DeLone, David R.

ObjectiveAlthough granulomatosis with polyangiitis (GPA; Wegener’s granulomatosis) is classically characterized by systemic disease involving the kidneys and airway, approximately 10% of patients who have it present with isolated central nervous system disease. When involving the skull base, GPA frequently mimics more common pathology, resulting in diagnostic challenges and delay. The primary objective of this study is to characterize the cranial base manifestations of GPA, highlighting aspects most relevant to the skull base surgeon.Study DesignRetrospective review.SettingTertiary academic referral center.Subjects and MethodsRetrospective analysis of all patients with skull base GPA treated at a tertiary referral center from January 1, 1996, to May 1, 2018.ResultsTwenty‐nine patients met inclusion criteria. Twenty‐one (72%) initially presented with skull base symptomatology as their cardinal manifestation of GPA. Twenty‐four (82%) presented with cranial neuropathy at some point in their disease course. The trigeminal nerve was most commonly involved (12 of 24, 50%), followed by the facial (11 of 24, 46%) and optic (8 of 24, 33%) nerves. Eighteen patients reported hearing loss attributed to the GPA disease process, presenting as conductive, sensorineural, or mixed. The most common locations for GPA‐derived inflammatory skull base disease on imaging included the cavernous sinus (12 of 29, 41%) and the orbit (7 of 29, 24%).ConclusionEstablishing the diagnosis of skull base GPA remains challenging. Cranial neuropathy is diverse in presentation and often mimics more common conditions. Imaging findings are also unpredictable and frequently nonspecific. Careful review of patient history, clinical presentation, serology and biopsy results, and imaging can reveal important clues toward the diagnosis.

Recognition of key concepts of structural and functional anatomy of the cerebellum can facilitate image interpretation and clinical correlation. Recently, the human brain mapping literature has increased our understanding of cerebellar anatomy, function, connectivity with the cerebrum, and significance of lesions involving specific areas. Both the common names and numerically based Schmahmann classifications of cerebellar lobules are illustrated. Anatomic patterns, or signs, of key fissures and white matter branching are introduced to facilitate easy recognition of the major anatomic features. Color-coded overlays of cross-sectional imaging are provided for reference of more complex detail. Examples of exquisite detail of structural and functional cerebellar anatomy at 7 T MRI are also depicted. The functions of the cerebellum are manifold with the majority of areas involved with non-motor association function. Key concepts of lesion–symptom mapping which correlates lesion location to clinical manifestation are introduced, emphasizing that lesions in most areas of the cerebellum are associated with predominantly non-motor deficits. Clinical correlation is reinforced with examples of intrinsic pathologic derangement of cerebellar anatomy and altered functional connectivity due to pathology of the cerebral hemisphere. The purpose of this pictorial review is to illustrate basic concepts of these topics in a cross-sectional imaging-based format that can be easily understood and applied by radiologists.

Objective The aim of this study was to quantitatively demonstrate radiation dose reduction for sinus and temporal bone examinations using high-resolution photon-counting detector (PCD) computed tomography (CT) with an additional tin (Sn) filter. Materials and Methods A multienergy CT phantom, an anthropomorphic head phantom, and a cadaver head were scanned on a research PCD-CT scanner using ultra-high-resolution mode at 100-kV tube potential with an additional tin filter (Sn-100 kV) and volume CT dose index of 10 mGy. They were also scanned on a commercial CT scanner with an energy-integrating detector (EID) following standard clinical protocols. Thirty patients referred to clinically indicated sinus examinations, and two patients referred to temporal bone examinations were scanned on the PCD-CT system after their clinical scans on an EID-CT. For the sinus cohort, PCD-CT scans were performed using Sn-100 kV at 4 dose levels at 10 mGy (n = 9), 8 mGy (n = 7), 7 mGy (n = 7), and 6 mGy (n = 7), and the clinical EID-CT was performed at 120 kV and 13.7 mGy (mean CT volume dose index). For the temporal bone scans, PCD-CT was performed using Sn-100 kV (10.1 mGy), and EID-CT was performed at 120 kV and routine clinical dose (52.6 and 66 mGy). For both PCD-CT and EID-CT, sinus images were reconstructed using H70 kernel at 0.75-mm slice thickness, and temporal bone images were reconstructed using a U70 kernel at 0.6-mm slice thickness. In addition, iterative reconstruction with a dedicated sharp kernel (V80) was used to obtain high-resolution PCD-CT images from a sinus patient scan to demonstrate improved anatomic delineation. Improvements in spatial resolution from the dedicated sharp kernel was quantified using modulation transfer function measured with a wire phantom. A neuroradiologist assessed the H70 sinus images for visualization of critical anatomical structures in low-dose PCD-CT images and routine-dose EID-CT images using a 5-point Likert scale (structural detection obscured and poor diagnostic confidence, score = 1; improved anatomic delineation and diagnostic confidence, score = 5). Image contrast and noise were measured in representative regions of interest and compared between PCD-CT and EID-CT, and the noise difference between the 2 acquisitions was used to estimate the dose reduction in the sinus and temporal bone patient cohorts. Results The multienergy phantom experiment showed a noise reduction of 26% in the Sn-100 kV PCD-CT image, corresponding to a total dose reduction of 56% compared with EID-CT (clinical dose) without compromising image contrast. The PCD-CT images from the head phantom and the cadaver scans demonstrated a dose reduction of 67% and 83%, for sinus and temporal bone examinations, respectively, compared with EID-CT. In the sinus cohort, PCD-CT demonstrated a mean dose reduction of 67%. The 10- and 8-mGy sinus patient images from PCD-CT were significantly superior to clinical EID-CT for visualization of critical sinus structures (median score = 5 ± 0.82 and P = 0.01 for lesser palatine foramina, median score = 4 ± 0.68 and P = 0.039 for nasomaxillary sutures, and median score = 4 ± 0.96 and P = 0.01 for anterior ethmoid artery canal). The 6- and 7-mGy sinus patient images did not show any significant difference between PCD-CT and EID-CT. In addition, V80 (sharp kernel, 10% modulation transfer function = 18.6 cm −1 ) PCD-CT images from a sinus patient scan increased the conspicuity of nasomaxillary sutures compared with the clinical EID-CT images. The temporal bone patient images demonstrated a dose reduction of up to 85% compared with clinical EID-CT images, whereas visualization of inner ear structures such as the incudomalleolar joint were similar between EID-CT and PCD-CT. Conclusions Phantom and cadaver studies demonstrated dose reduction using Sn-100 kV PCD-CT compared with current clinical EID-CT while maintaining the desired image contrast. Dose reduction was further validated in sinus and temporal bone patient studies. The ultra-high resolution capability from PCD-CT allowed improved anatomical delineation for sinus imaging compared with current clinical standard.

Background Carotid-cavernous fistulas (CCFs) are commonly misdiagnosed on computed tomography angiography (CTA). Purpose This study sought to identify the most sensitive and specific imaging features of CCFs on CTA. Material and Methods A retrospective review identified 18 consecutive patients suspected of having a CCF on CTA and subsequently underwent digital subtraction angiography (DSA). Two blinded reviewers assessed multiple findings on CTA: cavernous sinus asymmetry/enlargement; arterial-phase contrast in the cavernous sinus; proptosis; pre- or post-septal orbital edema; and dilated regional vasculature. Each was graded as positive, possible, and negative; “possible” was counted as positive. A third blinded reviewer served as a tiebreaker. Results Of 18 patients, nine were true-positive and nine were false-positive. Superior ophthalmic vein early enhancement and dilatation had 100.0% sensitivity (95% confidence interval [CI] 40.0–100.0) and 77.8% specificity (95% CI 44.4–100.0); arterial-phase contrast in the cavernous sinus had 88.9% sensitivity (95% CI 44.4–100.0) and 66.7% specificity (95% CI 18.5–90.1); peri-orbital edema had 88.9% sensitivity (95% CI 35.5–100.0) and 77.8% specificity (95% CI 22.2–100.0). The most specific markers of CCF were superior petrosal sinus and inferior ophthalmic vein dilatation/enhancement (100.0%, 95% CI 88.8–100.0 and 88.9%, 95% CI 44.4–100.0, respectively); the specificity of asymmetric cavernous enlargement was 44.4% (95% CI 11.1–77.7). Conclusions Among patients in whom a CCF is suspected on CTA, superior ophthalmic vein dilatation/enhancement and arterial-phase contrast within the cavernous sinus are the most sensitive findings. Asymmetric cavernous sinus enlargement has poor specificity and may result in false-positive diagnoses of CCFs. False positive cases were less likely to have an optimally timed contrast bolus.

AbstractObjectivesBoth projection and dual‐energy (DE)‐based methods have been used for metal artifact reduction (MAR) in CT. The two methods can also be combined. The purpose of this work was to evaluate these three MAR methods using phantom experiments for five types of metal implants.Materials and MethodsFive phantoms representing spine, dental, hip, shoulder, and knee were constructed with metal implants. These phantoms were scanned using both single‐energy (SE) and DE protocols with matched radiation output. The SE data were processed using a projection‐based MAR (iMAR, Siemens) algorithm, while the DE data were processed to generate virtual monochromatic images at high keV (Mono+, Siemens). In addition, the DE images after iMAR were used to generate Mono+ images (DE iMAR Mono+). Artifacts were quantitatively evaluated using CT numbers at different regions of interest. Iodine contrast‐to‐noise ratio (CNR) was evaluated in the spine phantom. Three musculoskeletal radiologists and two neuro‐radiologists independently ranked the artifact reduction.ResultsThe DE Mono+ at high keV resulted in reduced artifacts but also lower iodine CNR. The iMAR method alone caused missing tissue artifacts in dental phantom. DE iMAR Mono+ caused wrong CT numbers in close proximity to the metal prostheses in knee and hip phantoms. All musculoskeletal radiologists ranked SE iMAR > DE iMAR Mono+ > DE Mono+ for knee and hip, while DE iMAR Mono+ > SE iMAR > DE Mono+ for shoulder. Both neuro‐radiologists ranked DE iMAR Mono+ > DE Mono+ > SE iMAR for spine and DE Mono+ > DE iMAR Mono+ > SE iMAR for dental.ConclusionsThe SE iMAR was the best choice for the hip and knee prostheses, while DE Mono+ at high keV was best for dental implants and DE iMAR Mono+ was best for spine and shoulder prostheses. Artifacts were also introduced by MAR algorithms.

Background Dental hardware produces streak artifacts on computed tomography (CT) images reconstructed with the standard weighted filtered back projection (wFBP) method. Purpose To perform a preliminary evaluation of an iterative metal artifact reduction (IMAR) technique to assess its ability to improve anatomic visualization over wFBP in patients with dental amalgam or other hardware. Material and Methods CT images from patients with dental hardware were reconstructed using wFBP and IMAR software and soft-tissue or bone window/level settings. The anatomy most affected by metal artifacts was identified. Two neuroradiologists determined subjective and objective imaging features, including overall metal artifact score (1 = severe artifacts, 5 = no artifacts), soft-tissue visualization score of the most-compromised structure, and artifact length along the skin surface. CT numbers were used to quantify artifact severity. Results Twenty-four patients were included. IMAR improved overall metal artifact score in 18/24 cases (median =2 ± 0.9 vs. 1 ± 0.6, P < 0.001). Mean CT number in the most-affected anatomical structure significantly improved with IMAR (94.6 vs. 219 HU, P = 0.002) and length of affected skin surface decreased (40.4 mm vs. 118.7 mm, P < 0.001). However, osseous/dental artifactual defects were found in 22/24 cases with IMAR vs. 11/24 with wFBP. Conclusion IMAR software reduced metal artifact both subjectively and objectively and improved visualization of adjacent soft tissues. However, it produced a higher rate of artifactual defects in the teeth and bones than wFBP. Our findings support the use of IMAR as a valuable complement to, but not a replacement for, standard wFBP image reconstruction.

ABSTRACTPURPOSETo determine interobserver spatial variability in language area localization using three commonly employed language tasks.MATERIALS AND METHODSWith institutional review board approval, 125 fMRI time series from 50 different clinical language cases were retrospectively reviewed by three blinded readers who selected 3‐dimensional points representing the perceived center of Wernicke's and Broca's areas using three language tasks (semantic decision, SD; sentence comprehension, SC; and silent word generation, WG). Point dispersion values were then calculated using the perimeter of the 3‐dimensional triangle defined by the three readers’ selections.RESULTSAfter resolving interobserver laterality disagreements, there was no difference in spatial variability between the three tasks (P = .069). The SD task had the fewest interobserver laterality disagreements (P = .028) and the SC task had fewer failed localizations for Broca's area (P = .050) and Wernicke's area (P = .013).CONCLUSIONWhile there were no differences between interobserver spatial variability in language area localization between the three tasks, language task choice impacts the accuracy of fMRI language area identification because tasks vary in their rates of interobserver laterality disagreements and failed localizations. A combination of tasks including one with low laterality disagreements (eg, SD) and one with few failed localizations (eg, SC) may offer the best combination.

AbstractProgressive multifocal leukoencephalopathy (PML), a demyelinating disorder caused by brain infection with JC virus, is a neurological complication of immunocompromised states and immunosuppressive therapies. While most commonly seen in the HIV/AIDS population, patients with hematologic malignancies are also at risk following treatment protocols including monoclonal antibodies such as rituximab and after hematopoietic stem cell transplantation. Here, we present the case of PML following allogeneic HCT that highlights potential diagnostic difficulties. We also review the literature regarding PML following HCT and described therapies employed to attempt to treat this disorder.