Wood, Christopher P.

Superficial siderosis refers to hemosiderin deposition along the pial surface of the brain and spinal cord. It results from chronic and repetitive low-grade bleeding into the subarachnoid space. Dural tears are a common cause of superficial siderosis. Although such tears typically occur in the spine, dural tears can also occur in the posterior fossa. In many cases, posterior fossa dural tears are iatrogenic, and patients may present with neuroimaging evidence of postoperative pseudomeningoceles. We present a case of superficial siderosis caused by a persistent posterior fossa dural leak. The patient presented with superficial siderosis 30 years after a Chiari I malformation repair. A pinhole-sized dural tear was identified preoperatively using computed tomography cisternography. The dural defect was successfully repaired. An additional small tear that was not seen on imaging was also identified at surgery and successfully repaired.

Introduction There is increased interest in the use of artificial intelligence-based (AI) software packages in the evaluation of neuroimaging studies for acute ischemic stroke. We studied whether, compared to standard image interpretation without AI, Brainomix e-ASPECTS software improved interobserver agreement and accuracy in detecting ASPECTS regions affected in anterior circulation LVO. Methods We included 60 consecutive patients with anterior circulation LVO who had TICI 3 revascularization within 60 minutes of their baseline CT. A total of 16 readers, including senior neuroradiologists, junior neuroradiologists and vascular neurologists participated. Readers interpreted CT scans on independent workstations and assessed final ASPECTS and evaluated whether each individual ASPECTS region was affected. Two months later, readers again evaluated the CT scans, but with assistance of e-ASPECTS software. We assessed interclass correlation coefficient for total ASPECTS and interobserver agreement with Fleiss’ Kappa for each ASPECTS region with and without assistance of the e-ASPECTS. We also assessed accuracy for the readers with and without e-ASPECTS assistance. In our assessment of accuracy, ground truth was the 24 hour CT in this cohort of patients who had prompt and complete revascularization. Results Interclass correlation coefficient for total ASPECTS without e-ASPECTS assistance was 0.395, indicating fair agreement compared, to 0.574 with e-ASPECTS assistance, indicating good agreement (P < 0.01). There was significant improvement in inter-rater agreement with e-ASPECTS assistance for each individual region with the exception of M6 and caudate. The e-ASPECTS software had higher accuracy than the overall cohort of readers (with and without e-ASPECTS assistance) for every region except the caudate. Conclusions Use of Brainomix e-ASPECTS software resulted in significant improvements in inter-rater agreement and accuracy of ASPECTS score evaluation in a large group of neuroradiologists and neurologists. e-ASPECTS software was more predictive of final infarct/ASPECTS than the overall group interpreting the CT scans with and without e-ASPECTS assistance.

AbstractPurposeAn increasing number of implantable or external devices can impact whether patients can receive radiological imaging examinations. This study examines and tests the Neulasta (pegfilgrastim) Onpro on‐body injector in multiple imaging environments.MethodsThe injector was analyzed for four imaging modalities with testing protocols and strategies developed for each modality. In x‐ray and computed tomography (CT), scans with much higher exposure than clinical protocols were performed with the device attached to an anthropomorphic phantom. The device was monitored until the completion of drug delivery. For magnetic resonance imaging (MRI), the device was assessed using a hand‐held magnet and underwent the magnetically induced displacement testing in a 1.5T clinical MRI scanner room. For ultrasound, magnetic field changes were measured around an ultrasound scanner system with three transducers.ResultsFor x‐ray and CT no sign of device error was identified during or after the high radiation exposure scans. Drug delivery was completed at expected timing with expected volume. For MRI the device showed significant attractive force towards the hand‐held magnet and a 50‐degree deflection angle at 50 cm from the opening of the scanner bore. No further assessment from the gradient or radiofrequency field was deemed necessary. For ultrasound the maximum magnetic field change from baseline was measured to be +11.7 μT in comparison to +74.2 μT at 4 inches from a working microwave.ConclusionsNo device performance issue was identified under the extreme test conditions in x‐ray or CT. The device was found to be MR Unsafe. Magnetic field changes around an ultrasound system met the limitation set by manufacture. Patient ultrasound scanning is considered safe as long as the transducers do not inadvertently loosen the device.

AbstractBackgroundIntellectual disability is a complex multi‐faceted condition with diverse underlying etiologies. One rare form of intellectual disability is secondary to the loss of TRAPPC9, an activator of NF‐κB and a mediator of intracellular protein processing and trafficking. TRAPPC9 deficiency has been described in 48 patients with more than 15 pathologic variants.MethodClinical evaluation, magnetic resonance imaging, and whole‐exome sequencing were used to characterize the underlying cause of absent speech, restricted/repetitive behaviors, and worsening behavioral outbursts in 27‐year‐old man from Malta.ResultsMagnetic Resonance Imaging showed morphologic abnormalities, including global cerebral and cerebellar hypoplasia. Genetic analysis through Whole Exome Sequencing identified a homozygous deletion (c.568_574del) in TRAPPC9 resulting in a frameshift, premature stop codon, and ultimately a truncated protein (p.Trp190Argfs*95). In this case, the pathogenic variant was homozygous, identified in both of the parents without known consanguinity.ConclusionGiven the phenotype and genotype consistent with a deficiency in TRAPPC9, it is likely that this patient represents a novel case of this rare genetic syndrome. Specifically, this case, in the context of 48 total reported patients, raises questions as to the geographic origin of the pathologic variant and optimal detection and therapeutic intervention for this condition.

ObjectivesBiphenotypic sinonasal sarcoma (BSS) is a new, rare tumor characterized by concomitant neural and myogenic differentiation. The aim of this study is to describe the imaging characteristics and clinical outcomes of this neoplasm.MethodsA retrospective review of BSS patients surgically treated within a tertiary academic health care system was performed. Imaging characteristics and clinical outcomes were reviewed.ResultsFive patients underwent surgical resection of BSS tumors. Negative surgical margins were achieved in four (80%) patients. There were no deaths but two (40%) patients developed local recurrences during the postoperative follow‐up period (median follow‐up 31.4 months). Review of imaging characteristics revealed a median tumor size of 3.8 cm in greatest dimension. All tumors were unilateral and centered within the nasoethmoidal region. In all cases, the tumors extended to the nasal septum, lamina papyracea, and anterior skull base with variable degrees of erosion through these structures. On CT, involved bony structures demonstrated mixed lytic and sclerotic pattern, with definitive hyperostotic bone identified in four (80%) cases. On MRI, tumors were isointense‐to‐mixed iso/hypointense on both T1‐ and T2‐weighted sequences with one tumor demonstrating mixed T2 hyperintensity. All cases demonstrated gadolinium contrast enhancement.ConclusionsBSS is a locally aggressive tumor with a low risk of regional or distant metastases but has a significant rate of recurrence even with adequate resection. Despite its rarity, BSS should be considered in the differential diagnosis when imaging demonstrates a unilateral nasoethmoidal mass that is predominantly isointense to cerebral gray matter on T2‐weighted MRI and is hyperostotic on CT.Level of Evidence4

ObjectiveThe purpose of this article was to provide a combined pathologic and radiologic review of previous pathologically diagnosed facial nerve “hemangiomas” to confirm that these lesions are most characteristic of venous malformations rather than neoplasms.Study DesignRetrospective radiologic, clinical, and histopathologic review of all patients with a previous pathologically diagnosed facial nerve hemangioma of the temporal bone who underwent computed tomography or magnetic resonance imaging (MRI) were included. A consensus radiologic review for characteristic features and pathologic analysis was performed.Materials and MethodsA panel of 4 neuroradiologists retrospectively analyzed CT and MRI exams for 11 facial nerve hemangiomas and provided a consensus agreement on the characteristic imaging features. Concurrently, two neuropathologists reevaluated archived tissue specimens from these lesions and applied additional immunohistochemical and histochemical stains including D240, CD31, smooth muscle actin (SMA), Verhoeff Van Gieson (VVG) and glucose transporter 1 (GLUT1).ResultsLesions were composed of dilated vascular spaces with a simple, CD31‐positive endothelial lining and a smooth muscle component. All lesions were negative for markers found in arterial and lymphatic malformations and infantile hemangiomas. They had characteristic radiologic features previously ascribed to facial nerve hemangiomas. Namely, these lesions are typically T1 isointense or hypointense and T2 hyperintense relative to cerebral cortex and heterogeneously enhance on MRI. Bony canal expansion and erosion, intralesional calcification, and intracranial extension are common.ConclusionsOn the basis of this radiologic and pathologic review, these lesions are best characterized as venous malformations.Level of Evidence4