ASL Knockout Cell Lysate

Background: Anti-N-methyl-D-aspartate receptor encephalitis is a central nervous system inflammatory autoimmune disease affecting adults and children. The use of first- and second-line immunotherapies is supported. Recent reports suggest the efficacy of bortezomib in severe anti-N-methyl-D-aspartate encephalitis in adult patients not responsive to second-line treatment; there are no data about pediatric patients. patient description: We describe an eight-year-old child with anti-N-methyl-D-aspartate encephalitis not responsive to first- and second-line treatments who experienced marked clinical improvement after bortezomib administration. Discussion: Bortezomib is a selective and reversible inhibitor of the 26S proteasome, which is used to treat oncologic and rare autoimmune disorders in pediatric patients. As observed in adult patients, bortezomib administration induced anti-N-methyl-D-aspartate antibody titer decline and clinical improvement with an acceptable risk profile. Conclusion: This is the first report of the use of bortezomib in children with anti-N-methyl-D-aspartate encephalitis; it could be a useful therapeutic option in children with refractory anti-N-methyl-D-aspartate encephalitis. (C) 2019 Elsevier Inc. All rights reserved.

Background Myocardial perfusion is evaluated in first-pass MRI using a gadolinium-based contrast agent, which limits its repeatability and restricts its use in patients with abnormal kidney function. Arterial spin labeling (ASL) is a promising technique for measuring myocardial perfusion without contrast injection. The ratio of stress to rest perfusion, termed myocardial perfusion reserve (MPR), is an indicator of the severity of stenosis in patients with coronary artery disease (CAD). Purpose To quantify perfusion increases with pharmacological vasodilation, explore MPR differences between segments with and without perfusion defects, and examine the correlations between quantitative ASL and semiquantitative first-pass measurements. Study Type Prospective. Subjects Sixteen patients with suspected CAD: 10 classified as "healthy," having normal perfusion on first-pass and no enhancement on late gadolinium enhancement (LGE), and six as "nonhealthy," having hypoperfused segments including ischemic and infarcted. Field Strength/Sequence Flow-sensitive alternating inversion recovery (FAIR) rest-stress cardiac ASL with balanced steady-state free precession (bSSFP), rest-stress first-pass imaging using gradient-echo and LGE using a phase-sensitive inversion-recovery bSSFP at 1.5T. Assessment For healthy subjects, rest-stress perfusion data were compared in global, coronary artery territory, and segment regions of interest (ROIs). A segmental MPR comparison was performed between normal segments from healthy subjects and abnormal segments from nonhealthy subjects. Correlations between ASL and first-pass parameters were explored. Statistical Tests Wilcoxon-signed-rank test, nonparametric factorial analysis of variance (ANOVA), and Pearson's/Spearman's correlations. Results Perfusion increases were significant globally (P= 0.005), per coronary artery territory (P= 0.015), and per segment (P= 0.03 for all segments in ASL and first-pass, except anteroseptal in ASLP= 0.04). MPR differences between normal and abnormal segments were significant (P= 0.0028: ASL,P= 0.033: first-pass). ASL and first-pass measurements were correlated (MPR:r =0.64,P= 0.008 and perfusion:rho= 0.47,P= 0.007). Data Conclusion This study demonstrates the feasibility of ASL to detect hyperemia, the potential to differentiate segments with and without perfusion defects, and significant correlations between ASL and semiquantitative first-pass. Level of Evidence 2 Technical Efficacy Stage 1