2022

N. Matthew Ellinwood, Bethann N Valentine, Andrew S Hess, Jackie K Jens, Elizabeth M Snella, Maryam Jamil, Shannon J Hostetter, Nicholas D Jeffery, Jodi D Smith, Suzanne T Millman, Rebecca L Parsons, Mark T Butt, Sundeep Chandra, Martin T Egeland, Ana B Assis, Hemanth R Nelvagal, Jonathan D Cooper, Igor Nestrasil, Bryon A Mueller, Rene Labounek, Amy Paulson, Heather Prill, Xiao Ying Liu, Huiyu Zhou, Roger Lawrence, Brett E Crawford, Anita Grover, Ganesh Cherala, Andrew C Melton, Anu Cherukuri, Brian R Vuillemenot, Jill CM Wait, Charles A. O'Neill, Jason Pinkstaff, Joseph Kovalchin, Eric Zanelli and Emma McCullagh. Tralesinidase alfa enzyme replacement therapy prevents disease manifestations in a canine model of mucopolysaccharidosis type IIIB. June 18 [In Press]. Journal of Pharmacology and Experimental Therapeutics. doi: 10.1124/jpet.122.001119

Ivana Labounková, René Labounek, Radim Kolář, Ralf P. Tornow, Charles F. Babbs, Collin M. McClelland, Benjamin R. Miller & Igor Nestrašil. Heart rate and age modulate retinal pulsatile patterns. June 14. Communications Biology. doi: 10.1038/s42003-022-03441-6

Julien Cohen-Adad, Eva Alonso-Ortiz, Stephanie Alley, Maria Marcella Lagana, Francesca Baglio, Signe Johanna Vannesjo, Haleh Karbasforoushan, Maryam Seif, Alan C. Seifert, Junqian Xu, Joo-Won Kim, René Labounek, Lubomír Vojtíšek, Marek Dostál, Jan Valošek, Rebecca S. Samson, Francesco Grussu, Marco Battiston, Claudia A. M. Gandini Wheeler-Kingshott, Marios C. Yiannakas, Guillaume Gilbert, Torben Schneider, Brian Johnson, Ferran Prados. Comparison of multicenter MRI protocols for visualizing the spinal cord gray matter. Apr 5. Magnetic Resonance in Medicine. doi: 10.1002/mrm.29249

Stefania Aulická, Katarina Česká, Jiří Šána, František Siegl, Eva Brichtová, Hana Ošlejšková, Markéta Hermanová, Michal Hendrych, Elleni Ponechal Michu, Milan Brázdil, Ondřej Slabý, Igor Nestrašil. Cytokine-chemokine profiles in the hippocampus of patients with mesial temporal lobe epilepsy and hippocampal sclerosis. Feb 2022. Epilepsy Research. doi: 10.1016/j.eplepsyres.2022.106858

 

 

 

2021

Frits A. Wijburg, Fiona Heap, Stewart Rust, Jessica de Ruijter, Evelien Tump, Jan Pieter Marchal, Igor Nestrasil, Elsa Shapiro, Simon A. Jones, David Alexanderian. Long-term safety and clinical outcomes of intrathecal heparan-N-sulfatase in patients with Sanfilippo syndrome type A. Dec. Molecular Genetics and Metabolism. doi: 10.1016/j.ymgme.2021.09.003

Kurt G. Schilling...Igor Nestrasil, René Labounek, Christophe Lenglet, Amy Paulson, Stefania Aulicka, et al. Tractography dissection variability. Nov. Neuroimage. doi: 10.1016/j.neuroimage.2021.118502

Dinesh K. Deelchand, Mai-Lan Ho, Igor Nestrasil. Ultra-High-Field Imaging of the Pediatric Brain and Spinal Cord. Nov. Magnetic Resonance Imaging Clinics of North America. doi: 10.1016/j.mric.2021.06.013

Julien Cohen-Adad...René Labounek, Christophe Lenglet, Igor Nestrasil, et al. Generic acquisition protocol for quantitative MRI of the spinal cord. Oct. Nature Protocols. doi: 10.1038/s41596-021-00588-0

Julien Cohen-Adad...René Labounek, Christophe Lenglet, Igor Nestrasil, et al. Open-access quantitative MRI data of the spinal cord and reproducibility across participants, sites and manufacturers. Aug 16. Scientific Data. doi: 10.1038/s41597-021-00941-8

René Labounek, Zhuolin Wu, David A. Bridwell, Milan Brázdil, Jiří Jan, Igor Nestrašil. Blind Visualization of Task-Related Networks From Visual Oddball Simultaneous EEG-fMRI Data: Spectral or Spatiospectral Model? Apr 26. Frontiers in Neurology. doi: 10.3389/fneur.2021.644874

Ivana Labounkova, Rene Labounek, Igor Nestrasil, Jan Odstrcilik,
Ralf P. Tornow, and Radim Kolar. Blind Source Separation of Retinal Pulsatile Patterns in Optic Nerve Head Video-Recordings. Nov 24. IEEE Transactions on Medical Imaging. doi: 10.1109/TMI.2020.3039917

 

 

2020

Jitka Majovska, Igor Nestrasil, Amy Paulson, David Nascene, Katarina Jurickova, Anna Hlavata, Troy Lund, Paul J. Orchard, Manuela Vaneckova, Jiri Zeman, Martin Magner, Petr Dusek. White matter alteration and cerebellar atrophy are hallmarks of brain MRI in alpha-mannosidosis. Dec 3. Molecular Genetics and Metabolism. doi: 10.1016/j.ymgme.2020.11.008

René Labounek, Jan Valošek, Tomáš Horák, Alena Svátková, Petr Bednařík, Lubomír Vojtíšek, Magda Horáková, Igor Nestrašil, Christophe Lenglet, Julien Cohen-Adad, Josef Bednařík & Petr Hluštíl. HARDI-ZOOMit protocol improves specificity to microstructural changes in presymptomatic myelopathy. Oct 16. Scientific Reports. doi: 10.1038/s41598-020-70297-3

Steven Q. Le, Igor Nestrasil, Shih-hsin Kan, Martin Egeland, Jonathan D. Cooper, David Elashoff, Rong Guo, Jakub Tolar, Jennifer K. Yee, Patricia I. Dickson. Myelin and Lipid Composition of the Corpus Callosum in Mucopolysaccharidosis Type I Mice. June 14. Lipids. doi: 10.1002/lipd.12261

Pavel Filip, Alena Svatkova, Adam F Carpenter, Lynn E Eberly, Igor Nestrasil, Mikko J Nissi, Shalom Michaeli, Silvia Mangia. Rotating frame MRI relaxations as markers of diffuse white matter abnormalities in multiple sclerosis. March 2. NeuroImage: Clinical. doi: 10.1016/j.nicl.2020.102234

Stephanie I. W. van de Stadt, Wouter J. C. van Ballegoij, René Labounek, Irene C. Huffnagel, Stephan Kemp, Igor Nestrasil, Marc Engelen. Spinal cord atrophy as a measure of severity of myelopathy in adrenoleukodystrophy. Feb 19. Journal of Inherited Metabolic Disease. doi: 10.1002/jimd.12226

 

 

2019

Agnes H. Chen, Paul Harmatz, Igor Nestrasil, Julie B. Eisengart, Kelly E. King, Kyle Rudser, Alexander M. Kaizer, Alena Svatkovac, Amy Wakumoto, Steven Q. Le, Jacqueline Madden, Sarah Young, Haoyue Zhang, Lynda E. Polgreen, Patricia I. Dickson. Intrathecal enzyme replacement for cognitive decline in mucopolysaccharidosis type I, a randomized, open-label, controlled pilot study. Nov 30. Molecular Genetics and Metabolism. doi: 10.1016/j.ymgme.2019.11.007

Ivana Labounková, René Labounek, Jan Odstrčilík, Michal Hracho, Igor Nestrašil, Ralf P. Tornow, Radim Kolář. Blind Source Separation of Different Retinal Pulsatile Patterns from Simultaneous Long-term Binocular Ophthalmoscopic Video-records. Oct 7. (Conference Paper) Proceedings of the Annual International Conference of the IEEE Engineering in Medicine and Biology Society, EMBS. doi: 10.1109/EMBC.2019.8857560

René Labounek, Khoi Mai, Bryon Mueller, N. Matthew Ellinwood, Patricia Dickson, Igor Nestrašil. In-vivo cortical thickness estimation from high-resolution T1w MRI scans in healthy and mucopolysaccharidosis affected dogs. Oct 7. (Conference Paper)
Proceedings of the Annual International Conference of the IEEE Engineering in Medicine and Biology Society, EMBS. 
doi: 10.1109/EMBC.2019.8856826

Kelly E. King, Kyle D. Rudser, Igor Nestrasil, Victor Kovac, Kathleen A. Delaney, Jeffrey R. Wozniak, Bryon A. Mueller, Kelvin O. Lim, Julie B. Eisengart, Eva G. Mamak, Julian Raiman, Nadia Ali, Stephanie Cagle, Paul Harmatz, Chester B. Whitley, Elsa G. Shapiro. Attention and corpus callosum volumes in individuals with mucopolysaccharidosis type i. Apr 12. Neurology. doi: 10.1212/WNL.0000000000007496

 

 

2018

Roberto Giugliani, Luciana Giugliani, Fabiano de Oliveira Poswar, Karina Carvalho Donis, Amauri Dalla Corte, Mathias Schmidt, Ruben J. Boado, Igor Nestrasil, Carol Nguyen, Steven Chen & William M. Pardridge. Neurocognitive and somatic stabilization in pediatric patients with severe Mucopolysaccharidosis Type i after 52 weeks of intravenous brain-penetrating insulin receptor antibody-iduronidase fusion protein (valanafusp alpha): An open label phase 1-2 trial. July 5. Orphanet Journal of Rare Diseases. doi: 10.1186/s13023-018-0849-8

Igor Nestrasil, Alena Svatkova, Kyle D. Rudser, Ravishankar Chityala, Amy Wakumoto, Bryon A. Mueller, Petr Bednařík, Paul Tuite, Xiang Wu, Khalaf Bushara. White matter measures correlate with essential tremor severity—A pilot diffusion tensor imaging study. July 2. Brain and Behavior. doi: 10.1002/brb3.1039

Chester B Whitley, Maureen Cleary, Karl Eugen Mengel, Paul Harmatz, Elsa Shapiro, Igor Nestrasil, Patrick Haslett, David Whiteman, David Alexanderian. Observational Prospective Natural History of Patients with Sanfilippo Syndrome Type B. Apr 13. Journal of Pediatrics. doi: 10.1016/j.jpeds.2018.01.044

 

 

2017

Igor Nestrasil, Alia Ahmed, Josephine M. Utz, Kyle Rudser, Chester B. Whitley, Jeanine R. Jarnes-Utz. Distinct progression patterns of brain disease in infantile and juvenile gangliosidoses: Volumetric quantitative MRI study. Dec 20. Molecular Genetics and Metabolism. doi: 10.1016/j.ymgme.2017.12.432

Silvia Mangia, Alena Svatkova, Daniele Mascali, Mikko J. Nissi, Philip C. Burton, Petr Bednarik, Edward J. Auerbach, Federico Giove, Lynn E. Eberly, Michael J. Howell, Igor Nestrasil, Paul J. Tuite and Shalom Michaeli. Multi-modal brain MRI in subjects with PD and iRBD. Dec 19. Frontiers in Neuroscience. doi: 10.3389/fnins.2017.00709

Julie B. Eisengart, Jeanine Jarnes, Alia Ahmed, Igor Nestrasil, Richard Ziegler, Kathleen Delaney, Elsa Shapiro, Chester Whitley. Long-term cognitive and somatic outcomes of enzyme replacement therapy in untransplanted Hurler syndrome. Sep 27. Molecular Genetics and Metabolism Reports. doi: 10.1016/j.ymgmr.2017.07.012

Igor Nestrasil, Leonardo Vedolin. Quantitative neuroimaging in mucopolysaccharidoses clinical trials. Sep 15. Molecular Genetics and Metabolism. doi: 10.1016/j.ymgme.2017.09.006

Magy Abdelwahab, Michael Potegal, Elsa G. Shapiro, Igor Nestrasil. Previously unrecognized behavioral phenotype in Gaucher disease type 3. May 23. Neurology: Genetics. doi: 10.1212/NXG.0000000000000158

Igor Nestrasil, Elsa Shapiro, Alena Svatkova, Patricia Dickson, Agnes Chen, Amy Wakumoto, Alia Ahmed, Edward Stehel, Sarah McNeil, Curtis Gravance, Elizabeth Maher. Intrathecal enzyme replacement therapy reverses cognitive decline in mucopolysaccharidosis type I. Feb 17. American Journal of Medical Genetics, Part A.  doi: 10.1002/ajmg.a.38073

 

 

2016

Anthony Tabet, Matthew Gardner, Sebastian Swanson, Sydney Crump, Austin McMeekin, Diana Gong, Rebecca Tabet, Benjamin Hacker, Igor Nestrasil. Low-cost, rapidly-developed, 3D printed in vitro corpus callosum model for mucopolysaccharidosis type I. Dec 2
(Revised 3/16/2017). F1000Research. doi: 10.12688/f1000research.9861.2

Ulrike Löbel, Jan Sedlacik, Michael Nickel, Susanne Lezius, Jens Fiehler, Igor Nestrasil, Alfried Kohlschütter and Angela Schulz. Volumetric Description of Brain Atrophy in Neuronal Ceroid Lipofuscinosis 2: Supratentorial Gray Matter Shows Uniform Disease Progression. Oct 7. American Journal of Neuroradiology. doi: 10.3174/ajnr.A4816

Alena Svatkova, Igor Nestrasil, Kyle Rudser, Jodene Goldenring Fine, Jesse Bledsoe, and Margaret Semrud-Clikeman. Unique white matter microstructural patterns in ADHD presentations—a diffusion tensor imaging study. May 9. Human Brain Mapping. doi: 10.1002/hbm.23243

Elsa Shapiro, Kelly King, Alia Ahmed, Kyle Rudser, R. Rumsey, Brianna D. Yund, Kathleen A. Delaney, Igor Nestrasil, Chester. Whitley, Michael Potegal. The neurobehavioral phenotype in mucopolysaccharidosis Type IIIB: An exploratory study. Feb 2. Molecular Genetics and Metabolism Reports. doi: 10.1016/j.ymgmr.2016.01.003

Elsa G. Shapiro, Igor Nestrasil, Kathleen A. Delaney, Kyle Rudser, Victor Kovac, Nitin Nair, Charles W. Richard 3rd, Patrick Haslett, Chester B. Whitley. A Prospective Natural History Study of Mucopolysaccharidosis Type IIIA. Jan 16. The Journal of Pediatrics. doi: 10.1016/j.jpeds.2015.11.079

 

 

2015

James M. Provenzale, Igor Nestrasil, Steven Chen, Shih-hsin Kan, Steven Q. Le, Jacqueline K. Jens, Elizabeth M. Snella, Kristen N. Vondrak, Jennifer K.Yee, Charles H. Vite, David Elashoff, Lewei Duan, Raymond Y. Wang, N. Matthew Ellinwood, Miguel A. Guzman, Elsa G.Shapiro, Patricia I. Dickson. Diffusion tensor imaging and myelin composition analysis reveal abnormal myelination in corpus callosum of canine mucopolysaccharidosis I. July 26. Experimental Neurology. doi: 10.1016/j.expneurol.2015.07.021

Elsa G. Shapiro, Igor Nestrasil, Kyle Rudser, Kathleen Delaney, Victor Kovac, Alia Ahmed, Brianna Yund, Paul J. Orchard, Julie Eisengart, Gregory R. Niklason, Julian Raiman, Eva Mamak, Morton J. Cowan, Mara Bailey-Olson, Paul Harmatz, Suma P. Shankar, Stephanie Cagle, Nadia Ali, Robert D. Steiner, Jeffrey Wozniak, Kelvin O. Lim, Chester B. Whitley. Neurocognition across the spectrum of mucopolysaccharidosis type I: Age, severity, and treatment. June 17. Molecular Genetics and Metabolism. doi: 10.1016/j.ymgme.2015.06.002

Elsa G. Shapiro, Igor Nestrasil, Alia Ahmed, Andrew Wey, Kyle R. Rudser, Kathleen A. Delaney, Robin K. Rumsey, Patrick A. J. Haslett, Chester B. Whitley, Michael Potegal. Quantifying behaviors of children with Sanfilippo syndrome: The Sanfilippo Behavior Rating Scale. Mar 5. Molecular Genetics and Metabolism. doi: 10.1016/j.ymgme.2015.02.008

 

 

2014

Brianna Yund, Kyle Rudser, Alia Ahmed, Victor Kovac, Igor Nestrasil, Julian Raiman, Eva Mamak, Paul Harmatz, Robert Steiner, Heather Lau, Pooja Vekaria, Jeffrey R. Wozniak, Kelvin O. Lim, Kathleen Delaney, Chester Whitley, Elsa G. Shapiro. Cognitive, medical, and neuroimaging characteristics of attenuated mucopolysaccharidosis type II. Dec 9. Molecular Genetics and Metabolism. doi: 10.1016/j.ymgme.2014.12.299

 

 

2013

Raphael Schiffmann, Joan Mayfield, Caren Swift, Igor Nestrasil. Quantitative neuroimaging in mucolipidosis type IV. Nov 21 Molecular Genetics and Metabolism. doi: 10.1016/j.ymgme.2013.11.007

Michael Potegal, Brianna Yund, Kyle Rudser, Alia Ahmed, Kate Delaney, Igor Nestrasil, Chester B. Whitley & Elsa G. Shapiro. Mucopolysaccharidosis Type IIIA presents as a variant of Klüver-Bucy syndrome. June 8. Journal of Clinical and Experimental Neuropsychology. doi: 10.1080/13803395.2013.804035

Charles H. Vite, Igor Nestrasil, Anton Mlikotic, Jackie K. Jens, Elizabeth M. Snella, William Gross, Elsa G. Shapiro, Victor Kovac, James M. Provenzale, Steven Chen, Steven Q. Le, Shih-hsin Kan, Shida Banakar, Raymond Y. Wang, Mark E. Haskins, N. Matthew Ellinwood, Patricia I. Dickson. Features of brain MRI in dogs with treated and untreated mucopolysaccharidosis type i. Apr 1. Comparative Medicine. PMCID: PMC3625057