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Biomarkers and diagnostic guidelines for sporadic Creutzfeldt-Jakob disease.

Peter Hermann, Brian Appleby, Jean-Philippe Brandel, Byron Caughey, Steven Collins, Michael D Geschwind, Alison Green, Stephane Haïk, Gabor G Kovacs, Anna Ladogana, Franc Llorens, Simon Mead, Noriyuki Nishida, Suvankar Pal, Piero Parchi, Maurizio Pocchiari, Katsuya Satoh, Gianluigi Zanusso, Inga Zerr,

Sporadic Creutzfeldt-Jakob disease is a fatal neurodegenerative disease caused by misfolded prion proteins (PrP<sup>Sc</sup>). Effective therapeutics are currently not available and accurate diagnosis can be challenging. Clinical diagnostic criteria use a combination of characteristic neuropsychiatric symptoms, CSF proteins 14-3-3, MRI, and EEG. Supportive biomarkers, such as high CSF total tau, ... Read more >>

Lancet Neurol (The Lancet. Neurology)
[2021, 20(3):235-246]

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Subtype Diagnosis of Sporadic Creutzfeldt-Jakob Disease with Diffusion Magnetic Resonance Imaging.

Alberto Bizzi, Riccardo Pascuzzo, Janis Blevins, Marco E M Moscatelli, Marina Grisoli, Raffaele Lodi, Fabio M Doniselli, Gianmarco Castelli, Mark L Cohen, Aymeric Stamm, Lawrence B Schonberger, Brian S Appleby, Pierluigi Gambetti,

<h4>Objective</h4>Sporadic Creutzfeldt-Jakob disease (sCJD) comprises several subtypes as defined by genetic and prion protein characteristics, which are associated with distinct clinical and pathological phenotypes. To date, no clinical test can reliably diagnose the subtype. We established two procedures for the antemortem diagnosis of sCJD subtype using diffusion magnetic resonance imaging ... Read more >>

Ann Neurol (Annals of neurology)
[2021, 89(3):560-572]

Cited: 1 time

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Skin RT-QuIC Assays are More Sensitive than CSF RT-QuIC in Prion Detection for Chinese Probable Sporadic Creutzfeldt-Jakob Disease

Kang Xiao, Xue-Hua Yang, Wei Zhou, Cao Chen, Brian Appleby, Wen-Quan Zou, XiaoPing Dong, Qi Shi,

<h4>Background: </h4> The definite diagnosis of human sporadic Creutzfeldt-Jakob disease (sCJD) largely depends on postmortem neuropathology and PrP Sc detection in the brain. The development of prion RT-QuIC of cerebrospinal fluid (CSF) samples makes it possible for premortem diagnosis for sCJD. However the diagnostic potential of RT-QuIC of skin specimen ... Read more >>

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Identification of novel risk loci and causal insights for sporadic Creutzfeldt-Jakob disease: a genome-wide association study.

Emma Jones, Holger Hummerich, Emmanuelle Viré, James Uphill, Athanasios Dimitriadis, Helen Speedy, Tracy Campbell, Penny Norsworthy, Liam Quinn, Jerome Whitfield, Jacqueline Linehan, Zane Jaunmuktane, Sebastian Brandner, Parmjit Jat, Akin Nihat, Tze How Mok, Parvin Ahmed, Steven Collins, Christiane Stehmann, Shannon Sarros, Gabor G Kovacs, Michael D Geschwind, Aili Golubjatnikov, Karl Frontzek, Herbert Budka, Adriano Aguzzi, Hata Karamujić-Čomić, Sven J van der Lee, Carla A Ibrahim-Verbaas, Cornelia M van Duijn, Beata Sikorska, Ewa Golanska, Pawel P Liberski, Miguel Calero, Olga Calero, Pascual Sanchez-Juan, Antonio Salas, Federico Martinón-Torres, Elodie Bouaziz-Amar, Stéphane Haïk, Jean-Louis Laplanche, Jean-Phillipe Brandel, Phillipe Amouyel, Jean-Charles Lambert, Piero Parchi, Anna Bartoletti-Stella, Sabina Capellari, Anna Poleggi, Anna Ladogana, Maurizio Pocchiari, Serena Aneli, Giuseppe Matullo, Richard Knight, Saima Zafar, Inga Zerr, Stephanie Booth, Michael B Coulthart, Gerard H Jansen, Katie Glisic, Janis Blevins, Pierluigi Gambetti, Jiri Safar, Brian Appleby, John Collinge, Simon Mead,

<h4>Background</h4>Human prion diseases are rare and usually rapidly fatal neurodegenerative disorders, the most common being sporadic Creutzfeldt-Jakob disease (sCJD). Variants in the PRNP gene that encodes prion protein are strong risk factors for sCJD but, although the condition has similar heritability to other neurodegenerative disorders, no other genetic risk loci ... Read more >>

Lancet Neurol (The Lancet. Neurology)
[2020, 19(10):840-848]

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Evaluation of a New Criterion for Detecting Prion Disease With Diffusion Magnetic Resonance Imaging.

Alberto Bizzi, Riccardo Pascuzzo, Janis Blevins, Marina Grisoli, Raffaele Lodi, Marco E M Moscatelli, Gianmarco Castelli, Mark L Cohen, Lawrence B Schonberger, Aaron Foutz, Jiri G Safar, Brian S Appleby, Pierluigi Gambetti,

<h4>Importance</h4>Early diagnosis is a requirement for future treatment of prion diseases. Magnetic resonance imaging (MRI) with diffusion-weighted images and improved real-time quaking-induced conversion (RT-QuIC) in cerebrospinal fluid (CSF) have emerged as reliable tests.<h4>Objectives</h4>To assess the sensitivity and specificity of diffusion MRI for the diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD) with ... Read more >>

JAMA Neurol (JAMA neurology)
[2020, 77(9):1141-1149]

Cited: 2 times

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Human Prion Disease Surveillance in Washington State, 2006-2017.

Liliana Sánchez-González, Ryan A Maddox, Larissa C Lewis, Janis E Blevins, Elizabeth J Harker, Brian S Appleby, Marissa K Person, Lawrence B Schonberger, Ermias D Belay, Chas DeBolt, Kathryn H Lofy,

<h4>Importance</h4>Human prion disease surveillance is critical to detect possible cases of variant Creutzfeldt-Jakob disease and other acquired forms of prion disease in the United States. Results are presented here that describe 12 years of surveillance in Washington, the only US state that has reported the presence of classic bovine spongiform ... Read more >>

JAMA Netw Open (JAMA network open)
[2020, 3(10):e2020690]

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Diagnosis of prion diseases by RT-QuIC results in improved surveillance.

Daniel D Rhoads, Aleksandra Wrona, Aaron Foutz, Janis Blevins, Kathleen Glisic, Marissa Person, Ryan A Maddox, Ermias D Belay, Lawrence B Schonberger, Curtis Tatsuoka, Mark L Cohen, Brian S Appleby,

<h4>Objective</h4>To present the National Prion Disease Pathology Surveillance Center's (NPDPSC's) experience using CSF real-time quaking-induced conversion (RT-QuIC) as a diagnostic test, to examine factors associated with false-negative RT-QuIC results, and to investigate the impact of RT-QuICs on prion disease surveillance.<h4>Methods</h4>Between May 2015 and April 2018, the NPDPSC received 10,498 CSF ... Read more >>

Neurology (Neurology)
[2020, 95(8):e1017-e1026]

Cited: 2 times

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Case Report: Histopathology and Prion Protein Molecular Properties in Inherited Prion Disease With a De Novo Seven-Octapeptide Repeat Insertion.

Ignazio Cali, Laura Cracco, Dario Saracino, Rossana Occhipinti, Cinzia Coppola, Brian Stephen Appleby, Gianfranco Puoti,

The insertion of additional 168 base pair containing seven octapeptide repeats in the prion protein (PrP) gene region spanning residues 51-91 is associated with inherited prion disease. In 2008, we reported the clinical features of a novel <i>de novo</i> seven-octapeptide repeat insertion (7-OPRI) mutation coupled with codon 129 methionine (M) ... Read more >>

Front Cell Neurosci (Frontiers in cellular neuroscience)
[2020, 14:150]

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Prion propagation estimated from brain diffusion MRI is subtype dependent in sporadic Creutzfeldt-Jakob disease.

Riccardo Pascuzzo, Neil P Oxtoby, Alexandra L Young, Janis Blevins, Gianmarco Castelli, Sara Garbarino, Mark L Cohen, Lawrence B Schonberger, Pierluigi Gambetti, Brian S Appleby, Daniel C Alexander, Alberto Bizzi,

Sporadic Creutzfeldt-Jakob disease (sCJD) is a transmissible brain proteinopathy. Five main clinicopathological subtypes (sCJD-MM(V)1, -MM(V)2C, -MV2K, -VV1, and -VV2) are currently distinguished. Histopathological evidence suggests that the localisation of prion aggregates and spongiform lesions varies among subtypes. Establishing whether there is an initial site with detectable imaging abnormalities (epicentre) and ... Read more >>

Acta Neuropathol (Acta neuropathologica)
[2020, 140(2):169-181]

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Genome-wide association study identifies risk variants for sporadic Creutzfeldt-Jakob disease in STX6 and GAL3ST1

Emma Jones, Holger Hummerich, Emmanuelle Viré, James Uphill, Athanasios Dimitriadis, Helen Speedy, Tracy Campbell, Penny Norsworthy, Liam Quinn, Jerome Whitfield, Jacqueline Linehan, Zane Jaunmuktane, Sebastian Brandner, Parmjit Jat, Akin Nihat, Tze How Mok, Parvin Ahmed, Steven Collins, Christiane Stehmann, Shannon Sarros, Gabor Kovacs, Michael Geschwind, Aili Golubjatnikov, Karl Fronztek, Herbert Budka, Adriano Aguzzi, Hata Karamujić-Čomić, Sven van der Lee, Carla Ibrahim-Verbaas, Cornelia Van Duijn, Beata Sikorska, Ewa Golanska, Pawel Liberski, Miguel Calero, Olga Calero, Pascual Sanchez Juan, Antonio Salas, Federico Martinón-Torres, Elodie Bouaziz-Amar, Stephane Haik, Jean-Louis Laplanche, Jean-Phillipe Brandel, Phillipe Amouyel, Jean-Charles Lambert, Piero Parchi, Anna Bartoletti-Stella, Sabina Capellari, Anna Poleggi, Anna Ladogana, Maurizio Pocchiari, Serena Aneli, Giuseppe Matullo, Richard Knight, Saima Zafar, Inga Zerr, Stephanie Booth, Michael Coulthart, Gerard Jansen, Katie Glisic, Janis Blevins, Pierluigi Gambetti, Jiri Safar, Brian Appleby, John Collinge, Simon Mead,

Mammalian prions are lethal pathogens composed of fibrillar assemblies of misfolded prion protein. Human prion diseases are rare and usually rapidly fatal neurodegenerative disorders, the most common being sporadic Creutzfeldt-Jakob disease (sCJD). Variants in the gene that encodes prion protein ( PRNP ) are strong risk factors for sCJD, but ... Read more >>

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Revised Self-Monitoring Scale: A potential endpoint for frontotemporal dementia clinical trials.

Gianina Toller, Kamalini Ranasinghe, Yann Cobigo, Adam Staffaroni, Brian Appleby, Danielle Brushaber, Giovanni Coppola, Bradford Dickerson, Kimiko Domoto-Reilly, Julie Fields, Jamie Fong, Leah Forsberg, Nupur Ghoshal, Neill Graff-Radford, Murray Grossman, Hilary Heuer, Gink-Yuek Hsiung, Edward Huey, David Irwin, Kejal Kantarci, Daniel Kaufer, Diana Kerwin, David Knopman, John Kornak, Joel Kramer, Irene Litvan, Ian Mackenzie, Mario Mendez, Bruce Miller, Rosa Rademakers, Eliana Ramos, Katya Rascovsky, Erik Roberson, Jeremy Syrjanen, Carmela Tartaglia, Sandra Weintraub, Brad Boeve, Adam Boxer, Howard Rosen, Katherine Rankin, ,

<h4>Objective</h4>To investigate whether the Revised Self-Monitoring Scale (RSMS), an informant measure of socioemotional sensitivity, is a potential clinical endpoint for treatment trials for patients with behavioral variant frontotemporal dementia (bvFTD).<h4>Methods</h4>We investigated whether RSMS informant ratings reflected disease severity in 475 participants (71 bvFTD mutation+, 154 bvFTD mutation-, 12 behavioral mild ... Read more >>

Neurology (Neurology)
[2020, 94(22):e2384-e2395]

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Diagnostic value of plasma phosphorylated tau181 in Alzheimer's disease and frontotemporal lobar degeneration.

Elisabeth H Thijssen, Renaud La Joie, Amy Wolf, Amelia Strom, Ping Wang, Leonardo Iaccarino, Viktoriya Bourakova, Yann Cobigo, Hilary Heuer, Salvatore Spina, Lawren VandeVrede, Xiyun Chai, Nicholas K Proctor, David C Airey, Sergey Shcherbinin, Cynthia Duggan Evans, John R Sims, Henrik Zetterberg, Kaj Blennow, Anna M Karydas, Charlotte E Teunissen, Joel H Kramer, Lea T Grinberg, William W Seeley, Howie Rosen, Bradley F Boeve, Bruce L Miller, Gil D Rabinovici, Jeffrey L Dage, Julio C Rojas, Adam L Boxer, ,

With the potential development of new disease-modifying Alzheimer's disease (AD) therapies, simple, widely available screening tests are needed to identify which individuals, who are experiencing symptoms of cognitive or behavioral decline, should be further evaluated for initiation of treatment. A blood-based test for AD would be a less invasive and ... Read more >>

Nat Med (Nature medicine)
[2020, 26(3):387-397]

Cited: 40 times

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Co-existence of PrPD types 1 and 2 in sporadic Creutzfeldt-Jakob disease of the VV subgroup: phenotypic and prion protein characteristics.

Ignazio Cali, Gianfranco Puoti, Jason Smucny, Paul Michael Curtiss, Laura Cracco, Tetsuyuki Kitamoto, Rossana Occhipinti, Mark Lloyd Cohen, Brian Stephen Appleby, Pierluigi Gambetti,

We report a detailed study of a cohort of sporadic Creutzfeldt-Jakob disease (sCJD) VV1-2 type-mixed cases (valine homozygosity at codon 129 of the prion protein, PrP, gene harboring disease-related PrP, PrP<sup>D</sup>, types 1 and 2). Overall, sCJDVV1-2 subjects showed mixed clinical and histopathological features, which often correlated with the relative ... Read more >>

Sci Rep (Scientific reports)
[2020, 10(1):1503]

Cited: 3 times

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Clinical and volumetric changes with increasing functional impairment in familial frontotemporal lobar degeneration.

Nicholas T Olney, Elise Ong, Sheng-Yang M Goh, Lynn Bajorek, Reilly Dever, Adam M Staffaroni, Yann Cobigo, Meredith Bock, Kevin Chiang, Peter Ljubenkov, John Kornak, Hilary W Heuer, Ping Wang, Katya Rascovsky, Amelia Wolf, Brian Appleby, Jessica Bove, Yvette Bordelon, Patrick Brannelly, Danielle Brushaber, Christine Caso, Giovanni Coppola, Bradford C Dickerson, Susan Dickinson, Kimiko Domoto-Reilly, Kelly Faber, Jessica Ferrall, Julie Fields, Ann Fishman, Jamie Fong, Tatiana Foroud, Leah K Forsberg, Debra J Gearhart, Behnaz Ghazanfari, Nupur Ghoshal, Jill Goldman, Jonathan Graff-Radford, Neill R Graff-Radford, Ian Grant, Murray Grossman, Dana Haley, Gingyuek Hsiung, Edward D Huey, David J Irwin, David T Jones, Kejal Kantarci, Anna M Karydas, Daniel Kaufer, Diana Kerwin, David S Knopman, Joel H Kramer, Ruth Kraft, Walter Kremers, Walter Kukull, Maria I Lapid, Irene Litvan, Ian R Mackenzie, Miranda Maldonado, Masood Manoochehri, Scott M McGinnis, Emily C McKinley, Mario F Mendez, Bruce L Miller, Chiadi Onyike, Alex Pantelyat, Rodney Pearlman, Len Petrucelli, Madeleine Potter, Rosa Rademakers, Eliana M Ramos, Katherine P Rankin, Erik D Roberson, Emily Rogalski, Pheth Sengdy, Leslie M Shaw, Jeremy Syrjanen, M Carmela Tartaglia, Nadine Tatton, Joanne Taylor, Arthur Toga, John Q Trojanowski, Sandra Weintraub, Bonnie Wong, Zbigniew Wszolek, Adam L Boxer, Brad F Boeve, Howard J Rosen, ,

<h4>Introduction</h4>The Advancing Research and Treatment in Frontotemporal Lobar Degeneration and Longitudinal Evaluation of Familial Frontotemporal Dementia Subjects longitudinal studies were designed to describe the natural history of familial-frontotemporal lobar degeneration due to autosomal dominant mutations.<h4>Methods</h4>We examined cognitive performance, behavioral ratings, and brain volumes from the first time point in 320 ... Read more >>

Alzheimers Dement (Alzheimer's & dementia : the journal of the Alzheimer's Association)
[2020, 16(1):49-59]

Cited: 5 times

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Assessment of executive function declines in presymptomatic and mildly symptomatic familial frontotemporal dementia: NIH-EXAMINER as a potential clinical trial endpoint.

Adam M Staffaroni, Lynn Bajorek, Kaitlin B Casaletto, Yann Cobigo, Sheng-Yang M Goh, Amy Wolf, Hilary W Heuer, Fanny M Elahi, Peter A Ljubenkov, Reilly Dever, John Kornak, Brian Appleby, Jessica Bove, Yvette Bordelon, Patrick Brannelly, Danielle Brushaber, Christina Caso, Giovanni Coppola, Christina Dheel, Bradford C Dickerson, Susan Dickinson, Sophia Dominguez, Kimiko Domoto-Reilly, Kelly Faber, Jessica Ferrall, Julie A Fields, Ann Fishman, Jamie Fong, Tatiana Foroud, Leah K Forsberg, Ralitza Gavrilova, Debra Gearhart, Behnaz Ghazanfari, Nupur Ghoshal, Jill Goldman, Jonathan Graff-Radford, Neill Graff-Radford, Ian Grant, Murray Grossman, Dana Haley, Ging-Yuek Hsiung, Edward D Huey, David J Irwin, David T Jones, Lynne Jones, Kejal Kantarci, Anna Karydas, Daniel I Kaufer, Diana R Kerwin, David S Knopman, Ruth Kraft, Walter K Kremers, Walter A Kukull, Irene Litvan, Diane Lucente, Codrin Lungu, Ian R Mackenzie, Miranda Maldonado, Masood Manoochehri, Scott M McGinnis, Emily McKinley, Mario F Mendez, Bruce L Miller, Namita Multani, Chiadi Onyike, Jaya Padmanabhan, Alex Pantelyat, Rodney Pearlman, Len Petrucelli, Madeline Potter, Rosa Rademakers, Eliana Marisa Ramos, Katherine P Rankin, Katya Rascovsky, Erik D Roberson, Emily Rogalski, Pheth Sengdy, Leslie M Shaw, Jeremy Syrjanen, M Carmela Tartaglia, Nadine Tatton, Joanne Taylor, Arthur Toga, John Q Trojanowski, Sandra Weintraub, Ping Wang, Bonnie Wong, Zbigniew Wszolek, Adam L Boxer, Brad F Boeve, Joel H Kramer, Howard J Rosen, ,

<h4>Introduction</h4>Identifying clinical measures that track disease in the earliest stages of frontotemporal lobar degeneration (FTLD) is important for clinical trials. Familial FTLD provides a unique paradigm to study early FTLD. Executive dysfunction is a clinically relevant hallmark of FTLD and may be a marker of disease progression.<h4>Methods</h4>Ninety-three mutation carriers with ... Read more >>

Alzheimers Dement (Alzheimer's & dementia : the journal of the Alzheimer's Association)
[2020, 16(1):11-21]

Cited: 2 times

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Genetic screening of a large series of North American sporadic and familial frontotemporal dementia cases.

Eliana Marisa Ramos, Deepika Reddy Dokuru, Victoria Van Berlo, Kevin Wojta, Qing Wang, Alden Y Huang, Sandeep Deverasetty, Yue Qin, Marka van Blitterswijk, Jazmyne Jackson, Brian Appleby, Yvette Bordelon, Patrick Brannelly, Danielle E Brushaber, Bradford Dickerson, Susan Dickinson, Kimiko Domoto-Reilly, Kelley Faber, Julie Fields, Jamie Fong, Tatiana Foroud, Leah K Forsberg, Ralitza Gavrilova, Nupur Ghoshal, Jill Goldman, Jonathan Graff-Radford, Neill Graff-Radford, Ian Grant, Murray Grossman, Hilary W Heuer, Ging-Yuek R Hsiung, Edward Huey, David Irwin, Kejal Kantarci, Anna Karydas, Daniel Kaufer, Diana Kerwin, David Knopman, John Kornak, Joel H Kramer, Walter Kremers, Walter Kukull, Irene Litvan, Peter Ljubenkov, Codrin Lungu, Ian Mackenzie, Mario F Mendez, Bruce L Miller, Chiadi Onyike, Alexander Pantelyat, Rodney Pearlman, Len Petrucelli, Madeline Potter, Katherine P Rankin, Katya Rascovsky, Erik D Roberson, Emily Rogalski, Leslie Shaw, Jeremy Syrjanen, Maria Carmela Tartaglia, Nadine Tatton, Joanne Taylor, Arthur Toga, John Q Trojanowski, Sandra Weintraub, Bonnie Wong, Zbigniew Wszolek, Rosa Rademakers, Brad F Boeve, Howard J Rosen, Adam L Boxer, , Giovanni Coppola,

<h4>Introduction</h4>The Advancing Research and Treatment for Frontotemporal Lobar Degeneration (ARTFL) and Longitudinal Evaluation of Familial Frontotemporal Dementia Subjects (LEFFTDS) consortia are two closely connected studies, involving multiple North American centers that evaluate both sporadic and familial frontotemporal dementia (FTD) participants and study longitudinal changes.<h4>Methods</h4>We screened the major dementia-associated genes in ... Read more >>

Alzheimers Dement (Alzheimer's & dementia : the journal of the Alzheimer's Association)
[2020, 16(1):118-130]

Cited: 5 times

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Utility of the global CDR® plus NACC FTLD rating and development of scoring rules: Data from the ARTFL/LEFFTDS Consortium.

Toji Miyagawa, Danielle Brushaber, Jeremy Syrjanen, Walter Kremers, Julie Fields, Leah K Forsberg, Hilary W Heuer, David Knopman, John Kornak, Adam Boxer, Howard J Rosen, Bradley F Boeve, Brian Appleby, Yvette Bordelon, Jessica Bove, Patrick Brannelly, Christina Caso, Giovanni Coppola, Reilly Dever, Christina Dheel, Bradford Dickerson, Susan Dickinson, Sophia Dominguez, Kimiko Domoto-Reilly, Kelley Faber, Jessica Ferrell, Ann Fishman, Jamie Fong, Tatiana Foroud, Ralitza Gavrilova, Debra Gearhart, Behnaz Ghazanfari, Nupur Ghoshal, Jill S Goldman, Jonathan Graff-Radford, Neill Graff-Radford, Ian Grant, Murray Grossman, Dana Haley, Robin Hsiung, Edward Huey, David Irwin, David Jones, Lynne Jones, Kejal Kantarci, Anna Karydas, Daniel Kaufer, Diana Kerwin, Ruth Kraft, Joel Kramer, Walter Kukull, Irene Litvan, Diane Lucente, Codrin Lungu, Ian Mackenzie, Miranda Maldonado, Masood Manoochehri, Scott McGinnis, Emily McKinley, Mario F Mendez, Bruce Miller, Namita Multani, Chiadi Onyike, Jaya Padmanabhan, Alexander Pantelyat, Rodney Pearlman, Leonard Petrucelli, Madeline Potter, Rosa Rademakers, Eliana M Ramos, Kate Rankin, Katya Rascovsky, Erik D Roberson, Emily Rogalski, Pheth Sengdy, Leslie Shaw, Maria C Tartaglia, Nadine Tatton, Joanne Taylor, Arthur Toga, John Q Trojanowski, Ping Wang, Sandra Weintraub, Bonnie Wong, Zbigniew Wszolek,

<h4>Introduction</h4>We created global rating scoring rules for the CDR<sup>®</sup> plus NACC FTLD to detect and track early frontotemporal lobar degeneration (FTLD) and to conduct clinical trials in FTLD.<h4>Methods</h4>The CDR plus NACC FTLD rating was applied to 970 sporadic and familial participants from the baseline visit of Advancing Research and Treatment ... Read more >>

Alzheimers Dement (Alzheimer's & dementia : the journal of the Alzheimer's Association)
[2020, 16(1):106-117]

Cited: 3 times

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Individualized atrophy scores predict dementia onset in familial frontotemporal lobar degeneration.

Adam M Staffaroni, Yann Cobigo, Sheng-Yang M Goh, John Kornak, Lynn Bajorek, Kevin Chiang, Brian Appleby, Jessica Bove, Yvette Bordelon, Patrick Brannelly, Danielle Brushaber, Christina Caso, Giovanni Coppola, Reilly Dever, Christina Dheel, Bradford C Dickerson, Susan Dickinson, Sophia Dominguez, Kimiko Domoto-Reilly, Kelly Faber, Jessica Ferrall, Julie A Fields, Ann Fishman, Jamie Fong, Tatiana Foroud, Leah K Forsberg, Ralitza Gavrilova, Debra Gearhart, Behnaz Ghazanfari, Nupur Ghoshal, Jill Goldman, Jonathan Graff-Radford, Neill Graff-Radford, Ian Grant, Murray Grossman, Dana Haley, Hilary W Heuer, Ging-Yuek Hsiung, Edward D Huey, David J Irwin, David T Jones, Lynne Jones, Kejal Kantarci, Anna Karydas, Daniel I Kaufer, Diana R Kerwin, David S Knopman, Ruth Kraft, Joel H Kramer, Walter K Kremers, Walter A Kukull, Irene Litvan, Peter A Ljubenkov, Diane Lucente, Codrin Lungu, Ian R Mackenzie, Miranda Maldonado, Masood Manoochehri, Scott M McGinnis, Emily McKinley, Mario F Mendez, Bruce L Miller, Namita Multani, Chiadi Onyike, Jaya Padmanabhan, Alex Pantelyat, Rodney Pearlman, Len Petrucelli, Madeline Potter, Rosa Rademakers, Eliana Marisa Ramos, Katherine P Rankin, Katya Rascovsky, Erik D Roberson, Emily Rogalski, Pheth Sengdy, Leslie M Shaw, Jeremy Syrjanen, M Carmela Tartaglia, Nadine Tatton, Joanne Taylor, Arthur Toga, John Q Trojanowski, Sandra Weintraub, Ping Wang, Bonnie Wong, Zbigniew Wszolek, Adam L Boxer, Brad F Boeve, Howard J Rosen, ,

<h4>Introduction</h4>Some models of therapy for neurodegenerative diseases envision starting treatment before symptoms develop. Demonstrating that such treatments are effective requires accurate knowledge of when symptoms would have started without treatment. Familial frontotemporal lobar degeneration offers a unique opportunity to develop predictors of symptom onset.<h4>Methods</h4>We created dementia risk scores in 268 ... Read more >>

Alzheimers Dement (Alzheimer's & dementia : the journal of the Alzheimer's Association)
[2020, 16(1):37-48]

Cited: 7 times

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Nonlinear Z-score modeling for improved detection of cognitive abnormality.

John Kornak, Julie Fields, Walter Kremers, Sara Farmer, Hilary W Heuer, Leah Forsberg, Danielle Brushaber, Amy Rindels, Hiroko Dodge, Sandra Weintraub, Lilah Besser, Brian Appleby, Yvette Bordelon, Jessica Bove, Patrick Brannelly, Christina Caso, Giovanni Coppola, Reilly Dever, Christina Dheel, Bradford Dickerson, Susan Dickinson, Sophia Dominguez, Kimiko Domoto-Reilly, Kelley Faber, Jessica Ferrall, Ann Fishman, Jamie Fong, Tatiana Foroud, Ralitza Gavrilova, Deb Gearhart, Behnaz Ghazanfari, Nupur Ghoshal, Jill Goldman, Jonathan Graff-Radford, Neill Graff-Radford, Ian M Grant, Murray Grossman, Dana Haley, John Hsiao, Robin Hsiung, Edward D Huey, David Irwin, David Jones, Lynne Jones, Kejal Kantarci, Anna Karydas, Daniel Kaufer, Diana Kerwin, David Knopman, Ruth Kraft, Joel Kramer, Walter Kukull, Maria Lapid, Irene Litvan, Peter Ljubenkov, Diane Lucente, Codrin Lungu, Ian Mackenzie, Miranda Maldonado, Masood Manoochehri, Scott McGinnis, Emily McKinley, Mario Mendez, Bruce Miller, Namita Multani, Chiadi Onyike, Jaya Padmanabhan, Alexander Pantelyat, Rodney Pearlman, Len Petrucelli, Madeline Potter, Rosa Rademakers, Eliana Marisa Ramos, Katherine Rankin, Katya Rascovsky, Erik D Roberson, Emily Rogalski-Miller, Pheth Sengdy, Les Shaw, Adam M Staffaroni, Margaret Sutherland, Jeremy Syrjanen, Carmela Tartaglia, Nadine Tatton, Joanne Taylor, Arthur Toga, John Trojanowski, Ping Wang, Bonnie Wong, Zbigniew Wszolek, Brad Boeve, Adam Boxer, Howard Rosen, ,

<h4>Introduction</h4>Conventional Z-scores are generated by subtracting the mean and dividing by the standard deviation. More recent methods linearly correct for age, sex, and education, so that these "adjusted" Z-scores better represent whether an individual's cognitive performance is abnormal. Extreme negative Z-scores for individuals relative to this normative distribution are considered ... Read more >>

Alzheimers Dement (Amst) (Alzheimer's & dementia (Amsterdam, Netherlands))
[2019, 11:797-808]

Cited: 1 time

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Prion disease incidence in the United States: 2003-2015.

Ryan A Maddox, Marissa K Person, Janis E Blevins, Joseph Y Abrams, Brian S Appleby, Lawrence B Schonberger, Ermias D Belay,

<h4>Objective</h4>To report the incidence of prion disease in the United States.<h4>Methods</h4>Prion disease decedents were retrospectively identified from the US national multiple cause-of-death data for 2003-2015 and matched with decedents in the National Prion Disease Pathology Surveillance Center (NPDPSC) database through comparison of demographic variables. NPDPSC decedents with neuropathologic or genetic ... Read more >>

Neurology (Neurology)
[2020, 94(2):e153-e157]

Cited: 2 times

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Shortening heparan sulfate chains prolongs survival and reduces parenchymal plaques in prion disease caused by mobile, ADAM10-cleaved prions.

Patricia Aguilar-Calvo, Alejandro M Sevillano, Jaidev Bapat, Katrin Soldau, Daniel R Sandoval, Hermann C Altmeppen, Luise Linsenmeier, Donald P Pizzo, Michael D Geschwind, Henry Sanchez, Brian S Appleby, Mark L Cohen, Jiri G Safar, Steven D Edland, Markus Glatzel, K Peter R Nilsson, Jeffrey D Esko, Christina J Sigurdson,

Cofactors are essential for driving recombinant prion protein into pathogenic conformers. Polyanions promote prion aggregation in vitro, yet the cofactors that modulate prion assembly in vivo remain largely unknown. Here we report that the endogenous glycosaminoglycan, heparan sulfate (HS), impacts prion propagation kinetics and deposition sites in the brain. Exostosin-1 ... Read more >>

Acta Neuropathol (Acta neuropathologica)
[2020, 139(3):527-546]

Cited: 3 times

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Clinical Laboratory Tests Used To Aid in Diagnosis of Human Prion Disease.

Allyson Connor, Han Wang, Brian S Appleby, Daniel D Rhoads,

Prion diseases are a group of rapidly progressive and always fatal neurodegenerative disorders caused by misfolded prion protein in the brain. Although autopsy remains the gold-standard diagnostic tool, antemortem laboratory testing can be performed to aid in the diagnosis of prion disease. This review is meant to help laboratory directors ... Read more >>

J Clin Microbiol (Journal of clinical microbiology)
[2019, 57(10):]

Cited: 6 times

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Correction to: In Vitro Seeding Activity of Glycoform-Deficient Prions from Variably Protease-Sensitive Prionopathy and Familial CJD Associated with PrPV180I Mutation.

Zerui Wang, Jue Yuan, Pingping Shen, Romany Abskharon, Yue Lang, Johnny Dang, Alise Adornato, Ling Xu, Jiafeng Chen, Jiachun Feng, Mohammed Moudjou, Tetsuyuki Kitamoto, Hyoung-Gon Lee, Yong-Sun Kim, Jan Langeveld, Brian Appleby, Jiyan Ma, Qingzhong Kong, Robert B Petersen, Wen-Quan Zou, Li Cui,

The original version of this article unfortunately contained a mistake. The email address Dr. Wen-Quan Zou, one of the corresponding authors should be written as "[email protected]" instead of "[email protected]". ... Read more >>

Mol Neurobiol (Molecular neurobiology)
[2019, 56(8):5470]

Cited: 0 times

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Chronic Wasting Disease in Cervids: Implications for Prion Transmission to Humans and Other Animal Species.

Michael T Osterholm, Cory J Anderson, Mark D Zabel, Joni M Scheftel, Kristine A Moore, Brian S Appleby,

Chronic wasting disease (CWD) is a prion-related transmissible spongiform encephalopathy of cervids, including deer, elk, reindeer, sika deer, and moose. CWD has been confirmed in at least 26 U.S. states, three Canadian provinces, South Korea, Finland, Norway, and Sweden, with a notable increase in the past 5 years. The continued geographic ... Read more >>

mBio (mBio)
[2019, 10(4):]

Cited: 10 times

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Human prion diseases.

Han Wang, Daniel D Rhoads, Brian S Appleby,

<h4>Purpose of review</h4>Prion diseases are rapidly progressive neurodegenerative conditions that can be difficult to diagnose and are transmissible under specific circumstances. The authors will provide background regarding prion disease and focus on diagnostic tools.<h4>Recent findings</h4>Prion disease is caused by misfolded prion protein. The three possible causes of prion disease include ... Read more >>

Curr Opin Infect Dis (Current opinion in infectious diseases)
[2019, 32(3):272-276]

Cited: 1 time

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