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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.)
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D. Sondhi
bGenetic Medicine (D.S., C.H., D.M., S.M.K., R.G.C., D.B.)
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  • ORCID record for D. Sondhi
H.U. Voss
aFrom the Departments of Radiology (J.P.D., H.U.V., L.A.H., D.B.)
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K. Yohay
cPediatrics (K.Y., B.K.)
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C. Hollmann
bGenetic Medicine (D.S., C.H., D.M., S.M.K., R.G.C., D.B.)
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  • ORCID record for C. Hollmann
D. Mancenido
bGenetic Medicine (D.S., C.H., D.M., S.M.K., R.G.C., D.B.)
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S.M. Kaminsky
bGenetic Medicine (D.S., C.H., D.M., S.M.K., R.G.C., D.B.)
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L.A. Heier
aFrom the Departments of Radiology (J.P.D., H.U.V., L.A.H., D.B.)
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K.D. Rudser
eDivision of Biostatistics (K.D.R.), Clinical and Translational Science Institute, University of Minnesota, Minneapolis, Minnesota.
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B. Kosofsky
cPediatrics (K.Y., B.K.)
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B.J. Casey
dPsychiatry (B.J.C.), Weill Cornell Medical College, New York, New York
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R.G. Crystal
bGenetic Medicine (D.S., C.H., D.M., S.M.K., R.G.C., D.B.)
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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.)
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American Journal of Neuroradiology: 37 (6)
American Journal of Neuroradiology
Vol. 37, Issue 6
1 Jun 2016
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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

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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
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  • 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
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  • Clinical challenges and future therapeutic approaches for neuronal ceroid lipofuscinosis
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    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
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  • Photosensitivity is an early marker of neuronal ceroid lipofuscinosis type 2 disease
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    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

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