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2. Subcortical T1-Rho MRI Abnormalities in Juvenile-Onset Huntington’s Disease

Author

Alexander V. Tereshchenko, Jordan L. Schultz, Ansley J. Kunnath, Joel E. Bruss, Eric A. Epping, Vincent A. Magnotta, and Peg C. Nopoulos

Subjects
juvenile-onset Huntington’s disease, T1-Rho, neuroimaging, Neurosciences. Biological psychiatry. Neuropsychiatry, RC321-571
Abstract

Huntington’s disease (HD) is a fatal neurodegenerative disease caused by the expansion of cytosine-adenine-guanine (CAG) repeats in the huntingtin gene. An increased CAG repeat length is associated with an earlier disease onset. About 5% of HD cases occur under the age of 21 years, which are classified as juvenile-onset Huntington’s disease (JOHD). Our study aims to measure subcortical metabolic abnormalities in JOHD participants. T1-Rho (T1ρ) MRI was used to compare brain regions of 13 JOHD participants and 39 controls. Region-of-interest analyses were used to assess differences in quantitative T1ρ relaxation times. We found that the mean relaxation times in the caudate (p < 0.001), putamen (p < 0.001), globus pallidus (p < 0.001), and thalamus (p < 0.001) were increased in JOHD participants compared to controls. Furthermore, increased T1ρ relaxation times in these areas were significantly associated with lower volumes amongst participants in the JOHD group. These findings suggest metabolic abnormalities in brain regions previously shown to degenerate in JOHD. We also analyzed the relationships between mean regional T1ρ relaxation times and Universal Huntington’s Disease Rating Scale (UHDRS) scores. UHDRS was used to evaluate participants’ motor function, cognitive function, behavior, and functional capacity. Mean T1ρ relaxation times in the caudate (p = 0.003), putamen (p = 0.005), globus pallidus (p = 0.009), and thalamus (p = 0.015) were directly proportional to the UHDRS score. This suggests that the T1ρ relaxation time may also predict HD-related motor deficits. Our findings suggest that subcortical metabolic abnormalities drive the unique hypokinetic symptoms in JOHD.

Published
2020
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3. The Association between CAG Repeat Length and Age of Onset of Juvenile-Onset Huntington’s Disease

Author

Jordan L. Schultz, Amelia D. Moser, and Peg C. Nopoulos

Subjects
CAG, juvenile-onset Huntington’s disease, motor onset, Neurosciences. Biological psychiatry. Neuropsychiatry, RC321-571
Abstract

There is a known negative association between cytosine–adenine–guanine (CAG) repeat length and the age of motor onset (AMO) in adult-onset Huntington’s Disease (AOHD). This relationship is less clear in patients with juvenile-onset Huntington’s disease (JOHD), however, given the rarity of this patient population. The aim of this study was to investigate this relationship amongst a relatively large group of patients with JOHD using data from the Kids-JOHD study. Additionally, we analyzed data from the Enroll-HD platform and the Predict-HD study to compare the relationship between CAG repeat length and AMO amongst patients with AOHD to that amongst patients with JOHD using linear regression models. In line with previous reports, the variance in AMO that was predicted by CAG repeat length was 59% (p < 0.0001) in the Predict-HD study and 57% from the Enroll-HD platform (p < 0.0001). However, CAG repeat length predicted 84% of the variance in AMO amongst participants from the Kids-JOHD study (p < 0.0001). These results indicate that there may be a stronger relationship between CAG repeat length and AMO in patients with JOHD as compared to patients with AOHD. These results provide additional information that may help to model disease progression of JOHD, which is beneficial for the planning and implementation of future clinical trials.

Published
2020
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4. Autonomic Changes in Juvenile-Onset Huntington’s Disease

Author

Jordan L. Schultz and Peg C. Nopoulos

Subjects
juvenile-onset Huntington’s Disease, autonomic, neurodegeneration, Neurosciences. Biological psychiatry. Neuropsychiatry, RC321-571
Abstract

Patients with adult-onset Huntington’s Disease (AOHD) have been found to have dysfunction of the autonomic nervous system that is thought to be secondary to neurodegeneration causing dysfunction of the brain–heart axis. However, this relationship has not been investigated in patients with juvenile-onset HD (JOHD). The aim of this study was to compare simple physiologic measures between patients with JOHD (n = 27 participants with 64 visits) and participants without the gene expansion that causes HD (GNE group; n = 259 participants with 395 visits). Using data from the Kids-JOHD study, we compared mean resting heart rate (rHR), systolic blood pressure (SBP), and diastolic blood pressure (DBP) between the JOHD and GNE groups. We also divided the JOHD group into those with childhood-onset JOHD (motor diagnosis received before the age of 13, [n = 16]) and those with adolescent-onset JOHD (motor diagnosis received at or after the age of 13 [n = 11]). We used linear mixed-effects models to compare the group means while controlling for age, sex, and parental socioeconomic status and including a random effect per participant and family. For the primary analysis, we found that the JOHD group had significant increases in their rHR compared to the GNE group. Conversely, the JOHD group had significantly lower SBP compared to the GNE group. The JOHD group also had lower DBP compared to the GNE group, but the results did not reach significance. SBP and DBP decreased as disease duration of JOHD increased, but rHR did not continue to increase. Resting heart rate is more sensitive to changes in autonomic function as compared to SBP. Therefore, these results seem to indicate that early neurodegenerative changes of the central autonomic network likely lead to an increase in rHR while later progression of JOHD leads to changes in blood pressure. We hypothesize that these later changes in blood pressure are secondary to neurodegeneration in brainstem regions such as the medulla.

Published
2020
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5. Autonomic changes in Huntington’s disease correlate with altered central autonomic network connectivity

Author

Jordan L Schultz, Amanda E Heinzerling, Alivia N Brinker, Lyndsay A Harshman, Vincent A Magnotta, John A Kamholz, Aaron D Boes, and Peg C Nopoulos

Subjects
General Engineering
Abstract

Autonomic dysfunction has been described in patients with Huntington’s disease, but it is unclear if these changes in autonomic tone are related to the central autonomic network. We performed a pilot study to investigate the relationship between the integrity of the central autonomic network and peripheral manifestiations of autonomic dysfunction in premanifest Huntington’s disease. We recruited male participants with pre-motor-manifest Huntington’s disease and a comparison group consisting of healthy, male participants of approximately the same age. As this was a pilot study, only males were included to reduce confounding. Participants underwent a resting-state functional magnetic resonance imaging study to quantify functional connectivity within the central autonomic network, as well as a resting 3-lead ECG to measure heart rate variability with a particular focus on the parasympathetic time-domain measures of root mean square of successive differences between normal heartbeats. The pre-motor-manifest Huntington’s disease participants had significantly decreased root mean square of successive differences between normal heartbeats values compared with the healthy comparison group. The pre-motor-manifest Huntington’s disease group had significantly lower functional connectivity within the central autonomic network, which was positively correlated with root mean square of successive differences between normal heartbeats. Patients with pre-motor-manifest Huntington’s disease have reduced functional connectivity within the central autonomic network, which is significantly associated with observed changes in autonomic function.

Published
2022

6. The Cerebellum and Implicit Sequencing: Evidence from Cerebellar Ataxia

Author

Owen P, Morgan, Mitchell B, Slapik, Katherine G, Iannuzzelli, Stephen M, LaConte, Jonathan M, Lisinski, Peg C, Nopoulos, Ashley M, Cochran, Sharif I, Kronemer, Liana S, Rosenthal, and Cherie L, Marvel

Subjects
Adult, Male, Cerebellar Ataxia, Cerebellum, Humans, Learning, Female, Middle Aged, Aged
Abstract

The cerebellum recognizes sequences from prior experiences and uses this information to generate internal models that predict future outcomes in a feedforward manner [Front Hum Neurosci 8: 475, 2014; Cortex 47: 137-44, 2011; Cerebellum 7: 611-5, 2008; J Neurosci 26: 9107-16, 2006]. This process has been well documented in the motor domain, but the cerebellum's role in cognitive sequencing, within the context of implicit versus explicit processes, is not well characterized. In this study, we tested individuals with cerebellar ataxia and healthy controls to clarify the role of the cerebellum sequencing using variations on implicit versus explicit and motor versus cognitive demands across five experiments. Converging results across these studies suggest that cerebellar feedforward mechanisms may be necessary for sequencing in the implicit domain only. In the ataxia group, rhythmic tapping, rate of motor learning, and implicit sequence learning were impaired. However, for cognitive sequencing that could be accomplished using explicit strategies, the cerebellar group performed normally, as though they shifted to extra-cerebellar mechanisms to compensate. For example, when cognitive and motor functions relied on cerebellar function simultaneously, the ataxia group's motor function was unaffected, in contrast to that of controls whose motor performance declined as a function of cognitive load. These findings indicated that the cerebellum is not critical for all forms of sequencing per se. Instead, it plays a fundamental role for sequencing within the implicit domain, whether functions are motor or cognitive. Moreover, individuals with cerebellar ataxia are generally able to compensate for cognitive sequencing when explicit strategies are available in order to preserve resources for motor function.

Published
2020

7. Comprehensive Cleft Care, Volume 1

Author

Gretchen Probst, Eric J.W. Liou, Elizabeth J. Leslie, Michael T. Mennuti, Mary Breen, Michael L. Cunningham, Jingtao Li, Linda L. D’Antonio, Nicholas J.V. Hogg, Diane L. Sabo, Jesse A. Goldstein, Donna Cutler-Landsman, Kathleen C.Y. Sie, David K. Chong, Barry L. Ramsey, Alex Campbell, Alexander L. Figueroa, Gregory S. Antonarakis, Robert M. Greene, Lacey Sischo, Richard A. Hopper, Lisa L. Repaske, Jodi E. Gustave, John B. Mulliken, Mohammad Mazaheri, Laura E. Mitchell, Peg C. Nopoulos, Adriane L. Baylis, Michael C. Kao, Kelly N. Evans, Barry H. Grayson, Martin H.S. Huang, Ana M. Mercado, Todd D. Otteson, John Daskalogiannakis, Nancy J. Scherer, Jeffrey L. Marsh, Philip Kuo-Ting Chen, Stephanie E. Watkins, Christopher R. Forrest, Marilyn C. Jones, Peter J. Taub, Thomas Samson, Patricia Daly Chibbaro, Yu-Ray Chen, Dennis R. Warner, Ryan C. Ransom, Robert J. Havlik, Darren M. Smith, Oksana A. Jackson, Faisal Al-Mufarrej, Valerie Pereira, M. Samuel Noordhoff, Dina Ricciardi, Lawrence E. Brecht, Carrie L. Heike, Lyndon M. Hill, Rebecca Gaither, Daniela Damian, Shu-Jin Lee, Robert M. Menard, Kenneth L. Jones, Bernard J. Costello, Donna M. McDonald-McGinn, Ross E. Long, Elaine H. Zackai, Farah Sheikh, Randy Sherman, Brett F. Michelotti, Alexandre Marchac, David L. Jones, Henry K. Kawamoto, Edward P. Buchanan, Hillary L. Broder, Donald R. Mackay, Jamie L. Perry, Earl A. Gage, Lauren DiCairano, Mark Splaingard, Laura A. Monson, Ann W. Kummer, Alison Kaye, Court B. Cutting, William C. Shaw, Cathy R. Henry, Jill A. Helms, Bruce B. Horswell, Gary C. Burget, Richard E. Kirschner, Arun K. Gosain, Seng-Teik Lee, David M. Fisher, Franklyn P. Cladis, Kathleen A. Kapp-Simon, Paula G. Klaiman, Steven T. Lanier, John W. Polley, M. Michele Pisano, Jasmine Y. Rodriguez, Karen W.Y. Wong, Roberto L. Flores, Mary L. Marazita, Scott A. Deacon, Seth M. Weinberg, Mark P. Mooney, Larry H. Hollier, Marilyn A. Cohen, Matthew D. Ford, Peter Randall, Ronald P. Strauss, Christina Tragos, Alvaro A. Figueroa, Joseph Michienzi, Virginia A. Hinton, Anette Lohmander, David P. Kuehn, Scott A. Dailey, Philip J. Lupo, Amy L. Conrad, Lynn Marty Grames, Charles D. Bluestone, Oluwaseun A. Adetayo, Lynn C. Richman, H. Steve Byrd, Donald V. Huebener, Kenneth E. Salyer, Judah S. Garfinkle, Debbie Sell, David E. Kauffman, Sanjay Naran, Stephen B. Baker, Joseph E. Losee, Katherine W.L. Vig, Evan W. Beale, Ramon L. Ruiz, Gunvor Semb, David J. Reisberg, Brian C. Sommerlad, Justine C. Lee, and Susan M. Salkowitz

Subjects
medicine.medical_specialty, Plastic surgery, business.industry, medicine, business, Surgery, Volume (compression)
Published
2016

8. Comprehensive Cleft Care, Volume 2

Author

Mark P. Mooney, Kelly N. Evans, Alison Kaye, David J. Reisberg, Court B. Cutting, Valerie Pereira, M. Samuel Noordhoff, Lawrence E. Brecht, Mark Splaingard, Brian C. Sommerlad, Mary L. Marazita, Lyndon M. Hill, Judah S. Garfinkle, Robert M. Menard, Randy Sherman, Justine C. Lee, Peter Randall, Patricia Daly Chibbaro, Yu-Ray Chen, Darren M. Smith, John Daskalogiannakis, Christina Tragos, Faisal Al-Mufarrej, David L. Jones, Jasmine Y. Rodriguez, Barry H. Grayson, Elaine H. Zackai, Alvaro A. Figueroa, Jeffrey L. Marsh, Jesse A. Goldstein, Virginia A. Hinton, Richard E. Kirschner, Donna Cutler-Landsman, Franklyn P. Cladis, Todd D. Otteson, Robert M. Greene, Joseph Michienzi, Farah Sheikh, Laura A. Monson, Lynn Marty Grames, David K. Chong, Jill A. Helms, Ana M. Mercado, Charles D. Bluestone, Oluwaseun A. Adetayo, Linda L. D’Antonio, Evan W. Beale, Thomas Samson, Donald V. Huebener, Ramon L. Ruiz, Jingtao Li, Bruce B. Horswell, Gary C. Burget, Ross E. Long, Peter J. Taub, Gunvor Semb, M. Michele Pisano, Paula G. Klaiman, Christopher R. Forrest, Henry K. Kawamoto, Roberto L. Flores, Diane L. Sabo, Mohammad Mazaheri, Steven T. Lanier, Scott A. Deacon, Seth M. Weinberg, Larry H. Hollier, Marilyn A. Cohen, Lacey Sischo, Dennis R. Warner, Ryan C. Ransom, John W. Polley, Eric J.W. Liou, John B. Mulliken, Alex Campbell, Gretchen Probst, Nancy J. Scherer, Peg C. Nopoulos, Kenneth L. Jones, Marilyn C. Jones, Laura E. Mitchell, Anette Lohmander, Oksana A. Jackson, Alexander L. Figueroa, Kathleen C.Y. Sie, David P. Kuehn, Bernard J. Costello, Joseph E. Losee, Stephanie E. Watkins, Hillary L. Broder, Cathy R. Henry, Scott A. Dailey, Jodi E. Gustave, Gregory S. Antonarakis, Earl A. Gage, Kathleen A. Kapp-Simon, Ann W. Kummer, Ronald P. Strauss, Philip Kuo-Ting Chen, William C. Shaw, Edward P. Buchanan, Seng-Teik Lee, Philip J. Lupo, Lynn C. Richman, H. Steve Byrd, Matthew D. Ford, Michael C. Kao, Donald R. Mackay, Lauren DiCairano, Donna M. McDonald-McGinn, Jamie L. Perry, Debbie Sell, Arun K. Gosain, Sanjay Naran, Stephen B. Baker, David E. Kauffman, Katherine W.L. Vig, Karen W.Y. Wong, Susan M. Salkowitz, Amy L. Conrad, Kenneth E. Salyer, Michael L. Cunningham, Barry L. Ramsey, Richard A. Hopper, Lisa L. Repaske, Daniela Damian, Shu-Jin Lee, David M. Fisher, Brett F. Michelotti, Alexandre Marchac, Martin H.S. Huang, Robert J. Havlik, Rebecca Gaither, Elizabeth J. Leslie, Nicholas J.V. Hogg, Michael T. Mennuti, Mary Breen, Dina Ricciardi, Carrie L. Heike, and Adriane L. Baylis

Subjects
medicine.medical_specialty, Plastic surgery, business.industry, Medicine, business, Surgery, Volume (compression)
Published
2016

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