Tumefactive Primary Central Nervous System Vasculitis: Imaging Findings of a Rare and Underrecognized Neuroinflammatory Disease.

Primary central nervous system vasculitis (PCNSV) is a poorly understood neuroinflammatory disease of the CNS affecting the intracranial vasculature. Although PCNSV classically manifests as a multifocal beaded narrowing of the intracranial vessels, some patients may not have angiographic abnormalities. A rare subset of patients with PCNSV present with masslike brain lesions mimicking a neoplasm. In this article, we retrospectively review 10 biopsy-confirmed cases of tumefactive PCNSV (t-PCNSV). All cases of t-PCNSV in our series that underwent CTA or MRA were found to have normal large and medium-sized vessels. T-PCNSV had a variable MR imaging appearance with most cases showing cortical/subcortical enhancing masslike lesion (70%), often with microhemorrhages (80%). Diffusion restriction was absent in all lesions. In summary, normal vascular imaging does not exclude the diagnosis of t-PCNSV. Advanced imaging techniques including MR perfusion and MR spectroscopy failed to demonstrate specific findings for t-PCNSV but assisted in excluding neoplasm in the differential diagnosis. Biopsy remains mandatory for definitive diagnosis.

The Central Vein Sign in Radiologically Isolated Syndrome.

BACKGROUND AND PURPOSE: Radiologically isolated syndrome describes asymptomatic individuals with incidental radiologic abnormalities suggestive of multiple sclerosis. Recent studies have demonstrated that >40% of white matter lesions in MS (and often substantially more) have visible central veins on MR imaging. This "central vein sign" reflects perivenous inflammatory demyelination and can assist in differentiating MS from other white matter disorders. We therefore hypothesized that >40% of white matter lesions in cases of radiologically isolated syndrome would show the central vein sign. MATERIALS AND METHODS: We recruited 20 participants diagnosed with radiologically isolated syndrome after evaluation by a neurologist. We performed 3T MR imaging of the brain and cervical spinal cord. White matter lesions were analyzed for the central vein sign. RESULTS: Of 391 total white matter lesions, 292 (75%) demonstrated the central vein sign (central vein sign+). The median proportion of central vein sign+ lesions per case was 87% (range, 29%-100%). When the "40% rule" that has been proposed to distinguish MS from other disorders was applied, of 20 participants, 18 cases of radiologically isolated syndrome (90%) had ≥40% central vein sign+ lesions (range, 55%-100%). Two participants (10%) had <40% central vein sign+ lesions (29% and 31%). When the simpler "rule of 6" was applied, 19 participants (95%) met these criteria. In multivariable models, the number of spinal cord and infratentorial lesions was associated with a higher proportion of central vein sign+ lesions (P = .002; P = .06, respectively). CONCLUSIONS: Most cases of radiologically isolated syndrome had a high proportion of central vein sign+ lesions, suggesting that lesions in these individuals reflect perivenous inflammatory demyelination. Moreover, we found correlations between the proportion of central vein sign+ lesions and spinal cord lesions, a known risk factor for radiologically isolated syndrome progressing to MS. These findings raise the possibility, testable prospectively, that the central vein sign may have prognostic value in distinguishing patients with radiologically isolated syndrome at risk of developing clinical MS from those with white matter lesions of other etiologies.

HARMless: Transient Cortical and Sulcal Hyperintensity on Gadolinium-Enhanced FLAIR after Elective Endovascular Coiling of Intracranial Aneurysms.

BACKGROUND AND PURPOSE: Cortical and sulcal hyperintensity on gadolinium-enhanced FLAIR has been increasingly recognized after iodinated contrast medium exposure during angiographic procedures. The goal of this study was to assess the relationship of cortical and sulcal hyperintensity on gadolinium-enhanced FLAIR against various variables in patients following elective endovascular treatment of intracranial aneurysms. MATERIALS AND METHODS: We performed a retrospective review of 58 patients with 62 MR imaging studies performed within 72 hours following endovascular treatment of intracranial aneurysms. Patient demographics, aneurysm location, and vascular territory distribution of cortical and sulcal hyperintensity on gadolinium-enhanced FLAIR were documented. Analysis of cortical and sulcal hyperintensity on gadolinium-enhanced FLAIR with iodinated contrast medium volume, procedural duration, number of angiographic runs, and DWI lesions was performed. RESULTS: Cortical and sulcal hyperintensity on gadolinium-enhanced FLAIR was found in 32/62 (51.61%) post-endovascular treatment MR imaging studies, with complete resolution of findings in all patients on the available follow-up studies (27/27). Angiographic iodinated contrast medium injection and arterial anatomy matched the vascular distribution of cortical and sulcal hyperintensity on gadolinium-enhanced FLAIR. No significant association was found between cortical and sulcal hyperintensity on gadolinium-enhanced FLAIR with iodinated contrast medium volume (P = .56 value) and the presence of DWI lesions (P = .68). However, a significant association was found with procedural time (P = .001) and the number of angiographic runs (P = .019). No adverse clinical outcomes were documented. CONCLUSIONS: Cortical and sulcal hyperintensity on gadolinium-enhanced FLAIR is a transient observation in the arterial territory exposed to iodinated contrast medium during endovascular treatment of intracranial aneurysms. Cortical and sulcal hyperintensity on gadolinium-enhanced FLAIR is significantly associated with procedural time, and the frequency of angiographic runs suggesting a potential technical influence on the breakdown of the BBB, but no reported adverse clinical outcome or association with both iodinated contrast medium volume and DWI lesions was found. Recognition of cortical and sulcal hyperintensity on gadolinium-enhanced FLAIR as a benign incidental finding is vital to avoid unnecessary investigation.

Prospective Multicenter Study of Changes in MTT after Aneurysmal SAH and Relationship to Delayed Cerebral Ischemia in Patients with Good- and Poor-Grade Admission Status.

BACKGROUND AND PURPOSE: Patients with aneurysmal SAH and good clinical status at admission are considered at a lower risk for delayed cerebral ischemia. Prolonged MTT may be associated with an increased risk. It is unclear whether this is dependent on clinical status. Our purpose was to determine whether increased MTT within 3 days of aneurysmal SAH compared with baseline is associated with a higher risk of delayed cerebral ischemia in patients with good (World Federation of Neurosurgical Societies I-III) versus poor (World Federation of Neurosurgical Societies IV-V) admission status. MATERIALS AND METHODS: This prolonged MTT was a multicenter, prospective cohort investigation of 87 patients with aneurysmal SAH. MTT was measured at admission before aneurysm treatment (MTT1) and following repair (MTT2) within 3 days of admission; MTTdiff was calculated as the difference between MTT2 and MTT1. Changes in MTT across time were assessed with repeated measures analyses. Risk of delayed cerebral ischemia or death was determined with multivariate logistic regression analysis. RESULTS: In patients with a good grade (n = 49), MTT was prolonged in patients who developed delayed cerebral ischemia, with MTTdiff significantly greater (0.82 ± 1.5) compared with those who did not develop delayed cerebral ischemia (-0.14 ± 0.98) (P = .03). Prolonged MTT was associated with a significantly higher risk of delayed cerebral ischemia or death (OR = 3.1; 95% CI, 1.3-7.4; P = .014) on multivariate analysis. In patients with poor grades (n = 38), MTTdiff was not greater in patients who developed delayed cerebral ischemia; MTT1 was significantly prolonged compared with patients with a good grade. CONCLUSIONS: Patients in good clinical condition following aneurysmal SAH but with increasing MTT in the first few days after aneurysmal SAH are at high risk of delayed cerebral ischemia and warrant close clinical monitoring.

Differentiation of Enhancing Glioma and Primary Central Nervous System Lymphoma by Texture-Based Machine Learning.

BACKGROUND AND PURPOSE: Accurate preoperative differentiation of primary central nervous system lymphoma and enhancing glioma is essential to avoid unnecessary neurosurgical resection in patients with primary central nervous system lymphoma. The purpose of the study was to evaluate the diagnostic performance of a machine-learning algorithm by using texture analysis of contrast-enhanced T1-weighted images for differentiation of primary central nervous system lymphoma and enhancing glioma. MATERIALS AND METHODS: Seventy-one adult patients with enhancing gliomas and 35 adult patients with primary central nervous system lymphomas were included. The tumors were manually contoured on contrast-enhanced T1WI, and the resulting volumes of interest were mined for textural features and subjected to a support vector machine-based machine-learning protocol. Three readers classified the tumors independently on contrast-enhanced T1WI. Areas under the receiver operating characteristic curves were estimated for each reader and for the support vector machine classifier. A noninferiority test for diagnostic accuracy based on paired areas under the receiver operating characteristic curve was performed with a noninferiority margin of 0.15. RESULTS: The mean areas under the receiver operating characteristic curve were 0.877 (95% CI, 0.798-0.955) for the support vector machine classifier; 0.878 (95% CI, 0.807-0.949) for reader 1; 0.899 (95% CI, 0.833-0.966) for reader 2; and 0.845 (95% CI, 0.757-0.933) for reader 3. The mean area under the receiver operating characteristic curve of the support vector machine classifier was significantly noninferior to the mean area under the curve of reader 1 (P = .021), reader 2 (P = .035), and reader 3 (P = .007). CONCLUSIONS: Support vector machine classification based on textural features of contrast-enhanced T1WI is noninferior to expert human evaluation in the differentiation of primary central nervous system lymphoma and enhancing glioma.

First-Pass Contrast-Enhanced MRA for Pretherapeutic Diagnosis of Spinal Epidural Arteriovenous Fistulas with Intradural Venous Reflux.

BACKGROUND AND PURPOSE: Spinal epidural AVFs are rare spinal vascular malformations. When there is associated intradural venous reflux, they may mimic the more common spinal dural AVFs. Correct diagnosis and localization before conventional angiography is beneficial to facilitate treatment. We hypothesize that first-pass contrast-enhanced MRA can diagnose and localize spinal epidural AVFs with intradural venous reflux and distinguish them from other spinal AVFs. MATERIALS AND METHODS: Forty-two consecutive patients with a clinical and/or radiologic suspicion of spinal AVF underwent MR imaging, first-pass contrast-enhanced MRA, and DSA at a single institute (2000-2015). MR imaging/MRA and DSA studies were reviewed by 2 independent blinded observers. DSA was used as the reference standard. RESULTS: On MRA, all 7 spinal epidural AVFs with intradural venous reflux were correctly diagnosed and localized with no interobserver disagreement. The key diagnostic feature was arterialized filling of an epidural venous pouch with a refluxing radicular vein arising from the arterialized epidural venous system. CONCLUSIONS: First-pass contrast-enhanced MRA is a reliable and useful technique for the initial diagnosis and localization of spinal epidural AVFs with intradural venous reflux and can distinguish these lesions from other spinal AVFs.

First-Pass Contrast-Enhanced MR Angiography in Evaluation of Treated Spinal Arteriovenous Fistulas: Is Catheter Angiography Necessary?

BACKGROUND AND PURPOSE: Catheter angiography is typically used for follow-up of treated spinal AVFs. The purpose of this study was to determine the diagnostic performance and utility of first-pass contrast-enhanced MRA in the posttreatment evaluation of spinal AVFs compared with DSA. MATERIALS AND METHODS: A retrospective review was performed of all patients at our tertiary referral hospital (from January 2000 to April 2015) who underwent spine MR imaging, first-pass contrast-enhanced MRA, and DSA after surgical and/or endovascular treatment of a spinal AVF. Presence of recurrent or residual fistula on MRA, including vertebral level of the recurrent/residual fistula, was evaluated by 2 experienced neuroradiologists blinded to DSA findings. Posttreatment conventional MR imaging findings were also evaluated, including presence of intramedullary T2 hyperintensity, perimedullary serpentine flow voids, and cord enhancement. The performance of MRA and MR imaging findings for diagnosis of recurrent/residual fistula was determined by using DSA as the criterion standard. RESULTS: In total, 28 posttreatment paired MR imaging/MRA and DSA studies were evaluated in 22 patients with prior spinal AVF and 1 patient with intracranial AVF with prior cervical perimedullary venous drainage. Six image sets of 5 patients demonstrated recurrent/residual disease at DSA. MRA correctly identified all cases with recurrent/residual disease with 1 false-positive (sensitivity, 100%; specificity 95%; P < .001), with correct localization in all cases without interobserver disagreement. Conventional MR imaging parameters were not significantly associated with recurrent/residual spinal AVF. CONCLUSIONS: First-pass MRA demonstrates high sensitivity and specificity for identifying recurrent/residual spinal AVFs and may potentially substitute for DSA in the posttreatment follow-up of patients with spinal AVFs.

Comparison of Time-Resolved and First-Pass Contrast-Enhanced MR Angiography in Pretherapeutic Evaluation of Spinal Dural Arteriovenous Fistulas.

BACKGROUND AND PURPOSE: Different MRA techniques used to evaluate spinal dural arteriovenous fistulas offer unique advantages and limitations with regards to temporal and spatial resolution. The purpose of this study was to compare the efficacy and interobserver agreement of 2 commonly used contrast-enhanced spinal MRA techniques, multiphase time-resolved MRA and single-phase first-pass MRA, in assessment of spinal dural arteriovenous fistulas. MATERIALS AND METHODS: Retrospective review of 15 time-resolved and 31 first-pass MRA studies in patients with clinical suspicion of spinal dural arteriovenous fistula was performed by 2 independent, blinded observers. DSA was used as the reference standard to compare the diagnostic performance of the 2 techniques. RESULTS: There were 10 cases of spinal dural arteriovenous fistula in the time-resolved MRA group and 20 in the first-pass MRA group. Time-resolved MRA detected spinal dural arteriovenous fistulas with sensitivity and specificity of 100% and 80%, respectively, with 100% correct-level localization rate. First-pass MRA detected spinal dural arteriovenous fistulas with sensitivity and specificity of 100% and 82%, respectively, with 87% correct-level localization rate. Interobserver agreement for localization was excellent for both techniques; however, it was higher for time-resolved MRA. In 5 cases, the site of fistula was not included in the FOV, but a prominent intradural radicular vein was observed at the edge of the FOV. CONCLUSIONS: Multiphase time-resolved MRA and single-phase first-pass MRA were comparable in diagnosis and localization of spinal dural arteriovenous fistulas and demonstrated excellent interobserver agreement, though there were more instances of ambiguity in fistula localization on first-pass MRA.

Use of a flow-diverting stent for ruptured dissecting aneurysm treatment in a patient with sickle cell disease.

INTRODUCTION: Sickle cell disease (SCD) is a clinical phenotype that presents a unique challenge to the interventionalist, particularly when stent therapy is contemplated. Homozygous individuals are likely at increased risk for thromboembolic complications. There are no formal guidelines regarding antiplatelet therapy in the short or long term for intracranial stent use in SCD. The authors describe the novel use of a pipeline embolization device (PED) to treat a ruptured dissecting bilobed/fusiform vertebral artery V4 aneurysm in an SCD patient complicated by tortuous proximal anatomy and the anterior spinal artery arising from the diseased segment. Considerations regarding antiplatelet therapy in this scenario are discussed. CASE REPORT: A 50-year-old woman with homozygous recessive SCD was transported to the emergency department and presented with diffuse subarachnoid hemorrhage. CT angiography demonstrated a left-sided 3 × 5 mm fusiform bi-lobulated presumed dissecting vertebral artery aneurysm, immediately distal to the origin of the posterior inferior cerebellar artery (PICA). A PED was deployed within the V4 segment across the aneurysm. Post-treatment angiography showed patency of the parent artery, and patency of the "jailed" anterior spinal artery and of the PICA. DISCUSSION: Selecting a treatment method in SCD patients with a ruptured intracranial aneurysm is challenging and there are no clinical trials comparing treatment methods in this population. The authors demonstrate that flow diversion is feasible in SCD, which has not been described in the literature. Additionally, the case stresses the peri- and post-procedural management of SCD, as well as long-term considerations with a flow-diverting stent in place.

Comparison of Sagittal FSE T2, STIR, and T1-Weighted Phase-Sensitive Inversion Recovery in the Detection of Spinal Cord Lesions in MS at 3T.

BACKGROUND AND PURPOSE: Determining the diagnostic accuracy of different MR sequences is essential to design MR imaging protocols. The purpose of the study was to compare 3T sagittal FSE T2, STIR, and T1-weighted phase-sensitive inversion recovery in the detection of spinal cord lesions in patients with suspected or definite MS. MATERIALS AND METHODS: We performed a retrospective analysis of 38 patients with suspected or definite MS. Involvement of the cervical and thoracic cord segments was recorded on sagittal FSE T2, STIR, and T1-weighted phase-sensitive inversion recovery sequences independently by 2 readers. A consensus criterion standard read was performed with all sequences available. Sensitivity, specificity, and interobserver agreement were calculated for each sequence. RESULTS: In the cervical cord, the sensitivity of T1-weighted phase-sensitive inversion recovery (96.2%) and STIR (89.6%) was significantly higher (P < .05) than that of FSE T2 (50.9%), but no significant difference was found between T1-weighted phase-sensitive inversion recovery and STIR. In the thoracic cord, sensitivity values were 93.8% for STIR, 71.9% for FSE T2, and 50.8% for T1-weighted phase-sensitive inversion recovery. Significant differences were found for all comparisons (P < .05). No differences were detected in specificity. Poor image quality and lower sensitivity of thoracic T1-weighted phase-sensitive inversion recovery compared with the other 2 sequences were associated with a thicker back fat pad. CONCLUSIONS: The use of an additional sagittal sequence other than FSE T2 significantly increases the detection of cervical and thoracic spinal cord lesions in patients with MS at 3T. In the cervical segment, both STIR and T1-weighted phase-sensitive inversion recovery offer high sensitivity and specificity, whereas in the thoracic spine, STIR performs better than T1-weighted phase-sensitive inversion recovery, particularly in patients with a thick dorsal fat pad.

Pixel-by-Pixel Comparison of Volume Transfer Constant and Estimates of Cerebral Blood Volume from Dynamic Contrast-Enhanced and Dynamic Susceptibility Contrast-Enhanced MR Imaging in High-Grade Gliomas.

BACKGROUND AND PURPOSE: Estimates of blood volume and volume transfer constant are parameters commonly used to characterize hemodynamic properties of brain lesions. The purposes of this study were to compare values of volume transfer constant and estimates of blood volume in high-grade gliomas on a pixel-by-pixel basis to comprehend whether they provide different information and to compare estimates of blood volume obtained by dynamic contrast-enhanced MR imaging and dynamic susceptibility contrast-enhanced MR imaging. MATERIALS AND METHODS: Thirty-two patients with biopsy-proved grade IV gliomas underwent dynamic contrast-enhanced MR imaging and dynamic susceptibility contrast-enhanced MR imaging, and parametric maps of volume transfer constant, plasma volume, and CBV maps were calculated. The Spearman rank correlation coefficients among matching values of CBV, volume transfer constant, and plasma volume were calculated on a pixel-by-pixel basis. Comparison of median values of normalized CBV and plasma volume was performed. RESULTS: Weak-but-significant correlation (P < .001) was noted for all comparisons. Spearman rank correlation coefficients were as follows: volume transfer constant versus CBV, ρ = 0.113; volume transfer constant versus plasma volume, ρ = 0.256; CBV versus plasma volume, ρ = 0.382. We found a statistically significant difference (P < .001) for the estimates of blood volume obtained by using dynamic contrast-enhanced MR imaging (mean normalized plasma volume, 13.89 ± 11.25) and dynamic susceptibility contrast-enhanced MR imaging (mean normalized CBV, 4.37 ± 4.04). CONCLUSIONS: The finding of a very weak correlation between estimates of microvascular density and volume transfer constant suggests that they provide different information. Estimates of blood volume obtained by using dynamic contrast-enhanced MR imaging are significantly higher than those obtained by dynamic susceptibility contrast-enhanced MR imaging in human gliomas, most likely due to the effect of contrast leakage.

Evaluation of three-dimensional finite element-based deformable registration of pre- and intraoperative prostate imaging.

In this report we evaluate an image registration technique that can improve the information content of intraoperative image data by deformable matching of preoperative images. In this study, pretreatment 1.5 tesla (T) magnetic resonance (MR) images of the prostate are registered with 0.5 T intraoperative images. The method involves rigid and nonrigid registration using biomechanical finite element modeling. Preoperative 1.5 T MR imaging is conducted with the patient supine, using an endorectal coil, while intraoperatively, the patient is in the lithotomy position with a rectal obturator in place. We have previously observed that these changes in patient position and rectal filling produce a shape change in the prostate. The registration of 1.5 T preoperative images depicting the prostate substructure [namely central gland (CG) and peripheral zone (PZ)] to 0.5 T intraoperative MR images using this method can facilitate the segmentation of the substructure of the gland for radiation treatment planning. After creating and validating a dataset of manually segmented glands from images obtained in ten sequential MR-guided brachytherapy cases, we conducted a set of experiments to assess our hypothesis that the proposed registration system can significantly improve the quality of matching of the total gland (TG), CG, and PZ. The results showed that the method statistically-significantly improves the quality of match (compared to rigid registration), raising the Dice similarity coefficient (DSC) from prematched coefficients of 0.81, 0.78, and 0.59 for TG, CG, and PZ, respectively, to 0.94, 0.86, and 0.76. A point-based measure of registration agreement was also improved by the deformable registration. CG and PZ volumes are not changed by the registration, indicating that the method maintains the biomechanical topology of the prostate. Although this strategy was tested for MRI-guided brachytherapy, the preliminary results from these experiments suggest that it may be applied to other settings such as transrectal ultrasound-guided therapy, where the integration of preoperative MRI may have a significant impact upon treatment planning and guidance.

Interpretation errors in CT angiography of the head and neck and the benefit of double reading.

BACKGROUND AND PURPOSE: CTA provides high-resolution imaging of the head and neck vasculature but also of the soft tissues and bones. This results in a large volume of information to be interpreted. This study examines interpretation errors with head and neck CTAs and assesses whether double reading reduces miss rates. MATERIALS AND METHODS: Consecutive CTAs of the neck and intracranial circulation were retrospectively identified and reviewed for vascular and nonvascular findings by a consensus of 2 neuroradiologists. The results were compared with the official report. Significant discrepancies were considered those that would have influenced follow-up or management. RESULTS: We reviewed 503 studies; 144 were originally reported by a staff neuroradiologist alone, 209 by staff and diagnostic radiology resident, and 150 by staff and neuroradiology fellow. Twenty-six significant discrepancies were discovered in 20 studies, corresponding to 4.0% of studies with at least 1 miss, and an overall miss rate per study of 5.2%. There was at least 1 miss in 6.3% of studies interpreted by a staff neuroradiologist alone, 3.3% by staff and resident, and 2.7% by staff and fellow. The miss rate differences were not statistically significant. The most common misses were small aneurysms (50% of misses). CONCLUSIONS: CTA neck and head datasets are now large, and there is a potential for missed findings. Significant discrepancies can occur with a low but not insignificant rate. Arterial pathology accounted for most discrepancies. This study emphasizes the need for careful systematic scrutiny for both vascular and nonvascular pathology regardless of indication. Double reading reduces error rates.