whole-brain structural MRI connectome in the developing brain and pay a special attention to the challenges associated with the rapid changes that the brain is undergoing during maturation. The two main steps in constructing a structural brain network are (i) choosing connectivity measures that will serve as the network "edges" and (ii) finding an appropriate way to divide the brain into regions that will serve as the network "nodes". We will discuss how these two steps are usually performed in developmental studies and the rationale behind different strategies. Changes in local and global network properties that have been described during maturation in neonates and children will be reviewed, along with differences in network topology between typically and atypically developing subjects, for example, owing to pre-mature birth or hypoxic ischaemic encephalopathy. Finally, future directions of connectomics will be discussed, addressing important steps necessary to advance the study of the structural MRI connectome in development.

Quantitative 7T phase imaging in premanifest Huntington disease.

BACKGROUND AND PURPOSE: In vivo MR imaging and postmortem neuropathologic studies have demonstrated elevated iron concentration and atrophy within the striatum of patients with Huntington disease, implicating neuronal loss and iron accumulation in the pathogenesis of this neurodegenerative disorder. We used 7T MR imaging to determine whether quantitative phase, a measurement that reflects both iron content and tissue microstructure, is altered in subjects with premanifest Huntington disease. MATERIALS AND METHODS: Local field shift, calculated from 7T MR phase images, was quantified in 13 subjects with premanifest Huntington disease and 13 age- and sex-matched controls. All participants underwent 3T and 7T MR imaging, including volumetric T1 and 7T gradient recalled-echo sequences. Local field shift maps were created from 7T phase data and registered to caudate ROIs automatically parcellated from the 3T T1 images. Huntington disease-specific disease burden and neurocognitive and motor evaluations were also performed and compared with local field shift. RESULTS: Subjects with premanifest Huntington disease had smaller caudate volume and higher local field shift than controls. A significant correlation between these measurements was not detected, and prediction accuracy for disease state improved with inclusion of both variables. A positive correlation between local field shift and genetic disease burden was also found, and there was a trend toward significant correlations between local field shift and neurocognitive tests of working memory and executive function. CONCLUSIONS: Subjects with premanifest Huntington disease exhibit differences in 7T MR imaging phase within the caudate nuclei that correlate with genetic disease burden and trend with neurocognitive assessments. Ultra-high-field MR imaging of quantitative phase may be a useful approach for monitoring neurodegeneration in premanifest Huntington disease.

Changes in the thalamus in atypical parkinsonism detected using shape analysis and diffusion tensor imaging.

BACKGROUND AND PURPOSE: The thalamus is interconnected with the nigrostriatal system and cerebral cortex and has a major role in cognitive function and sensorimotor integration. The purpose of this study was to determine how regional involvement of the thalamus differs among Parkinson disease, progressive supranuclear palsy, and corticobasal syndrome. MATERIALS AND METHODS: Nine patients with Parkinson disease, 5 with progressive supranuclear palsy, and 6 with corticobasal syndrome underwent 3T MR imaging along with 12 matched, asymptomatic volunteers by using a protocol that included volumetric T1 and diffusion tensor imaging. Acquired data were automatically processed to delineate the margins of the motor and nonmotor thalamic nuclear groups, and measurements of ADC were calculated from the DTI data within these regions. Thalamic volume, shape, and ADC were compared across groups. RESULTS: Thalamic volume was smaller in the progressive supranuclear palsy and corticobasal syndrome groups compared with the Parkinson disease and control groups. Shape analysis revealed that this was mainly due to the diminished size of the lateral thalamus. Overall, ADC measurements were higher in the progressive supranuclear palsy group compared with both the Parkinson disease and control groups, and anatomic subgroup analysis demonstrated that these changes were greater within the motor regions of the thalamus in progressive supranuclear palsy and corticobasal degeneration. CONCLUSIONS: Reduced size and increased ADC disproportionately involve the lateral thalamus in progressive supranuclear palsy and corticobasal syndrome, consistent with selective neurodegeneration and atrophy in this region. Because these findings were not observed in Parkinson disease, they may be more specific markers of tau-related neurodegeneration.

An expanded role for neuroimaging in the evaluation of memory impairment.

SUMMARY: Alzheimer disease affects millions of people worldwide. The neuropathologic process underlying this disease begins years, if not decades, before the onset of memory decline. Recent advances in neuroimaging suggest that it is now possible to detect Alzheimer-associated neuropathologic changes well before dementia onset. Here, we evaluate the role of recently developed in vivo biomarkers in the clinical evaluation of Alzheimer disease. We discuss how assessment strategies might incorporate neuroimaging markers to better inform patients, families, and clinicians when memory impairment prompts a search for diagnosis and management options.

Super-resolution track density imaging of glioblastoma: histopathologic correlation.

BACKGROUND AND PURPOSE: Super-resolution track density imaging generates anatomic images with submillimeter voxel resolution by using high-angular-resolution diffusion imaging and fiber-tractography. TDI within the diseased human brain has not been previously described. The purpose of this study was to correlate TDI with histopathologic features of GBM. MATERIALS AND METHODS: A total of 43 tumor specimens (24 contrast-enhancing, 12 NE, and 7 centrally necrotic regions) were collected from 18 patients with treatment-naïve GBM by use of MR imaging-guided neurosurgical techniques. Immunohistochemical stains were used to evaluate the following histopathologic features: hypoxia, architectural disruption, microvascular hyperplasia, and cellular proliferation. We reconstructed track density maps at a 0.25-mm isotropic spatial resolution by using probabilistic streamline tractography combined with constrained spheric deconvolution (model order, 8; 0.1-mm step size; 1 million seed points). Track density values were obtained from each tissue site. A P value of .05 was considered significant and was adjusted for multiple comparisons by use of the false discovery rate method. RESULTS: Track density was not significantly different between contrast-enhancing and NE regions but was more likely to be elevated within regions demonstrating aggressive histopathologic features (P < .05). Significant correlation between relative track density and hypoxia (odds ratio, 3.52; P = .01), architectural disruption (odds ratio, 3.49; P = .03), and cellular proliferation (odds ratio, 1.70; P = .05) was observed irrespective of the presence or absence of contrast enhancement. CONCLUSIONS: Numeric values of track density correlate with GBM biologic features and may be clinically useful for identification of regions of tumor infiltration within both enhancing and NE components of GBM.

Reducing patient radiation dose during CT-guided procedures: demonstration in spinal injections for pain.

BACKGROUND AND PURPOSE: CT guidance may improve precision for diagnostic and therapeutic spinal injections, but it can increase patient radiation dose. This study examined the impact of reducing tube current on patient radiation exposure and the technical success for these procedures, by using axial acquisitions for short scan lengths and eliminating nonessential imaging. MATERIALS AND METHODS: Our institutional review board approved retrospective analysis of records from 100 consecutive outpatients undergoing spinal injections for pain before and after the CT protocol modification to reduce radiation dose. Data collected included patient age and sex, response to injection, number of sites and spinal levels treated, injection type, performing physician, CT acquisition method, number of imaging series, tube current, scan length, and DLP. RESULTS: Image contrast was reduced with the low-dose protocol, but this did not affect technical success or immediate pain relief. Mean DLP for all procedures decreased from 1458 ± 1022 to 199 ± 101 mGy · cm (P < .001). The range of radiologist-dependent DLP per procedure also was reduced significantly with the modified protocol. Selective nerve root blocks, lumbar injections, multiple injection sites, and the lack of prior imaging were each associated with a slightly higher DLP (<50 mGy · cm). CONCLUSIONS: Radiation to patients undergoing CT-guided spinal injections can be decreased significantly without affecting outcome by reducing tube current, using axial acquisitions for short scan lengths, and eliminating nonessential imaging guidance. These measures also decrease variability in radiation doses between different practitioners and should be useful for other CT-guided procedures in radiology.

Intraosseous CSF fistula in a patient with Gorham disease resulting in intracranial hypotension.

A 7-year-old girl with a history of headaches and Gorham disease was surgically treated in infancy for Chiari I malformation. Subsequent investigation revealed that her cerebellar tonsillar ectopia was due to a long-standing spinal CSF-lymphatic fistula causing intracranial hypotension. Percutaneous fistula closure was performed several times, resulting in transient symptomatic improvement.

Hippocampal CA1 apical neuropil atrophy in mild Alzheimer disease visualized with 7-T MRI.

OBJECTIVES: In Alzheimer disease (AD), mounting evidence points to a greater role for synaptic loss than neuronal loss. Supporting this notion, multiple postmortem studies have demonstrated that the hippocampal CA1 apical neuropil is one of the earliest sites of pathology, exhibiting tau aggregates and then atrophy before there is substantial loss of the CA1 pyramidal neurons themselves. In this cross-sectional study, we tested whether tissue loss in the CA1 apical neuropil layer can be observed in vivo in patients with mild AD. METHODS: We performed ultra-high-field 7-T MRI on subjects with mild AD (n = 14) and age-matched normal controls (n = 16). With a 2-dimensional T2*-weighted gradient-recalled echo sequence that was easily tolerated by subjects, we obtained cross-sectional slices of the hippocampus at an in-plane resolution of 195 µm. RESULTS: On images revealing the anatomic landmarks of hippocampal subfields and strata, we observed thinning of the CA1 apical neuropil in subjects with mild AD compared to controls. By contrast, the 2 groups exhibited no difference in the thickness of the CA1 cell body layer or of the entire CA1 subfield. Hippocampal volume, measured on a conventional T1-weighted sequence obtained at 3T, also did not differentiate these patients with mild AD from controls. CONCLUSIONS: CA1 apical neuropil atrophy is apparent in patients with mild AD. With its superior spatial resolution, 7-T MRI permits in vivo analysis of a very focal, early site of AD pathology.

Cervical and intracranial arterial anomalies in 70 patients with PHACE syndrome.

BACKGROUND AND PURPOSE: Cerebral and cervical arterial abnormalities are the most common non-cutaneous anomaly in PHACE syndrome, but the location and type of arterial lesions that occur have not been systematically assessed in a large cohort. Our aim was to characterize the phenotypic spectrum of arteriopathy, assess the frequency with which different arteries are involved, and evaluate spatial relationships between arteriopathy, brain structural lesions, and hemangiomas in PHACE syndrome. MATERIALS AND METHODS: Intracranial MRA and/or CTA images from 70 children and accompanying brain MR images in 59 patients with arteriopathy and PHACE syndrome were reviewed to identify the type and location of arterial lesions and brain abnormalities. Five categories of arteriopathy were identified and used for classification: dysgenesis, narrowing, nonvisualization, primitive embryonic carotid-vertebrobasilar connections, and anomalous arterial course or origin. Univariate logistic regression analyses were performed to test for associations between arteriopathy location, hemangiomas, and brain abnormalities. RESULTS: By study design, all patients had arterial abnormalities, and 57% had >1 form of arteriopathy. Dysgenesis was the most common abnormality (56%), followed by anomalous course and/or origin (47%), narrowing (39%), and nonvisualization (20%). Primitive embryonic carotid-vertebrobasilar connections were present in 20% of children. Hemangiomas were ipsilateral to arteriopathy in all but 1 case. The frontotemporal and/or mandibular facial segments were involved in 97% of cases, but no other specific associations between arteriopathy location and hemangioma sites were detected. All cases with posterior fossa anomalies had either ICA anomalies or persistent embryonic carotid-basilar connections. CONCLUSIONS: The arteriopathy of PHACE syndrome commonly involves the ICA and its embryonic branches, ipsilateral to the cutaneous hemangioma, with dysgenesis and abnormal arterial course the most commonly noted abnormalities. Brain abnormalities are also typically ipsilateral.