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Quantitative magnetic resonance evaluation of the trigeminal nerve in familial dysautonomia

Won, Eugene; Palma, Jose-Alberto; Kaufmann, Horacio; Milla, Sarah S; Cohen, Benjamin; Norcliffe-Kaufmann, Lucy; Babb, James S; Lui, Yvonne W
PURPOSE/OBJECTIVE:Familial dysautonomia (FD) is a rare autosomal recessive disease that affects the development of sensory and autonomic neurons, including those in the cranial nerves. We aimed to determine whether conventional brain magnetic resonance imaging (MRI) could detect morphologic changes in the trigeminal nerves of these patients. METHODS:Cross-sectional analysis of brain MRI of patients with genetically confirmed FD and age- and sex-matched controls. High-resolution 3D gradient-echo T1-weighted sequences were used to obtain measurements of the cisternal segment of the trigeminal nerves. Measurements were obtained using a two-reader consensus. RESULTS:in controls (P < 0.001). No association between trigeminal nerve area and age was found in patients or controls. CONCLUSIONS:Using conventional MRI, the caliber of the trigeminal nerves was significantly reduced bilaterally in patients with FD compared to controls, a finding that appears to be highly characteristic of this disorder. The lack of correlation between age and trigeminal nerve size supports arrested neuronal development rather than progressive atrophy.
PMID: 30783821
ISSN: 1619-1560
CID: 3686212

MRI, arthroscopic and histopathologic cross correlation in biceps tenodesis specimens with emphasis on the normal appearing proximal tendon

Burke, Christopher J; Mahanty, Scott R; Pham, Hien; Hoda, Syed; Babb, James S; Gyftopoulos, Soterios; Jazrawi, Laith; Beltran, Luis
PURPOSE/OBJECTIVE:To correlate the histopathologic appearances of resected long head of the biceps tendon (LHBT) specimens following biceps tenodesis, with pre-operative MRI and arthroscopic findings, with attention to the radiologically normal biceps. MATERIAL AND METHODS/METHODS:Retrospective analysis of patients who had undergone preoperative MRI, subsequent arthroscopic subpectoral tenodesis for SLAP tears and histopathologic inspection of the excised sample between 2013 and 16. Those with a normal MRI appearance or mildly increased intrasubstance signal were independently analyzed by 2 blinded radiologists. A blinded orthopedic surgeon and pathologist reviewed all operative imaging and pathologic slides, respectively. RESULTS:Twenty-three LHBT resected samples were identified on MRI as either normal (Reader 1 n = 15; Reader 2 n = 14) or demonstrating low-grade increased signal (Reader 1 n = 8; Reader 2 n = 9). Of these, 86.9% demonstrated a histopathological abnormality. 50% of samples with histopathological abnormality demonstrated normal appearance on MRI. The most common reported histopathology finding was myxoid degeneration (73.9%) and fibrosis (52.2%). The most common arthroscopic abnormality was fraying (18.2%) and erythema (13.6%). Utilizing histopathology as the gold standard, the two radiologists demonstrated a sensitivity of 35.0% v 42.9%, specificity of 66.7% v 100%, PPV of 87.5% v 100%, and NPV of 13.3% v 14.3%. Corresponding arthroscopic inspection demonstrated a sensitivity of 31.6%, specificity of 66.6%, PPV 85.7% and NPV of 13.3%. There was moderate agreement between the two radiologists, κ = 0.534 (95% CI, 0.177 to 0.891), p = 0.01. CONCLUSION/CONCLUSIONS:Histopathological features of low grade tendinosis including mainly myxoid degeneration and fibrosis are frequently occult on MR imaging.
PMID: 30639523
ISSN: 1873-4499
CID: 3595162

Preliminary analysis: Background parenchymal 18F-FDG uptake in breast cancer patients appears to correlate with background parenchymal enhancement and to vary by distance from the index cancer

Kim, Eric; Mema, Eralda; Axelrod, Deborah; Sigmund, Eric; Kim, Sungheon Gene; Babb, James; Melsaether, Amy N
PURPOSE/OBJECTIVE:To investigate how breast parenchymal uptake (BPU) of 18F-FDG on positron emission tomography/ magnetic resonance imaging (PET/MRI) in patients with breast cancer is related to background parenchymal enhancement (BPE), amount of fibroglandular tissue (FGT), and age, as well as whether BPU varies as a function of distance from the primary breast cancer. MATERIALS AND METHODS/METHODS:volume of interest 1) in the same quadrant of the ipsilateral breast, 5 mm from the index lesion; 2) in the opposite quadrant of the ipsilateral breast; and 3) in contralateral breast, quadrant matched to the opposite quadrant of the ipsilateral breast. The maximum standardized uptake value (SUVmax) of the index cancer was measured using a VOI that included the entire volume of the index lesion. Bleed from the primary tumor was corrected for (PET edge, MIM). FGT and BPE was assessed by 2 readers on a 4-point scale in accordance with BI-RADS lexicon. The Wilcoxon signed rank test and the Spearman rank correlation test were performed. RESULTS:BPU was significantly greater in the same quadrant as the breast cancer as compared with the opposite quadrant of the same breast (p < 0.001 for both readers) and was significantly greater in the opposite quadrant of the same breast compared to the matched quadrant of the contralateral breast (p = 0.002 for reader 1 and <0.001 for reader 2). While the FGT SUVmax in the same quadrant as the cancer correlated significantly with SUVmax of the index lesion, the FGT SUVmax in the opposite quadrant of the same breast and in the matched quadrant of the contralateral breast did not. The FGT SUVmax in the contralateral breast positively correlated with the degree of BPE and negatively correlated with age, but did not show a significant correlation with the amount of FGT for either reader. CONCLUSION/CONCLUSIONS:There appears to be an inverse correlation between metabolic activity of normal breast parenchyma and distance from the index cancer. BPU significantly correlates with BPE.
PMID: 30599855
ISSN: 1872-7727
CID: 3562812

Rapid Radial T1 and T2 Mapping of the Hip Articular Cartilage With Magnetic Resonance Fingerprinting

Cloos, Martijn A; Assländer, Jakob; Abbas, Batool; Fishbaugh, James; Babb, James S; Gerig, Guido; Lattanzi, Riccardo
BACKGROUND:Quantitative MRI can detect early changes in cartilage biochemical components, but its routine clinical implementation is challenging. PURPOSE/OBJECTIVE:along radial sections of the hip for accurate and reproducible multiparametric quantitative cartilage assessment in a clinically feasible scan time. STUDY TYPE/METHODS:Reproducibility, technical validation. SUBJECTS/PHANTOM/UNASSIGNED:A seven-compartment phantom and three healthy volunteers. FIELD STRENGTH/SEQUENCE/UNASSIGNED:at 3 T was developed. Automatic positioning and semiautomatic cartilage segmentation were implemented to improve consistency and simplify workflow. ASSESSMENT/RESULTS:Intra- and interscanner variability of our technique was assessed over multiple scans on three different MR scanners. STATISTICAL TESTS/UNASSIGNED:over six radial slices was calculated. Restricted maximum likelihood estimation of variance components was used to estimate intrasubject variances reflecting variation between results from the two scans using the same scanner (intrascanner variance) and variation among results from the three scanners (interscanner variance). RESULTS:. DATA CONCLUSION/UNASSIGNED:Our method, which includes slice positioning, model-based parameter estimation, and cartilage segmentation, is highly reproducible. It could enable employing quantitative hip cartilage evaluation for longitudinal and multicenter studies. LEVEL OF EVIDENCE/METHODS:1 Technical Efficacy: Stage 1 J. Magn. Reson. Imaging 2018.
PMID: 30584691
ISSN: 1522-2586
CID: 3560362

Different Relationship Between Systolic Blood Pressure and Cerebral Perfusion in Subjects With and Without Hypertension

Glodzik, Lidia; Rusinek, Henry; Tsui, Wai; Pirraglia, Elizabeth; Kim, Hee-Jin; Deshpande, Anup; Li, Yi; Storey, Pippa; Randall, Catherine; Chen, Jingyun; Osorio, Ricardo S; Butler, Tracy; Tanzi, Emily; McQuillan, Molly; Harvey, Patrick; Williams, Stephen K; Ogedegbe, Gbenga; Babb, James S; de Leon, Mony J
Although there is an increasing agreement that hypertension is associated with cerebrovascular compromise, relationships between blood pressure (BP) and cerebral blood flow are not fully understood. It is not known what BP level, and consequently what therapeutic goal, is optimal for brain perfusion. Moreover, there is limited data on how BP affects hippocampal perfusion, a structure critically involved in memory. We conducted a cross-sectional (n=445) and longitudinal (n=185) study of adults and elderly without dementia or clinically apparent stroke, who underwent clinical examination and brain perfusion assessment (age 69.2±7.5 years, 62% women, 45% hypertensive). Linear models were used to test baseline BP-blood flow relationship and to examine how changes in BP influence changes in perfusion. In the entire group, systolic BP (SBP) was negatively related to cortical (β=-0.13, P=0.005) and hippocampal blood flow (β=-0.12, P=0.01). Notably, this negative relationship was apparent already in subjects without hypertension. Hypertensive subjects showed a quadratic relationship between SBP and hippocampal blood flow (β=-1.55, P=0.03): Perfusion was the highest in subjects with mid-range SBP around 125 mm Hg. Longitudinally, in hypertensive subjects perfusion increased with increased SBP at low baseline SBP but increased with decreased SBP at high baseline SBP. Cortical and hippocampal perfusion decrease with increasing SBP across the entire BP spectrum. However, in hypertension, there seems to be a window of mid-range SBP which maximizes perfusion.
PMID: 30571554
ISSN: 1524-4563
CID: 3556742

Epigenetic Therapy with Panobinostat Combined with Bicalutamide Re-challenge in Castration-Resistant Prostate Cancer

Ferrari, Anna C; Alumkal, Joshi J; Stein, Mark N; Taplin, Mary-Ellen; Babb, James S; Barnett, Ethan S; Gomez-Pinillos, Alejandro; Liu, Xiaomei; Moore, Dirk F; DiPaola, Robert S; Beer, Tomasz M
PURPOSE/OBJECTIVE:LAARx). EXPERIMENTAL DESIGN/METHODS:The CWR22PC xenograft and isogenic cell line were tested for drug interactions on tumor cell growth and androgen receptor (AR), AR-splice variant7 and AR targets. A phase I trial had a 3x3 panobinostat dose-escalation design. The phase II randomized 55 patients to panobinostat 40mg (A-arm) or 20mg (B-arm) triweekly x2 weeks with bicalutamide 50mg/day in 3-week cycles. The primary endpoint was percent of patients radiographic progression-free (rPF) at 36 weeks versus historical high-dose bicalutamide. RESULTS:In the model, panobinostat/bicalutamide demonstrated synergistic antitumor effect while reducing AR activity. The dose-limiting toxicity was not reached. The probability of remaining rPF exceeded protocol-specified 35% in the A- and B-arms (47.5%; 38.5%). The A-arm but not the B-arm exceeded expectations for times (medians) to rP (33.9 and 10 weeks), and from PSA progression to rP (24 and 5.9 weeks). A-arm/B-arm: adverse events (AEs), 62%/19%; treatment stopped for AEs, 27.5%/11.5%; dose reduction required, 41%/4%. Principal A-arm grade≥3 AEs: thrombocytopenia (31%), fatigue (14%). CONCLUSIONS:LAARx. Panobinostat toxicity was tolerable with dose reductions. Epigenetic HDACI therapy reduces AR-mediated resistance to bicalutamide in CRPC models with clinical benefit in patients. The combination merits validation using a second-generation antiandrogen.
PMID: 30224345
ISSN: 1078-0432
CID: 3300352

Correction to: MRI assessment of the thigh musculature in dermatomyositis and healthy subjects using diffusion tensor imaging, intravoxel incoherent motion and dynamic DTI [Correction]

Sigmund, E E; Baete, S H; Luo, T; Patel, K; Wang, D; Rossi, I; Duarte, A; Bruno, M; Mossa, D; Femia, A; Ramachandran, S; Stoffel, D; Babb, J S; Franks, A G; Bencardino, J
The original version of this article, published on 04 June 2018, unfortunately contained a mistake.
PMID: 29987417
ISSN: 1432-1084
CID: 3191822

Online Interactive Case-Based Instruction in Prostate Magnetic Resonance Imaging Interpretation Using Prostate Imaging and Reporting Data System Version 2: Effect for Novice Readers

Rosenkrantz, Andrew B; Begovic, Jovan; Pires, Antonio; Won, Eugene; Taneja, Samir S; Babb, James S
PURPOSE/OBJECTIVE:To assess the effect on reader performance of an interactive case-based online tutorial for prostate magnetic resonance imaging (MRI) interpretation using Prostate Imaging and Reporting Data System (PI-RADS). METHODS:An educational website was developed incorporating scrollable multiparametric prostate MRI examinations with annotated solutions based on PI-RADS version 2. Three second-year radiology residents evaluated a separate set of 60 prostate MRI examinations both before and after review of the online case material, identifying and scoring dominant lesions. These 60 examinations included 30 benign cases and 30 cases with a dominant lesion demonstrating Gleason score ≥3 + 4 tumor on fusion-targeted biopsy. The readers' pooled performance was compared between the 2 sessions using logistic regression and Wilcoxon signed rank tests. RESULTS:All readers completed the online material within four-hours. Review of the online material significantly improved sensitivity (from 57.8%-73.3%, P = 0.003) and negative predictive value (from 69.2%-78.2%, P = 0.049), but not specificity (from 70.0%-67.8%, P = 0.692) or positive predictive value (from 59.6%-64.7%, P = 0.389). Reader confidence (1-10 scale; 10 = maximal confidence) also improved significantly (from 5.6 ± 2.7 to 6.3 ± 2.6, P = 0.026). However, accuracy of assigned PI-RADS scores did not improve significantly (from 45.5%-53.3%, P = 0.149). CONCLUSION/CONCLUSIONS:An online interactive case-based website in prostate MRI interpretation improved novice readers' sensitivity and negative predictive value for tumor detection, as well as readers' confidence. This online material may serve as a resource complementing existing traditional methods of instruction by providing a more flexible educational experience among a larger volume of learners. However, further more targeted educational initiatives regarding the proper application of PI-RADS remain warranted.
PMID: 29428182
ISSN: 1535-6302
CID: 2990062

Use of Shoulder Imaging in the Outpatient Setting: A Pilot Study

Garwood, Elisabeth R; Mittl, Gregory S; Alaia, Michael J; Babb, James; Gyftopoulos, Soterios
PURPOSE/OBJECTIVE:Characterize the clinical utility of diagnostic shoulder imaging modalities commonly used in the outpatient workup of shoulder pain. MATERIALS/METHODS/METHODS:Retrospective review of adults imaged for outpatient shoulder pain from 1/1/2013 to 9/1/2015. To be categorized as "useful", a study had to meet one of the following criteria: change the clinical diagnosis or treatment plan, provide a final diagnosis, or guide definitive treatment. A utility score was assigned to each study based on the number of utility criteria met (range 0-4). A score of 1 was considered low utility; a score of greater than or equal to 2 was considered high utility. Statistical analysis included binary logistic regression and generalized estimating equations. RESULTS:210 subjects (65% male); mean age 47 (range 18-84), underwent 302 imaging studies (159 X-ray, 137 MRI, 2 CT, 4 ultrasound) during the study period. 92.1% of all studies met minimum criteria for utility (score >1). Most commonly, diagnostic studies obtained during the outpatient workup of shoulder pain were found to guide definitive treatment (70.5%) or provide a final diagnosis (53%). Most X-rays were categorized as no or low utility (85.5%). 97.8% of the MRI studies were categorized as useful with most being high utility (73%). Overall, MRI was the most useful modality in all clinical scenarios (P = 0.002) and more likely to be high utility (P < 0.001) compared to X-rays. None of the investigated patient or injury characteristics were significant predictors of useful imaging. CONCLUSION/CONCLUSIONS:Our study suggests that both radiographs and MRI are useful in the evaluation of adult unilateral shoulder pain in the outpatient setting. MRI appears to be the most useful imaging modality in terms of helping guide diagnosis and treatment selection. This serves as a potential first step towards the development of evidence based imaging algorithms that can be used and tested in future studies.
PMID: 29203263
ISSN: 1535-6302
CID: 2907722

Diffusional kurtosis imaging of the corpus callosum in autism

Sui, Yu Veronica; Donaldson, Jeffrey; Miles, Laura; Babb, James S; Castellanos, Francisco Xavier; Lazar, Mariana
Background/UNASSIGNED:The corpus callosum is implicated in the pathophysiology of autism spectrum disorder (ASD). However, specific structural deficits and underlying mechanisms are yet to be well defined. Methods/UNASSIGNED:) diffusivities, which reflect myelination and microstructural organization of the extracellular space. The relationships between DKI metrics and processing speed, a cognitive feature known to be impaired in ASD, were also examined. Results/UNASSIGNED: > .05). Conclusion/UNASSIGNED:Decreased DKI metrics suggested that ASD may be associated with axonal deficits such as reduced axonal caliber and density in the corpus callosum, especially in the mid and posterior callosal areas. These data suggest that impaired interhemispheric connectivity may contribute to decreased processing speed in ASD participants.
PMCID:6293510
PMID: 30559954
ISSN: 2040-2392
CID: 3556472