Skip to main content
Advertisement

Main menu

  • Home
  • Content
    • Current Issue
    • Accepted Manuscripts
    • Article Preview
    • Past Issue Archive
    • Video Articles
    • AJNR Case Collection
    • Case of the Week Archive
    • Case of the Month Archive
    • Classic Case Archive
  • Special Collections
    • AJNR Awards
    • Low-Field MRI
    • Alzheimer Disease
    • ASNR Foundation Special Collection
    • Photon-Counting CT
    • View All
  • Multimedia
    • AJNR Podcasts
    • AJNR SCANtastic
    • Trainee Corner
    • MRI Safety Corner
    • Imaging Protocols
  • For Authors
    • Submit a Manuscript
    • Submit a Video Article
    • Submit an eLetter to the Editor/Response
    • Manuscript Submission Guidelines
    • Statistical Tips
    • Fast Publishing of Accepted Manuscripts
    • Graphical Abstract Preparation
    • Imaging Protocol Submission
    • Author Policies
  • About Us
    • About AJNR
    • Editorial Board
    • Editorial Board Alumni
  • More
    • Become a Reviewer/Academy of Reviewers
    • Subscribers
    • Permissions
    • Alerts
    • Feedback
    • Advertisers
    • ASNR Home

User menu

  • Alerts
  • Log in

Search

  • Advanced search
American Journal of Neuroradiology
American Journal of Neuroradiology

American Journal of Neuroradiology

ASHNR American Society of Functional Neuroradiology ASHNR American Society of Pediatric Neuroradiology ASSR
  • Alerts
  • Log in

Advanced Search

  • Home
  • Content
    • Current Issue
    • Accepted Manuscripts
    • Article Preview
    • Past Issue Archive
    • Video Articles
    • AJNR Case Collection
    • Case of the Week Archive
    • Case of the Month Archive
    • Classic Case Archive
  • Special Collections
    • AJNR Awards
    • Low-Field MRI
    • Alzheimer Disease
    • ASNR Foundation Special Collection
    • Photon-Counting CT
    • View All
  • Multimedia
    • AJNR Podcasts
    • AJNR SCANtastic
    • Trainee Corner
    • MRI Safety Corner
    • Imaging Protocols
  • For Authors
    • Submit a Manuscript
    • Submit a Video Article
    • Submit an eLetter to the Editor/Response
    • Manuscript Submission Guidelines
    • Statistical Tips
    • Fast Publishing of Accepted Manuscripts
    • Graphical Abstract Preparation
    • Imaging Protocol Submission
    • Author Policies
  • About Us
    • About AJNR
    • Editorial Board
    • Editorial Board Alumni
  • More
    • Become a Reviewer/Academy of Reviewers
    • Subscribers
    • Permissions
    • Alerts
    • Feedback
    • Advertisers
    • ASNR Home
  • Follow AJNR on Twitter
  • Visit AJNR on Facebook
  • Follow AJNR on Instagram
  • Join AJNR on LinkedIn
  • RSS Feeds

AJNR Awards, New Junior Editors, and more. Read the latest AJNR updates

Research ArticlePediatric Neuroimaging
Open Access

Brain Region–Specific Degeneration with Disease Progression in Late Infantile Neuronal Ceroid Lipofuscinosis (CLN2 Disease)

J.P. Dyke, D. Sondhi, H.U. Voss, K. Yohay, C. Hollmann, D. Mancenido, S.M. Kaminsky, L.A. Heier, K.D. Rudser, B. Kosofsky, B.J. Casey, R.G. Crystal and D. Ballon
American Journal of Neuroradiology June 2016, 37 (6) 1160-1169; DOI: https://doi.org/10.3174/ajnr.A4669
J.P. Dyke
aFrom the Departments of Radiology (J.P.D., H.U.V., L.A.H., D.B.)
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for J.P. Dyke
D. Sondhi
bGenetic Medicine (D.S., C.H., D.M., S.M.K., R.G.C., D.B.)
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for D. Sondhi
H.U. Voss
aFrom the Departments of Radiology (J.P.D., H.U.V., L.A.H., D.B.)
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for H.U. Voss
K. Yohay
cPediatrics (K.Y., B.K.)
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for K. Yohay
C. Hollmann
bGenetic Medicine (D.S., C.H., D.M., S.M.K., R.G.C., D.B.)
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for C. Hollmann
D. Mancenido
bGenetic Medicine (D.S., C.H., D.M., S.M.K., R.G.C., D.B.)
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for D. Mancenido
S.M. Kaminsky
bGenetic Medicine (D.S., C.H., D.M., S.M.K., R.G.C., D.B.)
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for S.M. Kaminsky
L.A. Heier
aFrom the Departments of Radiology (J.P.D., H.U.V., L.A.H., D.B.)
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for L.A. Heier
K.D. Rudser
eDivision of Biostatistics (K.D.R.), Clinical and Translational Science Institute, University of Minnesota, Minneapolis, Minnesota.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for K.D. Rudser
B. Kosofsky
cPediatrics (K.Y., B.K.)
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for B. Kosofsky
B.J. Casey
dPsychiatry (B.J.C.), Weill Cornell Medical College, New York, New York
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for B.J. Casey
R.G. Crystal
bGenetic Medicine (D.S., C.H., D.M., S.M.K., R.G.C., D.B.)
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for R.G. Crystal
D. Ballon
aFrom the Departments of Radiology (J.P.D., H.U.V., L.A.H., D.B.)
bGenetic Medicine (D.S., C.H., D.M., S.M.K., R.G.C., D.B.)
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for D. Ballon
  • Article
  • Figures & Data
  • Supplemental
  • Info & Metrics
  • Responses
  • References
  • PDF
Loading

Article Information

vol. 37 no. 6 1160-1169
DOI 
https://doi.org/10.3174/ajnr.A4669
PubMed 
26822727

Published By 
American Journal of Neuroradiology
Print ISSN 
0195-6108
Online ISSN 
1936-959X
History 
  • Received April 8, 2015
  • Accepted after revision November 30, 2015
  • Published online June 13, 2016.

Article Versions

  • Latest version (January 28, 2016 - 06:51).
  • You are viewing the most recent version of this article.
Copyright & Usage 
© 2016 by American Journal of Neuroradiology Indicates open access to non-subscribers at www.ajnr.org

Author Information

  1. J.P. Dykea,
  2. D. Sondhib,
  3. H.U. Vossa,
  4. K. Yohayc,
  5. C. Hollmannb,
  6. D. Mancenidob,
  7. S.M. Kaminskyb,
  8. L.A. Heiera,
  9. K.D. Rudsere,
  10. B. Kosofskyc,
  11. B.J. Caseyd,
  12. R.G. Crystalb and
  13. D. Ballona,b
  1. aFrom the Departments of Radiology (J.P.D., H.U.V., L.A.H., D.B.)
  2. bGenetic Medicine (D.S., C.H., D.M., S.M.K., R.G.C., D.B.)
  3. cPediatrics (K.Y., B.K.)
  4. dPsychiatry (B.J.C.), Weill Cornell Medical College, New York, New York
  5. eDivision of Biostatistics (K.D.R.), Clinical and Translational Science Institute, University of Minnesota, Minneapolis, Minnesota.
  1. Please address correspondence to Jonathan P. Dyke, PhD, Department of Radiology, Weill Cornell Medical College, 1300 York Ave, Box 234, New York, NY 10021; e-mail: jpd2001{at}med.cornell.edu
View Full Text

Altmetrics

Cited By...

  • 20 Citations
  • 21 Citations
  • Google Scholar

This article has been cited by the following articles in journals that are participating in Crossref Cited-by Linking.

  • Clinical challenges and future therapeutic approaches for neuronal ceroid lipofuscinosis
    Sara E Mole, Glenn Anderson, Heather A Band, Samuel F Berkovic, Jonathan D Cooper, Sophia-Martha Kleine Holthaus, Tristan R McKay, Diego L Medina, Ahad A Rahim, Angela Schulz, Alexander J Smith
    The Lancet Neurology 2019 18 1
  • Slowing late infantile Batten disease by direct brain parenchymal administration of a rh.10 adeno-associated virus expressing CLN2
    Dolan Sondhi, Stephen M. Kaminsky, Neil R. Hackett, Odelya E. Pagovich, Jonathan B. Rosenberg, Bishnu P. De, Alvin Chen, Benjamin Van de Graaf, Jason G. Mezey, Grace W. Mammen, Denesy Mancenido, Fang Xu, Barry Kosofsky, Kaleb Yohay, Stefan Worgall, Robert J. Kaner, Mark Souwedaine, Bruce M. Greenwald, Michael Kaplitt, Jonathan P. Dyke, Douglas J. Ballon, Linda A. Heier, Szilard Kiss, Ronald G. Crystal
    Science Translational Medicine 2020 12 572
  • Photosensitivity is an early marker of neuronal ceroid lipofuscinosis type 2 disease
    Nicola Specchio, Marcello Bellusci, Nicola Pietrafusa, Marina Trivisano, Luca de Palma, Federico Vigevano
    Epilepsia 2017 58 8
  • Neuronal Ceroid Lipofuscinosis: Potential for Targeted Therapy
    Nicola Specchio, Alessandro Ferretti, Marina Trivisano, Nicola Pietrafusa, Chiara Pepi, Costanza Calabrese, Susanna Livadiotti, Alessandra Simonetti, Paolo Rossi, Paolo Curatolo, Federico Vigevano
    Drugs 2021 81 1
  • Neuronal Ceroid Lipofuscinosis: The Multifaceted Approach to the Clinical Issues, an Overview
    Alessandro Simonati, Ruth E. Williams
    Frontiers in Neurology 2022 13
  • <p>Changing Times for CLN2 Disease: The Era of Enzyme Replacement Therapy</p>
    Nicola Specchio, Nicola Pietrafusa, Marina Trivisano
    Therapeutics and Clinical Risk Management 2020 Volume 16
  • Advances in the treatment of neuronal ceroid lipofuscinosis
    Jonathan B. Rosenberg, Alvin Chen, Stephen M. Kaminsky, Ronald G. Crystal, Dolan Sondhi
    Expert Opinion on Orphan Drugs 2019 7 11
  • Extracellular Vesicles as Drug Carriers for Enzyme Replacement Therapy to Treat CLN2 Batten Disease: Optimization of Drug Administration Routes
    Matthew J. Haney, Yuling Zhao, Yeon S. Jin, Elena V. Batrakova
    Cells 2020 9 5
  • Expanding the Neuroimaging Phenotype of Neuronal Ceroid Lipofuscinoses
    A. Biswas, P. Krishnan, A. Amirabadi, S. Blaser, S. Mercimek-Andrews, M. Shroff
    American Journal of Neuroradiology 2020 41 10
  • Computed tomography provides enhanced techniques for longitudinal monitoring of progressive intracranial volume loss associated with regional neurodegeneration in ovine neuronal ceroid lipofuscinoses
    Katharina N. Russell, Nadia L. Mitchell, Nigel G. Anderson, Craig R. Bunt, Martin P. Wellby, Tracy R. Melzer, Graham K. Barrell, David N. Palmer
    Brain and Behavior 2018 8 9
  • Recent Insight into the Genetic Basis, Clinical Features, and Diagnostic Methods for Neuronal Ceroid Lipofuscinosis
    Konrad Kaminiów, Sylwia Kozak, Justyna Paprocka
    International Journal of Molecular Sciences 2022 23 10
  • Gait phenotype in Batten disease: A marker of disease progression
    John R. Ostergaard
    European Journal of Paediatric Neurology 2021 35
  • Managing CLN2 disease: a treatable neurodegenerative condition among other treatable early childhood epilepsies
    Maria Mazurkiewicz-Bełdzińska, Mireia del Toro, Göknur Haliloğlu, Hidde H. Huidekoper, Ružica Kravljanac, Chris Mühlhausen, Brian Nauheimer Andersen, Igor Prpić, Pasquale Striano, Stéphane Auvin
    Expert Review of Neurotherapeutics 2021 21 11
  • Natural history of MRI brain volumes in patients with neuronal ceroid lipofuscinosis 3: a sensitive imaging biomarker
    Jan-Niklas Hochstein, A. Schulz, M. Nickel, S. Lezius, M. Grosser, J. Fiehler, J. Sedlacik, U. Löbel
    Neuroradiology 2022 64 10
  • Unraveling neuronal ceroid lipofuscinosis type 2 (CLN2) disease: A tertiary center experience for determinants of diagnostic delay
    Didem Ardicli, Goknur Haliloglu, Rahsan Gocmen, Ceren Gunbey, Meral Topcu
    European Journal of Paediatric Neurology 2021 33
  • Movement Disorders in Childhood
    Harvey S. Singer, Jonathan W. Mink, Donald L. Gilbert, Joseph Jankovic
    2022
  • Glucose metabolism impairment as a hallmark of progressive myoclonus epilepsies: a focus on neuronal ceroid lipofuscinoses
    Lorenzo Santucci, Sara Bernardi, Rachele Vivarelli, Filippo Maria Santorelli, Maria Marchese
    Frontiers in Cellular Neuroscience 2024 18
  • Magnetic resonance brain volumetry biomarkers of CLN2 Batten disease identified with miniswine model
    Kevin Knoernschild, Hans J. Johnson, Kimberly E. Schroeder, Vicki J. Swier, Katherine A. White, Takashi S. Sato, Christopher S. Rogers, Jill M. Weimer, Jessica C. Sieren
    Scientific Reports 2023 13 1
  • Position of Experts Regarding Follow-Up of Patients with Neuronal Ceroid Lipofuscinosis-2 Disease in Latin America
    Norberto Guelbert, Nora Atanacio, Inés Denzler, Emília Katiane Embiruçu, Nury Mancilla, Ricardo Naranjo, André Pessoa, Norma Spécola, Lina Tavera, Mónica Troncoso, Diane Vergara
    Journal of Inborn Errors of Metabolism and Screening 2020 8
  • Enzyme Replacement Therapy for CLN2 Disease: MRI Volumetry Shows Significantly Slower Volume Loss Compared with a Natural History Cohort
    Pritika Gaur, Paul Gissen, Asthik Biswas, Kshitij Mankad, Sniya Sudhakar, Felice D’Arco, Angela Schulz, Jens Fiehler, Jan Sedlacik, Ulrike Löbel
    American Journal of Neuroradiology 2024 45 11
  • Magnetic Resonance Imaging as a Readout of CLN5 Gene Therapy Efficacy in Sheep
    Samantha J. Murray, Mustafa M. Almuqbel, Simon A. Felton, Nickolas J. Palmer, Ashley R. Deane, Daniel J. Myall, Reza Shoorangiz, Arsène Ella, Matthieu Keller, David N. Palmer, Tracy R. Melzer, Nadia L. Mitchell
    Brain and Behavior 2025 15 4
PreviousNext
Back to top

In this issue

American Journal of Neuroradiology: 37 (6)
American Journal of Neuroradiology
Vol. 37, Issue 6
1 Jun 2016
  • Table of Contents
  • Index by author
  • Complete Issue (PDF)
Advertisement
Print
Download PDF
Email Article

Thank you for your interest in spreading the word on American Journal of Neuroradiology.

NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. We do not capture any email address.

Enter multiple addresses on separate lines or separate them with commas.
Brain Region–Specific Degeneration with Disease Progression in Late Infantile Neuronal Ceroid Lipofuscinosis (CLN2 Disease)
(Your Name) has sent you a message from American Journal of Neuroradiology
(Your Name) thought you would like to see the American Journal of Neuroradiology web site.
CAPTCHA
This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.
Cite this article
J.P. Dyke, D. Sondhi, H.U. Voss, K. Yohay, C. Hollmann, D. Mancenido, S.M. Kaminsky, L.A. Heier, K.D. Rudser, B. Kosofsky, B.J. Casey, R.G. Crystal, D. Ballon
Brain Region–Specific Degeneration with Disease Progression in Late Infantile Neuronal Ceroid Lipofuscinosis (CLN2 Disease)
American Journal of Neuroradiology Jun 2016, 37 (6) 1160-1169; DOI: 10.3174/ajnr.A4669

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero
0 Responses
Respond to this article
Share
Bookmark this article
Brain Region–Specific Degeneration with Disease Progression in Late Infantile Neuronal Ceroid Lipofuscinosis (CLN2 Disease)
J.P. Dyke, D. Sondhi, H.U. Voss, K. Yohay, C. Hollmann, D. Mancenido, S.M. Kaminsky, L.A. Heier, K.D. Rudser, B. Kosofsky, B.J. Casey, R.G. Crystal, D. Ballon
American Journal of Neuroradiology Jun 2016, 37 (6) 1160-1169; DOI: 10.3174/ajnr.A4669
del.icio.us logo Twitter logo Facebook logo Mendeley logo
  • Tweet Widget
  • Facebook Like
  • Google Plus One
Purchase

Jump to section

  • Article
    • Abstract
    • ABBREVIATION:
    • Materials and Methods
    • Results
    • Discussion
    • Conclusions
    • Acknowledgments
    • Footnotes
    • References
  • Figures & Data
  • Supplemental
  • Info & Metrics
  • Responses
  • References
  • PDF

Related Articles

  • PubMed
  • Google Scholar

Cited By...

  • Enzyme Replacement Therapy for CLN2 Disease: MRI Volumetry Shows Significantly Slower Volume Loss Compared with a Natural History Cohort
  • Slowing late infantile Batten disease by direct brain parenchymal administration of a rh.10 adeno-associated virus expressing CLN2
  • Expanding the Neuroimaging Phenotype of Neuronal Ceroid Lipofuscinoses
  • Crossref (21)
  • Google Scholar

This article has been cited by the following articles in journals that are participating in Crossref Cited-by Linking.

  • Clinical challenges and future therapeutic approaches for neuronal ceroid lipofuscinosis
    Sara E Mole, Glenn Anderson, Heather A Band, Samuel F Berkovic, Jonathan D Cooper, Sophia-Martha Kleine Holthaus, Tristan R McKay, Diego L Medina, Ahad A Rahim, Angela Schulz, Alexander J Smith
    The Lancet Neurology 2019 18 1
  • Slowing late infantile Batten disease by direct brain parenchymal administration of a rh.10 adeno-associated virus expressing CLN2
    Dolan Sondhi, Stephen M. Kaminsky, Neil R. Hackett, Odelya E. Pagovich, Jonathan B. Rosenberg, Bishnu P. De, Alvin Chen, Benjamin Van de Graaf, Jason G. Mezey, Grace W. Mammen, Denesy Mancenido, Fang Xu, Barry Kosofsky, Kaleb Yohay, Stefan Worgall, Robert J. Kaner, Mark Souwedaine, Bruce M. Greenwald, Michael Kaplitt, Jonathan P. Dyke, Douglas J. Ballon, Linda A. Heier, Szilard Kiss, Ronald G. Crystal
    Science Translational Medicine 2020 12 572
  • Photosensitivity is an early marker of neuronal ceroid lipofuscinosis type 2 disease
    Nicola Specchio, Marcello Bellusci, Nicola Pietrafusa, Marina Trivisano, Luca de Palma, Federico Vigevano
    Epilepsia 2017 58 8
  • Neuronal Ceroid Lipofuscinosis: Potential for Targeted Therapy
    Nicola Specchio, Alessandro Ferretti, Marina Trivisano, Nicola Pietrafusa, Chiara Pepi, Costanza Calabrese, Susanna Livadiotti, Alessandra Simonetti, Paolo Rossi, Paolo Curatolo, Federico Vigevano
    Drugs 2021 81 1
  • Neuronal Ceroid Lipofuscinosis: The Multifaceted Approach to the Clinical Issues, an Overview
    Alessandro Simonati, Ruth E. Williams
    Frontiers in Neurology 2022 13
  • <p>Changing Times for CLN2 Disease: The Era of Enzyme Replacement Therapy</p>
    Nicola Specchio, Nicola Pietrafusa, Marina Trivisano
    Therapeutics and Clinical Risk Management 2020 Volume 16
  • Advances in the treatment of neuronal ceroid lipofuscinosis
    Jonathan B. Rosenberg, Alvin Chen, Stephen M. Kaminsky, Ronald G. Crystal, Dolan Sondhi
    Expert Opinion on Orphan Drugs 2019 7 11
  • Extracellular Vesicles as Drug Carriers for Enzyme Replacement Therapy to Treat CLN2 Batten Disease: Optimization of Drug Administration Routes
    Matthew J. Haney, Yuling Zhao, Yeon S. Jin, Elena V. Batrakova
    Cells 2020 9 5
  • Expanding the Neuroimaging Phenotype of Neuronal Ceroid Lipofuscinoses
    A. Biswas, P. Krishnan, A. Amirabadi, S. Blaser, S. Mercimek-Andrews, M. Shroff
    American Journal of Neuroradiology 2020 41 10
  • Computed tomography provides enhanced techniques for longitudinal monitoring of progressive intracranial volume loss associated with regional neurodegeneration in ovine neuronal ceroid lipofuscinoses
    Katharina N. Russell, Nadia L. Mitchell, Nigel G. Anderson, Craig R. Bunt, Martin P. Wellby, Tracy R. Melzer, Graham K. Barrell, David N. Palmer
    Brain and Behavior 2018 8 9

More in this TOC Section

  • CHARGE fetal MRI clival cleft
  • fetal brain development of 10 weeks gestation
  • Neuroimaging Delineation and Progression of SLSMD
Show more Pediatric Neuroimaging

Similar Articles

Advertisement

Indexed Content

  • Current Issue
  • Accepted Manuscripts
  • Article Preview
  • Past Issues
  • Editorials
  • Editor's Choice
  • Fellows' Journal Club
  • Letters to the Editor
  • Video Articles

Cases

  • Case Collection
  • Archive - Case of the Week
  • Archive - Case of the Month
  • Archive - Classic Case

More from AJNR

  • Trainee Corner
  • Imaging Protocols
  • MRI Safety Corner
  • Book Reviews

Multimedia

  • AJNR Podcasts
  • AJNR Scantastics

Resources

  • Turnaround Time
  • Submit a Manuscript
  • Submit a Video Article
  • Submit an eLetter to the Editor/Response
  • Manuscript Submission Guidelines
  • Statistical Tips
  • Fast Publishing of Accepted Manuscripts
  • Graphical Abstract Preparation
  • Imaging Protocol Submission
  • Evidence-Based Medicine Level Guide
  • Publishing Checklists
  • Author Policies
  • Become a Reviewer/Academy of Reviewers
  • News and Updates

About Us

  • About AJNR
  • Editorial Board
  • Editorial Board Alumni
  • Alerts
  • Permissions
  • Not an AJNR Subscriber? Join Now
  • Advertise with Us
  • Librarian Resources
  • Feedback
  • Terms and Conditions
  • AJNR Editorial Board Alumni

American Society of Neuroradiology

  • Not an ASNR Member? Join Now

© 2025 by the American Society of Neuroradiology All rights, including for text and data mining, AI training, and similar technologies, are reserved.
Print ISSN: 0195-6108 Online ISSN: 1936-959X

Powered by HighWire