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Select item 105233141.

Treatment of non-epileptic episodes of anxious, fearful behavior in adolescent juvenile neuronal ceroid lipofuscinosis (CLN3 disease)

John R. Ostergaard

Front Neurol. 2023; 14: 1216861. Published online 2023 Sep 12. doi: 10.3389/fneur.2023.1216861

PMCID:: PMC10523314

Abstract Article PDF–977K

Select item 104621912.

Assessing the integrity of auditory sensory memory processing in CLN3 disease (Juvenile Neuronal Ceroid Lipofuscinosis (Batten disease)): An auditory evoked potential study of the duration-evoked mismatch negativity (MMN)

Tufikameni Brima, Edward G. Freedman, Kevin D. Prinsloo, Erika F. Augustine, Heather R. Adams, Kuan Hong Wang, Jonathan W. Mink, Luke H. Shaw, Emma P. Mantel, John J. Foxe

Version 1. Res Sq. Preprint. 2023 Aug 17. doi: 10.21203/rs.3.rs-3203894/v1

Published in:: J Neurodev Disord. 2024; 16: 3.

PMCID:: PMC10462191

Abstract Article PDF–1.4M

Select item 101263973.

Etiology of anxious and fearful behavior in juvenile neuronal ceroid lipofuscinosis (CLN3 disease)

John R. Ostergaard

Front Psychiatry. 2023; 14: 1059082. Published online 2023 Apr 11. doi: 10.3389/fpsyt.2023.1059082

PMCID:: PMC10126397

Abstract Article PDF–187K

Select item 89600594.

Pacemaker Implantation in Juvenile Neuronal Ceroid Lipofuscinosis (CLN3)–A Long-Term Follow-Up Study

Mette Møller Handrup, Henning Mølgaard, Brian N. Andersen, John R. Ostergaard

Front Neurol. 2022; 13: 846240. Published online 2022 Mar 10. doi: 10.3389/fneur.2022.846240

PMCID:: PMC8960059

Abstract Article PDF–2.5M

Select item 86401395.

A Novel CLN6 Variant Associated With Juvenile Neuronal Ceroid Lipofuscinosis in Patients With Absence of Visual Loss as a Presenting Feature

Paschalis Nicolaou, George A. Tanteles, Christina Votsi, Eleni Zamba-Papanicolaou, Savvas S. Papacostas, Kyproula Christodoulou, Yiolanda-Panayiota Christou

Front Genet. 2021; 12: 746101. Published online 2021 Nov 19. doi: 10.3389/fgene.2021.746101

PMCID:: PMC8640139

Abstract Article PDF–1.9M

Select item 79303566.

Paroxysmal sympathetic hyperactivity following status epilepticus in a 22-year-old with Juvenile Neuronal Ceroid Lipofuscinosis: A case report

Molly D. Himmelrich, Jennifer M. Pritchard, Camilo Gutierrez, Tara L.M. Dutta

Epilepsy Behav Rep. 2021; 15: 100427. Published online 2021 Jan 19. doi: 10.1016/j.ebr.2021.100427

PMCID:: PMC7930356

Abstract Article PDF–1.2M

Select item 76554327.

Global and Widespread Local White Matter Abnormalities in Juvenile Neuronal Ceroid Lipofuscinosis

U. Roine, T.J. Roine, A. Hakkarainen, A. Tokola, M.H. Balk, M. Mannerkoski, L.E. Åberg, T. Lönnqvist, T. Autti

AJNR Am J Neuroradiol. 2018 Jul; 39(7): 1349–1354. doi: 10.3174/ajnr.A5687

PMCID:: PMC7655432

Abstract Article PDF–1.3M

Select item 74531058.

Imaging data on characterization of retinal autofluorescent lesions in a mouse model of juvenile neuronal ceroid lipofuscinosis (CLN3 disease)

Qing Jun Wang, Kyung Sik Jung, Kabhilan Mohan, Mark E. Kleinman

Data Brief. 2020 Oct; 32: 106076. Published online 2020 Jul 25. doi: 10.1016/j.dib.2020.106076

PMCID:: PMC7453105

Abstract Article PDF–2.6M

Select item 72334139.

Astrocytes in Juvenile Neuronal Ceroid Lipofuscinosis (CLN3) display metabolic and calcium signaling abnormalities

Megan E. Bosch, Tammy Kielian

J Neurochem. Author manuscript; available in PMC 2020 May 18.

Published in final edited form as: J Neurochem. 2019 Mar; 148(5): 612–624. Published online 2018 Aug 16. doi: 10.1111/jnc.14545

PMCID:: PMC7233413

Abstract Article PDF–954K

Select item 697534110.

Topological Alterations of the Structural Brain Connectivity Network in Children with Juvenile Neuronal Ceroid Lipofuscinosis

T. Roine, U. Roine, A. Tokola, M.H. Balk, M. Mannerkoski, L. Åberg, T. Lönnqvist, T. Autti

AJNR Am J Neuroradiol. 2019 Dec; 40(12): 2146–2153. doi: 10.3174/ajnr.A6306

PMCID:: PMC6975341

Abstract Article PDF–2.1M

Select item 693781211.

Synapse alterations precede neuronal damage and storage pathology in a human cerebral organoid model of CLN3-juvenile neuronal ceroid lipofuscinosis

Gemma Gomez-Giro, Jonathan Arias-Fuenzalida, Javier Jarazo, Dagmar Zeuschner, Muhammad Ali, Nina Possemis, Silvia Bolognin, Rashi Halder, Christian Jäger, Willemijn F. E. Kuper, Peter M. van Hasselt, Holm Zaehres, Antonio del Sol, Herman van der Putten, Hans R. Schöler, Jens C. Schwamborn

Acta Neuropathol Commun. 2019; 7: 222. Published online 2019 Dec 30. doi: 10.1186/s40478-019-0871-7

PMCID:: PMC6937812

Abstract Article PDF–4.4M

Select item 632301212.

Short-Term Administration of Mycophenolate Is Well-Tolerated in CLN3 Disease (Juvenile Neuronal Ceroid Lipofuscinosis)

Erika F. Augustine, Christopher A. Beck, Heather R. Adams, Sara Defendorf, Amy Vierhile, Derek Timm, Jill M. Weimer, Jonathan W. Mink, Frederick J. Marshall

JIMD Rep. 2019; 43: 117–124. Published online 2018 Jun 20. doi: 10.1007/8904_2018_113

PMCID:: PMC6323012

Abstract Article PDF–224K

Select item 617699913.

Immunomodulation with minocycline rescues retinal degeneration in juvenile neuronal ceroid lipofuscinosis mice highly susceptible to light damage

Katharina Dannhausen, Christoph Möhle, Thomas Langmann

Dis Model Mech. 2018 Sep 1; 11(9): dmm033597. Published online 2018 Sep 5. doi: 10.1242/dmm.033597

PMCID:: PMC6176999

Abstract Article

Select item 605309314.

Juvenile neuronal ceroid lipofuscinosis (Batten disease): current insights

John R Ostergaard

Degener Neurol Neuromuscul Dis. 2016; 6: 73–83. Published online 2016 Aug 1. doi: 10.2147/DNND.S111967

PMCID:: PMC6053093

Abstract Article PDF–197K

Select item 564590915.

Glial cells are functionally impaired in juvenile neuronal ceroid lipofuscinosis and detrimental to neurons

Lotta Parviainen, Sybille Dihanich, Greg W. Anderson, Andrew M. Wong, Helen R. Brooks, Rosella Abeti, Payam Rezaie, Giovanna Lalli, Simon Pope, Simon J. Heales, Hannah M. Mitchison, Brenda P. Williams, Jonathan D. Cooper

Acta Neuropathol Commun. 2017; 5: 74. Published online 2017 Oct 17. doi: 10.1186/s40478-017-0476-y

PMCID:: PMC5645909

Abstract Article PDF–6.5M

Select item 556996916.

Aberrant adhesion impacts early development in a Dictyostelium model for juvenile neuronal ceroid lipofuscinosis

Robert J. Huber, Michael A. Myre, Susan L. Cotman

Cell Adh Migr. 2017; 11(4): 399–418. Published online 2016 Sep 26. doi: 10.1080/19336918.2016.1236179

PMCID:: PMC5569969

Abstract Article PDF–1.5M

Select item 489081917.

OPTICAL COHERENCE TOMOGRAPHY IN JUVENILE NEURONAL CEROID LIPOFUSCINOSIS

Michael S. Hansen, Marianne N. Hove, Hanne Jensen, Michael Larsen

Retin Cases Brief Rep. 2016 Spring; 10(2): 137–139. Published online 2016 Apr 4. doi: 10.1097/ICB.0000000000000200

PMCID:: PMC4890819

Abstract Article PDF–376K

Select item 474944318.

Remote Assessment of Cognitive Function in Juvenile Neuronal Ceroid Lipofuscinosis (Batten disease) – a Pilot Study of Feasibility and Reliability

Shayne N. Ragbeer, Erika F. Augustine, Jonathan W. Mink, Alyssa R. Thatcher, Amy E. Vierhile, Heather R. Adams

J Child Neurol. Author manuscript; available in PMC 2017 Mar 1.

Published in final edited form as: J Child Neurol. 2016 Mar; 31(4): 481–487. Published online 2015 Sep 2. doi: 10.1177/0883073815600863

PMCID:: PMC4749443

Abstract Article PDF–160K

Select item 461025219.

Standardized assessment of seizures in patients with juvenile neuronal ceroid lipofuscinosis

Erika F Augustine, Heather R Adams, Christopher A Beck, Amy Vierhile, Jennifer Kwon, Paul G Rothberg, Frederick Marshall, Robert Block, James Dolan, Jonathan W Mink

Dev Med Child Neurol. Author manuscript; available in PMC 2016 Apr 1.

Published in final edited form as: Dev Med Child Neurol. 2015 Apr; 57(4): 366–371. Published online 2014 Nov 11. doi: 10.1111/dmcn.12634